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Query: UMLS:C0027947 (neutropenia)
 17,527 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The fine structures of the red pulp of the spleen and the liver of a patient with autoimmune hemolytic anemia, neutropenia and thrombocytopenia associated with systemic lupus erythematosus are described. The red blood cells were phagocytized in toto by the splenic macrophages. These also contained neutrophils and platelets in various stages of degradation. Sinus endothelial cells revealed occasional erythrophagocytosis. The Kupffer cells in the liver occasionally contained red cells and platelets. These morphological findings and marked improvement of hematological abnormalities following splenectomy suggested that the spleen was the major site of destruction of blood cells. Undulating tubules associated with the endoplasmic reticulum were present in the sinus endothelial cells of the spleen.
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PMID:Fine structure of the spleen in autoimmune hemolytic anemia associated with systemic lupus erythematosus. 63 98

Retyculocytopenia and neutropenia or both abnormalities are very infrequent findings during the evolution of patients with acquired autoimmune hemolytic anemia (AIHA). In this paper we describe the clinical cases of three patients with AIHA in whom six different periods of reticulocytopenia were identified; the first two patients also had neutropenia. At the moment of the diagnosis of the cytopenias, the patients did not have a systemic disease, a viral infection or received immunosuppression able to produce them. In every patient the bone marrow cellularity was increased mostly at the expense of normal and macroerythroblasts, containing significant amounts of the other hematopoietic cells. After the diagnosis of AIHA, the patients received supplementary treatment with folic acid which did not produce a remission. The remissions were related to steroids: the neutrophil counts increased importantly one to ten days after starting the steroid administration, whereas reticulocytes reappeared more slowly from 7 to 30 days post-initiation. In patients with AIHA the occurrence of reticulocytopenia and neutropenia have been related to several causes. The favorable responses to steroids in our cases suggests a relationship of steroid with the immunological changes in AHA. It should be pointed out that it is necessary to prolong the followup as done in our three cases. Although these cytopenias may be independent from the hemolytic anemia, we feel our cases suggest that they could be manifestations of a pathologic state not yet characterized.
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PMID:[Reticulocytopenia and neutropenia in 3 patients with idiopathic autoimmune hemolytic anemia]. 186 1

T-gamma-lymphoproliferative disorder, a syndrome of T-cell lymphocytosis with neutropenia has been described in patients with various autoimmune disorders, especially rheumatoid arthritis. We report a case of T-gamma-lymphoproliferative disorder occurring in a 42-year-old white woman with a long history of dermatitis herpetiformis and subsequent development of Coomb's positive autoimmune hemolytic anemia and polymyositis. The peripheral blood lymphocytes showed the T-suppressor cell phenotype (CD2-, CD3-, CD8-, and CD4-). DNA analysis of the peripheral blood lymphocytes revealed a T-cell receptor beta-chain gene rearrangement and an immunoglobulin heavy-chain gene rearrangement. The patient's course was marked by numerous bouts of infection. The unique factor in this patient was the development of a plasma cell dyscrasia and amyloidosis prior to death.
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PMID:T-gamma-lymphoproliferative disorder arising in a background of autoimmune disease and terminating in plasma cell dyscrasia with primary amyloidosis. 189 15

A 47-year-old female developed autoimmune hemolytic anemia, autoimmune neutropenia, and autoimmune thrombocytopenia 19 months following allogeneic bone marrow transplantation for chronic myelogenous leukemia. Treatment with high-dose corticosteroids resulted in marked improvement in all three cell lines.
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PMID:Autoimmune pancytopenia following allogeneic bone marrow transplantation. 196 93

Autoimmune hemolytic anemia (AIHA) has been considered to be unresponsive to intravenous gammaglobulin (IVGG) at the doses that are effective in immune thrombocytopenic purpura and autoimmune neutropenia (usually 2 g/kg total dose). This study reports the use of a higher dose (5 g/kg total dose over 5 days) in four severe cases of AIHA which resulted in a sustained remission in two patients, a transient response in the third, and a failure in the forth patient. These data suggest that larger quantities of IVGG may be needed in this disease, possibly because the reticuloendothelial system appears to be enlarged in AIHA patients.
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PMID:Intravenous treatment of autoimmune hemolytic anemia with very high dose gammaglobulin. 243 27

In 1981, Imbach et al. (Lancet, 1, 1228-1231) reported that infusion of intravenous immunoglobulin (IVIG) would substantially elevate platelet counts in children with acute or chronic idiopathic thrombocytopenic purpura (ITP). Subsequent studies confirmed these findings and extended the effect to adults and to newborns with passive immune thrombocytopenia. Studies in children with acute ITP demonstrated that administration of IVIG was the fastest way to increase a patient's platelet count, and that this agent could be given at doses as high as 1 g/kg/day so that the course of therapy and response to treatment would be more rapid. Reports of effective treatment of patients with autoimmune neutropenia and autoimmune hemolytic anemia by IVIG broadened the scope of its usefulness. In addition, several studies in children and adults with chronic ITP suggested that repeated infusions were a safe and effective way to maintain an adequate platelet count in such patients, and might also gradually lead to lasting improvement. These studies and Imbach's controlled trial in children with acute ITP suggested that IVIG therapy might provide a curative effect in addition to the acute effect. To combine all of these clinical effects with a multitude of in vitro observations to explain the mechanism of action of IVIG is complicated. Fehr et al. (N. Engl. J. Med. 306, 1254-1258, 1982) showed that Fc receptor blockade occurs following administration of IVIG. This effect is clearly demonstrated in vivo by the delayed removal from the vascular space of antibody-coated red blood cells following infusion of IVIG. In addition, many less well-defined effects occur in relation to immunoglobulin production, induction of suppressor cells, antiplatelet antibody levels, and bone marrow platelet production. Studies continue to try to define which effects actually underlie the clinical effects seen, and which are merely test-tube phenomena.
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PMID:The use of intravenous gamma-globulin in idiopathic thrombocytopenic purpura. 247 85

