Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027947 (neutropenia)
 17,527 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Subpopulational composition of peripheral blood and bone marrow lymphocytes was studied in 17 children with different forms of immunodeficiency, combined with hematological shifts (agammaglobulinemia--6, mucocutaneous candidiasis--2, selective IgG-deficiency--2, hyper-IgM syndrome--3, cephalo-oculocutaneous telangiectasia (COCT)--2, general, variable immunodeficiency--2 patients; neutropenia was observed in all the patients, lymphopenia--in 13, anemia-in 6 patients. Surface markers were assayed by flow cytofluorometry with monoclonal antibodies OKT3, OKT4, OKT8, OKB7, produced by "Ortho diagnostics". Changes characteristic of certain forms of primary immunodeficiency have been revealed in the subpopulational composition of peripheral blood and bone marrow lymphocytes: decreased helper potential in patients with general variable immunodeficiency, T-lymphocyte deficiency in patients with COCT increased number of phenotype T3 cells and decreased amount of B-cell in agammaglobulinemia patients. Significant heterogeneity has been noted in the parameters of hemogram, myelogram and in the subpopulational composition of peripheral blood and bone marrow lymphocytes in each nosologic form, the group of patients with hyper-IgM syndrome has proved to be most heterogeneic. It has been suggested that the changes in the subpopulational composition of bone marrow lymphocytes may be responsible for primary immunodeficiency and disorders in hemopoiesis.
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PMID:[Phenotyping of blood and bone marrow lymphocytes in children with primary immunologic deficiency]. 237 47

Ataxia-telangiectasia (AT) is an autosomal recessive syndrome of combined immunodeficiency. Hallmarks of the disease comprise progressive cerebellar ataxia, oculocutaneous telangiectasia, cancer susceptibility and variable humoral and cellular immunodeficiency. We describe a patient with AT presenting with autoimmune haemolytic anaemia, neutropenia, hepatosplenomegaly, lymphadenopathy and hyper-IgM at the age of 6 months. At the age of 26 months she developed persistent fever, progressive lymphadenopathy and pulmonary nodular infiltrates, which were responsive to steroid therapy.
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PMID:Unusual and severe disease course in a child with ataxia-telangiectasia. 1291 15

Clonal disorders of large granular lymphocytes (LGL) represent a rare spectrum of biologically distinct lymphoproliferative diseases originating either from mature T cells or natural killer cells. Both subtypes can manifest as indolent or aggressive disorders. We report a 77-year-old woman with rheumatoid arthritis, splenomegaly, and neutropenia who developed a painful leg ulcer refractory to treatment and thigh telangiectatic lesions. Because of the association of rheumatoid arthritis, splenomegaly, and nonspecific neutropenia, the diagnosis of Felty syndrome was initially made. Further investigation allowed the diagnosis of a CD56(-) natural killer-cell LGL leukemia and documented skin infiltration by natural killer cells. Cutaneous manifestations of LGL leukemia have been rarely reported. This report of pseudo-Felty syndrome with CD56(-) LGL leukemia, presenting with a leg ulcer and telangiectasia, enhances the role of dermatology in the diagnosis of hematologic neoplasia.
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PMID:Leg ulcer and thigh telangiectasia associated with natural killer cell CD56(-) large granular lymphocyte leukemia in a patient with pseudo-Felty syndrome. 1996 15