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)

All members in one generation and two of six members of the next generation were found to have familial thrombocytopenia. The disorder was transmitted as an autosomal dominant trait. Easy bruising and a bleeding tendency were the common features. No member had splenomegaly. Platelet counts varied from 8 to 75 x 10-9/liter, and there were no apparent morphologic abnormalities. Platelet survival studies using sodium chromate Cr 51-labeled autologous platelets were done for three members and showed shortened survival times. Two members were treated with corticosteroids and splenectomy without benefit. Three members tolerated surgery (splenectomy, polypectomy) without undue difficulty. Defective or ineffective thrombocytopoiesis appeared to be the basis of this hereditary disorder.
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PMID:Case report: familial thrombo-cytopenia in seven members of three generations. 65 72

In order to study the effect of oxymetholone therapy in advanced myelofibrosis, 11 patients (4 females, 7 males) were given, 3--5 mg per kg body weight, long-term oxymetholone treatment in a prospective multicenter study. Five cases had previously had a diagnosis of polycythemia vera. All patients had anemia initially, 4 leukocytopenia and 10 thrombocytopenia in addition. Hepato-splenomegaly was present in all cases but in varying degree. Five patients required regular blood transfusions before treatment. In 9 of the 15 courses given, there was normalization of the peripheral blood or substantial improvement (better than 3 g hemoglobin/dl or 50 X 10(9) platelets/1) after androgens. Significant effects were noted both on hemoglobin values and platelet counts. The need for blood transfusions ceased completely in all 5 cases. When oxymetholone treatment was reduced or interrupted 4 patients relapsed; 2 of them responded to a renewed course. The red cell counts returned to previous polycythemic values in one patient and another died from acute leukemia. The results of this study suggest that androgens might be of value in advanced cases of myelofibrosis with transfusion-requiring anemia or severe thrombocytopenia.
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PMID:Oxymetholone treatment in myelofibrosis. 66 66

Hemodynamic studies were performed in 5 vinyl chloride monomer workers in whom splenomegaly or thrombocytopenia was detected during a screening program at major chemical plant. Three patients had portal hypertension and collateral venous circulations, with intrasplenic pressures between 20 and 29 mm Hg and normal wedged hepatic venous pressures, but the gradient between the wedged and free hepatic vein pressures was also increased. Splenic blood flows were increased in both hypertensive and normotensive patients. There was no correlation between the splenic blood flow and the portal pressure or the presence of portal fibrosis. The portal hypertension associated with vinyl chloride exposure is mainly presinusoidal in type, and may be attributed to an abnormality of the portal vein radicles, or hepatic sinusoids.
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PMID:Portal hypertension in vinyl chloride monomer workers. A hemodynamic study. 66 6

Four patients with malignant histiocytosis are described. Major clinical signs were fever, lymphadenopathy, hepatomegaly and splenomegaly. Laboratory studies showed leukopenia, thrombocytopenia, low leukocyte alkaline phosphatase (LAP) score, normal erythrocyte sedimentation rate (ESR) and elevated serum triglycerides. There was evidence of bone marrow involvement in all four cases. Typical changes were seen in the histological study.
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PMID:Malignant histiocytosis. A clinical and morphological study of four cases. 67 3

We studied the clinical and laboratory characteristics of 71 patients with hairy cell leukemia to ascertain whether prognostic factors important for the therapy of the disease existed. Granulocytopenia of less than 500/mu 1 was associated with the occurrence of a serious infection in 46% of the patients, compared to 19% of patients whose absolute granulocyte count was greater than 500/mu 1 (P less than 0.05). In most patients with symptoms of thrombocytopenia or leukopenia, splenectomy led to improved blood counts. Patients treated with splenectomy only had a significantly longer survival than those treated with steroids only (P less than 0.05). A group of patients who were slightly older and had minimal splenomegaly and few hairy cells in the peripheral blood did well without therapeutic intervention. Twenty-four patients have died, 16 of an infectious process. The actuarial survival for the group is 51% at 4 years, and there has been no change subsequently.
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PMID:Hairy cell leukemia: a clinical review based on 71 cases. 71 40

