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Hairy Cell Leukemia in the Thai Population: a Case Report

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Hairy Cell Leukemia in the Thai Population: a Case Report Case Report A Rare Occurrence of Hairy Cell Leukemia in the Thai Population: A Case Report Ekapun Karoopongse MD*, Archrob Khuhapinant PhD, MD*, Chirayu U Auewarakul MD, PhD* *Division of Hematology, Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand Hairy cell leukemia (HCL) has been mainly reported from the Western countries. Herein we describe a case of HCL diagnosed in a Thai patient. A 36-year-old man presented with abdominal discomfort, frequent gum bleeding and significant weight loss for 2 months. Physical examination revealed moderate anemia, petechial hemorrhage on the extremities and an enlarged spleen down to the umbilicus. No hepatomegaly or lymphadenopathy was detected. Complete blood counts revealed a hemoglobin (Hb) of 6.6 g/dL, a white blood cell (WBC) count of 1.6x109/L (neutrophil 16%, lymphocyte 71%, monocyte 11%, atypical lymphocyte 1%), and a platelet (PLT) count of 17x109/L. Abnormal large mononuclear cells with villous projections were seen in the blood smear. Although bone marrow (BM) aspiration resulted in a dry tap, abnormal lymphocytes with villous projections could again be identified in the touch preparation. Flow cytometric analysis showed a distinct population above the normal lymphocyte region on CD45/SSC gates with a strong expression of CD19, CD20, CD22, CD25, CD11c, and kappa. CD5, CD23, CD10, CD4, and CD8 were all negative. BM biopsy was consistent with HCL. The patient was treated with splenectomy followed by 8 cycles of fludarabine and cyclophosphamide chemotherapy. At 21 months after diagnosis, the patient was doing well with a Hb of 16.9 g/dl, a WBC count of 6.8x109/L, neutrophil 49.9%, lymphocyte 39.6%, monocyte 8.6%, and a PLT count of 329x109/L). No abnormal lymphoid cells were detected in the blood smear. This present report represents the first Thai HCL case that was immunophenotypically confirmed by flow cytometry and successfully treated at Siriraj Hospital. Keywords: Hairy cell leukemia, lymphoid neoplasms, flow cytometry, splenectomy, fludarabine J Med Assoc Thai 2010; 93 (Suppl. 1): S196-202 Full text. e-Journal: http://www.mat.or.th/journal Hairy cell leukemia (HCL) is an uncommon neutropenia, and thrombocytopenia, although some type of mature B-cell lymphoid neoplasm characterized patients may be asymptomatic and some may have el- by splenomegaly and pancytopenia(1,2). The diagnos- evated white blood cell (WBC) counts(1,6). HCL occurs tic hallmark of HCL is the detection of abnormal lym- more frequently in Caucasians, particularly in Ashkenazi phoid cells with circumferential hair-like cytoplasmic Jews, with rare occurrence in persons of Asian and projections in the blood, marrow, spleen, and/or liver, African descents. The incidence of this disease in the and unique exhibition of mature B-cell antigens and United States and the United Kingdom appear to be monoclonal surface immunoglobulin(3-5). Typically, pa- similar, with approximately 600-800 newly diagnosed tients are middle-aged to elderly men who present with cases a year(6-8). In Asian countries, HCL cases were a palpable spleen, usually a least 5 cm below the left rarely reported(8-13). Herein a clinical description of a costal margin as well as symptoms related to anemia, classical case of HCL in a Thai man is presented, which epitomizes the first successfully treated HCL ever re- Correspondence to: Auewarakul C, Division of Hematology, ported from Thailand. Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkoknoi, Bangkok 10700, Thailand. Phone: 0-2419-4418, Fax: 0-2411-2012. Email: sicaw@ Case Report mahidol.ac.th A 36-year-old Thai man presented with a his- S196 J Med Assoc Thai Vol. 93 Suppl. 1 2010 tory of abdominal discomfort for 4 months. The patient CD10, CD4, and CD8 were negative. The pathological had been in good health until four months earlier when and immunophenotypic results were thus consistent he started to feel that his abdomen was gradually en- with HCL. larged. A firm mass in the left upper abdomen was noted. Due to symptomatic pancytopenia and sple- He felt generally tired and had a 15-kilogram weight nomegaly, it was decided to perform splenectomy to loss without spiking fevers. He subsequently devel- relieve the symptoms. The patient received pneumoccal oped petechial hemorrhage all over his arms and legs and hemophilus influenza vaccines and blood transfu- associated with frequent episodes of bleeding per gum sion with 2 units of packed red blood cells and 12 units which prompted him to seek medical advice. The pa- of platelet concentrates in preparation for surgery. The tient denied tobacco and alcohol abuse and was not spleen was removed with no immediate complications. using any