Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023418 (leukemia)
 93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The prolymphocytic variant of hairy-cell leukemia (HCL-V) is relatively rare and differs from typical hairy-cell leukemia (HCL) both clinically and morphologically. Recognition of HCL-V is important due to therapeutic impact. We report on a case of HCL-V, atypical in its degree of marrow fibrosis, IgM/lambda monoclonality, expression of CD24, and the ultrastructural presence of ribosomal lamellar complexes. The patient was treated with splenectomy followed by pentostatin, and he achieved a partial response.
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PMID:Atypical prolymphocytic variant of hairy-cell leukemia: case report and review of the literature. 889 38

Hairy cell leukemia variant is an uncommon chronic B-cell lymphoproliferative disorder characterized clinically by splenomegaly and marked leukocytosis. Cytologically, the leukemic cells are distinguishable from those of classical hairy cell leukemia by the presence of single, central, and vesicular nucleoli. Cytogenetic information for this uncommon leukemia is scanty, although structural abnormalities involving 7q34 have been reported in few cases. We report a patient with hairy cell leukemia variant who has t(2;8)(p12;q34) but without [corrected] c-MYC oncogene rearrangement.
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PMID:Hairy cell leukemia variant with t(2;8)(p12;q24) abnormality. 933 72

Hairy cell leukaemia variant is a very rare chronic lymphoproliferative disorder and is closely related to hairy cell leukemia. We hereby describe a case of hairy cell leukaemia variant for the first time in Saudi Arabia. An elderly Saudi man presented with pallor, massive splenomegaly, and moderate hepatomegaly. Hemoglobin was 7.7 g/dl, Platelets were 134 x109/l and white blood count was 140x10 9/l with 97% being abnormal lymphoid cells with cytoplasmic projections. The morphology, cytochemistry, and immunophenotype of the lymphoid cells were classical of hairy cell leukaemia variant. The bone marrow was easily aspirated and findings were consistent with hairy cell leukaemia variant.
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PMID:Hairy cell leukemia-variant. 1125 16

The aim of the article is to present a rare case of Hairy cell leukaemia variant (HCl-V) which is a distinct clinico-pathological entity with intermediate features between classical HCl (HCl-C) and B-cell prolymphocytic leukaemia. It is an uncommon disorder accounting for approximately 0.4% of chronic lymphoid malignancies and 10% of all HCl cases. A 58 year old woman presented with pain abdomen and loss of weight. On examination she had massive splenomegaly. Peripheral smear was reported as chronic lymphoproliferative disorder (? Hairy cell leukemia or splenic lymphoma with villous lymphocytes). On Bone marrow examination, differential diagnosis was given as splenic lymphoma with villous lymphocytes (SLVL) and prolymphocytic variant of Hairy cell leukemia. On flow cytometric analysis, these cells were positive for CD11c, CD19, CD20, and CD22. Based on the clinical, peripheral smear, bone marrow and flow cytometry findings, a diagnosis of hairy cell leukaemia variant was confirmed. The differential diagnosis should always include SLVL, HCL-C and Japanese variant HCL because they have different clinical and biological features, particularly regarding their response to purine analogue-based treatment or splenectomy.
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PMID:A hairy cell leukaemia variant - a rare case report. 2354 22