Between 1960 and 1977, 50 patients with agnogenic myeloid metaplasia were splenectomized. Twenty-five of 26 patients with painful splenomegaly, 4 of 9 patients with refractory hemolytic anemia, 4 of 10 patients with refractory thrombocytopenia, and 4 of 4 patients with portal hypertension showed significant benefit from the procedure. There were five immediate postoperative deaths. Four of these deaths occurred early in our series of splectomies for myeloid metaplasia before 1970. Only one death has occurred in the last 21 patients operated on. Survival following splenectomy averaged 25.5 mo.
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PMID:Splenectomy in myeloid metaplasia. 76 Aug 65

Clinical data on 24 civilian patients hospitalized for malaria in The New York Hospital were analyzed. Of 16 patients infected with Plasmodium falciparum, 14 acquired the disease in West Africa. Only three of the 24 had taken recommended courses of prophylaxis. Diagnosis was invariably, and often dangerously, delayed because physicians often made diagnoses of viral syndromes or used antibiotics; only one patient had a blood smear taken by a personal physician. Although all patients had fever and chills, classic malarial fever was seen in only seven patients; nausea, vomiting and diarrhea were common. Hepatomegaly and splenomegaly occurred in about half the patients. Blood smears stained in routine fashion by Wright's stain were positive in 23 of 24 patients. A normal leukocyte count was present in 19 of the 24 patients and thrombocytopenia in 16 of 23. The most frequent complications were those of central nervous system involvement. Therapy consisted mainly of chloroquine phosphate but other drugs, including quinine, pyrimethamine, sulfonamides and primaquine, were used in special situations. Suggestions for prophylaxis, diagnosis and therapy were made.
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PMID:Malaria - the mime. Recent lessons from a group of civilian travellers. 78 38

A 69-year-old woman was referred to our department because of moderate anemia and thrombocytopenia. On admission the spleen was slightly enlarged. On the basis of histological examination of biopsy specimens from spinal processes the diagnosis of myelofibrosis was made. The subsequent clinical course was progressively downhill. Although splenomegaly was of only moderate degree, severe anemia and thrombocytopenia supervened. Platelet mean life span was dramatically shortened (1.8 hours) and platelet production rate considerably increased (about 18xnormal). Neither corticosteroid therapy nor splenectomy alleviated the thrombocytopenia. Extremely large platelets, with diameters of up to 10 mum, were seen in the peripheral blood. The mean platelet diameter and percentage of megathrombocytes reached peak values about 2 weeks after splenectomy. It is suggested that the immunologic background of the rapid thrombocytolysis is similar to that which governs platelet destruction in idiopathic thrombocytopenic purpura.
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PMID:Myelofibrosis and rapid thrombocytolysis. A case report. 84 65

An unusual family is described with a congenital bleeding disorder present in four males belonging to three generations. Of the three surviving affected males, all had splenomegaly and petechiae. The three had moderate thrombocytopenia (55-90 X 10(9)/liter) and markedly prolonged Ivy-template bleeding times (greater than 30 min). They were also noted to have reticulocytosis and, upon further investigation, imbalanced globin chain synthesis resembling that of beta-thalassemia minor. Studies on nine additional family members in four generations were normal except for slight elevations of reticulocyte counts in female members, one of whom had the abnormal globin chain synthesis ratio. In male members, the bleeding tendency and clinical signs always occurred in the presence of the globin chain synthesis defect and reticulocytosis. This previously undescribed condition was apparently transmitted as an X-linked disorder.
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PMID:X-linked syndrome of platelet dysfunction, thrombocytopenia, and imbalanced globin chain synthesis with hemolysis. 87 27

Thrombocytopenia is often one of the first signs of vinylchloride disease. Morphological investigations showed that it is caused by splenic alterations. Besides splenomegaly, there are significant structural changes in the red pulp cords affecting the microcirculation. These quantitative and qualitative alterations cause increased platelet pooling and and increased destruction of platelets, which result in a decrease in thrombocytes in the peripheral blood.
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PMID:[Aetiology of the thrombocytopenia in vinylchloride disease (author's transl)]. 91 12


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