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)

Three patients had leukocytosis of large granular lymphocytes and chronic neutropenia. Clonal chromosomal abnormalities (trisomy 8 and trisomy 14) and lymphocytic infiltration of splenic red pulp, hepatic sinusoids, and bone marrow indicated the neoplastic nature of the large granular lymphocytes. Demonstration of a T3+, T8+, HNK-1 + phenotype and low natural killer cell activity that was augmented by interferon treatment showed the leukemic cells to be immature natural killer cells. Multiple autoantibodies were present and included rheumatoid factor and antinuclear, antineutrophil, antiplatelet, and antierythrocyte antibodies, suggesting a defect of B-cell immunoregulation. In addition, in-vitro studies showed impaired suppression of immunoglobulin biosynthesis by abnormal cells from one patient. Antineutrophil antibodies and absence of direct cell-mediated inhibition of granulocyte-macrophage colony formation supported a humoral immune mechanism for the neutropenia. In these patients the syndrome of splenomegaly, multiple autoantibodies with neutropenia, and lymphocytosis of large granular lymphocytes is due to a neoplastic proliferation of immature natural killer cells.
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PMID:Leukemia of large granular lymphocytes: association with clonal chromosomal abnormalities and autoimmune neutropenia, thrombocytopenia, and hemolytic anemia. 396 54

A spontaneous multifocal subcutaneous fibromatosis is described in 6 pig-tailed macaques (Macaca nemestrina) with simian acquired immune deficiency syndrome (simian AIDS). The lesions consisted of a proliferation of vascular fibrous tissue that was infiltrated by lymphocytes and plasma cells. One animal also had retroperitoneal fibromatosis, which has also been found in this colony of pig-tailed macaques. Progressive weight loss, diarrhea, lymphadenopathy, and neutropenia were seen. Peripheral lymph nodes were hyperplastic, and there was splenomegaly. Aggregates of lymphocytes were present in the bone marrow, kidneys, liver, and lungs. Type D retrovirus particles were found in three nodules by electron microscopy; intracytoplasmic type A and budding particles were identified in fibroblasts. In a setting of acquired immunodeficiency, these subcutaneous tumors in pig-tailed macaques present a striking analogy to Kaposi's sarcoma in human AIDS.
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PMID:Subcutaneous fibromatosis associated with an acquired immune deficiency syndrome in pig-tailed macaques. 401 42

Visceral leishmaniasis (kala-azar) is an important cause of morbidity and mortality in widely scattered areas of the world. To better characterize the South American form of the disease, the clinical and laboratory manifestations of 29 patients admitted to hospital (18 male and 11 female patients, mean age 4.9 years), were assessed in an endemic area in northeastern Brazil. Fever, weight loss, pronounced splenomegaly, hepatomegaly, anemia, thrombocytopenia, relative neutropenia, hypoalbuminemia and hypergammaglobulinemia were found in the majority of patients. Symptoms were often present for two or more months before diagnosis. Secondary infections complicated many cases; there were ten cases of pneumonia and half of the patients had one or more intestinal parasites. The average length of hospital stay was 27 days; all patients were treated with meglumine antimoniate (Glucantime). The mortality rate was 3%. American visceral leishmaniasis remains an important disease among children living in endemic areas.
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PMID:American visceral leishmaniasis (kala-azar). 402 31

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

The kinetics of blood neutrophils was investigated by means of the in vitro radioactive diisopropyl fluorophosphate method in 35 patients with a chronic, steady-state neutropenia. There were 17 patients in whom the half disappearance time of neutrophils was normal. In 10 of these patients, the production of neutrophils was low and in 7, production was normal. In 18 patients the half disappearance time of neutrophilic granulocytes was shorter than normal. The production of neutrophilic granulocytes was low in five of these patients, normal in eight patients, and increased in five. An attempt was made to correlate other laboratory measurements with the kinetic picture, but no relationship was found; the marrow neutrophil reserve as measured by endotoxin or cortisol injection; marrow cellularity on aspiration or biopsy; in vitro-labeling index with (3)HTdR; or serum lysozyme concentration proved of no value in identifying the various kinetic groups. The only finding that seemed to correlate with the kinetic picture was the presence or absence of splenomegaly. In 12 of the 18 patients with a short half disappearance time, splenomegaly was present whereas in 15 of 17 patients with a normal half disappearance time, there was no splenomegaly. Of 20 patients with greater than 1000 neutrophils per mm(3), 17 were found to have a normal total-blood neutrophil pool. Thus these patients, with many of their cells marginated, agree to have a "shift neutropenia."Myelocyte to blood transit time and myelocyte generation time, as measured in seven patients by in vivo labeling with diisopropy fluorophosphate, proved to be essentially normal. Thus, it appears that in chronic neutropenia, increased or decreased production of neutrophils is accomplished by increasing or decreasing early precursor input into the system.
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PMID:Leukokinetic studies. XIV. Blood neutrophil kinetics in chronic, steady-state neutropenia. 509 74

A disease that is similar to human AIDS may occur in monkeys. Simian AIDS (SAIDS) was experimentally transmitted from 2 rhesus monkeys dying of the disease to 4 cytomegalovirus (CMV) antibody-negative rhesus monkeys. The inocula consisted of the supernatant fluid from 10% homogenates of various tissues with or without buffy-coat cells from blood. Lymphadenopathy, splenomegaly, neutropenia, polymyositis, and other signs of the disease appeared in recipients within a few weeks after inoculation. Two animals developed Kaposi-like "patch" and "plaque" skin lesions and one died of sepsis and profound lymphoid depletion. A second animal also died with lymphoid depletion. All animals became infected with CMV but antibody levels were low in two animals, appeared and then disappeared in one, and never developed in the second monkey which died.
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PMID:Experimental transmission of simian acquired immunodeficiency syndrome (SAIDS) and Kaposi-like skin lesions. 613 95

