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

Several immune-mediated blood disorders in both children and adults have findings compatible with an autoimmune etiology, although the autoantigen in most instances is not clearly established. In the present report, we review briefly the clinical features of immune hemolytic anemia, immune thrombocytopenic purpura, and immune neutropenia in children and discuss the possible pathogenetic mechanisms based on present experimental and clinical observations. The role of the spleen in the pathophysiology of these disorders is emphasized.
 ...
PMID:The role of the spleen in "autoimmune" blood disorders. 57 5

In this 16 year old boy a syndrome, characterized by high fever, generalized lymphadenopathy, splenomegaly, diffuse skin rash, facial and periorbital edema, neutropenia, thrombocytopenia, elevated serum glutamic oxaloacetic transaminase (SGOT) levels and transient electrocardiographic changes, appeared 2 weeks after the institution of diphenylhydantoin therapy. Lymph node biopsy, performed at the height of the illness, revealed widespread subendothelial fibrin exudation and fibrin-platelet thrombi in the lymph node microvasculature, a finding most consistent with thrombotic thrombocytopenic purpura. Although many types of abnormal lymph node histology have been described with diphenylhydantoin, this appears to be the first instance of this histologic picture. This syndrome may be related to a serum sickness-like illness which triggered an episode of localized coagulopathy.
 ...
PMID:Diphenylhydantoin-induced serum sickness with fibrin-platelet thrombi in lymph node microvasculature. 116 93

4 patients had typical features of thrombotic thrombocytopenic purpura (TTP) after 3 to 8 weeks of ticlopidine therapy. In 2 ticlopidine was the only medication taken before TTP; in another, rechallenge with drugs other than ticlopidine that the patient had been taking did not produce relapse. In all patients the delay between start of ticlopidine and TTP was in the same range as the latent period before ticlopidine-induced neutropenia. No relapse occurred in the 4 to 43 months of follow-up in the 3 surviving patients.
 ...
PMID:Thrombotic thrombocytopenic purpura related to ticlopidine. 167 52

Anti-neutrophil antibodies have been described in a variety of clinical conditions associated with neutropenia. However, relatively little is known about the antigenic specificities of naturally occurring anti-neutrophil autoantibodies. We investigated the possibility that anti-neutrophil antibodies specific for the neutrophil adhesion glycoprotein (GP) complex CD11b/CD18 might be present in the sera of some patients with autoimmune neutropenia. These membrane GPs have been shown to be highly immunogenic in the production of murine monoclonal antibodies against neutrophil antigens. Moreover, autoantibodies to the platelet membrane GP complex IIb/IIIa, another member of the integrin family of cell adhesion proteins, have been demonstrated in immune thrombocytopenic purpura. Sera from 50 patients known to have anti-neutrophil IgG antibodies were evaluated using an immunobead "antigen capture" assay, modeled after a method used to identify anti-platelet GPIIb/IIIa autoantibodies. This assay detected anti-CD11b/CD18 autoantibodies in seven of the 50 sera. Each of these seven sera demonstrated decreased IgG binding to the neutrophils of a patient with congenital deficiency of CD11b/CD18. The patient with the highest levels of anti-CD11b/CD18 suffered recurrent skin infections and cellulitis, and died of respiratory failure during one of multiple episodes of pneumonia. Purified IgGs from five of these patients demonstrated effects on adhesion and/or opsonin receptor-mediated functions when tested with intact neutrophils in vitro. Our findings indicate that some patients with autoimmune neutropenia have autoantibodies specific for the functionally important neutrophil adhesion proteins CD11b/CD18. Our findings also raise the possibility that these autoantibodies may, in some cases, interfere with neutrophil function, thereby amplifying the risk of infection associated with neutropenia.
 ...
PMID:Identification of autoantibodies specific for the neutrophil adhesion glycoproteins CD11b/CD18 in patients with autoimmune neutropenia. 167 88

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.
 ...
PMID:Intravenous treatment of autoimmune hemolytic anemia with very high dose gammaglobulin. 243 27

High dose intravenous gammaglobulin (0.4 g/kg/day) for five days, causes a rapid rise in the platelet count in immune thrombocytopenic purpura (ITP). The response in chronic ITP is transient making it useful in emergency situations. Efficacy in immune mediated neutropenia and warm antibody haemolytic anaemia has been reported but in limited numbers. We have used this therapy in twenty-three adults with ITP, one patient with immune neutropenia, four patients with warm antibody autoimmune haemolytic anaemia and one case of thrombotic thrombocytopenic purpura (TTP). In ITP only four of the twenty patients failed to respond to gammaglobulin and of these, three had a positive antinuclear factor but no other signs of systemic lupus erythematosis. The sole patient with neutropenia treated with IgG failed to show any response. In three patients with haemolytic anaemia parameters of haemolysis improved during therapy. The patient with TTP suffered a cerebrovascular accident during therapy prior to a documented response. High dose intravenous immunoglobulin rapidly elevates the platelet count in acute and chronic ITP. Its use in other immune mediated haematological conditions has yet to be fully evaluated.
 ...
PMID:High dose intravenous gammaglobulin in immune haematological disease. 244 Jul 41

In this paper we review our experience with HIV-related thrombocytopenic purpura (TP) in 24 patients seen from October, 1985 through April, 1988: the median follow-up was 16 months (range 3-32). All patients belonged to risk groups for AIDS and intravenous drug abusers represented 83% of the entire cohort. The male/female ratio was 4, and most of the patients were Walter Reed stage 3. The mean value of platelets at diagnosis was 33 x 10(9)/liter (range 6-120), and half of the patients had severe thrombocytopenia with hemorrhagic symptoms. Anemia and/or neutropenia were concomitant with TP in 21% and 17% of cases; four cases had pancytopenia. Marrow pictures showed megakaryocytic hyperplasia in 68% of cases; myelodysplasia or hypoplasia were observed in 14% and 18% of patients, respectively; lymphoid aggregates were present in two cases. Antiplatelet antibodies and circulating immune complexes were detected in 40% and 50% of cases, and the mean T4/T8 ratio was 0.9 (range 0.4-1.8). Half of the patients did not require specific therapy due to lack of bleeding; however no spontaneous reversions to normal platelet values occurred. The response to steroids and to immunoglobulins (either high-dose or anti-D) was good but temporary, and required maintenance therapy. The 2-year actuarial risk of evolution into overt AIDS was 30%, with a crude rate of 4 cases over 365 person-months at risk. The events which determined AIDS were opportunistic infections in two cases, Kaposi's sarcoma and malignant lymphoma in the other two. A comparison with the features of idiopathic TP is made and hypotheses regarding the pathogenetic mechanisms are discussed.
 ...
PMID:HIV-related thrombocytopenic purpura: a study of 24 cases. 249 84

The systemic rheumatic diseases are commonly complicated by hematologic abnormalities. Five frequently encountered and clinically relevant complications are reviewed. They include the anemia of chronic disease, neutropenia, autoimmune thrombocytopenic purpura, the lupus inhibitor, and hematologic malignancies. The pathophysiology and treatment of each of these conditions are discussed in this review.
 ...
PMID:Hematologic complications of rheumatic disease. 311 57

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.
 ...
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.
 ...
PMID:Efficacy of intravenous gamma globulin in autoimmune-mediated pediatric blood dyscrasias. 311 7


1 2 3 4 5 Next >>