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Team D: pathology, allergy & immunology, dermatology, plastic & reconstructive surgery, and rheumatology.

PRIMARY CUTANEOUS B CELL LYMPHOMA ASSOCIATED WITH ALOPECIA. Monica K. Bedi, MD. Tampa, Fla.

Primary cutaneous B cell lymphomas represent a small percentage of all cutaneous lymphomas. Most present as a solitary violaceous papule or nodule on the head neck or trunk. We describe an unusual case of marginal zone B cell lymphoma that presented with small perifollicular papules on the scalp associated with alopecia. Histopathology shows an atypical lymphoid infiltrate of L26 positive B cells centered around the hair follicles. Gene rearrangement and flow cytometry confirmed the diagnosis of B cell lymphoma. We are unaware of any other cases of primary cutaneous B cell lymphoma with pilotropism and alopecia as seen in our case.

IMMUNOHISTOCHEMICAI EVALUATION OF MORPHEAFORM AND INFUNDIBULOCYSTIC VARIANTS OF BASAL CELL CARCINOMA, DESMOPLASTIC TRICHOEPITHELIOMA, TRICHOBLASTOMA, AND BASALOID FOLLICULAR HARMARTOMA USING BCL-2, K167 AND PCNA. P.H. Bowman, MD, R.A. Abdelsayed, DDS, N.A. Ibrahim, PhD, and 0.P. Sangueza, MD. Medical College of Georgia, Augusta.

Distinction between benign and malignant basal cell neoplasms with similar histologic characteristics is sometimes difficult. The purpose of this investigation was to determine whether there are differences in cellular expression of Bcl-2, Ki67, and PCNA between morpheaform basal cell carcinoma (MBC) vs desmoplastic trichoepithelioma (DTE), and between infundibulocystic basal cell carcinoma (IBC) vs trichoblastoma (TB) vs basaloid follicular hamartoma (BFH). Forty-three classic, unequivocal tumor cases (19 MBCs, 4 DTEs, 6 IBCs, 12 TBs, and 2 BFHs) were selected from the archives of the surgical pathology service at the Medical College of Georgia and stained with antibodies for Bcl-2, Ki67, and PCNA. Each case was assigned a staining intensity score based on the percentage of cells staining as follows: 1+, 0% to 24% cells staining; 2+, 25% to 50% cells staining; 3+, 51% to 75% cells staining; 4+, 76% to 100% cells staining. Staining intensity scores for each neoplastic group were then tabulated and examined st atistically using the analysis of variance (ANOVA) technique. Bcl-2 staining was similar for all tumor types studied. PCNA staining was more variable but could not reliably distinguish between any of the tumor types. Ki67 expression between IBC, TB, and BFH was not different enough to tell the tumors apart. In contrast, all of the MBCs showed intense expression of Ki67 (mean staining intensity 3.53), while none of the DTEs did (staining intensity 1+ for all tumors). This difference in Ki67 expression between MBC and DYE was found to be statistically significant. Differences in Ki67 expression may have value in differentiating between MBC and DTE when routine histologic findings are inconclusive.

TUBEROUS SCLEROSIS IN A MOTHER AND HER TEENAGE DAUGHTER. Beth Brogan, MD, and John Zic, MD. The Vanderbilt Clinic, Nashville, Tenn.

A 44-year-old woman, who was diagnosed with tuberous sclerosis five months earlier, presented with her 14-year-old daughter. The woman had a twenty-year history of painful periungual fibromas and multiple angiofibromas in her mesolabial folds, many of which had been surgically removed. As part of her initial work-up for tuberous sclerosis, in the previous month she had an abdominal ultrasound and echocardiogram, both which were normal. As a work-up for chronic headaches several years earlier, a head CT scan had been reportedly normal, as were past routine eye examinations. She had no history of seizure disorder or learning disability and had no family history of tuberous sclerosis. Physical examination revealed multiple 1-to-3 mm firm, erythematous papules studding her bilateral mesolabial folds. On her bilateral hands and feet, multiple flesh-colored verrucous papules projected from underneath the proximal nail folds, causing longitudinal ridging on the nail plates. A Wood's lamp exam revealed an 8 x 5 mm hy popigmented, oval patch on the left lateral lower leg and scattered hypopigmented 2 to 4 mm macules primarily on the lower extremities. Likewise, the patient's daughter had no history of seizure disorder or mental retardation, and this was her first evaluation for tuberous sclerosis. She had recently noted a growth near a toenail, and her orthodontist had pointed out gingival papules. Physical exam revealed two intraoral 3 to 4 mm pink papules on upper and lower gingiva. A 4 mm smooth papule projected from the proximal nail fold of her left second toe, and a longitudinal groove on a fingernail could be traced to an additional proximal nail fold papule. There was a pebbled texture of the mesolabial fold without discrete papules. A wood's lamp revealed two coalescing hypopigmented patches on her right arm. MR scan of the head in the 44-year-old patient revealed multiple cortical tubers and subependymal nodules. MR scan of her daughter additionally revealed cortical tubers. A renal ultrasound in the teenager was normal. Both patients were referred for carbon dioxide laser ablation of their periungual fibromas and for genetic counseling. These cases highlight several of the diagnostic criteria of tuberous sclerosis. Additionally, it is clear that signs and symptoms of tuberous sclerosis may be subtle without serious neurologic symptoms, and the diagnosis may be made at any age. The case also emphasizes the sensitivity of an MR scan, as opposed to a CT scan, in diagnosing cortical tubers. Finally various specialties can aid in the diagnosis and management of tuberous sclerosis, including dermatologists, primary care physicians, neurologists, radiologists, dentists and genetic counselors.

INFLIXIMAB, A UNIQUE CHIMERIC ANTI-TNF[alpha] MONOCLONAL ANTIBODY FOR THE TREATMENT OF AN EXFOLIATIVE ERXTHRODERMA. Jennifer H. Alberts, MD, and Jami L. Miller, MD. Department of Medicine, Division of Dermatology, Vanderbilt University, Nashville, Tenn.

