Leukemia & Lymphoma, March 2011; 52(3): 539540

LETTER TO THE EDITOR

Hairy cell leukemia with ascites: an unusual presentation

SMITA KAYAL1, VENKATRAMAN RADHAKRISHNAN1, SARIKA SINGH2, ANITA CHOPRA3, RAJIVE KUMAR2, & VINOD RAINA1
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Departments of 1Medical Oncology, 2Laboratory Oncology, and 3Hematology, Dr. B. R. A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India (Received 6 May 2010; accepted 8 May 2010)

Hairy cell leukemia (HCL) is a rare chronic lymphoproliferative disorder characterized by the presence of splenomegaly, pancytopenia, and the presence of abnormal B-lymphocytes with cytoplasmic projections giving the appearance of hairs projecting from their surface [1]. Unusual presentations of HCL include marked leukocytosis, spontaneous splenic rupture, bulky chest or abdominal lymphadenopathy, destructive bone lesions, and neurological complications including meningitis and peripheral nerve compression [2]. Ascites has been rarely reported as a presenting feature of hairy cell leukemia [3]. A 52-year-old male presented with history of intermittent fever, fatigue, weight loss of 5 months duration, and abdominal swelling of 2 weeks duration. On examination he had massive ascites. He had no peripheral lymph node enlargement or clinical signs of chronic liver failure. Ultrasonography of the abdomen showed gross ascites, splenomegaly (15 cm), normal liver size and echotexture, and no lymph node enlargement. A computed tomography (CT) scan of the abdomen showed splenomegaly with multiple focal lesions, ascites, and mild right pleural effusion. His complete blood count was hemoglobin 9.3 g/dL, white blood cell (WBC) count 1500/dL, absolute neutrophil count 1200/dL, and platelets 64 000/dL. The differential count showed 30 neutrophils, 15 lymphocytes, two mononuclear cells, and three eosinophils out of 50 cells. Bone marrow aspirate showed 40% abnormal lymphoid cells with typical hairy cell morphology [Figure 1(a)]. Bone marrow biopsy revealed 3035% cellularity,

with inltration by abnormal CD20-positive lymphocytes showing the classical fried-egg appearance described in HCL. Flow cytometric analysis of the bone marrow aspirate showed a population of abnormal lymphoid cells positive for CD19, CD20, CD11c, CD25, and CD103 and kappa light chain restriction consistent with HCL. Abdominal paracentesis revealed straw-colored ascitic uid with a serum/ascitic uid albumin gradient (SAAG) ratio of 51.1, consistent with exudative nature of the uid. The ascitic uid showed WBC count 840/dL with 40% polymorphs and 60% lymphocytes, red blood cells (RBCs) 1200/dL, and numerous atypical lymphoid cells with hairy cell morphology on Jenner Gimesa stain [Figure 1(b)]. Flow cytometric analysis of a centrifuged specimen of ascitic uid showed a small population (*0.7%) of CD19 cells coexpressing CD11c and CD25 and showing kappa light chain restriction compatible with the presence of HCL cells in the ascitic uid. Acid-fast bacilli staining for tuberculosis and bacterial culture from the ascitic uid were negative. Liver function studies were normal except for a low albumin of 2.9 g/dL. Antibody screening tests for hepatitis B, hepatitis C, and human immunodeciency virus were negative. An upper gastrointestinal endoscopy study was normal. The patient was treated with cladribine 0.14 mg/kg/day as 2 h infusion for 5 days. He had a signicant reduction in the ascites and improvement of general well-being within 2 weeks of starting the treatment. Ascites in HCL is very rare [25], and ascites as the presenting feature of HCL has been reported only

Correspondence: Dr. Vinod Raina, Professor of Medical Oncology, Department of Medical Oncology, Dr. B. R. A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India. Tel: 91-11-26593679. Fax: 91-11-26588408. E-mail: vinodraina@hotmail.com ISSN 1042-8194 print/ISSN 1029-2403 online 2011 Informa UK, Ltd. DOI: 10.3109/10428194.2010.492538

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Letter to the Editor

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Figure 1. (a) Bone marrow showing atypical lymphoid cells with abundant cytoplasm, ne chromatin, prominent nucleolus, and cytoplasmic projections (61000 magnication, JennerGiemsa stain). (b) Ascitic uid showing atypical lymphoid cells with abundant cytoplasm and ne cytoplasmic projections (61000 magnication, JennerGiemsa stain).

Table I. Ascites due to hairy cell leukemia: a review of published case reports. Age in years/sex 35/M 37/M 41/M 49/M 52/M Time from diagnosis 10 months 11 months 16 months Baseline Baseline Abdominal disease LN LN LN Peritoneal deposits None Nature of uid Exudative Exudative Chylous Exudative Exudative Hairy cells in ascitic uid Yes Yes Mononuclear cells Yes Yes Pleural effusion Yes Yes No Yes No

Case report Bouroncle [2] Davies et al. [4] Dincol et al. [3] Present case

Treatment RT CDA RT CDA CDA

Outcome CR PR CR CR PR

M, male; LN, lymphadenopathy; RT, radiotherapy; CDA, cladribine; CR, complete response; PR, partial response.

once in the literature [3]. The pathogenesis of ascites in HCL is not known; it is hypothesized that abdominal lymph nodes and/or peritoneal deposits in HCL give rise to ascites, similar to what is seen in patients with non-Hodgkin lymphoma. So far, all the cases of ascites due to HCL reported in the literature have been associated with abdominal lymphadenopathy and/or peritoneal deposits, unlike our case where there was no evidence of the same; this suggests that there may be other mechanisms for the pathogenesis of ascites in HCL. Our case presented with splenomegaly, ascites, and pancytopenia. This constellation of ndings can also be seen with liver disease and portal hypertension. Cases of HCL with histologically proven cirrhosis of the liver have been described in the literature [3]. However, liver cirrhosis and portal hypertension were ruled out after adequate investigations in our case. Ascites in HCL can be chylous or non-chylous, and is usually associated with pleural effusion. In our setting, abdominal tuberculosis is an important differential diagnosis, especially in HCL, as patients are at a higher risk of tuberculosis due to a suppressed cellmediated immune system secondary to the disease and treatment [1]. Ascites in HCL responds to treatment with cladribine [2,4], and patients have

also shown complete response with abdominal radiotherapy [2,3]. A summary of case reports in the literature on ascites in HCL is provided in Table I. The presence of ascites at baseline can misguide the physician from a diagnosis of HCL, as chronic liver disease and non-Hodgkin lymphoma would be a more appropriate diagnosis in such a setting. The pathologist should also be alerted, when sent uid samples, to carefully look for the presence of hairy cells. This case highlights that ascites can be a rare presenting feature of HCL, hairy cells can be demonstrated in the ascitic uid, and therapy with cladribine is effective in treating ascites. References
1. Grever MR. How I treat hairy cell leukemia. Blood 2010;115:2128. 2. Bouroncle BA. Unusual presentations and complications of hairy cell leukemia. Leukemia 1987;1:288293. 3. Dincol G, Doan O, Kucukkaya RD, Gul E, Kahraman R, g Aan M. Hairy cell leukaemia presenting with ascites, pleural g effusion and increased CA 125 serum level. Neth J Med 2008;66:2326. 4. Davies GE, Wiernik PH. Hairy cell leukemia with chylous ascites. JAMA 1977;238:15411542. 5. Krause JR, Dekker A. Hairy cell leukemia (leukemic reticuloendotheliosis) in serous effusions. Acta Cytol 1978;22:8082.