Document Detail


Hairy cell leukemia and variant in Taiwan: report of a variant case and literature review.
MedLine Citation:
PMID:  21326814     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
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:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't; Review     Date:  2011-01-05
Journal Detail:
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:
Created Date:  2011-02-17     Completed Date:  2011-06-14     Revised Date:  2013-06-30    
Medline Journal Info:
Nlm Unique ID:  101480565     Medline TA:  Int J Clin Exp Pathol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  183-9     Citation Subset:  IM    
Affiliation:
Department of Pathology, Chi-Mei Medical Center, Yung-Kang, Tainan, Taiwan.
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MeSH Terms
Descriptor/Qualifier:
Acid Phosphatase / 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:
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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