Pediatric management problems.
Mrs. MacMillan brings her 10-year-old daughter Jessica to her pediatrician's office for evaluation of her skin. Jessica states that a "weird rash" began on the left side of her neck 1 day ago. She noticed the rash after waking up from a nap that she took after school. Jessica had shown the rash, which she described as "un-itchy dots" to her mother. They both dismissed it as an insect bite rash. Last night, Jessica had spontaneous gum bleeding that resolved with light pressure after a few minutes. Today, Jessica woke up with the taste of blood in her mouth and noticed that the rash was now on her left temple, lower legs, ankles, and feet. Upon further questioning, it was learned that 1 week earlier, Jessica had bleeding from an old left earlobe piercing. She has also noticed more than the usual amount of bruising within the past week, often for unexplained reasons. Jessica has been afebrile and denies epistaxis, visible blood in the urine or stool, or other symptomatology. She has not yet reached menarche. Tick bites, recent exposure to wooded areas, recent injuries, and recent travel were also denied. Two weeks prior to this visit, Jessica had an upper respiratory infection (presumed to be viral), which has since resolved.
Past Medical and Family History
Previous episodes of easy bruising or bleeding were denied. Past medical history is unremarkable. Previous surgeries and significant injuries were denied. Jessica takes no medications including vitamins, over-the-counter drugs, and herbal remedies. She has no known allergies to environmental allergens, medications, or foods. Immunizations are up-to-date. Jessica is a gregarious fifth grader who likes school and gets good grades. She practices cheerleading after school. Jessica lives with her parents and a younger brother. Her father is employed outside of the home, and her mother is a homemaker. Family history is positive for heavy menstrual cycles in her mother and a questionable "clotting disease" in her paternal grandfather and paternal aunt.
Vital signs: T - 98.8 [degrees] F (37.1 [degrees] C) orally; P - 100; R - 24; BP - 117/62.
Height: 4'9" (144.7 cm) (75th percentile).
Weight: 63.2 lbs. (31.6 kg) (between 25th and 50th percentiles).
General: Tall and slender for age, friendly. Appears happy, healthy, and in no distress.
Skin: Multiple petechiae on left temple, left lateral neck, left hip, buttocks, lower legs, ankles, and dorsal, medial, lateral, and plantar aspects of both feet. One 5 cm ecchymosis noted to the left of the mid spine. One 4 cm ecchymosis on the right elbow. Several smaller bruises over both knees and shins.
HEENT: Normocephalic. Sclerae and conjunctivae clear. Red reflex present bilaterally. Corneal light reflexes normal. Fundoscopic exam reveals no hemorrhages. PERRLA. Extra-ocular movements intact. Tympanic membranes clear. Nares patent bilaterally with no epistaxis or other drainage. Oral mucous membranes are pale pink with diffuse buccal, gingival, and palatal petechiae noted. A small amount of blood is visualized in the oral cavity. Tonsils are clear. Neck is supple with full range of motion, no masses.
Lymphatic system: No nodes palpable.
Thorax/lungs: Breasts Tanner stage III; equal chest expansion; lungs clear to auscultation.
Cardiovascular: Heart rate and rhythm regular; S1, S2 clearly auscultated. No extra sounds, murmurs, or rubs noted.
Gastrointestinal: Abdomen soft, nontender, nondistended. No masses or hepatosplenomegaly. Normoactive bowel sounds in 4 quadrants. Anal orifice clean without lesions or bleeding.
Genitourinary: Tanner stage III; labia pink, clean; hymen intact; no vaginal bleeding.
Musculoskeletal: Gait steady. Spine midline. Muscle strength equal in all extremities.
Neurologic: Alert and oriented.
Laboratory results: WBC 7.8 k/ul; RBC 4.5 m/ul; hemoglobin 13.5 gm/dl; hematocrit 38.9%; platelet count 9 k/ul; absolute neutrophil count (ANC) 4.7. Peripheral smear showed no blasts and was essentially normal except for the presence of larger than usual sized platelets.
What is Your Assessment?
The differential diagnosis for Jennifer included the following: (a) acute leukemia; (b) trauma; (c) infectious thrombocytopenia; (d) Henoch-Schonlein purpura (HSP); and (e) idiopathic thrombocytopenic purpura (ITP).
