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)

A case of agranulocytosis in a man with nonspecific seronegative polyarthritis treated with levamisole in the form of a proprietary veterinary anthelmintic is described. At the time of presentation he had a relative monocytosis. Recovery was uneventful and was heralded and paralleled by an increase in monocytes, serum vitamin B12 and total vitamin B12 binding capacity. Levamisole induced agranulocytosis and the significance of monocytosis and serum vitamin B12 binding proteins in neutropenia are briefly reviewed.
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PMID:Agranulocytosis in an arthritic patient treated with levamisole. A case report. 42 68

We report a patient with the syndrome of large granular lymphocytes in whom the initial clinical features were polyarthritis, hepatosplenomegaly and neutropenia. Relative lymphocytosis was also demonstrated at the expense of a subpopulation with morphology and surface markers characteristic of large granular lymphocytes (CD2+, CD8+, CD16+ and HNK-1+). After 6 months of asymptomatic course, without changes in clinical or laboratory data, the patient died from an acute abdomen with mesenteric ischemia of different likely causes as suggested by necropsy data (multivisceral diffuse infiltrate by large granular lymphocytes, systemic vasculitis and Clostridium sepsis). The association between this syndrome and systemic vasculitis is discussed.
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PMID:[Vasculitis associated with proliferation of large granular lymphocytes]. 225 May 16

Large granular lymphocyte (LGL) leukemia is a rare disease characterized by clonal expansion of LGL associated with chronic neutropenia, multiple auto-antibodies, and occasionally polyarthritis. We studied cell surface antigen expression and functional activity of leukemic LGL from ten such patients. Using two-color flow cytometric analysis, we found that leukemic LGL from all ten patients expressed the CD3 and HNK-1 markers, while cells from only four patients expressed IgG Fc receptors (FcR). The LGL leukemic cells had little or no NK activity (defined as MHC-nonrestricted cytotoxicity against K562 target cells); however, NK activity could be induced in leukemic LGL by in vitro treatment with as little as 0.05 microgram/mL of anti-CD3 monoclonal antibody. Cell sorting experiments demonstrated that NK activity was induced in CD3+ leukemic LGL (either CD3+, HNK-1+ or CD3+, FcR+) with anti-CD3 monoclonal antibody but not in normal CD3+, FcR- T cells. Treatment with purified interleukin 2 (IL 2) also caused direct activation of some CD3+ leukemic LGL. Despite induction with anti-CD3 MAb or IL 2, activated leukemic LGL did not proliferate or express high density IL 2 receptors detectable by cell sorter analysis. Treatment with alpha interferon had minimal effect on NK activity of LGL leukemic cells. These results suggest that leukemic LGL may provide a useful model for examining the signals required for LGL maturation and activation.
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PMID:Induction of NK activity in large granular lymphocyte leukemia: activation with anti-CD3 monoclonal antibody and interleukin 2. 309 27

The authors have studied the case of a female patient with rheumatoid polyarthritis, who developed a lymphocytic proliferation in the blood, the marrow, and the liver, associated with a neutropenia. Several similar cases have been recently reported in the literature. The cellular proliferation is made of large granulous lymphocytes and the study of membrane markers enables to find the following homogeneous phenotype: E rosette+, CD8+, HNK-1+, FcR+, CD4-luminal diameter "divided by degrees - -, IgS-, HLA class II-. This lymphocytic sub-population produces little interleukin-2, responds weakly to mitogens (PHA, CON A, PWM), and inhibits the response of normal lymphocytes to the same mitogens. These lymphocytes have a weak natural killer activity but, on the contrary, develop a very strong cytotoxic activity which is antibody-dependent. Clinically, splenomegaly, anemia and infections are frequent and hepatomegaly or thrombopenia more rare. Adenopathies are never present. The evolution is chronic in nature and not very aggressive, although the lymphocytic proliferation is monoclonal in origin, as demonstrated in molecular biology studies. The neutropenia might be secondary to an inhibiting effect of lymphocytes on the granular precursors of the bone marrow. There is a definite association between this lympho-proliferative syndrome and rheumatoid polyarthritis, and this association appears to be different from the Felty's syndrome.
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PMID:[Characterization of chronic lymphocytic proliferation in a female patient having rheumatoid polyarthritis with neutropenia]. 361 55

Five patients with polyarthritis and neutropenia had numerous circulating large granular lymphocytes with a phenotype attributed to immature natural killer cells. All five had splenomegaly and recurrent infections. Arthritis was most prominent at the wrists and hands, and all patients were considered to have atypical cases of Felty's syndrome. Antinuclear antibodies, rheumatoid factor, antineutrophil antibodies, and immune complexes were detected in most patients. Bone marrow biopsies revealed a maturation arrest at the myelocyte stage and lymphoid infiltrates. Large lymphocytes with azurophilic cytoplasmic granules were found on peripheral blood smears and showed a characteristic reactivity pattern with monoclonal antibodies suggesting a natural killer cell lineage. Peripheral blood mononuclear cells showed less than normal natural killer activity against K562 target cells. Increased numbers of large granular lymphocytes with a phenotype of immature natural killer cells may be important in the pathogenesis of neutropenia, humoral immune disturbances, and synovitis in a subset of patients with Felty's syndrome.
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PMID:Polyarthritis and neutropenia associated with circulating large granular lymphocytes. 402 84

