Cases reported "Leukemia, Hairy Cell"

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1/36. Chronic urticaria as a presenting sign of hairy cell leukemia.

    Chronic urticaria is a common clinical disorder that is idiopathic in over 75% of cases. Less commonly, urticaria may be the presenting manifestation of an allergic or infectious disease, endocrinopathy, inherited syndrome, or autoimmune disorder. Rarely, urticaria may be a sign of underlying malignancy, including leukemia. C.C. is a 48-year-old white female who was referred for evaluation of recurrent urticaria for 3 years. The pruritic, erythematous wheals were pinpoint, and appeared to be precipitated by heat, stress, and effort. Prick tests were negative except to D. pteronyssinus. CBCs over the past 5 years revealed WBCs of 2,300-5,000 cells/mm3. skin biopsy revealed interstitial edema with infiltration of eosinophils and mast cells consistent with urticaria. The impression was probable cholinergic urticaria, for which hydroxyzine was prescribed with fair symptomatic control. One year later, she presented with bright red blood per rectum. Repeat physical examination revealed lymphadenopathy and splenomegaly. Subsequent laboratory studies showed pancytopenia. endoscopy was normal except for small, nonbleeding hemorrhoids. Bone marrow biopsy revealed histologic evidence of hair, cell leukemia that was treated with 2-chlorodeoxyadenosine. Upon initiation of chemotherapy her pruritus and urticaria subsided. Recent CBC revealed Hgb 9.2 g/dL, platelets 290,000 cells/mm3, and WBC 4,100 cells/mm3. Peripheral blood smear showed no hairy cells.
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2/36. An otherwise typical case of non-Japanese hairy cell leukemia with CD10 and CDw75 expression: response to cladaribine phosphate therapy.

    Hairy cell leukemia (HCL) in Western patients typically expresses CD19, CD20, CD11c, CD25, HLA-DR, and IgG/lambda and lacks expression of CD5 and CD10. The immunophenotype is in contrast to Japanese HCL which typically expresses CD5 and CD10. Western and Japanese HCL also differ in their clinical presentation and response to treatment with alpha-interferon. We report a case of non-Japanese HCL which presented typically with pancytopenia; however, the immunophenotype was atypical with expression of CD10 and CDw75. CDw75 expression has not previously been described in either Japanese or non-Japanese HCL. The patient achieved a marked partial pathologic response and complete clinical response to treatment with cladaribine phosphate.
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3/36. Chimeric monoclonal anti-CD20 antibody (rituximab)--an effective treatment for a patient with relapsing hairy cell leukaemia.

    A case story is presented, describing a 46 y old man, with a relapsing hairy cell leukaemia. After treatment with monoclonal anti CD-20 antibodies (rituximab) 375mg/week, four times, a complete remission was obtained which has lasted >9 months. The rituximab treatment produced a better remission than earlier treatments with alpha-interferon and chlorodeoxyadenosine. In addition, in contrast to other treatments, no initial worsening of the pancytopenia was observed.
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4/36. Fatal cold anti-i autoimmune haemolytic anaemia complicating hairy cell leukaemia.

    Hairy cell leukaemia (HCL) is a rare lymphoproliferative disorder associated with pancytopenia, splenomegaly and the presence of typical hairy B lymphocytes in the bone marrow and/or peripheral blood. The most significant complication relates to opportunistic infections that arise as a consequence of neutropenia and monocytopenia. HCL is occasionally associated with systemic autoimmune disorders including polyarteritis nodosa and rheumatoid disease. Secondary autoimmune haemolytic anaemia (AIHA) appears to be rare. We report on two cases of HCL complicated by fatal cold anti-i AIHA. Fulminant haemolysis causing death is rare in cold AIHA and only a few individual cases have been reported, none having anti-i specificity.
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5/36. 2-chlorodeoxyadenosine (cladribine) in the treatment of hairy cell leukemia.

