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A case of adult acute T-cell lymphoblastic leukemia presented with hemophagocytic syndrome/Hemofagositik sendromla prezente olan akut T-hucreli lenfoblastik losemi olgusu.

Patients with acute lymphoblastic leukemia (ALL) present most frequently with signs and symptoms of the uncontrolled growth of leukemic cells in bone marrow, lymphoid organs and other sites of extramedullary spreading such as cerebrovascular disease, pleural effusions, blindness, etc. [1-3]. Hemophagocytic syndrome (HPS) is an unusual acute syndrome presenting with fever, hepatosplenomegaly, and cytopenias. The hallmark of HPS is the accumulation of activated macrophages that phagocyte hematopoietic cells in the reticuloendothelial system. Most cases of HPS in adults are secondary to some disorders such as infection or malignancy, and thus investigation of the underlying disease is necessary. Herein, we report a case of ALL presented with HPS.

A 22-year-old male patient referred to our clinic because of pancytopenia. The patient experienced febrile attacks one month before hospitalization but at that time no etiological factor that could cause fever had been demonstrated. The patient was hospitalized by the Department of Pulmonary Diseases because of pulmonary infiltrates. Febrile attacks did not resolve despite use of broad spectrum antibiotics and antifungal drug. However Candida spp. was detected with bronchoalveolar lavage. Complete blood count values were reported to be normal in this period. The patient consulted the Hematology Department for development of anemia. Atypical cells with lymphomonocytoid appearance were present in peripheral blood film. Bone marrow aspiration and biopsy were performed. Bone marrow aspiration showed an increase in histiocytes, the cells phagocytizing erythrocytes and platelets (Figure 1). There was no malignant infiltration in the marrow. Periodic acid-Schiff and peroxidase staining were negative. The patient remained febrile during this period and neutropenia and thrombocytopenia developed in addition to anemia. Intravenous immunoglobulin treatment (1 g/kg for 2 days) was given. Ten days after immunoglobulin treatment, firstly thrombocytopenia and later neutropenia resolved. Peripheral blood film examination and white blood cell differential were normal. Bone marrow was reevaluated and findings of hemophagocytosis had disappeared and bone marrow was normal in appearance. The patient was no longer febrile. Intravenous antifungal treatment was switched to oral voriconazole and the patient was discharged with regular follow-up. The patient admitted with the findings of pancytopenia one month later. Peripheral blood film showed atypical lymphomonocytoid cells and we proceeded to bone marrow examination. There was blastic infiltration of 50%. The blasts had a scanty, agranular, basophilic cytoplasm and a large nucleus with 1 to 3 nucleoli. Analysis of surface antigens showed that 49% of cells were blast cells positive for CD7, cytoplasmic CD3 and terminal deoxynucleotidyl transferase (TdT). Cytogenetic examination revealed normal karyotype. The diagnosis of T-cell lymphoblastic lymphoma was made. Four-drug remission induction chemotherapy including cyclophosphamide 1200 mg/[m.sup.2] once on day 1, vincristine 2 mg once weekly (days 1, 8, 15 and 22), daunorubicin 45 mg/[m.sup.2] for 3 days and prednisolone 60 mg/[m.sup.2] po daily for 21 days was started. The patient reached hematological remission after one cycle of chemotherapy and we proceeded to central nervous system prophylaxis with cranial radiotherapy and intrathecal methotrexate. The patient experienced an early relapse two weeks after completion of radiotherapy.


ALL might present with different symptoms and signs. Herein, we present a case of adult T-cell ALL who presented with HPS. Although we could not clarify the underlying cause of hemophagocytosis, it was possibly related to an infection. The leukemia developed almost two months after successful treatment of hemophagocytosis.

A 20-year-old male patient was reported by Nishiki et al. [4] as the first adult leukemia/lymphoma patient presenting with hemophagocytosis. Suzuki et al. [5] reported development of lymphoblastic leukemia after hemophagocytosis and they interpreted this condition as a kind of secondary leukemia. Goldschmidt et al. [6] reported a similar case of ALL developing after hemophagocytosis in 2005 who was diagnosed after repeated bone marrow biopsies. There is a one- or two-month interval between HPS and development of acute leukemia in most of the cases reported in the literature, as was the case in our patient. Our case and the other reported cases of acute T-cell lymphoblastic leukemia demonstrate the importance of follow-up and repeated testing in cases of HPS.


[1.] Arboix A, Besses C. Cerebrovascular disease as the initial clinical presentation of haematological disorders. Eur Neurol 1997; 37: 207-11.

[2.] Mayo GL, Carter JE, McKinnon SJ. Bilateral optic disk edema and blindness as initial presentation of acute lymphocytic leukemia. Am J Ophthalmol 2002; 134: 141-2.

[3.] Chaves FP, Quillen K, Xu D. Pericardial effusion: a rare presentation of adult T-cell leukemia/lymphoma. Am J Hematol 2004; 77: 381-3.

[4.] Nishiki S, Yamane T, Hino M, Ohta K, Sannomiya Y, Inoue T et al. B precursor lymphoblastic leukemia/lymphoma manifested at onset as hemophagocytic syndrome. Rinsho Byori 2000; 48: 975-9 (abstract).

[5.] Suzuki T, Mugishima H, Yamada A, Nagata T, Shichino H, Chin M et al. Development of acute lymphoblastic leukemia following hemophagocytic lymphohistiocytosis: is it secondary leukemia? Int J Hematol 1999; 70: 58-9.

[6.] Goldschmidt N, Gural A, Kornberg A, Spectre G, Shopen A, Paltiel O. Prolonged fever of unknown origin and hemophagocytosis evolving into acute lymphoblastic leukemia. Am J Hematol 2005; 78: 246-7.

Ahmet Ifran (1), Fahri Sahin (2), Kursat Kaptan (1), Mukerrem Safali (3), Cengiz Beyan (1)

(1) Department of Hematology, Gulhane Military Medical Academy, Ankara, Turkey

(2) Department of Hematology, Ege University, Medical Faculty, Izmir, Turkey

(3) Department of Pathology Gulhane Military Medical Academy, Ankara, Turkey

Address for Correspondence: Dr. Ahmet Ifran, Gulhane Military Medical Academy Hematology Department 06010 Etlik, Ankara, Turkey

Phone: +90 312 304 41 04 Fax: +90 312 304 41 00 E-mail:
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Title Annotation:Letter to the Editor
Author:Ifran, Ahmet; Sahin, Fahri; Kaptan, Kursat; Safali, Mukerrem; Beyan, Cengiz
Publication:Turkish Journal of Hematology
Article Type:Report
Geographic Code:7TURK
Date:Sep 1, 2008
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