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)

From 1969--1975 patients with predominantly megakaryocytic myelosis were observed. Diagnosis was made up after histologic examination of iliac crest bone biopsy specimens embedded in methacrylate. From 6 patients rebiopsies and from further eleven ones the removed spleens were available. Autopsy was performed in 12 cases. In our material predominantly megakaryocytic myelosis was especially observed in male patients of advanced ages. The course of the disease was rarely acute, most often chronic and associated with the development of an extreme splenomegaly. Transition into osteomyelofibrosis or osteomyelosclerosis was frequent, the latter condition tended to develop a so-called myelosarcoma. The cause of death often was a generalized hemorrhagic diathesis with gastrointestinal hemorrhage or an fatal infection.
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PMID:[Pathologc findings in megakaryocytic myelosis (author's transl)]. 89 19

A 29-year-old male was diagnosed as having essential thrombocythemia (ET) in 1975. From that time, his platelet count gradually increased to more than 2 X 10(6)/microliter until 1979. However, his platelet count gradually decreased to less than 6 X 10(5)/microliter in 1985. Also, in 1982, erythroblasts and immature myeloid cells began to appear in the peripheral blood, and the liver and spleen became palpable in 1985. Bone marrow then revealed osteomyelosclerosis. These findings suggested that ET had transformed to myelofibrosis with myeloid metaplasia. Increased hepatosplenomegaly was accompanied by the appearance of ascites in June, 1988, and an esophageal varix ruptured in December of the same year. The varix was resected and the spleen was removed. After the operation, ascites did not recur and his condition became stable. Portal hypertension in this patient was considered to be due mainly to increased blood flow from the enlarged spleen.
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PMID:[Essential thrombocythemia transformed to myelofibrosis with myeloid metaplasia after seven years]. 204 Nov 65

A hemorrhage from esophageal varices is a rare complication of osteomyelosclerosis. Treatment and pathogenesis of this life-threatening complication is controversial. Following splenectomy in two patients, esophageal varices and ascites disappeared. The theory of an increased portal blood flow secondary to splenomegaly is supported with these two cases.
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PMID:[Hemorrhaging of esophageal varices associated with osteomyelosclerosis]. 696 5

Idiopathic myelofibrosis (IMF) is characterized by excessive accumulation of connective tissue in the bone marrow as part of a clinical syndrome which in its classical form is featured by leukoerythroblastic anemia and huge splenomegaly at the time of diagnosis. An acute variant of the disease exists being featured by pancytopenia, nor or minimal splenomegaly and a rapidly fatal clinical course. This review describes the relationship of IMF to other chronic myeloproliferative disorders and highlights current concepts of the pathogenesis of bone marrow fibrosis, implicating the intramedullary release of various growth factors, including platelet-derived growth factor beta. In a subgroup of patients bone marrow fibrosis may develop consequent to autoimmune bone marrow damage. The clinical and laboratory findings in some of the larger series of patients are presented and the reasons for the highly variable clinical presentation and prognosis are critically discussed. It is proposed that studies on prognosis in IMF are based upon simple prognostic staging systems, which should include the Hb-concentration, platelet count, spleen size and the presence/absence of osteomyelosclerosis on X-ray. Using these parameters the patients are easily categorized into three prognostic groups with highly different survival times.
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PMID:Idiopathic myelofibrosis--an update with particular reference to clinical aspects and prognosis. 840 Mar 33

The chronic myeloproliferative syndromes are bone marrow stem cell disorders. An increase of cell counts of one or rather all three blood cell types is characteristic for these disorders. The most important diseases in this group are: chronic myelogenous/granulocytic leukemia, polycythemia rubra vera, osteomyelosclerosis or agnogenic myeloid metaplasia and essential thrombocythemia. The cells are normally differentiating in these diseases, while the control of cell dividing is abnormal and therefore the cells are produced and accumulated in excess. Splenomegaly is a common and characteristic clinical finding. The fibrotic or sclerotic transformation of the bone marrow can take place in all forms of the syndrome. Extramedullary haematopoiesis can occur in all of the above diseases, but it is most common in myelofibrosis/agnogenic myeloid metaplasia. In the last phase of the disease a terminal blastic crisis may terminate the course of chronic myeloproliferative diseases. The myeloproliferative disorders can be transformed in each other--the most common transformation is that of the polycythemia rubra vera into myelofibrosis. The greatest progress in the therapy of chronic myeloproliferative diseases is achieved in chronic myelogenous leukemia: bone marrow transplantation and interferon treatment (the latter also in essential thrombocythemia and polycythemia rubra vera) are routine modalities worldwide. A new drug, anagrelide is effective in the treatment of myeloproliferative thrombocytosis and thrombocythemia.
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PMID:[Chronic myeloproliferative diseases]. 971 44