    Hairy cell leukemia is an uncommon chronic B cell lymphoproliferative disorder in taiwan and only sporadic cases have been noted. This disease is often found in middle-aged men with the typical manifestations of pancytopenia and splenomegaly. While tartrate-resistant acid phosphatase (TRAP) stain of the neoplastic cells in peripheral blood smear is positive in 90% cases, bone marrow examination is still necessary for diagnosis. For treatment, splenectomy and interferon have been the standard strategies. Recently, newly-developed purine analogues, 2-chlorodeoxyadenosine (cladribine) and 2'-deoxycorfomycin (pentostatin), were found to achieve long lasting complete remission. Here we reported a 48-year-old men who was found to have splenomegaly and bicytopenia incidentally at routine physical checkup. Although the TRAP staining of peripheral blood showed negative result, hairy cell leukemia was finally diagnosed by bone marrow biopsy. The following splenectomy showed a typical picture of hairy cell leukemia on pathology, but cytopenia recovered only partially after surgery. One course of chemotherapy with cladribine (0.1 mg/kg/day continuous intravenous infusion for 7 days) was given after recovery from major surgery. The white cell and platelet counts normalized after treatment and he carried a stable condition thereafter. Since the disease is rare and purine analogues are newly developed, there has been no report on using 2-clorodeoxy-adenosine against hairy cell leukemia in our country. Here we suggest that newly developed purine analogues such as cladribine are the first choice of treatment in cases of hairy cell leukemia.
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6/36. Persistent remission after immunosuppressive therapy of hairy cell leukemia mimicking aplastic anemia: two case reports.

    Some patients with hairy cell leukemia (HCL) manifest pancytopenia and bone marrow hypoplasia without an apparent increase in atypical cells, so their disease resembles severe aplastic anemia at onset. We treated 2 HCL patients, who were initially diagnosed with aplastic anemia, with antithymocyte globulin (ATG) in combination with cyclosporine or antilymphocyte globulin (ALG). Both patients obtained partial remission in response to the immunosuppressive therapy and did not need transfusion treatment for more than 3 years. Sustained improvement of hematopoiesis in such B-cell malignancies after ATG/ ALG therapy suggests that the mechanisms underlying successful immunosuppressive therapy for aplastic anemia may involve B-cell suppression, inhibiting hematopoietic stem cells.
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7/36. Epstein-Barr virus positive large B-cell lymphoma arising in a patient previously treated with cladribine for hairy cell leukemia.

    We describe the case of a patient treated with 2-chloro-2'-deoxyadenosine, CdA or cladribine for hairy cell leukemia who subsequently developed an Epstein Barr virus (EBV)-positive polymorphous large B-cell lymphoma (p-LBCL). The time interval between cladribine therapy and development of p-BCL was 11 months and morphologically resembled an EBV-positive post transplant lymphoproliferative disorder (PTLD). Molecular genetic studies for EBV-clonality by Southern blot hybridization showed a clonal population of infected cells, implying that this was an EBV induced lesion. The chronology of events suggest that cladribine, a purine analog which has been previously described to induce long-lasting immunodeficiency, can, in some cases, weaken the host defense mechanism to a level at which an innocuous EBV infection may transform the normal lymphoid cells into an aggressive neoplasm. Unlike most methotrexate-related lymphoproliferative disorders (LPDs), which undergo spontaneous remission after discontinuation of therapy, LPDs secondary to purine analogs often fails to resolve after discontinuation of therapy and requires additional therapy. Our patient was treated with rituximab following the diagnosis of p-LBCL, with the goal of improving the pancytopenia to permit chemotherapy. However, the patient failed to show any dramatic improvements in counts, developed systemic symptoms and progressive ascites. He expired 3 weeks after a second dose of rituximab. cladribine is a potent immunosuppressive agent and should be included with the list of immunosuppressive agents that may be associated with EBV-related B-cell lymphoproliferative disorders.
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8/36. Acute myeloid leukemia following treatment with cladribine for hairy cell leukemia: a case report and review of the literature.