1. Leukemia, although unlikely, should always be considered in the differential diagnosis of a child with petechiae, increased bruising, and unusual bleeding. A child with acute leukemia would most likely present with additional complaints, such as fatigue, anorexia, weight loss, bone pain, and frequent or persistent infections (Lanzkowsky, 2000). Physical exam findings may also demonstrate the presence of fever, tachycardia, tachypnea, dyspnea, pallor, lymphadenopathy, hepatomegaly, and/or splenomegaly (Lanzkowsy, 2000). A complete blood count (CBC) would most likely demonstrate anemia; a low, normal, or elevated white cell count; and thrombocytopenia (Lanzkowsky, 2000). The differential would show neutropenia (an absolute neutrophil count [ANC] of < 1,000 per microliter) (Hay, Hayward, Lein, & Sondheimer, 1999). An increased number of immature white cells or "blasts" may also be seen on peripheral smear (Lanzkowsky, 2000). Although Jessica is severely thrombocytopenic, she is neither anemic nor neutropenic, and given that she is otherwise healthy with stable vital signs, no other complaints, and no symptoms of underlying disease, leukemia was not suspected. Of course, the only way to truly rule out leukemia is through bone marrow aspiration and biopsy.
2. Trauma was ruled out in Jessica's situation because petechiae or purpura that are caused by accidental or intentional injury usually fade within a few days and are confined to local areas (Fox, 1997). There also would be no history of spontaneous bleeding, as there was in Jessica's case. She and her mother offered no history of recent injury. In this situation it is obvious that severe thrombocytopenia, not trauma, is the cause of Jessica's signs and symptoms. It is important to keep in mind that when any child presents with petechiae and ecchymoses, the possibilities of accidental injury, abuse, any kind of mechanical pressure (such as cupping or coining, which are acceptable practices in some cultures), and excessive crying, coughing, or distress (mostly in infants and young children) can all cause petechiae (Fox, 1997).
3. Infectious thrombocytopenia can result from a number of potentially life-threatening bacterial, viral, or rickettsial infections. Examples of these include meningococcemia, septicemia, bacteremia, HIV infection, echovirus infection, and Rocky Mountain Spotted Fever. The classic signs of this diagnosis are fever with petechiae or purpura (Fox, 1997). The child would also most likely have a toxic appearance. Since Jessica has been afebrile, is healthy in appearance, has no signs or symptoms of infection, and had no history of recent exposure to wooded areas, this diagnosis was easily ruled out.
4. HSP is a common form of vasculitis in childhood that often follows an upper respiratory tract infection. The rash of HSP is maculopapular and characteristically confined to the buttocks and lower extremities. Since Jessica's rash was macular and the petechiae, bruising, and bleeding were diffuse, HSP was ruled out.
5. Acute ITP is the most common bleeding disorder of childhood. The classic signs of ITP are petechiae with mucocutaneous bleeding, purpura, and thrombocytopenia (platelet count of < 100 k/ul). ITP often occurs following a viral infection and is thought to be an autoimmune response that results in destruction of platelets. Although it is most commonly seen in 2- to 5-year-olds, it can occur at any age. This condition occurs in an otherwise healthy child, is self-limiting, and usually undergoes spontaneous remission within 9-12 months. Most, but not all cases of ITP, can be managed on an outpatient basis. Hemorrhage and bleeding into vital organs are rare but serious complications of ITP. In a small percentage of individuals, ITP can become recurrent or chronic (Fox, 1997; Hay et al., 1999; Lanzkowsky, 2000). Jessica displays the classic signs of ITP, making it the most likely diagnosis.
What is Your Management Plan?
Although most cases of ITP can be managed by the primary care practitioner, Jessica's case warranted immediate referral to a hematologist due to the severity of her thrombocytopenia and degree of spontaneous bleeding. She was re-evaluated in the pediatric hematology clinic at a large tertiary care hospital that same day. A repeat CBC with platelet count, differential, and peripheral smear, prothrombin time (PT), partial thromboplastin time (PTT), fibrinogen level, type and screen, stool for guaiac testing, and urine for microscopic analysis were ordered.
The repeat CBC with platelet count, differential, and peripheral smear were done to confirm the earlier finding of thrombocytopenia, as well as to detect other hematologic abnormalities of diagnostic significance. PT, PTT, and fibrinogen levels measure the extrinsic and intrinsic pathways of the clotting cascade, and thus were drawn to look for abnormalities of coagulation. A type and screen was drawn to determine Jessica's Rh status for possible treatment with Rho(D) immune globulin, which can only be given to Rh + patients (Siberry & Iannone, 2000). Stool for guaiac testing and microscopic urinalysis were done to check for occult blood loss, which may have occurred from these sites secondary to the thrombocytopenia.
Results of the repeat CBC confirmed the presence of severe thrombocytopenia, with the platelet count showing a decrease from 9 k/ul to 2 k/ul within 6 hours. Other CBC values were normal. Coagulation studies were normal. Type and screen was A+, Coomb's negative. Urine and stool were both negative for blood. With ITP, laboratory results will demonstrate thrombocytopenia in the absence of anemia or neutropenia, as well as normal bleeding times (in an otherwise well child). Because of this, the diagnosis of acute, severe ITP was confirmed by the hematologist.