T-cell chronic lymphocytic leukemia: clinical aspects and laboratory findings of five patients. This study illustrates the main clinical aspects and laboratory findings for five patients suffering from T-cell chronic lymphocytic leukemia. They make up 5% of our observations in the Department of Oncology-Hematology at the Busto Arsizio Hospital. Three patients with phenotype CD3+/CD4+ showed a fast course with skin involvement and poor response to chemotherapy (mean survival: 12 months). The course of a patient with unusual phenotype CD3-/CD4+ associated with autoimmune hemolytic anemia and end-stage prolymphocytic transformation was better (survival: 58 months). For 12 years we have been observing a woman suffering from the recently defined CD3+/CD8+/HNK1+ large granular lymphocytes chronic lymphocytosis with associated neutropenia. The disease has good prognosis, with poor symptomatology even without therapy. This study supports the immunological classification of the chronic lymphoproliferative diseases of leukemia and lymphoma with different clinical aspects and prognoses. This method of classification may be important in the consideration of some therapeutical approaches.
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PMID:[Chronic T-cell lymphocytic leukemia: clinical and laboratory aspects of 5 patients]. 278 7

Intravenous gamma globulin (IVIG) was used to treat autoimmune neutropenia of childhood and autoimmune hemolytic anemia, two autoimmune disorders not previously treated with this modality. Six children younger than two years of age, who presented with severe infections and persistent absolute neutrophil counts (300/mm3), were treated with 1 g of gamma globulin per kilo body weight until counts reached more than 1,000/mm3. The average response occurred with a dose of 3.0 g/kg within five to seven days and lasted an average of 14 days before counts decreased to baseline levels. Four patients with autoimmune hemolytic anemia were also treated with IVIG, 1 g/kg for five to seven days (average dose of 5 g/kg), for severe Coombs'-positive hemolytic anemia. Response of the hemolytic anemia to IVIG was excellent in one patient and good in two patients. No response occurred in the fourth patient. Response was slower in these patients than in patients treated for immune thrombocytopenic purpura (ITP). The average total amount of gamma globulin required for a response is markedly different: 1 g/kg for ITP, 3.0 g/kg for autoimmune neutropenia, and 5 g/kg for hemolytic anemia. Possible mechanisms of action include blockade of reticuloendothelial system Fc receptors, suppression of autoantibody production, and/or interference in the binding of autoantibodies to target cells.
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PMID:Use of intravenous gamma globulin for the treatment of autoimmune neutropenia of childhood and autoimmune hemolytic anemia. 311 5

Sixteen pediatric patients diagnosed with a variety of autoimmune-mediated hematocytopenias were treated with one to 50 courses of intravenous gamma globulin (IVIG), pH 4.25, over the course of one to 30 months. Thirteen patients had immune thrombocytopenic purpura (ITP), two had autoimmune neutropenia, and one had autoimmune hemolytic anemia. In one patient, chronic ITP was associated with systemic lupus erythematosis, and in a second patient, acute ITP was the presenting manifestation of infection with human immunodeficiency virus. Initial therapy consisted of 400 mg/kg/dose daily for five days for the first seven patients treated, and 1,000 mg/kg/dose daily for two days for the remaining nine patients. In 15 of 16 patients, there was a response to IVIG therapy. In nine of 16 patients, maintenance IVIG therapy for two to more than 30 months was required. Minimal toxicity was experienced in four of 210 separate infusions. Data are presented to support the use of IVIG in the management of childhood autoimmune disorders.
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PMID:Efficacy of intravenous gamma globulin in autoimmune-mediated pediatric blood dyscrasias. 311 7

We report an observation relating to a history of dissociated pancytopenia with unexpected severe hemolysis following surgery for colonic carcinoma. Following short-term monochemotherapy with 5-fluorouracil investigations revealed neutropenia and leukemoid syndrome. The anemia probably resulted from the interaction of 2 consecutive mechanisms: delayed post-transfusional hemolysis through anamnestic alloimmunization and an autoimmune hemolytic anemia whose biological evidence persisted for several months after the initial episode.
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PMID:[Delayed post-transfusional hemolysis, leukemoid syndrome, autoimmune hemolytic anemia. Coordinating Committee for the Specific Study of Anemias under the chairmanship of H. Rochant]. 345 6


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