Using a new adenoviral vector (Ad) construct, we expressed human thrombopoietin (TPO) cDNA (AdTPO) in mice with various inherited immune deficiency syndromes such as nude, SCID and NOD-SCID mice. Immune normal Balb/c mice and a vector construct without TPOcDNA (AdNull), respectively, were used for controls. All animals (3 per group) were treated with a single application of 10(9) PFU (plaque forming unit) of Ad (AdTPO or AdNull) intraperitoneally on day 0. Four to 5 weeks following AdTPO administration, SCID and NOD-SCID mice demonstrated peak concentration of PLT of 12- to 14-fold normal value simultaneously with maximum concentration of PMNs (10- to 12-fold normal value). Later on these animals had a chronic thrombocytosis. In contrast, Balb/c mice and nude mice experienced PLT peak concentration of 4- to 6-fold normal value without granulocytosis 1 to 2 weeks following AdTPO treatment. Only nude mice had chronically elevated PLTs. In contrast, Balb/c mice developed thrombocytopenia due to cross-reacting anti-TPO antibodies. Animals with chronic thrombocytosis revealed increased content of CFU-G/GM, CFU-GEMM and CFU-Meg in bone marrow compared with controls. In contrast, Balb/c mice showed decreased content of CFUs if anti-TPO-antibodies were present. Histologically, only SCID mice developed severe osteomyelofibrosis and osteomyelosclerosis, hepato-splenomegaly, extramedullary hematopoiesis in liver and lung and ultimately suffered of progressive pancytopenia, anisocytosis, fragmentocytosis and a lethal wasting syndrome. In contrast, NOD-SCID mice which demonstrated similar extent of TPO overexpression and in addition to the B- and T-cellular immune deficiency harbour defective monocytes and macrophages, did not develop fibrotic changes of the bone marrow. From these results, we conclude (1) chronic TPO overexpression in vivo may lead to thrombocytosis and granulocytosis with expansion of CFU-GM, -GEMM and -Meg; (2) in vivo expression of adenovirally mediated TPOcDNA depends on immune competency of the host; (3) functionally normal monocytes and macrophages are indispensable for development of secondary osteomyelofibrosis and (4) adenovirally mediated expression of xenogeneic transgenes may brake immune tolerance for the respective self protein leading to autoimmune phenomena. Our in vivo model might provide further insights into the pathophysiology of secondary osteomyelofibrosis and may prove useful in designing new strategies for immune therapies of cancer.
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PMID:[Adenovirus long-term expression of thrombopoietin in vivo: a new model for myeloproliferative syndrome and osteomyelofibrosis]. 982 87

A series of 24 consecutive patients affected by myelofibrosis with myeloid metaplasia was reviewed. The clinical-pathological features at onset were similar to those reported in literature; in particular in all the patients we observed splenomegaly and the typical leuko-erythroblastic picture in peripheral blood. The median survival of our series was of 57 months; the deaths were caused by severe anaemia and/or infection and/or haemorrhage; the blastic terminal transformations were rare. According to other authors, unfavourable prognostic factors in our patients were: male sex, advanced age, hepatomegaly, the presence of systemic signs, anaemia, leukocytosis, leukopenia, high number of circulating erythroblasts, thrombocytopenia, osteomyelosclerosis. We have confirmed the clinical value of three staging prognostic systems: the system proposed by Njoku based on haemoglobin level and reticulocytes number, the system proposed by Visani based on haemoglobin and circulating myeloid precursors number, the system proposed by Dupriez based on haemoglobin and white blood cells number.
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PMID:[Myelofibrosis with myeloid metaplasia. A study of 24 consecutive patients]. 1530 45

Idiopathic myelofibrosis is a chronic myeloproliferative disorder characterized by excessive connective tissue deposition in the bone marrow. It presents with leucoerythroblastic anemia and massive splenomegaly. It is termed osteomyelosclerosis in the presence of primitive bone formation in the bone marrow and radiological presence of osteosclerosis. A 40-year-old male, known case of thalassemia trait presented with fatigue and lump in the abdomen for two months. Physical examination showed splenomegaly 8.5 cm below costal margin. X-ray examination revealed multiple osteosclerotic lesions involving the pelvis and long bones. Hemogram showed: hemoglobin 7.8 gm/dl, TLC 47,500/mm(3); DLC was polymorphs 49%, lymphocytes 7%, eosinophils 4%, basophils 4%, blasts 5%, promyelocytes 2%, myelocytes 14%, metamyelocytes 10%. Platelet count was 60,000/mm(3). Peripheral blood film showed leucoerythroblastic blood picture with features of dysmyelopoiesis. Bone marrow aspiration was diluted with peripheral blood. Bone marrow biopsy showed replacement of marrow by grade III reticulin fibrosis. Bony trabaculae were wide and thick. Platelet function studies were abnormal. The clinical, radiological and hematological features suggested a diagnosis of osteomyelosclerosis. We present this case because no similar association of osteomyelosclerosis with thalassemia trait has been described in English literature to date. This is the first study from India, which describes platelet function tests in a patient with osteomyelosclerosis.
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PMID:Osteomyelosclerosis with thalassemia trait: report of a rare association, study of platelet function tests and review of literature. 1861 73