A 63-year-old white woman had generalized erythroderma in the spring of 1999. The erythroderma nearly cleared with IV steroids, but her disease flared whenever the prednisone was tapered below 40 mg QD. Several immunosuppressive agents were initiated including methotrexate, acitretin, and narrow-band UVB. The patient did not respond or had a severe reaction to these treatments. Her scaling and erythema persisted and ultimately required six hospital admissions within a 12-month period. The etiology of the patient's exfoliative erythroderma remains unclear, with psoriasis or atopic dermatitis as the most likely cause. Laboratory data obtained on admission was normal including two negative Sezary preps. Gene rearrangement studies were negative for malignancy. Chest x-ray showed no evidence of masses or adenopathy. A biopsy of the skin showed a spongiotic psoriasiform dermatitis without evidence of malignant lymphocytes. The primary pathophysiology of an exfoliative erythroderma is not well understood, but is bel ieved to be due to the interaction of many inflammatory cells and cytokines in the skin. In our patient, previous therapy with numerous immunosuppressant agents proved either to be ineffective or complicated by adverse drug side effects. Since the patient already had Crohn's disease, an indication for therapy with infliximab, we believed a trial for the treatment of her exfoliative erythroderma was warranted. Within 36 hours of receiving infliximab, 5 mg/kg intravenously, the patient's exfoliative erythroderma dramatically improved. She was discharged to home on cyclosporine A microemulsion 50 mg BID. Two weeks after the initial infliximab infusion, the patient was seen in follow-up. She reported doing well until approximately 24 hours earlier at which time she began to experience rapidly progressive pruritus and erythema. Examination revealed a hypothermic erythrodermic woman. The patient received a second infusion of infliximab 5 mg/kg without complications; as noted previously, the patient's erythroderma r apidly resolved over the next 24 hours.

IATROGENIC CALCINOSIS CUTIS. Jeff J. Ligon, MD, Jennifer Hoffman, MD, and John Zic, MD. Vanderbilt University, Department of Medicine, Division of Dermatology, Nashville, Tenn.

Calcinosis cutis is the cutaneous deposition of calcium salts in the dermis. It can occur iatrogenically when concentrated calcium solutions are given intraveniously. We present a case of calcinosis cutis in a neonate who required aggressive electrolyte management in the setting of resuscitation after cardiac surgery. A 14-day-old male born at 35 weeks gestation was first noted to have a violaceous patch on the right medial shin one day after a Norwood palliation procedure for hypoplastic left heart. This procedure was complicated post-operatively by cardiac arrest requiring extracorporeal membrane oxygenation and during which a total of 380 mg of CaCl was given in a 10% solution through a peripheral IV in the right foot. The patient's skin lesion became more indurated over the ensuing week and developed into a 2.0 by 2.6 cm yellow-white firm plaque with central ecchymosis with multiple similar 2 to 5 mm satellite white papules on the surrounding skin. A skin biopsy showed slight basophilia of the dermis sugg estive of diffuse calcim deposition which was confirmed with a von Kossa stain. Many infiltrating neutrophils and eosinophils were also noted. Most interestingly, dermal veins appeared darkened and ragged while arterioles appeared completely normal. The skin plaque showed no progression over the next 3 weeks. The patient expired at that time due to complications of his cardiac disease. When beterotropic calcification occurs in the setting of normal calcium levels, it is usually due to damaged tissue (i.e. trauma, collagen vascular disease). However, heterotopic calcification may occur in hypercalcemic states where it called "metastatic." Calcium salts are slightly acidic, hypertonic solutions that are capable of precipitating proteins. The concentration of calcium chloride given to our patient was 1000-fold higher than the normal intravascular level. This created a situation in which venous and dermal structures were damaged and large amounts of calcium were present for deposition. Prevention of iatrogenic ca lcinosis cutis entails using minimally concentrated solutions and administering them through central IV's. Calcium gluconate is better tolerated than calcium chloride. Treatment is usually supportive though some have recommended intralesional steroid injection. This case is instructive because it represents a complication of intravenous calcium administration of which clinicians should be aware.

TOPICAL TREATMENT OF PALMOPLANTAR PUSTULOSIS. Ronald A. Nelson, MD, and Jami L. Miller, MD. Department of Medicine, Division of Dermatology, Vanderbilt University Medical Center, Nashville, Tenn.

Palmoplantar pustulosis represents a therapeutic challenge, particularly for patients with relative contraindications to systemic agents. We report a 52-year-old white male treated with hydroxychloroquine, methotrexate and prednisone for long-standing rheumatoid arthritis who developed palmoplantar pustulosis. This patient responded to tazarotene 0.1% gel applied to the palms and soles nightly, augmented betamethasone dipropionate 0.05% applied to the palms twice a day, and flurandrenolide 4 [micro]g/cm patches applied to the soles daily. This topical combination treatment relieved the palmoplantar pustulosis without the potential side effects of systemic therapy.

MALIGNANT ATROPHIC PAPULOSIS (DEGOS' DISEASE). Ronald A. Nelson, MD. Department of Medicine, Division of Dermatology, Vanderbilt University Medical Center, Nashville, Tenn.

