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1. Quach H, Januszewicz H, Westerman D: Complete remission of hairy cell leukemia variant (HCL-v) complicated by red cell aplasia post treatment with rituximab. Haematologica; 2005 Nov;90 Suppl:ECR26
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Complete remission of hairy cell leukemia variant (HCL-v) complicated by red cell aplasia post treatment with rituximab.
  • Hairy cell leukemia variant (HCL-v) is a rare form of a chronic B-cell lymphoproliferative disorder.
  • Unlike typical hairy cell leukemia (HCL) where the complete response (CR) rate to 2-chlorodeoxyadenosine and 2'-deoxycoformycin can approach about 90%, in HCL-v CR is rare and partial response (PR) occurs in approximately 50% with these agents.
  • Rituximab treatment in relapsed or refractory HCL results in a CR of 13% to 53%, but its use in HCL-v has not been reported in the literature to our knowledge.
  • We describe a patient with HCL-v, whose course was previously complicated by pure red cell aplasia who achieved CR after treatment with rituximab.
  • We also briefly review outcomes of treatments used in HCL-v reported in the current literature.
  • [MeSH-major] Antibodies, Monoclonal / therapeutic use. Antineoplastic Agents / therapeutic use. Leukemia, Hairy Cell / drug therapy. Neoplasms, Second Primary / drug therapy. Red-Cell Aplasia, Pure / etiology
  • [MeSH-minor] 2-Chloroadenosine / administration & dosage. 2-Chloroadenosine / analogs & derivatives. Aged. Antibodies, Monoclonal, Murine-Derived. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Blood Transfusion. Combined Modality Therapy. Cyclophosphamide / administration & dosage. Deoxyadenosines / administration & dosage. Disease Progression. Drug Resistance, Neoplasm. Fatal Outcome. Humans. Interferon-alpha / administration & dosage. Male. Prednisolone / administration & dosage. Recurrence. Remission Induction. Rituximab. Splenectomy. Urinary Bladder Neoplasms / radiotherapy

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  • (PMID = 16266917.001).
  • [ISSN] 1592-8721
  • [Journal-full-title] Haematologica
  • [ISO-abbreviation] Haematologica
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Murine-Derived; 0 / Antineoplastic Agents; 0 / Deoxyadenosines; 0 / Interferon-alpha; 115044-75-2 / 2-chloro-3'-deoxyadenosine; 146-77-0 / 2-Chloroadenosine; 4F4X42SYQ6 / Rituximab; 8N3DW7272P / Cyclophosphamide; 9PHQ9Y1OLM / Prednisolone
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2. Makita M, Nakamura K, Kono A: [Successful rituximab treatment in a patient with refractory hairy cell leukemia-Japanese variant and suffering from acute respiratory distress]. Rinsho Ketsueki; 2005 Nov;46(11):1196-201
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  • [Title] [Successful rituximab treatment in a patient with refractory hairy cell leukemia-Japanese variant and suffering from acute respiratory distress].
  • A 69-year-old man was referred to our hospital because of thrombocytopenia and was diagnosed as having hairy cell leukemia-Japanese variant (HCL-Jv) in December 2000.
  • In addition, the number of hairy cells (HCs) in his peripheral blood (PB) began gradually to increase.
  • He received treatment with interferon-alpha and cladribine, but he failed to respond completely to these treatments.
  • His white blood cell count was 123.1 X 10(9)/L with 91% HCs.
  • Radiography and computed tomography of his chest revealed ground-glass opacity in both lungs.
  • After 8 courses of rituximab therapy, the HCs disappeared in his PB and BM, and his pulmonary infiltrates subsided.
  • These results suggest that rituximab may be a very effective treatment for refractory HCL-Jv.
  • [MeSH-major] Antibodies, Monoclonal / administration & dosage. Antineoplastic Agents / administration & dosage. Leukemia, Hairy Cell / complications. Leukemia, Hairy Cell / drug therapy. Respiratory Distress Syndrome, Adult / etiology
  • [MeSH-minor] Aged. Antibodies, Monoclonal, Murine-Derived. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Humans. Leukemic Infiltration / complications. Leukemic Infiltration / drug therapy. Lung / pathology. Male. Melphalan / administration & dosage. Rituximab