Immediate hospitalization and treatment with intravenous Rho(D) immune globulin (WinRho) was initiated per hematologist's orders. WinRho is an immune globulin specifically designed to treat ITP and has been shown to effectively slow the destruction of platelets in approximately 80% of patients treated (Hay et al., 1999, p. 755). Other medications used in the treatment of ITP include intravenous immune globulin (IGIV) and corticosteroids (Hay et al., 1999; Siberry & Iannone, 2000). The recommended initial dosage of WinRho is 50 mcg/kg IV and can be given as a single dose or in two divided doses on different days to children with a hemoglobin of greater than or equal to 10 mg/dl (Siberry & Iannone, 2000). Jessica received approximately 30 mcg/kg, or 950 mcg IV x 1 dose, with the plan for her to receive the additional 20 mcg/kg the next day. Unfortunately, she developed an adverse reaction to the first dose (temperature to 100 [degrees] F, rigors, severe headache, and nausea), and thus had to receive a total of 37.5 mg of Benadryl[R] IV, with resultant relief of symptoms. The additional dose of WinRho was discontinued and her drug allergy was documented. Jessica was alternatively treated with a 3-day inpatient course of high dose pulse steroids IV, which she tolerated well: SoluMedrol[R] at 30 mg/kg/dose, or 950 mg IV per dose. Her platelet counts were monitored closely every day while in the hospital. Jessica was discharged home after 5 days in stable condition on oral steroids and with a platelet count of 10 k/ul. Patients with overt bleeding or platelet counts less than 10 k/ul have often been treated with prednisone 2-4 mg/kg in daily divided doses for 1-4 weeks, followed by a slow taper (Hay et al., 1999). Jessica was sent home on prednisone 30 mg po BID x 4 weeks (2 mg/kg/day). Her dose was then tapered slowly over the next several weeks.
It is important to remember that many children require no treatment, and therapy is only indicated for patients with platelet counts less than 10 k/ul, less than 20 k/ul with bleeding, or at high risk of severe bleeding (Fox, 1997; Hay et al., 1999). Splenectomy is indicated only in cases of severe, acute ITP with serious, life-threatening bleeding that can not be controlled by other medical measures and with chronic, unremitting ITP that has been unresponsive to other therapies (Hay et al., 1999). Follow up for children with ITP is generally recommended once a week until platelet count is stable and then once a month until platelet count is normal (Fox, 1997; Lanzkowsky, 2000).
The main goal of care for the patient and family living with ITP is to prevent injury and to minimize bleeding episodes as much as possible until platelet count spontaneously normalizes. Jessica and her mother were instructed on the usual course of ITP; to observe for signs and symptoms of bleeding at home, including life threatening signs of intracranial hemorrhage (such as headache, vomiting, sleepiness, or change in mental status); and to immediately notify the hematologist if bleeding or impact injury, especially to the head, stomach, or back, occurs. Avoidance of medications that may precipitate bleeding such as aspirin and NSAIDs was discussed. Jessica was instructed to blow her nose very gently (only when necessary), to use a soft bristled toothbrush, and to avoid flossing until her platelet count was higher. Generous intake of fruit juices, water, and high fiber foods was advised in order to prevent constipation, which can cause rectal bleeding. Jessica was also told not to strain with bowel movements in order to prevent intracranial bleed. The importance of not participating in contact sports and cheerleading until permitted by the hematologist was discussed. Her medication regimen was reviewed, including drug, dose, route, frequency, duration of therapy, and possible side effects. Importance of compliance with the medication regimen as well as with appointments for follow-up and blood work to assess platelet count and response to treatment was emphasized. Jessica and her mother were also told that the school nurse needed to be notified of the diagnosis.
These instructions as well as other safety and comfort measures, such as the use of a seat belt whenever in any moving vehicle, the application of moisturizers and lip balm to prevent drying, cracking, and subsequent bleeding of skin, the use of saline nose drops and a cool mist humidifier in the bedroom to alleviate nasal dryness and prevent epistaxis, and the immediate application of pressure to any areas of bleeding, were also reviewed. Verbalization of questions and concerns was always encouraged.
Jessica has been experiencing occasional nosebleeds at home, but is otherwise doing well. Her platelet count 1 month after diagnosis was 82 k/ul and continues to improve.
Fox, J.A. (1997). Primary health care of children. St. Louis: Mosby-Year Book, Inc.
Hay, W.W., Hayward, A.R., Lein, M.J., & Sondheimer, J.M. (1999). Current pediatric diagnosis & treatment (14th edition). Stamford, CT: Appleton & Lange.
Lanzkowsky, R (2000). Manual of pediatric hematology and oncology (3rd edition). San Diego: Academic Press.
Siberry, G.K., & Iannone, R. (2000). The Harriet Lane handbook. St. Louis: Mosby, Inc.
Contributed by: Deborah Hernandez, MS, RN, PNP Graduate Student State University of New York at Stony Brook Stony Brook, NY
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|Author:||Belkengren, Richard; Sapala, Shirley|
|Date:||Jan 1, 2002|
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