Malignant atropic papulosis (Degos' disease) is a rare thrombo-occlusive disorder of uncertain etiology predominately affecting young men. Distinctive cutaneous manifestations are caused by occlusion of blood vessels in the dermis. Occlusive disease in the gastrointestinal tract and central nervous system results in disease complications, but there is typically no evidence of autoimmune vasculitis. Gastrointestinal disease can result in bowel perforation, peritonitis, and death. Consequently, Degos' disease carries a poor prognosis: over 50% of the first 110 reported cases died from complications of central nervous system or gastrointestinal involvement. Recent case reports, however, identified patients with a more benign variant of the illness with cutaneous, but not systemic, involvement. At present, treatment options are not satisfactory. A 27-year-old white male patient with a six year history of recurrent, erythematous papules recently presented for evaluation after failing treatment with colchicine and prednisone. Clinical examination demonstrated multiple erythematous papules with central erosions and overlying eschars in various stages of evolution. Skin biopsy showed a wedge-shaped area of dermal necrosis, superficial and deep perivascular inflammation, and vascular disruption. Extensive laboratory evaluation failed to show evidence of autoimmune disorder or systemic vasculitis. Upper gastrointestinal endoscopy showed no evidence of involvement. Overall, the clinical findings suggested Degos' disease presently limited to the skin. The patient showed minimal response to numerous treatments, including dipyridamole, danazol, celecoxib, dapsone, tetracycline, niacinamide, and high-potency topical corticosteroids. Treatment with methotrexate 10 mg weekly showed promise for controlling the number and frequency of new lesions. Keratoacanthoma is a dome-shaped cutaneous neoplasm characterized by its rapid growth and spontaneous involution. The solitary keratoacanthoma is the most common type, however, cases of g iant keratoacanthomas (larger than 2.5 cm) and multiple keratoacanthomas have been reported. Treatment of this neoplasm consists of surgery, curretage and dessication, liquid nitrogen destruction, radiation, and other intralesional, topical, or systemic agents. We describe a rare case of multiple giant keratoacanthomas successfully treated with subcutaneous interferon alfa-2a and oral isotretinoin. Keratoacanthoma is a dome-shaped cutaneous neoplasm characterized by its rapid growth and spontaneous involution. The solitary keratoacanthoma is the most common type of this tumor, however, cases of giant keratoacanthomas (larger than 2.5 cm) and multiple keratoacanthomas have been reported. Treatment of this neoplasm consists of surgery, curretage and dessication, liquid nitrogen destruction, radiation, and other intralesional, topical, or systemic agents. We describe a rare case of multiple giant keratoacanthomas successfully treated with subcutaneous interferon alfa-2a and oral isotretinoin. This is the first c ase to our knowlegde of successful combination therapy of keratoacanthomas with these agents.

MUILTIPLE GIANT KERATOACANTHOMAS TREATED WITH SUBCUTANEOUS INTERFERON [alpha]-2A AND ORAL ISOTRETINOIN. Pamela E. Sakalosky, MD, and Philip Shenefelt, MD. University of South Florida, Tampa, Fla.

Keratoacanthoma is a dome-shaped cutaneous neoplasm characterized by its rapid growth and spontaneous involution. The solitary keratoacanthoma is the most common type of this tumor, however, cases of giant keratoacanthomas ([greater than] 2.5 cm) and multiple keratoacanthomas have been reported. Treatment of this neoplasm consists of surgery, durretage and dessication, liquid nitrogen destruction, radiation, and other intralesional, topical or systemic agents. We describe a rare case of multiple giant keratoacanthomas successfully treated with subcutaneous interferon [alpha]-2a and oral isotretinoin. This is the first case to our knowledge of successful combination therapy of keratoacanthomas with these agents.

ACQUIRED PERFORATING DISORDER. Hunter H. Sams, MD. The Vanderbilt Clinic, Nashville, Tenn.

A 68-year-old white man with insulin-dependent diabetes mellitus presented to clinic with a two-year history of thick spots on his leg and abdomen. They were not particularly itchy, but with time, they had enlarged and became increasingly uncomfortable. He had been putting skin lotion on the spots, which was not helpful. Examination revealed one 3 x 1.2 cm plaque on the right thigh and multiple small papules on the right thigh and around the left flank and waist. Light brown densely hard keratinous material was present in the center of the lesions. The borders were heaped up and violaceous, with surrounding bright erythema. The diagnosis is acquired perforating disorder (APD). In this case, associated with diabetes mellitus. Although rare, these disorders are so distinctive that they are readily recognized. The spectrum of disease includes Kyrle's disease, elastosis perforans serpiginosa, perforating folliculitis, and reactive perforating collagenosis. It is not known whether these are distinct disorders or s pectrum of the same condition. Additionally, the etiology is unknown. Clinically, APD presents with a papule with a central dell that progresses to a keratotic plug which enlarges over time. Inflammation and erythema usually surrounds the lesions. Proposed diagnostic criteria for acquired APD include: (1) histopathologic findings of elimination of necrotic basophilic collagen tissue into a cup-shaped epidermal depression, (2) clinical presentation of umbilicated papules or nodules with a central adherent keratotic plug, and (3) onset of skin lesions after the age of 18 years. Histology of the lesions is characterized by hyperkeratosis and central parakeratotic column. Collagen and elastic fibers may track through the epidermis as if "perforating" to the surface. Additionally, a microvasculopathy may be noted.

LINEAR FIXED DRUG ERUPTION. Elizabeth B. Wiser, MD, and Alan S. Boyd, MD. Vanderbilt University Medical Center, Nashville, Tenn.

A 24-year-old white female presented with a one month history of a mildly pruritic rash on both legs, initially beginning on her right thigh and spreading distally. On examination, there were multiple 3 mm orange-pink, flat papules coalescing into narrow linear plaques, extending across the right anterior thigh and down the left posterior calf onto the foot. She was in good health and took no medications except for occasional naproxen. A punch biopsy revealed a slightly thinned epidermis with abundant dyskeratotic cells and vacuolar change along the basal layer. Pigment incontinence and rare eosinophils were present in the papillary dermis, features consistent with those of a fixed drug eruption. The patient was advised to discontinue naproxen. She was treated with topical mometosone furoate 0.1% cream twice daily with rapid diminution of the eruption leaving residual postinflammatory hyperpigmentation. Linear dermatoses may be classified as secondary to contact with exogenous agents, the Koebner effect, or a re distributed along dermatomal, vascular, or Blaschko's lines. We report a case of a linear fixed drug eruption secondary to naproxen, which appears to follow the lines of Blaschko.

TRANSBRONCHIAL FINE NEEDLE ASPIRATION: A REVIEW OF 94 CASES. Cesar V. Reyes, MD, and JoAnne Jensen, CT(ASCP) (MIAC). Cytology Section, Veterans Affairs Hospital, Hines, Ill.