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  • (PMID = 16440803.001).
  • [ISSN] 0485-1439
  • [Journal-full-title] [Rinshō ketsueki] The Japanese journal of clinical hematology
  • [ISO-abbreviation] Rinsho Ketsueki
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Murine-Derived; 0 / Antineoplastic Agents; 4F4X42SYQ6 / Rituximab; Q41OR9510P / Melphalan
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3. Imamura T, Ohtsuka E, Ogata M, Oka F, Kashima K, Kikuchi H, Nasu M: Successful induction of long-term remission using rituximab in a patient with refractory hairy cell leukemia-Japanese variant. Int J Hematol; 2004 Dec;80(5):432-4
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  • [Title] Successful induction of long-term remission using rituximab in a patient with refractory hairy cell leukemia-Japanese variant.
  • We report the case of a 76-year-old man with hairy cell leukemia (HCL)-Japanese variant who underwent rituximab therapy.
  • HCL proved refractory to treatment with pentostatin and cladribine, and the number of hairy cells in the peripheral blood continued to increase after splenectomy.
  • The patient was treated with rituximab (375 mg/m2 intravenously weekly for 4 cycles), and hairy cells disappeared from the peripheral blood on the day after the first administration.
  • Complete remission continued for 18 months after treatment.
  • Although they produce high response rates in typical HCL, nucleoside analogs are associated with an inferior clinical response in HCL-Japanese variant, and repetitive administration of these agents increases the risk of serious infections.
  • This encouraging result suggests that rituximab therapy should be considered as salvage therapy for refractory HCL-Japanese variant.
  • [MeSH-major] Antibiotics, Antineoplastic / administration & dosage. Antibodies, Monoclonal / administration & dosage. Antineoplastic Agents / administration & dosage. Cladribine / administration & dosage. Leukemia, Hairy Cell / drug therapy. Pentostatin / administration & dosage
  • [MeSH-minor] Aged. Antibodies, Monoclonal, Murine-Derived. Drug Therapy, Combination. Humans. Japan. Male. Remission Induction. Rituximab

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  • (PMID = 15646655.001).
  • [ISSN] 0925-5710
  • [Journal-full-title] International journal of hematology
  • [ISO-abbreviation] Int. J. Hematol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Antibiotics, Antineoplastic; 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Murine-Derived; 0 / Antineoplastic Agents; 395575MZO7 / Pentostatin; 47M74X9YT5 / Cladribine; 4F4X42SYQ6 / Rituximab
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4. Kristinsson SY, Vidarsson B, Agnarsson BA, Haraldsdottir V, Olafsson O, Johannesson GM, Eyjolfsson GI, Bjornsdottir J, Onundarson PT, Reykdal S: Epidemiology of hairy cell leukemia in Iceland. Hematol J; 2002;3(3):145-7
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  • [Title] Epidemiology of hairy cell leukemia in Iceland.
  • INTRODUCTION: Hairy cell leukemia (HCL) is a rare B-cell lymphoproliferative disorder.
  • This is the first study in which all cases diagnosed nationwide over a long period of time in a well defined population are analysed.
  • We report the epidemiology of all HCL patients in Iceland, their clinical characteristics, treatment and follow-up.
  • PATIENTS AND METHODS: : All patients diagnosed with HCL in Iceland over a 20 year period, were included in this study.
  • RESULTS: Sixteen patients, 13 males and three females were diagnosed with HCL in Iceland from 1981-2000, giving a mean incidence of 4.7/million/year (95% CI: 2.7-7.6) in the population 20 years and older.
  • Eleven patients were treated with a purine analogue, 10 of whom achieved CR.
  • One patient had a variant of HCL and did not respond to any therapy and one patient died of sepsis before any chemotherapy could be given.
  • Six patients with HCL have died, one from complications of HCL.
  • Three patients developed a second malignancy (19%).
  • CONCLUSIONS: The mean incidence of HCL in Iceland is 4.7/million/year.

