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)

A case of a 37-year-old woman presenting with acute agnogenic myeloid metaplasia (AAMM) is described. The disease had a stormy course and was characterized by moderate splenomegaly, persistently depressed WBC counts, extramedullary hemopoiesis and presence of a high percentage of atypical myeloblasts in the peripheral smear. Platelets were persistently low, reticulocytes significantly below normal, notwithstanding anemia. Hot tended to fall progressively to intolerably low values in the absence of transfusion. The chromosomal mapping of peripheral blood revealed the presence of a trisomy of chromosome No. 8. This abnormality already demonstrated in two previous cases of acute myelofibrosis and the clinical course of the disease suggest that acute myelofibrosis and AAMM could be the same disease while chronic myelofibrosis should be considered a separate entity. Also, it is possible that AAMM with trisomy of chromosome No. 8 and stormy clinical course may be a different entity from the acute myeloproliferative disorders associated with other chromosomal abnormalities.
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PMID:Acute agnogenic myeloid metaplasia with chromosomal abnormalities. 11 13

Eleven cases of myeloproliferative disease occurred in a group of 24 beagle dogs placed in a 60Co gamma-ray field at about 13 months of age and irradiated at an exposure rate of 5 R/22-hour day for duratior of life. Of these 11 dogs, 5 (described in this paper) were diagnosed as having erythroleukemia. The bone marrow showed marked erythroblastic hyperplasia, with maturation arrest of the erythroid elements, and increased numbers of myeloblasts and promyelocytes. The terminal peripheral blood was characterized by marked anemia and thrombocytopenia, with circulating erythrocytic precursors and abnormal erythrocyte morphology. Splenomegaly and hepatomegaly occurred in 4 of the 5 animals. In the spleens and livers of all 5, there was extensive leukemic infiltration and proliferation. The extent of leukemic involvement in other tissues and organs varied in individual dogs.
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PMID:Radiation-induced erythroleukemia in the beagle dog. A hematologic summary of five cases. 26 46

Malignant myelosclerosis has been described as a rare form of acute myeloproliferative disorder characterised by minimal or absent splenomegaly, cytopenias, circulating myeloblasts and diffuse marrow fibrosis, but distinction between this entity and acute myeloblastic leukaemia with marrow fibrosis is difficult. This report describes a 50-year-old man who presented with features similar to those of malignant myelosclerosis but ther terminal stage was characterised by generalised lymphadenopathy, bone tenderness, marked leucocytosis and myeloblastaemia. Post mortem examination showed multiple chloromata consisting of myeloblasts. These findings suggest that malignant myelosclerosis may be but a variant of acute myeloblastic leukaemia and probably should be treated similarly. A possible association between diagnostic medical irradiation and this acute myeloproliferative disorder is discussed.
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PMID:Acute myeloblastic leukaemia with marrow fibrosis (malignant myelosclerosis): acute leukaemia or malignant myelosclerosis? 27 42

This report discusses the case of a 60-year-old man who presented in 1969 with thrombocytopenia and mild marrow hypoplasia. A diagnosis of paroxysmal nocturnal haemoglobinuria (PNH), was established. The subsequent course included episodes of overt intravascular haemolysis. Thrombocytopenia reverted on several occasions during Oxymetholone therapy. The terminal phase of the illness was marked by the development of a leukocytosis and densely hypercellular bone marrow with splenomegaly. The features were those of a myeloproliferative disorder, although frank leukaemia did not develop.
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PMID:A case of paroxysmal nocturnal haemoglobinuria terminating in a myeloproliferative syndrome. 28 62

Two children who presented initially with a lymphoid malignancy were noted to develop recurrences with myeloid features late in the course of their disease. In both cases, evidence of lymphoid differentiation was present in the myelogenous cells that were Ph1 chromosome negative. The first patient had acute myelogenous leukemia and developed a recurrence with morphologic features of acute myelogenous leukemia. Terminal transferase was present in the myelogenous blasts. The second patient initially had a diffuse lymphoblastic non-Hodgkin lymphoma. During the course of her illness she developed a myeloproliferative disorder characterized by basophilic meningitis, splenomegaly, and hypereosinophilia. Lymphocyte T-cell (E-rosette) markers were present on the eosinophils. These observations lend further support to the hypothesis of varying lymphoid and myeloid differentiation in certain cases of leukemia.
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PMID:Myelogenous leukemia evolving during the course of lymphoid malignancy in children. 29 24