Wang's transbronchial fine-needle aspiration (TBFNA) is a safe and effective procedure in cancer diagnosis, staging of lung carcinomas, and recognition of mediastinal lymphomas. It reduces the number of mediastinoscopies that may be associated with more complications. We evaluated our TBFNA experience of 6 years at Hines VA Hospital to determine its strength as a diagnostic tool and the usefulness of liquid-based cytology and flow cytometry in such specimens. The 94 TBFNAs from 86 patients during January 1995 and December 2000 at Hines VA Hospital were reviewed and correlated with available clinical and imaging data. The patients were all male with age range of 46 to 87 years (mean, 62 years) and clinical diagnosis of mediastinal or hilar lymph nodes, mass, lung cancer, or recurrent lung cancer (n = 14), or suspected lymphoma. All patients showed mediastinal lymphadenopathy on imaging scan. Previous sputum cytologies (n = 34) were negative. No endobronchial lesion were seen on bronchoscopy. TBFNAs were perfor med by the pulmonologists in the presence of a cytopathologist who made on spot evaluation of Diff-Quik smears. Clusters of lymphoid cells were required for adequacy of TBFNA specimens. Smear inadequacy and negative for malignant cells prompted additional aspirations by the pulmonologist. After TBFNA completion, the needle/tubing was washed in Thin-Prep Cytolyt (n = 94) and, when lymphoma was suspected, in RPMI medium for flow cytometry (n = 7). Sixty-two of 94 cases showed positive for malignant cells: small cell carcinoma (n = 2), adenocarcinoma (n = 23), squamous cell carcinoma (n = 7), poorly differentiated large cell carcinoma (n = 2), and consistent with lymphoma (n = 2). There were 21 cases negative for malignancy. Five contained only reactive lymphoid cells. Six cases were unsatisfactory. Papanicolaou-stained thin-preparation smears of the TBFNA washings reaffirmed these findings. Flow cytometry of TBFNA washings confirmed the diagnosis of reactive lymphadenopathy (n = 5) and lymphoma (n = 2). Our res ults proved the utility of TBFNA in the diagnosis and staging of lung cancers, cytologic typing of lung carcinomas, and recognition of mediastinal lymphomas. Liquid-based cytology and flow cytometry likewise have adjunctive role in TBFNA specimen evaluations.

T-CELL LIKE NK POSYPRANSPLANT LARGE GRANULAR LYMPHOCYTOSIS: AN OVERVIEW OF OTHER LARGE GRANULAR LYMPHOCYTIC PROLIFERATIONS. Amilcar A. Castellano-Sanchez, MD, Beria Cabello-Inchausti, MD, Michael Schwartz, MD, and Morton J. Robinson, MD. Department of Pathology, Mount Sinai Medical Center, Miami Beach, Fla.

A 62-year-old man was sent for hematologic consultation due to unexplained progressive lymphocytosis. The patient's WBC had risen from normal values to 28.5 x 109/mL, with a predominance of lymphocytes. Medical history was remarkable for a cadaveric kidney transplant due to adult polycystic kidney disease in 1983 (native kidneys and spleen removed) and a 25 lb weight loss, attributed to poor appetite in the 2 months before this consultation. Associated conditions included gout and arterial hypertension. Physical examination was unremarkable. CT scans of the chest, abdomen, and pelvis revealed liver cysts. Hemoglobin was 10.7 g/dL, and platelets were 364,000/mL. Examination of the peripheral blood smear disclosed large lymphoid cells without granules and some with faint to prominent cytoplasmic granules. Immunophenotype by flow cytometry performed in peripheral blood demonstrated that 50% of the WBC were T lymphocytes with the following phenotype: CD3 [+ or -] CD16 [+ or -] coexpression of CD3/CD16 as well as other T cell markers. There was also positive gene rearrangement for the T cell receptor. All these findings were indicative of a T-cell like NK posttransplant lymphocytosis. The proliferations of the large granular lymphocytes (LGL) can be divided into posttransplant LGL lymphocytosis, LGL leukemias, and LGL lymphomas. LGL are a subset of larger lymphoid cells that show subtle to prominent cytoplasmic azurophilic granules and account for 10% to 15% of the total lymphocyte count. LGL can be further subdivided into two different populations by immunophenotypic and molecular means, thus there are T-cell like natural killer (NK) LGL and true NK LGL. T-cell like NK posttransplant lymphocytosis is an uncommon condition that usually presents late after solid organ transplantation. It corresponds to an increased number of CD3(+) cells that may or may not be clonal. Immunophenotype is of paramount importance for the diagnosis. Once suspected, a period of 6 months of follow-up is required to differentiate the reactive -transient from the persistent processes. The true NK form of this disorder is even more uncommon.

T-CELL LARGE GRANULAR LYMPHOCYTIC LEUKEMIA. G. Cusano, DO, W. Koss, MD, M. Holguin, MD, A.F. Harris, BA, CLSp (MB), and E. Rappaport, MD. Scott & White Memorial Hospital and the Texas A&M University System Health Science Center, College of Medicine, Temple, Tex.

Large granular lymphocytic leukemia (LGL) is a syndrome associated with the proliferation of large granular lymphocytes and neutropenia which was initially reported in 1977. T-cell LGL leukemia is one of the four T-cell leukemias recognized by the new WHO classification. LGL leukemia is a clonal disease that involves CD 3 positive T-cells and has a strong association with rheumatoid arthritis (RA) and Felty's Syndrome (RA, splenomegaly and neutropenia). In fact LGL leukemia with RA and Felty's Syndrome have similar joint manifestations and similar good response to methotrexate. Also up to 40% of patients with Felty's syndrome have LGL leukemia lending credence that they are part of the spectrum of the same disorder. This disease entity has been linked to several other autoimmune diseases including pure red cell aplasia. We present a case of LGL leukemia in an asymptomatic 39-year-old female who presented with severe neutropenia (absolute neutrophil count of 0.2 x 103). The peripheral blood lymphocytosis was c omposed of reactive-like mature large granular lymphocytes. She had no evidence of either splenomegaly or RA. The bone marrow aspirate and biopsy were non-diagnostic. The immunophenotype by flow cytometry demonstrated an almost exclusive population of T-cells (CD 2+ CD 3+ CD 7+ TCR n) in the peripheral blood. In addition approximately half of these T-cells expressed neither CD 4 nor CD 8. Molecular studies by PCR revealed a unique pattern of T-cell receptor gene rearrangements demonstrating clonality. A more in-depth presentation of this case and a review of this disease entity will be presented.

