Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0038002 (splenomegaly)
 9,873 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

The clinical and laboratory features of 23 patients with a characteristic form of splenic lymphoma with circulating "villous" lymphocytes (SLVL) are described and compared with those of other B cell disorders with preferential splenic involvement. SLVL affects predominantly men in their early 70's and is characterized by gross splenomegaly with little or no lymphadenopathy, presence of monoclonal gammopathy in two thirds of the cases, and infiltration of the peripheral blood by lymphocytes with a characteristic pattern of membrane irregularity. These lymphocytes are often confused with cells from hairy cell leukemia, from which they can be distinguished by a number of morphological features, by having a small cell volume, and by lack of expression of the HC2 and Tac antigens. The bone marrow is easily aspirated in the majority of cases and shows a relatively sparse infiltration. The spleen histology shows predominant white pulp involvement, although infiltration of the red pulp is seen in a small number of cases. The differential diagnosis of SLVL should also include hairy cell leukemia variant, prolymphocytic leukemia, and atypical forms of chronic lymphocytic leukemia.
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PMID:Splenic B cell lymphoma with "villous" lymphocytes in the peripheral blood: a disorder distinct from hairy cell leukemia. 349 40

Hairy-cell leukaemia-variant (HCL-variant) is a rare B-cell disorder which accounts for 10% of HCL cases. It affects elderly or middle-aged males. The main features are splenomegaly, lymphocytosis and cytopenias without monocytopenia. The circulating cells have a morphology intermediate between prolymphocytes and hairy cells. The immunophenotype shows a mature B-cell phenotype with expression of the B-cell antigens CD11c and CD103-but unlike typical HCL the cells are CD25- and HC2-negative. The histology of bone marrow and spleen shows a pattern of infiltration similar to that in HCL. There is no recurrent chromosomal abnormality but complex karyotypes and monoallelic p53 deletion by fluorescence in situ hybridization are common. Patients are resistant to alkylating agents and interferon-alpha (IFN-alpha) and only half achieve partial responses to pentostatin and/or cladribine. Splenectomy results in long-lasting partial responses in over two-thirds of the patients and is a good palliative treatment. Despite the lack of response to most therapies, the clinical course of HCL-variant is chronic. The median survival is 9 years and 42% of patients die of unrelated causes. Transformation to large cell is seen in 6% of patients. The inferior survival in HCL-variant compared with typical HCL cases may reflect the chemotherapy resistance.
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PMID:The variant form of hairy-cell leukaemia. 1267 Apr 64