medications. Physical examination revealed The gross review of the spleen showed a large spleen a moderately pale man without jaundice and fever. Scat- measuring 35 x 24 x 9 cm and weighing 4,000 gm. Histo- tered petechial hemorrhage was found on both of his pathology study showed extensive involvement by arms and lower legs. The respiratory, cardiovascular small lymphoid cells with monocytoid features and and nervous system was within normal limits. Abdomi- presence of few microscopic blood lakes consistent nal examination revealed a firmly enlarged spleen ex- with HCL. The follow-up CBCs after surgery are shown tending down to the level of his umbilicus. Liver was in Table 1. The patient was subsequently treated with not palpable and no sign of chronic liver disease nor oral chemotherapy regimen consisting of fludarabine ascites was present. All superficial lymph nodes were (F) 25 mg/m2 and cyclophosphamide (C) 150 mg/m2 for not palpable. The basic laboratory investigations were 4 days every 4 week. Co-trimoxazole was also prescribed performed as shown in Table 1. The patient had pancy- in order to prevent infection during chemotherapy. The topenia with hemoglobin (Hb) of 7.5 g/dL, a white blood patient did well and completed 8 cycles of FC regimen cell (WBC) count of 2.4 x 109/L, absolute neutrophil without complications. CBC at 21 months after the ini- count (ANC) of 0.48 x 109/L and a platelet (PLT) count tial diagnosis was normal. No abnormal cells were de- of 28 x 109/L. Peripheral blood smear showed normo- tected in the blood smear or by flow cytometry. Ab- chromic normocytic red blood cells with decreased dominal imaging study revealed a normal-sized liver platelets. Abnormal small to medium-sized mononuclear with no intraabdominal or intrapelvic lymph nodes. cells with circumferential cytoplasmic projections were found as shown in Fig. 1. Radiologic investigation was Discussion also performed which demonstrated marked splenom- Fifty years have passed since the original re- egaly with the dimension of 23 cm, mild hepatomegaly, port of HCL by Bouroncle et al in 1958(14). In the recent and celiac node enlargement. 2008 report by the US National Cancer Institute’s Sur- As the provisional diagnosis was HCL, bone veillance, Epidemiology and End Results (SEER) pro- marrow (BM) aspiration and biopsy were subsequently gram, there were 2,856 cases of HCL diagnosed during performed. BM aspiration resulted in a dry tap but nu- 1978-2004 with the age-specific incidence rates (IR) of merous mononuclear cells with hairy projections were 0.32 per 100,000 person-years(6). IRs were about 4-fold again identified in the touch preparation. BM biopsy greater among men than women and more than 3-fold revealed moderate to marked hypercellularity with dif- higher for whites than blacks. A bimodal age distribu- fuse infilttration by widely-spaced lymphoid cells with tion was revealed in this large study with a dominant abundant cytoplasm and prominent cell borders, pro- early-onset and late-onset modes near ages 45 and 80 ducing a “fried-egg” appearance (Fig. 2). These cells years, respectively, in females. Among males, the peak were intensely marked with CD20 (as shown in Fig. frequency was near age 55 years with a less prominent 3A), focally marked with CD79a, and not marked with late-onset mode. The majority of patients presented CD3 (Fig. 3B). with pancytopenia (50-70%) and splenomegaly (80%)(1). The BM biopsy staining for CD5, CD10, CD23, Other features include monocytopenia and myelofibro- CD 34, CD68, cyclin D1, TdT, myeloperoxidase, kappa sis(3). and lambda light chain was all negative. Flow cytometric In Asian countries, there are a limited number analysis of peripheral blood revealed a distinct popu- of reports on HCL. The largest study came from Japan lation of cells above the lymphocytic region in the CD45/ but the Japanese HCL cases appeared to have a differ- SSC gates with expression of CD19, CD20, CD22, CD25, ent feature from the classical HCL reported from the CD11c, and FMC7, as shown in Fig. 4. CD5, CD23, West. In a series of 40 cases reported by Machii T et al, J Med Assoc Thai Vol. 93 Suppl. 1 2010 S197 only 9 cases were classical HCL, 2 cases were HCL- series of 5 cases with the age range of 27 to 77, all 5 prolymphocytic variant and the remainder was the so- patients had splenomegaly, 3 of them had pancytope- called “HCL-Japanese variant” as they frequently pre- nia and 2 of them had leukocytosis(11). In another Chi- sented with a high WBC count and had a distinct CD25- nese series from Hongkong, 18 cases were reported negative hairy morphology(15). In a small Taiwanese with the incidence of HCL of 0.035 per 100,000 popula- tion per year which was much lower than the US SEER’s data(12). The clinical features such as male dominance and clinical presentations, however, were similar to the Western reports.
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