EFFECT OF TISSUE FIXATIVES ON ABH BLOOD GROUP ANTIGENS. Sumita Gokhale, MD, and Patrick A. Adegboyega, MD. Department of Pathology, University of Texas Medical Branch, Galveston, Tex.

The occurrence of tissue contaminants ("floaters") and specimen mix-ups are problems frequently encountered in surgical pathology. The use of blood group immunostains may sometimes be helpful in resolving these problems. Due to concerns about the toxicity of formalin, alternative tissue fixation methods are being explored, but the effect of different fixatives on the expression of blood group antigens is not known. Using immunohistochemistry with monoclonal antibodies to blood group isoantigens A, B, and H, we studied the effect of different fixatives on the expression of these isoantigens in tissues from different organs. The organs studied included: placenta (n 4), uterus (n = 2), ovary (n = 2), colon (n = 1), skin and subcutanoeus tissue (n = 1) and axillary soft tissue mass (n = 1). The following fixatives were used: Acetone, 70% Ethanol, B5, Bouin's, Carnoy's, methanol and 10% formalin. After fixation for 6, 12, and 72 hours, the tissue blocks were embedded in paraffin and immunohistochemistry was perfor med on 4-micron thick tissue sections using the avidin-biotin detection method. The expression of the blood groups isoantigen was concordant with the blood group of the patient in all the cases studied irrespective of the fixative and time of fixation. Immunohistochemical staining of paraffin-embedded tissue with ABH blood group antibodies is a rapid, reliable and cost effective method that may be helpful in sorting out interpretative problems of floaters and specimen mix-ups, irrespective of the fixative used.

QUANTITATIVE ANALYSIS OF THE BLOOD WASTAGE AS A RESULT OF DIAGNOSTIC PHLEBOTOMY WHAT CAN BE DONE? Vaishali Popat, MD, MPH, Firas Akhrass, MD, and David Wadhwani, MD. Union Memorial Hospital, Baltimore, Md.

For each patient, laboratories usually collect more blood than is needed for specific determinations. Although it has been shown that a few microliters are actually needed to perform a particular test, typically 5 to 7 mL of blood is drawn. There are two different tube sizes (5 mL and 7mL) available for the same exact tests in the hospitals. A survey of the phlebotomists at our institute showed that 5 mL/7 mL test tubes were being randomly selected for the blood draw. We conducted a cross sectional study to estimate the amount of blood wastage as a result of diagnostic phlebotomy in these two different tube sizes. In this study the amount of blood discarded after the completion of requested diagnostic testing was measured in a random sample in the hospital laboratory over 30-day period. The observations were made in three different departments namely chemistry, hematology and immunology and included all the tests ordered for that day from outpatient as well as inpatient services. A total of 1916 observations of discarded test tubes were made in the laboratory. The analysis showed that the volume of blood discarded in a 5 mL tube (2.5 [+ or -] 0.68 mL) was 31% lower than the volume of blood discarded in a 7 mL tube (3.6 [+ or -] 1.02 mL) (P [less than].001). There was no statistically significant difference between the number of tests ordered and amount of blood discarded per tube (P = .112) in both test tube sizes. Based on CDC-NICLTS study, our data can be extrapolated to all hospitals in USA which estimates that approximately 2.05 (1.78-2.32) million liters of blood is discarded per year as a result of diagnostic lab testing. Practical strategies for decreasing the amount of blood waste include a thorough knowledge of the volume of blood needed for each test, using a smaller test tube, and education of the phlebotomy personnel. Although substituting smaller tubes does not address the issue of excessive use of laboratory tests, smaller tubes may reduce the blood collected from the patients without compromising l aboratory test procedures.

JEJUNAL PANCREATIC HETEROTOPIA WITH CARCINOMA. NI. Gupta, MD, M. M. Nicolas, MD, and D. Duncan, MD. Department of Pathology, Orlando Regional Medical Center, Orlando, Fla.

The presence of aberrant or heterotopic pancreas in the gastrointestinal tract is an incidental though not an unusual finding. However, the jejunum is rarely involved, with a malignant transformation being even rarer. We report a case of a 68-year-old man with heterotopic pancreas in the jejunum with malignant transformation. The patient was admitted with sudden onset of abdominal pain and distension due to a partial small bowel obstruction. Laparotomy revealed a proximal jejunal mass, small bowel obstruction and adhesions. Histopathological examination of the resected proximal jejunal segment showed a subserosal nodule of heterotopic pancreas containing moderately differentiated adenocarcinoma invading through the jejunal muscularis propria into the submucosa. Severe chronic pancreatitis was also present. Only four cases of carcinoma in aberrant pancreatic tissue of the jejunum have so far been reported.

HIV-ASSOCIATED ATYPICAl LYMPHOID HYPERPLASIA OF MAJOR SALIVARY GLAND. A CASE REPORT. Maud Major-Nicolas, MD, Meenakshi Vij Gupta, MD, and Lizardo Cerezo, MD. Orlando Regional Healthcare System, Orlando, Fla.