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  • (PMID = 12111650.001).
  • [ISSN] 1466-4860
  • [Journal-full-title] The hematology journal : the official journal of the European Haematology Association
  • [ISO-abbreviation] Hematol. J.
  • [Language] ENG
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
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5. Telek B, Batár P, Udvardy M: [Successful alemtuzumab treatment of a patient with atypical hairy cell leukaemia variant]. Orv Hetil; 2007 Sep 23;148(38):1805-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Successful alemtuzumab treatment of a patient with atypical hairy cell leukaemia variant].
  • Although hairy cell leukaemia and hairy cell leukaemia variant are characterized by much alike clinical features, these two diseases are disparate in nature and treatment.
  • While hairy cell leukaemia responds quite well to 2-chlorodeoxyadenosine (cladribine) treatment, hairy cell leukaemia variant has much worse response rate and has no effective treatment option yet.
  • With other treatment modalities, including monoclonal antibody treatment, we have less experience.
  • Alemtuzumab (Campath-1H, MabCampath) treatment has been reported in a case with hairy cell leukaemia in relaps while there is no data with alemtuzumab therapy in the treatment of hairy cell leukaemia variant.
  • The authors present their case of a 58 year-old male who has been diagnosed with hairy cell leukaemia variant upon clinical findings and lymphocyte phenotyping.
  • Alemtuzumab treatment was started (3 x 30 mg/week s.c. for 12 weeks).
  • After 8 weeks of treatment haematologic remission was achieved; flow cytometry has revealed only 1.5% malignant cells.
  • Alemtuzumab treatment can be favourable in those cases of hairy cell leukaemia and hairy cell leukaemia variant which is dominated mainly by bone marrow infiltration and present no lymphadenomegaly or splenomegaly.
  • In our case the p53 mutation had no influence on the outcome of alemtuzumab treatment.
  • [MeSH-major] Antibodies, Monoclonal / therapeutic use. Antibodies, Neoplasm / therapeutic use. Antineoplastic Agents / therapeutic use. Leukemia, Hairy Cell / drug therapy
  • [MeSH-minor] Antibodies, Monoclonal, Humanized. Antigens, CD / blood. Biomarkers, Tumor / blood. Drug Administration Schedule. Humans. Male. Middle Aged. Treatment Outcome

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  • (PMID = 17872336.001).
  • [ISSN] 0030-6002
  • [Journal-full-title] Orvosi hetilap
  • [ISO-abbreviation] Orv Hetil
  • [Language] hun
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Hungary
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Humanized; 0 / Antibodies, Neoplasm; 0 / Antigens, CD; 0 / Antineoplastic Agents; 0 / Biomarkers, Tumor; 3A189DH42V / alemtuzumab
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6. Johnson SA: Use of fludarabine in the treatment of mantle cell lymphoma, Waldenström's macroglobulinemia and other uncommon B- and T-cell lymphoid malignancies. Hematol J; 2004;5 Suppl 1:S50-61
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  • [Title] Use of fludarabine in the treatment of mantle cell lymphoma, Waldenström's macroglobulinemia and other uncommon B- and T-cell lymphoid malignancies.
  • After initial efforts using fludarabine as a single agent in the treatment of acute leukemia, its activity at lower and safer doses was demonstrated in chronic lymphocytic leukemia (CLL) patients who were refractory or had relapsed from traditional chemotherapies, representing a highly effective therapy for this condition.
  • Fludarabine was also rapidly shown to be beneficial as first-line therapy in CLL.
  • There is now considerable evidence that fludarabine is an effective agent in non-Hodgkin's lymphoma and in combination therapy for acute myeloid leukemia.
  • The actions of fludarabine are not restricted to these settings and its potential role in the treatment of a range of uncommon T- and B-cell lymphoid malignancies is slowly emerging.
  • This review will focus on the characteristics and treatment options for two B-cell disorders, mantle cell lymphoma and Waldenström's macroglobulinemia, with emphasis on the clinical activity of fludarabine.
  • These include B-cell neoplasms such as the CLL variant prolymphocytic leukemia, hairy cell leukemia and mucosa-associated lymphoid tissue-derived lymphomas; the T-cell disorders cutaneous T-cell lymphoma, angioimmunoblastic lymphadenopathy and other rarer T-cell diseases; and aggressive variants of non-Hodgkin's lymphoma including Richter's syndrome.

