| Hairy cell leukemia and variant in Taiwan: report of a variant case and literature review. | |
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MedLine Citation:
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PMID: 21326814 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Hairy cell leukemia (HCL) is characterized by leukemic cells with abundant "hairy" cytoplasm, strong cytoplasmic positivity for tartrate-resistant acid phosphatase (TRAP), characteristic immunophenotype and sensitivity to treatment with purine nucleoside analogs. HCL-variant (HCL-v) encompasses chronic B-cell leukemias resembling classical HCL but exhibiting variant cytomorphology, variant immunophenotype and resistance to conventional HCL therapy. We present the case of a 67-year-old Taiwanese male with HCL-v who had leukocytosis and splenomegaly. His hairy leukemic cells were weakly positive for TRAP and expressed CDllc and CD103 but not CD25. He received oral chemotherapy with chlorambucil and in complete hematological remission in 9 months but relapsed 2 months later. Literature review revealed 9 cases of HCL and 3 cases of HCL-v including current case from Taiwan. All patients were adults with splenomegaly. The HCL patients had a significantly higher frequency of leukopenia (p = 0.024) and monocytopenia (p = 0.008) and a lower frequency of leukocytosis (p = 0.018) than HCL-v patients. All 8 HCL patients responded favorably to 2-chlorodeoxyadenosine with or without splenectomy. The 3 HCL-v patients had leukocytosis and received chemotherapy with variable outcome. HCL and HCL-v are rare in Taiwan and their pathological and immunophenotypical features were not fully characterized. A multimodality approach incorporating hematological findings, cytomorphology, histopathology, cytochemistry, complete immunophenotyping and clinical features is needed to identify and characterize such cases in Taiwan. |
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Authors:
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Yen-Chuan Hsieh; Shen-Tsung Chang; Shih-Sung Chuang; Chin-Li Lu; Chao-Jung Tsao; Ching-Nan Lin; Chin-Yang Li |
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Publication Detail:
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Type: Case Reports; Journal Article; Research Support, Non-U.S. Gov't; Review Date: 2011-01-05 |
Journal Detail:
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Title: International journal of clinical and experimental pathology Volume: 4 ISSN: 1936-2625 ISO Abbreviation: Int J Clin Exp Pathol Publication Date: 2011 |
Date Detail:
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Created Date: 2011-02-17 Completed Date: 2011-06-14 Revised Date: 2013-05-29 |
Medline Journal Info:
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Nlm Unique ID: 101480565 Medline TA: Int J Clin Exp Pathol Country: United States |
Other Details:
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Languages: eng Pagination: 183-9 Citation Subset: IM |
Affiliation:
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Department of Pathology, Chi-Mei Medical Center, Yung-Kang, Tainan, Taiwan. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Acid Phosphatase
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metabolism Aged Antigens, CD / metabolism Antineoplastic Agents, Alkylating / therapeutic use Bone Marrow / pathology Chlorambucil / therapeutic use Humans Isoenzymes / metabolism Leukemia, Hairy Cell / drug therapy, metabolism, pathology* Leukocytosis / metabolism, pathology Male Recurrence Remission Induction Splenomegaly / metabolism, pathology Tumor Markers, Biological / metabolism |
| Chemical | |
Reg. No./Substance:
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0/Antigens, CD; 0/Antineoplastic Agents, Alkylating; 0/Isoenzymes; 0/Tumor Markers, Biological; 305-03-3/Chlorambucil; EC 3.1.3.-/tartrate-resistant acid phosphatase; EC 3.1.3.2/Acid Phosphatase |
| Comments/Corrections | |
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