Though first described in 1985, it is not widely known that HIV lymphadenopathy may involve the major salivary glands. The lesion presents as either unilateral or bilateral neck masses with variable onset of symptoms and diagnosis depends upon pathologic examination to exclude fully developed lymphoma and infection. We report a case of a 30-year-old HIV-infected woman who presented with an acute onset neck mass. The gland was replaced by a florid hyperplasia of CD20 positive B-cells forming large reactive appearing follicles and diffuse areas. Duct epithelium was also heavily infiltrated by small lymphocytes. No extrafollicular foci of large cell proliferation were seen. No clonal light chain or p-53 expression were demonstrated. There has not been a precise study to date that assesses the risk of lymphoma arising within HIV-associated lymphoid hyperplasia of the salivary gland. Neither has there been a study that correlates whether an acute onset of the neck mass would favor a greater risk of a malignant les ion versus a benign inflammatory process. It seems likely that as in lymph nodes, HIV-associated lymphoid hyperplasia in salivary gland tissue may progress to either lymphoid atrophy or lymphoma. However, atypical HIV-associated lymphoid hyperplasia is not treatable and must be distinguished pathologically from lymphoma to spare the patient the added morbidity of chemotherapy.

REDUCTION MAMMAPLASTY-MODIFICATION OF THE INFERIOR PEDICLE TECHNIQUE ENHANCING NIPPLE AREOLAR PROJECTION AND CONTOUR. Mark D. McDonough, MD, Vanderbilt University Medical Center, Nashville, Tenn; and Calvin R. Peters, MD, Orlando, Fla.

Despite many reported modifications of the inferior pedicle technique, the best methods for optimizing contour and projection have yet to be determined for reduction mammaplasty. A simple and direct modification of the inferior pedicle technique is presented which employs a subdermal shelf to enhance upper-pole fullness and nipple projection while optimizing breast contour. Employment of a subdermal shelf as well as flexible preoperative plan and closure results in excellent nipple-areolar projection and contour. The subdermal shelf provides nipple-areolar support while attention to T closure technique enhances contour. Complications of reduction mammaplasty utilizing this technique have been negligible. Twenty-five patients underwent this procedure between 1997-2000 with only two minor complications. These resolved without surgical intervention. Reductions ranged from between 350 g and 1830 g with an average reduction of 731 g. This technique is a simple, direct and efficient method for optimizing nipple are olar projection and contour. It is reliable and produces an aesthetically pleasing result as well as satisfied patients.

SIMPLE SELF-RETAINING RING RETRACTOR FOR MINOR SURGICAL PROCEDURES. Carl B. Pearl, MD, A. Aguillon, MD, W. T. Majewski, MD, and Jack Yu, MD. Medical College of Georgia, Augusta.

The need for better exposure usually leads to hand-held retractors. The addition of another set of hands in the surgical field frequency deprives the assistant of direct view of the surgical field. More hands in the surgical field also increase the risk of needle stick or other injury. The traditional self retaining retractor offers an alternative to the hand-held retractor but is costly and must be sterilized after each case. Also, this retractor can exert great force on tissues. We offer an alternative to the more traditional self-retaining retractor that can be quickly assembled using a 1 L disposable plastic bowl, which is typically found in the sterile field. More importantly, our retractor limits the maximum tension that can be placed on a wound. Our retractor is fabricated by excising a ring 1 cm in depth from the bowl and then placing eight 5 mm slits evenly distributed around the cut surface of the ring. Sutures are then placed through the tissue to be retracted. The free ends of the suture are then placed through the slits with the frictional force between the suture and the slits maintaining exposure. If greater exposure is needed, hemostats can be placed on the suture outside the ring. We measured the maximum amount of force that could be applied to the suture placed through the slit using a Vitrodyne tensionometer. The average force required to dislodge the suture (4-0 Vicryl) is 0.92 N [+ or -] 0.5 N. This is well below the tension that causes tissue injury (The Sure-Closure device, which is currently approved for clinical use to approximate wound edges, can generate 29 N of maximum tensile force). When hemostats are applied to the suture to provide more tension, the minimum force required to deform the plastic rim is the maximum amount of tension applied to tissue. The force required to deform the rim itself is 1.9 N. Therefore, our retractor offers an alterative to traditional self-retaining retractors that does not damage delicate tissues. The retractor can be fabricated in approximately 3 minute s, less time than that required for local anesthetic with vasoconstrictor to take effect. Our retractor is effective, cost efficient, and fast and has proved helpful during its use over the past 24 months.

MAGNESIUM ABNORMALITIES IN MUSCLE DISORDERS: FIBROMYALGIA, SCLERODERMA, AND DERMATOMYOSITIS. Kenneth J. Niermann, BS, Jane H. Park, PhD, Lloyd E. King, MD, PhD, and Nancy J. Olsen, MD. Departments of Radiology and Medicine, Vanderbilt University School of Medicine, Nashville, Tenn.

Magnesium (Mg) is required for all enzymatic reactions involving ATP and therefore is critical in the production of energy for muscle contraction. Magnesium deficiency is known to produce neuromuscular symptoms of fatigue, weakness, and abnormal EMG. In this report, deficiencies in magnesium and ATP levels are compared in 3 muscle disorders with clinical symptoms of weakness and fatigue: fibromyalgia (FM), scleroderma (Scl), and dermatomyositis (DM). Quadricep muscles from 11 normal control subjects, 12 FM, 13 Scl, and 11 DM patients were examined using P-31 Magnetic Resonance Spectroscopy (MRS) to determine free and ATP-bound Mg. Thigh muscles were studied during 6 min of rest, 12 min of exercise, and finally 6 min of recovery. Free and ATP-bound Mg levels were calculated from the MR spectra according to the methods of Gupta. In all patient groups, levels of free Mg were normal at rest but were significantly lower than control values (27%-42%) during exercise. At rest and during exercise, the concentration o f the MgATP complex, which is the enzymatically active form of ATP, was 13% below normal in the FM muscles, and decreased in Scl and DM by 35% and 43%, respectively. The patients' deficiencies in free Mg and MgATP persisted during the recovery phase, which may be related to fatigue. A longitudinal study of 12 DM patients, who were treated with prednisone, showed that improved muscle function correlated with increased levels of free Mg and MgATP. FM patients showed the least amount of muscle weakness and smaller losses in magnesium than the DM or Scl patients. Muscle pathology in DM and Scl, including inflammation, fat infiltration, fibrosis, and atrophy, may promote increased losses of free Mg and MgATP. In DM patients, prednisone treatment resulted in improved strength and biochemical status. These MRS data suggest a significant role for Mg in the pathophysiology of muscle diseases.

