Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Autoimmune neutropenia is thought to be an uncommon disorder in adults. Over a 2-year period, however, autoimmune neutropenia was diagnosed in seven adults in a county with a population of approximately 105,000 people. The median age of the patients was 52 years old (range of 22 to 81 years), and five of the seven patients were women. All seven patients had at least one other symptom of autoimmune disease. Three patients had splenomegaly, three patients had positive direct antiglobulin tests, and two patients had immune thrombocytopenia. Antibodies reacting to neutrophils were detected by either granulocyte agglutination (GA) or granulocyte immunofluorescence (GIF) testing in five of the seven patients. Antibodies in four patients reacted with an 80 kd neutrophil membrane glycoprotein, and antibodies from two people reacted with a 60 kd membrane glycoprotein. Three patients were given treatment with splenectomy, which resulted in only transient improvement in the neutrophil counts. Serious infections occurred in only three patients over the 2 years of observation. In summary, autoimmune neutropenia in adults may occur more often than appreciated. Most cases of autoimmune neutropenia in adults appear to be associated with other autoimmune phenomena.
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PMID:Autoimmune neutropenia in Sheboygan County, Wisconsin. 159 17

Four case histories are reported: 1. A 37-year-old woman suffering from Glanzmann's thrombasthenia has been regularly seen since 1955. Characteristically (and in contrast to the first description by Glanzmann!) persistently prolonged bleeding times were noted. Clot retraction is severely diminished and the platelets fail to aggregate upon various stimuli. (Platelet agglutination upon addition of ristocetin to platelet rich plasma is normal.) The diagnosis of thrombasthenia was confirmed by demonstration of a deficiency of the membrane glycoprotein IIb/IIIa complex. In recent years the patient has become refractory to platelet transfusion therapy, a response shown to be due to antibodies against GPIIb/IIIa in the plasma. Spontaneous bleeding tendency has appeared to improve over the years. 2. Two patients with proliferation of B-lymphocytes are presented. a) Splenomegaly and an increase of B-lymphocytes in the peripheral blood were detected in a 45-year-old asymptomatic man. DNA analysis suggested that polyclonal proliferation of B-lymphocytes was present. Diagnostic considerations are discussed. b) In a 46-year-old male patient with subacute aleukemic leukemia of a pre-B-cell type diagnosed in 1981, the disease showed an unexpectedly benign course: after initial mild chemotherapy the patient has remained in a stable condition while off cytotoxic treatment for the last two years. Nevertheless, besides anemia necessitating regular transfusions, persistent agranulocytosis is present which is not explained by bone marrow infiltration. In vitro experiments suggest suppression of myelopoiesis by cellular interaction with leukemic cells or a deficiency of growth factors causing agranulocytosis. 3. An 81-year-old man showed signs and symptoms of lead intoxication which proved to be due to oral ingestion of a lead-containing ointment.
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PMID:[Unusual features in Glanzmann thrombasthenia; proliferation of B-lymphocytes, lead poisoning]. 305 86

The Friend spleen focus-forming virus induces a massive expansion of erythroid progenitor cells resulting in polycythemia and splenomegaly. The pathogenic agent is the membrane glycoprotein gp55, encoded by the env gene. Recent evidence indicates that gp55 binds to and activates the erythropoietin (Epo) receptor. It is not clear, however, whether gp55 completely mimics the natural receptor ligand (Epo). To directly compare both effectors, we constructed selectable retroviral vectors which carry either the env or the Epo gene. The selection marker allowed for clonal analysis of infected cells. After infection of DBA/2J mice, the spleen weight, hematological indices, and Epo titer of peripheral blood were monitored. Although both viruses induced an acute erythrocytosis, there were significant differences in disease phenotype and progression. The Epo virus caused an enhanced increase of hematocrit and erythrocytes, whereas with the env virus the pool of late progenitors (CFU-erythroid) was dramatically expanded, resulting in a more severe splenomegaly. The distribution of cytologically recognizable erythroid precursors was shifted towards immature cell types by the env vector compared with Epo. These data suggest that Epo and gp55 differentially affect proliferation and differentiation. Gp55 appears to promote proliferation over differentiation, whereas Epo preferentially drives differentiation.
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PMID:Selectable retrovirus vectors encoding Friend virus gp55 or erythropoietin induce polycythemia with different phenotypic expression and disease progression. 793 6

To aid in the rapid differential diagnosis of thrombocytopenia, the authors developed a latex agglutination test for glycocalicin, a proteolytic fragment of platelet membrane glycoprotein Ib. Plasma glycocalicin determinations were performed for 34 patients with thrombocytopenia. Plasma samples from four patients with aplastic anemia and ten patients with myelodysplastic syndromes, all with glycocalicin levels less than 0.6 mg/L by an enzyme-linked immunosorbent assay, all had negative results by the latex test. In contrast, positive latex agglutination titers were obtained for all 12 patients with idiopathic thrombocytopenic purpura. Eight patients with liver cirrhosis and splenomegaly had elevated levels of plasma glycocalicin, and all of their plasma samples produced agglutination. This latex agglutination test for glycocalicin allows a rapid discrimination of thrombocytopenia caused by impaired platelet production from that caused by increased platelet destruction; thus, it is suitable for use as a screening test in a routine clinical laboratory.
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PMID:Rapid detection of plasma glycocalicin by a latex agglutination test. A useful adjunct in the differential diagnosis of thrombocytopenia. 824 98