Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:3.4.24.11 (CD10)
 9,792 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 72-year-old woman was referred to our hospital for evaluation of leukocytosis revealed by a medical examination. Her physical examination demonstrated no splenomegaly and no palpable lymph nodes. Her white cell count was 10,900/microl with atypical lymphocytosis (84.5%). Her hemoglobin concentration was 10.4 g/dl, and platelet count 151,000/microl. On peripheral blood smears, the atypical lymphocytes had a hairy cell-like appearance, and phase-contrast microscopic and transmission electron microscopic findings revealed the lymphocytes had many long surface microvilli. Flowcytometric analysis of peripheral blood lymphocytes identified expanded B-lymphocytes as having the IgG+, CD5- CD10- CD11c+ CD19+ CD20+ CD23- CD25- and CD103- cell surface phenotype. Serum electrophoresis disclosed polyclonal elevation of IgG and IgM (2620 mg/dl and 840 mg/dl, respectively). No light-chain restriction and a polyclonal VH gene rearrangement pattern indicated the polyclonal proliferation of B cells. The patient was a nonsmoker and had HLA-DR4, as in previous reports which have suggested an association between hairy B-cell lymphoproliferative disorder (HBLD) and HLA-DR4. No chromosome 3 abnormality was observed. These findings were consistent with the characteristics of HBLD, but differed in some respects from those of persistent polyclonal B-cell lymphocytosis (PPBL). Therefore, we diagnosed this patient as having HBLD.
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PMID:[Polyclonal B-cell lymphocytosis with hairy cell appearance: hairy B-cell lymphoproliferative disorder]. 1786 2

An 80-year-old female patient showed persistent lymphocytosis morphologically resembling the Japanese variant of hairy cell leukemia (HCL). However, flow cytometric analysis determined that these lymphocytes were of polyclonal B-cell origin, showing CD5-, CD10(-), CD11c(+), CD19(+), CD20(+), CD23(-), CD103(-), FMC7(-), HLA-DR(+) and surface membrane immunoglobulin (smIg) G(+) phenotype. The female patient also showed polyclonal hypergammaglobulinemia with bone marrow plasmacytosis. The patient was diagnosed as having hairy B-cell lymphoproliferative disorder (HBLD). Serum interleukin-6 (IL-6) level was elevated at the time of diagnosis in this patient, but IL-6 receptor (CD126) was not expressed on the hairy B-cells. Intracellular IL-6 was not detected in these cells either, suggesting that IL-6 did not play an important role in the B-lymphocytosis present in our patient with HBLD.
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PMID:The role of interleukin-6 in a patient with polyclonal hairy B-cell lymphoproliferative disorder: a case report. 1819 43

Flow cytometric immunophenotpying (FCI) is a vital tool in the diagnosis of hairy cell leukemia (HCL). HCL has a distinctive immunophenotype based upon staining with antibodies to CD5 (negative), CD10 (negative), CD23 (negative), CD20 (abnormally bright), CD22 (abnormally bright), CD11c (abnormally bright), CD25 (abnormally bright), CD103 (positive), and CD123 (positive). Due to this unique immunophenotype, FCI can differentiate malignant hairy cells from normal B-cells and other lymphoproliferative disorders, especially the morphologically similar hairy cell leukemia variant and splenic lymphoma with villous lymphocytes. FCI is extremely sensitive in the detection of minimal disease and is useful in monitoring response to therapy.
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PMID:Diagnosis of hairy cell leukemia by flow cytometry. 2150 92