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  • (PMID = 15079153.001).
  • [ISSN] 1466-4860
  • [Journal-full-title] The hematology journal : the official journal of the European Haematology Association
  • [ISO-abbreviation] Hematol. J.
  • [Language] ENG
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] FA2DM6879K / Vidarabine; P2K93U8740 / fludarabine
  • [Number-of-references] 108
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7. Matutes E, Wotherspoon A, Catovsky D: The variant form of hairy-cell leukaemia. Best Pract Res Clin Haematol; 2003 Mar;16(1):41-56
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The variant form of hairy-cell leukaemia.
  • Hairy-cell leukaemia-variant (HCL-variant) is a rare B-cell disorder which accounts for 10% of HCL cases.
  • The circulating cells have a morphology intermediate between prolymphocytes and hairy cells.
  • The immunophenotype shows a mature B-cell phenotype with expression of the B-cell antigens CD11c and CD103-but unlike typical HCL the cells are CD25- and HC2-negative.
  • The histology of bone marrow and spleen shows a pattern of infiltration similar to that in HCL.
  • Splenectomy results in long-lasting partial responses in over two-thirds of the patients and is a good palliative treatment.
  • Despite the lack of response to most therapies, the clinical course of HCL-variant is chronic.
  • Transformation to large cell is seen in 6% of patients.
  • The inferior survival in HCL-variant compared with typical HCL cases may reflect the chemotherapy resistance.
  • [MeSH-major] Leukemia, Hairy Cell / pathology
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. B-Lymphocytes / immunology. B-Lymphocytes / pathology. Cladribine / therapeutic use. Diagnosis, Differential. Humans. Immunophenotyping. Interferon-alpha / therapeutic use. Male. Survival Analysis

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  • (PMID = 12670464.001).
  • [ISSN] 1521-6926
  • [Journal-full-title] Best practice & research. Clinical haematology
  • [ISO-abbreviation] Best Pract Res Clin Haematol
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Interferon-alpha; 47M74X9YT5 / Cladribine
  • [Number-of-references] 40
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8. Palomera L, Domingo JM, Sola C, Azaceta G, Calvo MT, Gutierrez M: Cladribine (2-chlorodeoxyadenosine) therapy in hairy cell leukemia variant. A report of three cases. Haematologica; 2002 Jan;87(1):107-8
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  • [Title] Cladribine (2-chlorodeoxyadenosine) therapy in hairy cell leukemia variant. A report of three cases.
  • [MeSH-major] Antimetabolites, Antineoplastic / therapeutic use. Cladribine / therapeutic use. Leukemia, Hairy Cell / drug therapy
  • [MeSH-minor] Aged. Combined Modality Therapy. Drug Evaluation. Female. Follow-Up Studies. Humans. Interferon-alpha / therapeutic use. Male. Middle Aged. Remission Induction. Salvage Therapy. Splenectomy. Treatment Outcome