MALACOPLAKIA OF THE GI TRACT IN A PATIENT WITH SLE. Janine Serebro, BA, and Leonard Serebro, MD. LSUHSC School of Medicine in New Orleans and Ochsner Clinic and Alton Ochsner Medical Foundation, New Orleans, La.

An 18-year-old woman with SLE for 6 months with arthritis, nephritis on biopsy, positive ANA, and positive double stranded DNA was treated by her local physician with 90 mg of prednisone for 2 months and then 60 mg of prednisone for 4 months. Three months previously she had had an appendectomy with an uneventful postoperative course. She presented to Ochsner with abdominal pain and had mild tenderness and rebound. Ultrasound of the abdomen showed fluid. A catheter was inserted and pus was obtained. During surgery, an abscess was found that perforated the anterior rectal wall and the left tube and ovary, requiring a right colectomy and left salpingo-oophorectomy. Pathologic examination revealed foamy histiocytes with spherical targetoid bodies stained with calcium and PAS. These Michaelis-Gutmann bodies and the foamy histiocytes are characteristic of malacoplakia, a chronic granulomatous disease that typically affects immunocompromised patients. Since there was no evidence of vasculitis, we believe the most li kely sequence of events is that malacoplakia developed in the patient's right retrocolic area and in the left tube and ovarian areas that resulted in fistulas and abscess formation, as well as perforation of the colon. After surgery, prednisone was rapidly tapered, and the patient was treated with an antibiotic with a good recovery and subsequent viable pregnancy. Malacoplakia is a rare condition that has been previously described in the renal tract and retroperitoneal areas. It has also been described in the GI tract in inherited autoimmune disorders and immunocompromised patients, but this is the first known SLE patient to have malacoplakia involving the colon and tubulo-ovarian areas. High doses of prednisone for a prolonged period caused her to be immunocompromised. We believe that the appendicitis that occurred 3 months previously may have initiated the process since the malakoplakia developed in the same area and involved the appendiceal stump.

ACQUIRED HEMOPHILIA IN SLE. Jennifer Pedersen, DO, John G. Paty, Jr., MD, and Mukta Panda, MD. University of Tennessee-Chattanooga Unit.

The purposes of this study are (1) to recognize the entity of acquired hemophilia due to anti-factor VIII antibodies and its association with autoimmune diseases and occult malignancy, and (2) to understand the indications for treatment of acquired hemophilia. A 76-year-old white woman with a history of SLE and polymyalgia rheumatica, in remission on daily prednisone and Plaquanil, presented to the office with a 1-week history of spontaneous, extensive bruising of the right hip and thigh after arising from a chair. She denied trauma, a personal or family history of easy bruising, or prolonged bleeding. Physical examination was unremarkable except for an extensive area of ecchymosis along the right inner and outer thigh. MRI revealed iliospoas hemorrhage. Initial laboratory values showed a normochromic, normocyctic anemia with a hemoglobin of 6 (1 week before admission her hemoglobin was 10). She had a normal platelet count, prothrombin, thrombin and bleeding times, but an elevated partial thromboplastin time (57.1 seconds). The PTT partially corrected with the addition of normal plasma, so factor VIII assay was done, which showed decreased activity at 8% with positive titers for factor VIII inhibitor (72 Bethesda, normal, 0.1 to 0.8). Lupus anticoagulant and antiphospholipid antibodies were negative. Subsequently, the patient had a spontaneous right retroperitoneal and left upper extremity hemorrhage and required factor VIII transfusions, high-dose corticosteroids and oral cytoxan. Six months later she remains symptom free on 5 mg of prednisone and Plaquenil 400 mg daily. Her hemoglobin is 11.8, PTT 28.6, and there is no detectable factor VIII inhibitor. Hemophilia A, as a result of factor VIII deficiency, can be inherited or acquired. Acquired inhibitors to factor VIII are endogenous IgG/IgM immunoglobulins, which interfere with normal blood coagulation. Acquired factor VIII inhibitors can occur in patients with inherited hemophilia A and postpartum women patients with collagen vascular diseases such as SLE, or idiopathically, especially in older patients. They are also associated with occult malignancy such as lymphoma and solid organ tumors. The syndrome usually presents with significant bleeding and soft tissue ecchymoses with minimal or no trauma. Treatment of active episodes of bleeding may involve administration of exogenous porcine or human factor VIII or treatment with activated prothrombin complex concentrates as in our patient. Prophylactic treatment with steroids, with or without immunosuppressive agents, is indicated in patients with significant bleeding or with high titers of inhibitor.

ALLERGIC GRANULOMATOSIS ANGIITIS, ASSOCIATED MYOCARDIAL ISCHEMIA, AND CONGESTIVE HEART FAILURE WITH FAVORABLE LONG-TERM RESPONSE TO TREATMENT. Gene Wallace, MD, and Christopher Parker, DO. Brooke Army Medical Center, Fort Sam Houston, Tex.

