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

A 83-year-old man was diagnosed with primary myelofibrosis based on the presence of leukoerythroblastosis, splenomegaly, chromosome 46 XY, a dry tap bone marrow aspiration and fibrosis on bone marrow biopsy, when he was admitted for herpes zoster in June 1987. He was admitted for a second time with multiple subcutaneous tumors over his entire body in July, 1989. He had mild splenomegaly, but no hepatomegaly nor lymphadenopathy. Laboratory tests were as follows: RBC 214 x 10(4)/microliters, Hb 5.1 g/dl, Ht 17.7%, WBC 3,200/microliters with leukoerythroblastosis, platelets 11.6 x 10(4)/microliters, s-lysozyme 251 micrograms/ml, u-lysozyme 770 micrograms/ml, NAP ratio 98%, score 278. Bone marrow aspiration resulted in a dry tap. Bone marrow biopsy showed marked fibrosis. Histologic examination of subcutaneous tumor biopsy specimens revealed a diffuse infiltration of monocytes with flexuous nuclei. These cells were positive for alpha-naphtyl butyrate esterase stain, and negative for peroxidase, alpha-naphtol ASD chloroacetate esterase stain and platelet glycoprotein IIb/IIIa stain (APAAP). Ultrastructurally, these cells were mostly monocytes and promonocytes, while phenotypically, CD11b, CD13, CD14, CD33 and HLA-DR were positive. These date indicated that the subcutaneous tumors originated from monocytes.
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PMID:[Primary myelofibrosis transforming into multiple subcutaneous monoblastoma--a case report]. 175 57

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

A 50-year-old man was admitted with acute inferior and anterior myocardial infarction. The patient was diagnosed with essential thrombocythemia (ET) based on the findings of marked thrombocytosis of 1,113 x 10(3)/mm3, splenomegaly, and numerous clumping megakaryocytes on bone marrow biopsy. Emergent coronary angiography revealed extensive multivessel thrombosis involving the left main coronary artery and completely occluding the proximal right coronary artery. In addition to standard therapy with aspirin, heparin, and primary angioplasty of the right coronary artery, the patient received additional antiplatelet therapy with ticlopidine, hydroxyurea, and the platelet glycoprotein IIb/IIIa receptor-inhibiting monoclonal antibody drug abciximab (ReoPro). Serial coronary angiograms 1 and 5 days following the infarction showed progressive thrombus resolution. The pathophysiologic mechanisms and therapeutic challenges of ET-associated coronary thrombosis are discussed in this report.
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PMID:Multivessel coronary thrombosis treated with abciximab (ReoPro) in a patient with essential thrombocythemia. 949 58

One of frequently occurring hematological disorders accompanying advanced liver diseases is thrombocytopenia (TP). Immunological disorders in patients with liver cirrhosis, loss of tolerance to own antigens, and the change of platelet antigenicity enable antiplatelet antibody formation under the influence of continuous activation. The aim of the study was to determine the rate of autoimmunological thrombocytopenia occurrence in patients with liver cirrhosis and TP. Antiplatelet autoantibody occurrence were determined in blood serum with the use of ELISA method in 15 patients with liver cirrhosis and TP (mean plt number 67.9 +/- 24.9 x 10(3)/microliter). Three patients (20%) presented anti-GPIIb/IIIa antibodies and 2 patients--anti-GPIa/IIa. These patients had liver failure (stage C according to Child-Pugh classification) and splenomegaly. Plt morphological parameters were also evaluated. The significant decrease of plt crit as well as the decrease of mean platelet volume (MPV) was observed in liver cirrhosis with thrombocytopenia. The increase of megathrombocyte population (MPV > 20fl) up to 5.5% of all plt was also observed. Megathrombocytes in healthy individuals were 2.25% of platelet population. Examinations confirmed that autoimmunological factors play an important role in the development of thrombocytopenia in liver cirrhosis.
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PMID:Platelet autoantibodies in liver cirrhosis and thrombocytopenia. 1171 41

Forty-five individuals with hepatosplenic schistosomiasis mansoni were studied with the aim of measuring levels of von Willebrand factor antigen (vWF:Ag), detecting abnormalities in platelet morphology and aggregation, and identifying changes to surface antigens. Haemograms, platelet aggregation tests, flow cytometry investigations of CD41/CD42b antibody and vWF:Ag assays were performed. Mean platelet counts were low (77,522/mm3) and 82.2% of patients presented thrombocytopenia. An inverse relationship between spleen size and platelet count was seen. Macroplatelets were found in 57.1% of patients, indicating good bone-marrow response, but were insufficient to compensate for the decrease in platelets due to splenomegaly. Decreased or absent platelet aggregation was seen in 50% of patients, probably due to low platelet counts. Markers for GPIIb/IIIa were normal in more than 90% of patients, not supporting the increased capture and destruction of platelets in the spleen that is hypothesized to occur with cirrhosis. Similar to cirrhosis, vWF:Ag levels were high or very high in 70.5% of patients. High levels of vWF:Ag were associated with platelet counts <100,000/mm3, larger spleen diameter and oesophageal varices. In conclusion, hepatosplenic schistosomiasis leads to a lower platelet count due to pooling in the spleen and, consequently, impaired aggregation, but not to increased capture and destruction of platelets in the spleen. High vWF:Ag levels probably promote stabilization of platelet microaggregates and prevent minor manifestations of thrombocytopenia such as petechiae, ecchymosis and gingival bleeding.
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PMID:Platelet function and the von Willebrand factor antigen in the hepatosplenic form of schistosomiasis mansoni. 1911 53