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

We describe the clinical and laboratory features of 17 adult patients with a variant form of hairy cell leukemia (HCL-V) studied over the last 7 years. The main findings were: splenomegaly, moderate anemia, thrombocytopenia, and a raised white blood cell count (median 116 x 10(9)/L; range 15 to 482). The circulating lymphoid cells had abundant villous cytoplasm and a round, occasionally bilobed nucleus, with a prominent nucleolus. Monocytopenia, a feature of typical HCL, was not seen; neither was tartrate-resistant acid phosphatase demonstrated in eight cases tested. HCL-V cells had a mature B-cell phenotype: CD19+, CD20+, CD22+, FMC7+, CD11c+, CD10-, CD5-, with light chain isotope restriction in 15 cases. In contrast to typical hairy cells, HCL-V cells were negative with the monoclonal antibodies anti-HC2 and anti-TAC (CD25). Immunoglobulin (Ig) was not detected in two cases and IgG was expressed in the cell membrane of 73% of cases. Bone marrow histology was different from HCL, showing interstitial infiltration by cells clumped together and a moderate amount of reticulin, but the spleen showed the typical red pulp expansion of HCL. HCL-V patients did not respond to splenectomy (5 of 7) or alpha-interferon (7 of 7); 2 of 3 patients had a partial response to 2'deoxycoformycin. The clinical course was benign with 15 patients alive with a median survival greater than 4 years. We confirm that HCL-V is a distinct clinico-pathologic entity with intermediate features between HCL and B-prolymphocytic leukemia.
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PMID:A variant form of hairy cell leukemia resistant to alpha-interferon: clinical and phenotypic characteristics of 17 patients. 236 67

We have analysed the immunological profile of cells from 194 patients with a B-cell disorder associated with circulating hairy or villous lymphocytes. These included: 69 typical HCL, 25 HCL-variant and 100 splenic lymphoma with villous lymphocytes (SLVL). All cases were HLA-DR+, CD19+, CD2- and most expressed Smlg with light chain restriction. The kappa/lambda ratios were: HCL, 1.2; SLVL, 1.5; and HCL-variant, 0.55. The majority were FMC7+ (89-96% of cases) and membrane CD22+ (73-98% of cases) and often negative with CD5 and CD23, markers characteristic of chronic lymphocytic leukemia. CD24 was variably expressed ranging from 21% of cases in HCL-variant to 60% in HCL and 89% in SLVL; CD10 and CD38 were positive in one third of SLVL but usually negative in HCL and HCL-variant. Of the four markers considered typical of HCL, CD11c, CD25, HC2 and B-ly-7, CD25 and HC2 were consistently negative in HCL-variant and a minority of SLVL cases expressed CD25 or HC2+ or B-ly-7+; CD11c was positive in all three disorders (47 to 97% of cases). Based on the most common phenotype of typical HCL: CD11c+, CD25+, HC2+ and B-ly-7+, we propose a scoring system which considers the reactivity with each of these four markers and gives 1 point if positive and 0 points if negative. Scores range from 4 (typical of HCL) to 0 (atypical of HCL). 98% of HCL had high scores (3 or 4) whereas 88% of HCL-variant and 77% of SLVL scored 1 or 2 and no single case scored 3 or 4.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:The immunophenotype of hairy cell leukemia (HCL). Proposal for a scoring system to distinguish HCL from B-cell disorders with hairy or villous lymphocytes. 782 54

Splenic lymphoma with villous lymphocytes (SLVL) is a low-grade disorder that regularly presents with peripheral blood involvement. We describe the immunophenotype of the circulating cells from 100 SLVL patients whose disease has been characterized on clinical, morphologic, and histologic grounds. Cells from all cases expressed B-cell antigens (CD19 and CD37) and/or HLA-Dr and showed light chain restriction (kappa/lambda: 1.5/1) with moderate to strong intensity of membrane Ig staining. Cells from most cases (> 80%) were CD24+, FMC7+, and expressed strongly membrane CD22. The monoclonal antibodies CD10, CD23, and CD38 were positive in one-third of the cases; CD11c in 47%; and CD25 in 25% of cases. A minority of cases (< 20%) were positive with HC2, B-ly-7, and CD5. However, none of the 19 CD5+ cases had the phenotype characteristic of chronic lymphocytic leukemia (CD5+, CD23+, FMC7-, weak surface Ig and membrane CD22). None of the 17 CD25+ cases had the immunophenotype typical of hairy cell leukemia (CD25+, CD11c+, HC2+, B-ly-7+). HC2 and B-ly-7 were the most useful reagents to distinguish SLVL from hairy cell leukemia. Our findings demonstrate that SLVL has a distinct immunologic profile and that monoclonal antibodies are important for the differential diagnosis between this disease and other B-lymphoproliferative disorders with which SLVL can be confused.
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PMID:The immunophenotype of splenic lymphoma with villous lymphocytes and its relevance to the differential diagnosis with other B-cell disorders. 812 45