Allergic granulomatosis angiitis, also known as ChurgStrauss syndrome, is a form of vasculitis that can affect small and medium-sized vessels. It affects men more commonly than women and usually develops in middle age. Typically, there are three phases to the disease: the prodromal allergic phase, peripheral blood and tissue eosinophilia, and life-threatening systemic vasculitis. Criteria for diagnosis includes asthma, eosinophilia, mononeuropathy or polyneuropathy, pulmonary infiltrates, paranasal sinus abnormality, and extravascular eosinophils. The major cause of death in these patients is myocardial infarction and congestive heart failure. The treatment of choice is glucocorticoids. When life-threatening organ involvement is present, cytotoxic therapy should be considered. We present a 73-year-old man with no known cardiac disease who was admitted with acute myocardial ischemia and congestive heart failure. His medical history was significant for recent development of asthma, two episodes of pneumonia, an d right foot drop. Chest film showed patchy infiltrates bilaterally. CBC displayed leukocytosis of 15.9, 30% eosinophilia, and ESR was 100 mm/hr. ECHO findings were positive for focal anterior and lateral segmental akinesis with an LVEF of 40% to 44%. However, coronary arteriography did not show evidence of atherosclerotic disease. Endomyocardial biopsy showed eosinophilic infiltration, vascular fibrinoid necrosis and giant cells. The patient was started on high-dose steroids with resolution of leukocytosis and peripheral eosinophilia. Within 1 month of initiating treatment, the ECHO demonstrated an improvement in LVEF to 50% to 59%. Repeated endomyocardial biopsy revealed resolution of the eosinophilic vasculitis. He completed a 6-month course of pulse cyclophosphamide. Several attempts were made to taper the prednisone dose, but daily doses below 5 mg resulted in a flare. Maintenance prednisone at 5 mg daily has resulted in stable disease activity for more than 28 months. Review of the literature regarding long-term treatment with cardiac involvement is not widely documented. We present a case of allergic granulomatosis angiitis that suggests the need for chronic daily low dose prednisone. This case shows the importance of reporting long-term data on successful management of patients with allergic granulomatosis angiitis.

BACK PAIN MIMICKING ANKYLOSING SPONDYLITIS IN A 17-YEAR-OLD BOY WITH TESTICULAR CANCER. Janine Serebro, BA, Kirk Beeson, MS, and Leonard Serebro, MD. LSUHSC School of Medicine in New Orleans and Ochsner Clinic and Alton Ochsner Medical Foundation, New Orleans, La.

A 17-year-old boy presented to the rheumatology clinic with low back pain for 3 months that started insidiously and was associated with morning stiffness lasting 1 hour. On examination, he had a Shober test of 3 cm and mild sacroiliac joint tenderness. Laboratory tests showed a sedimentation rate of 77 and C reactive protein of 8.2 (normal [less than]0.8). X-ray of the lumbar spine and Ferguson view of the SI joint were unremarkable. Presumptive diagnosis of ankylosing spondylitis was made, and the patient was given indomethacin 50 mg TID with only mild improvement in symptoms. Two weeks later, he was reevaluated, the indomethacin was increased to 200 mg a day, and he was given kenalog 120 mg IM. A repeat sedimentation rate and CRP were unchanged. He presented to the emergency department 10 days later with increasing back pain. An abdominal ultrasound showed large para-aortic lymphadenopathy and metastatic involvement of the liver. The patient denied any testicular pain, but careful examination indicated a sm all right testicular mass. Biopsy of the mass revealed a testicular germ cell tumor. He was treated with chemotherapy with eventual resolution of the cancer including the metastatic deposits and the back pain. Testicular tumors may present with symptoms mimicking ankylosing spondylitis that can result in a delay in diagnosis and poor outcome. Three lessons can be learned from this case and the review of the literature: 1. Testicular examination should be included in any young male who presents with lower back pain even if another diagnosis is suggested by history and physical examination. 2. A patient with refractory ankylosing spondylitis should have an abdominal ultrasound to rule out para-aortic involvement due to testicular cancer. 3. Lower back pain can also be a sign of recurrent testicular cancer. Early diagnosis results in the best therapeutic outcome.

THE ROLE OF THE MDR CHANNEL IN REGULATING INTRACELLULAR CONCENTRATION OF CELL PERMEABLE PEPTIDE ANTAGONIST OF CELL ADHESION. Allan F. Moore, BA, VMS III, Xue Yan Liu, MD, and Jacek Hawiger, MD, PhD. Department of Immunology and Microbiology, Vanderbilt University School of Medicine, Nashville, Tenn.

We have developed a new class of cell-permeable peptide antagonists of integrin-mediated adhesion of human erythroleukemic and endothelial cells. Integrins are major two-way signaling receptors that underlie developmental programming, homeostasis, thrombosis, inflammatory and immune responses, progression of atherosclerosis, and tumor metastesis. Cell-permeable peptides designed by us are composed of hydrophobic h region as membrane translocating sequence and functional "cargo" made of cell-adhesion regulatory domain (CARD) of human integrin b3. To determine whether this peptide "escapes" from cytoplasm through multiple drug resistance (MDR) channel, we tested its inhibitor, verapamil. We hypothesized that by blocking the MDR channel with verapamil we will enhance inhibiting potency of cell-permeable peptide antagonist of integrin-mediated adhesion. A cell adhesion assay was used to measure integrin aII[beta]3 human erythroleukemic (HEL) cell adhesion to fibrinogen-coated plates. The cells were pre-incubated with 1) anti-aIIb[beta]3 antibody, 2) cell permeable [beta]3-1S peptide, or 3) verapamil followed by cell permeable [beta]3-1S peptide. Cells were then centrifuged and washed to remove extracellular peptide, resuspended, stimulated with HEL antagonist, 4[beta]-phorbol 12-myristate 13-acetate (PMA), and incubated on a fibrinogen-coated 96 well microtype plate for two hours at 37 C in 5% [CO.sub.2]. Cell attachment was quantitated by a colorimetric reaction measuring endogenous cellular acid phosphatase activity and read on an ELISA plate reader at 405 nm. The adhesion of HEL cells was PMA-dependent reading maximum at 10 nM concentration. Blocking aIIb[beta]3 integrins with specific antibody reduced HEL cell adhesion to the baseline level. Cell-permeable [beta]3-1S peptide inhibited adhesion in a concentration-dependent manual. The concentration [beta]3-1S peptide causing 50% inhibition of adhesion (IC50) was 32mM. Addition of verapamil (100mM) reduced IC50 of [beta]3-1S peptide 3 fold. These results indicate t hat intracellular concentration of cell-permeable peptide antagonist of integrin-mediated cell adhesion can be increased by verapamil and possibly other MDR channel inhibitors.
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Publication:Southern Medical Journal
Article Type:Statistical Data Included
Geographic Code:1USA
Date:Oct 1, 2001
Words:7598
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