Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A further case of persistent polyclonal B-cell lymphocytosis is reported. This recently identified distinct clinicopathologic entity is frequently associated with the presence of the HLA-DR7 antigen. It follows a benign course and has so far been reported only in women smokers. The disorder is characterized by mild chronic peripheral lymphocytosis, the presence of characteristic binucleate lymphocytes on peripheral blood smears, and a polyclonal increase in serum IgM. In some cases, lymphadenopathies and/or splenomegaly are observed. Surface marker studies of peripheral lymphocytes demonstrate the polyclonal B-cell nature of this entity.
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PMID:[Persistent B-cell polyclonal lymphocytosis: a benign lymphoproliferative syndrome]. 194 54

A syndrome associating a polyclonal increase of B-cells with binucleate lymphocytes in peripheral blood and a polyclonal increase of serum IgM, accompanied or not by splenomegaly and lymphadenopathy, has been reported in young to middle-aged women who are cigarette smokers. The majority of patients were HLA-DR7 positive and their clinical course remained stable over periods of years. We report four cases of this syndrome. In all patients, DNA analysis provided additional evidence that the lymphocytosis was polyclonal, while in one patient who met all other symptomatic criteria the total lymphocyte count was normal. This would suggest that unless careful attention is paid to the morphology of the lymphocytes, the syndrome may go undiagnosed.
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PMID:Cigarette smoking and chronic polyclonal B-cell lymphocytosis. 833 50

This is the first report of the unusual syndrome of persistent polyclonal B-cell lymphocytosis occurring in monozygotic twins. The syndrome is characterized by a lymphocytosis, with circulating atypical, binucleated lymphocytes, mild splenomegaly and raised serum IgM. It occurs predominantly in females, with serological evidence of previous EBV infection, and is associated with cigarette smoking and HLA-DR7 phenotype. The association with .DR7 suggests a genetic predisposition. Its occurrence in identical twins, documented here, provides stronger support for a hereditary/genetic basis for the syndrome.
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PMID:Persistent polyclonal B-cell lymphocytosis in identical twins. 902 12

We report the occurrence of the syndrome of persistent polyclonal B-cell lymphocytosis in a brother and a sister. Both showed the morphological and immunophenotypic features of this rare disorder. In addition both had mild splenomegaly, increase of serum IgM and serological evidence of previous EBV infection. Of interest, two additional brothers had no evidence of PPBL but were indistinguishable in terms of HLA haplotype (HLA-DR7), smoking habits or evidence of EBV infection. These observations provide additional support for a genetic basis of the syndrome but suggest that pathogenic factors other than those known so far may be required for its full expression.
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PMID:Familial persistent polyclonal B-cell lymphocytosis. 1134 68