    The overall survival of patients with hairy cell leukemia (HCL) has significantly increased in recent years because of the development of effective treatments such as interferon alpha and purine analogs. Several reports have described an increased risk of secondary cancers, particularly solid tumors, in patients with HCL. We describe a case of a patient with HCL, who had prolonged pancytopenia after a single course of cladribine. Fifteen months after the diagnosis of HCL the patient developed acute myeloid leukemia (AML) and died shortly afterwards. review of the literature shows few reports of acute leukemia in HCL patients. All of the 11 reported cases of leukemia in patients with HCL have been in patients who have been treated with either interferon alpha or purine analogs, and developed several years (mean 4.3 years; range 1.6-6.4 years) after the diagnosis of HCL. Our case is unusual in that the patient developed AML shortly (1.2 years) after the diagnosis and treatment of HCL. Further studies are needed to clarify whether leukemias seen in patients following the treatment of their HCL are incidental findings or related to HCL and its treatments.
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9/36. sezary syndrome in a patient with hairy cell leukemia in remission.

    A 65-year-old man was evaluated for pancytopenia in March 1979, and found to have hairy cell leukemia (HCL). Treatment with splenectomy and subsequently interferon produced temporary remissions. In July 1985, the patient began intravenous deoxycoformycin (DCF) therapy, and after 1 year complete peripheral blood and bone marrow remission was achieved. Fourteen months after cessation of therapy, the patient developed a skin rash and was found to have cutaneous T-cell lymphoma and sezary syndrome. Morphologic study of the hairy cells (HC) in the peripheral blood at presentation and the Sezary cells was distinct by light and electron microscopic study. immunophenotyping of peripheral blood mononuclear cells showed clearly that the HC were of B-cell origin (CD20 , sIg ), whereas the lymphoid population at second presentation was T-cell (CD3 , CD4 , HLA-DR-). Clonal rearrangement of T-cell antigen receptor beta-chain gene was detected by Southern analysis of the Sezary cell population, whereas immunoglobulin heavy and light chain genes remained in germ line configuration. This is the first case of sezary syndrome developing in a patient previously treated for HCL where studies have confirmed distinct B-cell and T-cell origin of the two neoplasms. The authors suggest that treatment and disease-related immunosuppression are possible etiologic factors in the development of this second lymphoid neoplasm.
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10/36. Hairy cell leukemia: an elusive but treatable disease.

    Hairy cell leukemia (HCL) is a unique chronic lymphoproliferative disorder that can mimic or coexist with other clonal hematologic disorders and has been associated with autoimmune disorders. It should be entertained as an alternative diagnosis in patients with cytopenias being assigned the diagnosis of aplastic anemia, hypoplastic myelodysplastic syndrome, atypical chronic lymphocytic leukemia, B-prolymphocytic leukemia, or idiopathic myelofibrosis. Causative etiology or molecular defects remain unclear, although nonspecific chromosomal and molecular changes have been described. The typical presentation is that of a middle-aged man with an incidental finding of pancytopenia, splenomegaly, and inaspirable bone marrow. Treatment with a purine analogue, cladribine or pentostatin, results in extremely high, durable, overall, and complete response rates, although resistance and relapses do occur. A variant subtype exists and is frequently associated with a poor response. Because of its simplified dosing schedule, cladribine is commonly used as the initial therapy. Treatment of relapsed HCL is dictated by the duration of the preceding remission. Relapsed disease after a prolonged remission can often be successfully retreated with the same initial agent. Resistance in typical HCL is treated with the alternate purine analogue. New agents, such as rituximab and BL22, are actively being evaluated and show promising results in both HCL subtypes. This article uses two patients diagnosed with aplastic anemia and recently seen in consultation at our institution as a springboard to discuss the biology, pathogenesis, clinical presentation, diagnostic evaluation, and treatment options of HCL.
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