A 77-year-old man with a malignant pleural effusion was treated by aspiration of fluid and injection of Corynebacterium parvum vaccine for pleurodesis. Within 24 hr he had developed a temperature and a neutropenia followed by an acute crippling polyarthritis of his hands and wrists which subsequently responded to oral steroids and non-steroidal antiinflammatory drugs.
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PMID:Acute polyarthritis following the use of Corynebacterium parvum vaccine (Coparvax) for malignant pleural effusion. 665 46

Sulphasalazine has been established to be an effective drug for second line treatment of early mild to moderate rheumatoid arthritis. Its application for juvenile chronic arthritis (JCA) is limited so far and controversial results for the efficacy of this therapy have been published. We studied the efficacy and tolerance of the sulphasalazine treatment in 32 patients with JCA (10 with polyarthritis, 21 with pauciarthritis and 1 with systemic form). Our results revealed significant response of the treatment at the end of the 6th month in 24/31 patients (77%). In one patient the treatment was discontinued because of transitory neutropenia at the end of the 1st month. No significant difference was observed between the efficacy of the treatment in the polyarticular and pauciarticular disease, as well as newly-diagnosed cases and those with longstanding disease. From the group of 17 children treated up to the end of the 1st year 88% achieved complete remission. No serious toxic effects were observed, with the exception of two cases with transitory low-grade neutropenia. According to our results sulphasalazine is an effective and well tolerated drug for second line treatment of JCA-patients.
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PMID:Sulphasalazine. An alternative drug for second-line treatment of juvenile chronic arthritis. 1059 65

It has been suggested that some cases of nonimmune chronic idiopathic neutropenia of adults (NI-CINA) may be considered preleukemic disorders. This paper describes two patients with NI-CINA who developed acute myeloid leukemia (AML) 34 and 64 months, respectively, following NI-CINA diagnosis. Patient 1 presented erythema nodosum and patient 2 polyarthritis of the large joints 9 and 2 months, respectively, before AML. Patient 1 had AML M4 disease associated with aberrant expression of CD7 and CD19 cell surface markers and one abnormal clone in bone marrow karyotype. Patient 2 had myeloid/natural killer (NK) cell leukemia with expression of CD7 and CD56 molecules and four derivative abnormal clones in the karyotype. Both patients had del(5)(q22q35) in common. No mutations in the transmembrane or the intracytoplasmic domain of the granulocyte colony-stimulating factor (G-CSF) receptor were found. The first patient had disease resistant to chemotherapy from the beginning of the treatment and the second following a brief complete hematological remission. On the basis of these observations, we concluded that a causal link of AML with the underlying NI-CINA cannot be presently justified, but the unusual findings noted in our patients prompt the description of additional cases for a further investigation of the relationships, if any, between these two granulocytic disorders.
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PMID:Two patients with nonimmune chronic idiopathic neutropenia of adults developing acute myeloid leukemia with aberrant phenotype and complex karyotype but no mutations in granulocyte colony-stimulating factor receptor. 1180 37

We report a case of CD8(+)/V beta 5.1(+) T-cell large granular lymphocyte leukemia (T-LGL leukemia) presenting with mild lymphocytosis, severe autoimmune neutropenia, thrombocytopenia, polyarthritis and recurrent infections with a chronic disease course. Immunophenotyping showed an expansion of CD3(+)/TCR alpha beta(+)/CD8(+bright)/CD11c(+)/CD57(-)/CD56(-) large granular lymphocytes with expression of the TCR-V beta 5.1 family. Southern blot analysis revealed a clonal rearrangement of the TCR beta-chain gene. Hematopoietic growth factors, high dose intravenous immunoglobulin and corticosteroids were of limited therapeutic benefit to correct the cytopenias. During the disease course, the patient developed a severe cutaneous leg ulcer and bilateral vascular mammary skin lesions. Treatment with 2-deoxycoformycin resulted in both clinical and hematological complete responses, including the resolution of vascular skin lesions. Combined immuno-staining with relevant T-cell associated and anti-TCR-V beta monoclonal antibodies proved to be a sensitive method to assess the therapeutic effect of 2-deoxycoformicin and to evaluate the residual disease.
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PMID:Cd8(+)/V beta 5.1(+) large granular lymphocyte leukemia associated with autoimmune cytopenias, rheumatoid arthritis and vascular mammary skin lesions: successful response to 2-deoxycoformycin. 1211 71

Kikuchi-Fujimoto's disease (KFD) or histiocytic necrotizing lymphadenitis is a self-limiting condition characterized by fever, neutropenia and lymphadenopathy. It is rarely associated with systemic lupus erythematosus (SLE). The authors reported a case of Kikuchi's necrotizing lymphadenitis who presented with fever, generalized lymphadenopathy, moderate leukopenia, polyarthritis, vasculitis-like lesions and oral ulcers compatible with SLE but serologic tests for autoimmune disease were all negative. The clinical symptoms resolved spontaneously within 3 months without any treatment. Because there is an association between KFD and SLE, great care should be taken with a patient who presents with either KFD or SLE.
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PMID:Kikuchi-Fujimoto's disease, histiocytic necrotizing lymphadenitis, mimicking systemic lupus erythematosus. 1245 85


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