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  • (PMID = 11801472.001).
  • [ISSN] 0390-6078
  • [Journal-full-title] Haematologica
  • [ISO-abbreviation] Haematologica
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Antimetabolites, Antineoplastic; 0 / Interferon-alpha; 47M74X9YT5 / Cladribine
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9. Goldaniga M, Guffanti A, Gianelli U, Magni M, Lambertenghi Deliliers G, Baldini L: Clinical and molecular complete remission in a case of variant hairy cell leukemia treated with DHAP followed by high-dose chemotherapy plus rituximab. Haematologica; 2004 Nov;89(11):ECR41
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Clinical and molecular complete remission in a case of variant hairy cell leukemia treated with DHAP followed by high-dose chemotherapy plus rituximab.
  • [MeSH-major] Antibodies, Monoclonal / therapeutic use. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Leukemia, Hairy Cell / drug therapy
  • [MeSH-minor] Antibodies, Monoclonal, Murine-Derived. Antineoplastic Agents / administration & dosage. Antineoplastic Agents / therapeutic use. Cisplatin / administration & dosage. Cisplatin / therapeutic use. Combined Modality Therapy. Cytarabine / administration & dosage. Cytarabine / therapeutic use. Dexamethasone / administration & dosage. Dexamethasone / therapeutic use. Humans. Male. Middle Aged. Remission Induction. Rituximab. Stem Cell Transplantation

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  • (PMID = 15533846.001).
  • [ISSN] 1592-8721
  • [Journal-full-title] Haematologica
  • [ISO-abbreviation] Haematologica
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Murine-Derived; 0 / Antineoplastic Agents; 04079A1RDZ / Cytarabine; 4F4X42SYQ6 / Rituximab; 7S5I7G3JQL / Dexamethasone; Q20Q21Q62J / Cisplatin; DHAP protocol
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10. Sasaki M, Sugimoto K, Mori T, Karasawa K, Oshimi K: Effective treatment of a refractory hairy cell leukemia variant with splenic pre-irradiation and alemtuzumab. Acta Haematol; 2008;119(1):48-53
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Effective treatment of a refractory hairy cell leukemia variant with splenic pre-irradiation and alemtuzumab.
  • A 72-year-old Japanese man presented with 43.1 x 10(9)/l hairy cells and apparent splenomegaly.
  • The leukemia cells had unevenly distributed microvilli and round nuclei with dense chromatin and one or two clear nucleoli, lacked CD25 expression and were negative for tartrate-resistant acid phosphatase.
  • The case was diagnosed as hairy cell leukemia variant (HCLv) and proved refractory to various chemotherapies, including cladribine, pentostatin, interferon-alpha, CHOP and rituximab.
  • Pretreatment with 22.5 Gy to the spleen reduced the spleen size from 12 to 4 cm below the left costal margin, and the number of circulating leukemic cells decreased from 229.0 to 63.6 x 10(9)/l.
  • In vitro treatment with alemtuzumab confirmed the cytotoxic effect against the patient's leukemic cells in the presence of complement.
  • [MeSH-major] Antibodies, Monoclonal / therapeutic use. Antibodies, Neoplasm / therapeutic use. Antineoplastic Agents / therapeutic use. Leukemia, Hairy Cell / drug therapy. Leukemia, Hairy Cell / radiotherapy. Spleen / radiation effects
  • [MeSH-minor] Aged. Antibodies, Monoclonal, Humanized. Antigens, CD / immunology. Antigens, Neoplasm / immunology. Combined Modality Therapy. Fluorescent Antibody Technique. Glycoproteins / immunology. Humans. Immunophenotyping. Male

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  • [Copyright] 2008 S. Karger AG, Basel
  • (PMID = 18259114.001).
  • [ISSN] 1421-9662
  • [Journal-full-title] Acta haematologica
  • [ISO-abbreviation] Acta Haematol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Humanized; 0 / Antibodies, Neoplasm; 0 / Antigens, CD; 0 / Antigens, Neoplasm; 0 / Antineoplastic Agents; 0 / CD52 antigen; 0 / Glycoproteins; 3A189DH42V / alemtuzumab
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