Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Acute myelofibrosis is a rare but distinct accelerated variant of agnogenic myeloid metaplasia that is characterized by marked anemia, peripheral blood myeloblastosis and normoblastosis, a lack of teardrop poikilocytosis, and prominent myelofibrosis. There is usually no palpable hepatosplenomegaly or lymph node enlargement. The clinical course is remarkable short. We describe a 63-year-old man who presented with idiopathic acquired sideroblastic anemia and subsequently developed acute myelofibrosis. Intensive polychemotherapy with vincristine, cytosine arabinoside, and prednisone and a later trial of oxymetholone therapy were ineffective. He died 134 days after the diagnosis of acute myelofibrosis was established. The 11 previously reported cases of acute myelofibrosis are reviewed, and the relationships of acute myelofibrosis to other myeloproliferative disorders and to idiopathic acquired sideroblastic anemia are discussed.
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PMID:Idiopathic acquired sideroblastic anemia terminating in acute myelofibrosis: case report and review of leterature. 31 17

Acute myelosclerosis is an unusual myeloproliferative syndrome characterized by pancytopenia, absence of massive hepatosplenomegaly, and an absence of tear-drop poikilocytes. The marrow is usually fibrotic with atypical megakaryocytic and trilinear dyspoiesis. Median survival is approximately six months from onset of symptoms. The authors report a case of acute myelosclerosis that evolved in a patient two and one-half years after the onset of idiopathic acquired sideroblastic anemia. A review of the other previously reported case of this unusual transformation and comments on the pathogenesis of the transformation from idiopathic acquired sideroblastic anemia to acute myelofibrosis are included.
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PMID:Idiopathic acquired sideroblastic anemia terminating in acute myelosclerosis. 707 67

Acute myelofibrosis (AMF) was diagnosed in a 59-yr-old black male in September 1978, on the basis of pancytopenia, lack of hepatosplenomegaly, fibrosis of the marrow, and paucity of teardrop red blood cells in the peripheral blood. Since then the patient has demonstrated an unusually long survival of 36 mo with a changing cytogenetic course. His initial 46, XY normal karyotype changed in 20 mo to trisomy 8, followed 1 yr later by 1:4 translocation in peripheral blood. Simultaneously with these changes, the fibrosis in the bone marrow progressively decreased, ultimately terminating in chronic granulocytic leukemia-like presentation with reversal to 46, XY karyotype. Fibroblast culture failed to show any evidence of cytogenetic abnormalities. The disappearance of fibrosis confirmed by trichrome and reticulin stains and lack of cytogenetic abnormalities in fibroblasts confirms the secondary role of fibrosis.
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PMID:Karyotypic polymorphism in acute myelofibrosis. 711 54