Ten clinical observations concerning six families with familial myeloproliferative disorders are reported. Family no. 1 : two brothers, RES with myelosclerosis and ROS with chronic myeloid leukemia. Family no. 2 : PG atypical myeloproliferative syndrome and his brother polycythemia vera. Family no. 3 : DF myelosclerosis and her son (DR) polycythemia vera. Family no. 4 : DM, polycythemia vera, the mother and a sister with splenomegaly. The brother died with myelofibrosis. Family no. 5 : GA and ML, cousins with polycythemia vera. Family no. 6 : MB and ZG, a brother and sister with polycythemia vera. No consanguinity and no toxic, infections or malignant etiology were found in these families. The literature reviewed emphasises the rarity of the familial incidence of myeloproliferative disorders.
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PMID:[Familial myeloproliferative syndromes. Study of 6 families and review of literature]. 35 25

This study is based upon an analysis of the hematologic and pathologic material from seven patients with acute myelosclerosis, as well as a review of the literature of 49 cases reported under this designation, or one of its synonyms. Patients with this disease characteristically present with pancytopenia, minimal or absent anisocytosis and poikilocytosis, and a fibrotic bone marrow showing hyperplasia and immaturity of all three cell lines, with particular prominence of megakaryocytes and their precursors. In addition, clinical splenomegaly is almost always absent, and the disease has a rapidly fatal course. We consider only one-fourth of the cases reported in the literature to have the clinical and hematologic features consistent with the diagnosis of acute myelosclerosis; the remainder represent a variety of myeloproliferative disorders, including chronic myelosclerosis with an accelerated terminal phase, acute myeloblastic leukemia with bone marrow fibrosis, myeloproliferative diseases that cannot be subclassified, and cases in which the data are insufficient for analysis. Using strict clinical and hematological criteria, acute myelosclerosis can be separated from other myeloproliferative disorders as a distinct clinicopathologic entity.
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PMID:Acute ("malignant") myelosclerosis. 36 69

The aim of the present work was to investigate the effect of selective beta-I-blockade on the exchangeable splenic platelet pool (ESPP). Therefore, 50 mg of metoprolol (a selective beta-I-receptor blocking agent) was given by mouth to three groups of subjects: (1) 15 healthy volunteers, (2) five asplenic subjects, and (3) 10 patients with different myeloproliferative disorders (MPD) with and without splenomegaly. The platelet count was determined 90, 150, 210, 270 and 330 min after the ingestion of the drug. In the group of healthy controls a significant increase (P less than 0.005) in the platelet count occurred and amounted to about 10% over baseline. The average peak value was reached at 150 min and the elevation in platelet count was maintained more than 4 h. An increase in the platelet count was also induced in patients with MPD, but the mean for the peak value only reached borderline significance (0.10 greater than P greater than 0.05). In the group of asplenic subjects no change in the platelet count occurred. It is concluded that acute oral administration of metoprolol causes a significant release of platelets from the ESPP. This platelet release is, however, no enhanced if the ESPP is enlarged.
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PMID:The exchangeable splenic platelet pool in response to selective adrenergic beta-i-receptor blockade. 60 60

A woman aged 67 who developed polycythaemia vera 21 years ago has been seen regularly since the diagnosis was first made. She was treated initially with pyrimethamine and then for 10 years by repeated venesections. After a 4 year period when her myeloproliferative disease was considered to be transitional a complete transformation to myelofibrosis occurred. She was treated by splenic irradiation and later with alkylating agents in an attempt to give her symptomatic relief from massive splenomegaly. Repeated and massive haemorrhage from gastro-oesophageal varices was treated initially by percutaneous transhepatic sclerosis of the gastro-oesophageal collateral vessels. A subsequent dacron mesocaval jump graft operation was successful in preventing further haematemesis and melaena. During the last year transformation back to polycythaemia vera has occurred. The evolution of her myeloproliferative disease has been fully documented by detailed blood counting, bone marrow aspirates and trephine biopsies, blood volume studies and other radioisotope investigations including the quantitation of functional erythropoietic tissue with 52Fe.
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PMID:Polycythaemia vera-transformation to myelofibrosis and subsequent reversal. 62 32

Epidural myeloblastoma, which compressed the spinal cord, was the first evidence for chronic granulocytic leukemia, eosinophilic type, Ph chromosome negative. This manifestation was preceded by 3 years follow-up of a patient with persistent eosinophilia of 60% mature eosinophils. The only clues for the diagnosis of leukemia were splenomegaly and high serum vitamin B12, most of which was bound to transcobalamin I. The latter finding presents a useful diagnostic criterium in myeloproliferative disorders.
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PMID:Chronic eosinophilic leukemia complicated by epidural myeloblastoma. 80 41


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