A patient with low-grade lymphocytosis, splenomegaly, and neutropenia, but adequate myeloid leukogenesis, was found to have chronic lymphocytic leukemia, which represented a clonal proliferation of a distinct T-lymphocyte subset. The lymphocytes did not form E rosettes but had an OKT3+, OKT4+, OKT6+, OKT8+, OKT11+, HNK-1+, HNK-36+, OKIa1+, OKM1+ phenotype and functionally had suppressor and natural killer activity. Morphologically, they were large granular lymphocytes, which were strongly acid phosphatase positive and nonspecific esterase negative. They did not respond to mitogens, or to allogeneic cells. Initially, the spleen appeared to be the most involved organ and, judging from the high proportion of leukemic splenic lymphocytes in the S and G2/M phases of the cell cycle, was also the organ of origin of the leukemic cells. Only a few leukemic cells in the blood and bone marrow were in S and G2/M phases. After splenectomy, the lymphocyte count rose considerably and the bone marrow became progressively more infiltrated by tumor nodules. One year after diagnosis, the patient was started on chemotherapy because of progressive anemia. He responded to the chemotherapy by normalization of the hemoglobin and neutrophil count and had a moderate decrease in the bone marrow involvement and peripheral lymphocytosis.
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PMID:Natural killer and suppressor T-cell chronic lymphocytic leukemia. 622 21

4 patients are described with rheumatoid arthritis, splenomegaly, neutropenia and an unusual proliferation of T cells in the blood and marrow. These patients are clinically similar to patients with classical Felty's syndrome but can be distinguished from them by staining blood and marrow mononuclear cells with a panel of monoclonal anti-T cell antibodies. The T cells from patients with T cell proliferations stain with UCHT1 (OKT3 equivalent) and UCHT4 (OKT8 equivalent but do not stain with a panel of OKT1-like antibodies. In 7 patients with classical Felty's syndrome there was no increase of UCHT4 cells in the blood and the large majority of T cells stained with OKT1-like antibodies. The marrows from the patients with T cell proliferations contain plentiful haemopoietic progenitor cells and it is probable that the T lymphocytes suppress their normal maturation. There was a poor response to splenectomy in 2 patients with T cell proliferations, and single cytotoxic drug therapy may be more appropriate when therapy is required. The literature is reviewed and it is suggested that the T cell proliferations may be secondary to the rheumatoid process.
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PMID:Unusual T cell proliferations and neutropenia in rheumatoid arthritis: comparison with classical Felty's syndrome. 633 54

With the purpose of detecting granulocyte-specific membrane-directed autoantibodies, IgG was isolated from 14 patients with Felty's syndrome, from 5 patients with rheumatoid arthritis associated with neutropenia, and from 3 rheumatoid factor positive patients with autoimmune neutropenia. By means of indirect immunofluorescence suspensions of leukocytes from healthy controls were tested for their ability to bind whole IgG fractions or F(ab')2 fragments of IgG isolated from the neutropenic patients. By this method, whole IgG preparations from neutropenic patients and normal controls were invariably found to bind to the surface of granulocytes and a minor proportion of lymphocytes. In contrast. F(ab')2 fragments of IgG from the neutropenic patients failed to bind to the surface of granulocytes. Both whole IgG fractions and F(ab')2 fragments displayed displayed antinuclear antibody activity ruling out the possibility of loss of antibody reactivity during the preparation of F(ab')2 fragments. The neutropenia seen in rheumatoid arthritis with or without splenomegaly thus does not seem to be induced by granulocyte-specific membrane-directed IgG autoantibodies, but rather by a non-specific attachment of IgG most probably in immune complex bound form to Fc gamma receptors on neutrophils which thereby acquire surface properties facilitating removal from the circulating blood cell pool.
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PMID:Lack of evidence for granulocyte specific membrane-directed autoantibodies in neutropenic cases of rheumatoid arthritis and in autoimmune neutropenia. 634 99

Patients with HCL are subject to a variety of medical problems. Many of these complications are caused by the cytopenias and splenomegaly produced by proliferating neoplastic cells. Infection is a common cause of morbidity in HCL, but it is not clear whether there is an inherent defect in the immune system. The incidence of infection is related to neutropenia and is increased by the administration of cytotoxic drugs and corticosteroids; such drugs should be used cautiously in these patients. Opportunistic or unusual pathogens occur frequently in HCL, but recovery from such infections is the rule if the diagnosis is made early. Marrow hypoplasia is not infrequently seen and may present diagnostic difficulties. Such patients may have a lower tumor burden and clinically milder anemia. Hemorrhagic complications are unusual in HCL, though many patients have platelet function abnormalities. Other medical problems occur with increased frequency in HCL, and failure to recognize them leads to increased morbidity in this disease. Autoimmune disease is seen in up to one fourth of patients. It takes the form of self-limited skin and joint disease, or a more progressive, systemic of patients. It takes the form of self-limited skin and joint disease, or a more progressive, systemic vasculitis. Both forms can usually be treated with splenectomy or corticosteroids, but alkylating agents can also be used successfully. Bone disease is usually localized and responds well to radiotherapy. Other problems such as amyloidosis, multiple myeloma, and paraproteinemia are uncommon in HCL.
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PMID:Clinical problems in hairy cell leukemia: diagnosis and management. 639 Jun 85


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