A new cell line, designated "HDS", was established in a suspension culture derived from the peripheral blood of a patient with hairy cell leukemia (HCL) who developed clinical resistance to alpha-interferon (aIFN) therapy. The patient exhibited a clinical picture characteristic of HCL, including splenomegaly, cytopenias, and tartrate-resistant acid phosphatase (TRAP)-positive "hairy" cells in blood and marrow. Chromosomal studies revealed that the cultured cells possess the chromosomal abnormality +12. Cytochemical and immunologic studies show the HDS cell line had the phenotype of a B-lymphocyte. HDS cells expressed the HLA-DR and CD19 surface antigens, but were negative for early B (CD10) and T (CD2, CD3) cell markers. The cells are also negative for other T-cell, granulocytic and monocytic markers and for typical HCL markers such as CD11c and CD22. However, the expression of these antigens was induced by in-vitro treatment of the cells with the differentiation-inducing agent tetradecanoyl phorbol acetate (TPA). Ultrastructural analyses of the cultured cells revealed a display of surface microvilli mixed with ruffles in a classical hairy cell pattern. It is therefore highly likely that the HDS cells represent HCL cells in an atypical stage of differentiation.
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PMID:HDS: Establishment of a New Cell Line from a Hairy Cell Leukemia Patient with Resistance to Alpha-Interferon Therapy. 2746 53

T-cell large granular lymphocytic leukemia (T-LGLL) is an uncommon but probably underdiagnosed disease caused by clonal proliferation of large granular lymphocytes. Diagnosis is typically based on the high number of morphologically characteristic lymphoid cells and finding of an abnormal immunophenotype by flow cytometry. Because of its relatively indolent clinical behavior, observation is often an appropriate therapy. Here we present a case of a 53-year-old male admitted to the hospital because of abdominal pain. Blood examination revealed mild mycrocitic anemia and multiplied lactate dehydrogenase level. Abdominal ultrasound showed splenomegaly of 16 cm, with no lymphadenopathy. Fine needle aspiration of bone marrow revealed hypocellular marrow with 50% of atypical lymphoid cells. There were 81% of atypical medium sized granular lymphocytes with irregularly shaped nuclei in peripheral blood, so the cytologic diagnosis was lymphoproliferative process. Bone marrow biopsy showed nodular and interstitial proliferation of small, partially atypical T lymphocytic cells positive for CD2, CD3, CD5, CD8, granzyme and TIA, and negative for hairy cell markers, CD10, MUM 1, bcl 1, CD4 and CD56. The finding was consistent with T-LGLL. Due to splenomegaly, the patient was treated with cyclosporine and gradually reduced dose of corticosteroids, leading to regression of splenomegaly and normalization of lactate dehydrogenase level.
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PMID:T-Cell Large Granular Lymphocytic Leukemia - Case Reports. 3043 31

According publications' data, hairy cell leukosis encounters in humans aged from 26 to 70 years and in males four times more often than in females. The disease is manifested by impotence, splenomegaly, and presence in blood and bone marrow clone of lymphoid cells with particular morphology, cytochemic markers and immune phenotype (sIg+, CD19+, CD20+, CD5-, CD10-, with marked expression of CD25+, CD103+). The presented clinical case of illness with hairy cell leucosis is the first one detected in pediatric practice in adolescent of 16 years old.
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PMID:[The case of hairy cell leukosis in pediatrics]. 3058 41

A 75-year-old male evaluated for pancytopenia. Abnormal lymphocytes with hairy projections noted on peripheral blood. Bone marrow examination showed diffuse proliferation of CD20+ B-lymphocytes. Flowcytometry revealed monoclonal lambda-restricted B-cells expressing CD19, CD20, CD11c, CD103, CD25 and CD123, negative for CD5 and CD10. Additional staining showed positivity for cyclin-D1, Annexin-A1 and TRAP. FISH identified t(11;14). PCR was positive for BRAF V600E. Given the above findings, nonspecificity of t(11;14) and the presence of BRAF V600E; the diagnosis of HCL was favored. Patient achieved CR with infusional cladribine. Herein, we report the co-occurrence of CCND1/IGH and BRAF V600E in HCL, a rare scenario that could characterize a new subtype of HCL.
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PMID:Hairy cell leukemia with CCND1/IGH fusion gene and BRAF V600E mutation. 3225 95


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