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)

Thirty-five patients who fulfilled the FAB diagnosis criteria of chronic myelomonocytic leukemia (CMML), i.e., myelodysplastic features, monocytosis over 10(9)/liter, bone marrow monocyte infiltration, blast cells less than 5% in the peripheral blood and less than 30% in the bone marrow, are analyzed. CMML appears as an entity distinct from myelodysplastic and myeloproliferative disorders. Splenomegaly, anemia, thrombocytopenia, leukocytosis with monocytes and granulocytic cells in all stages of development, increased blood and urine lysozyme levels without renal failure, and polyclonal hyperimmunoglobulinemia are its main clinical and biologic features. With conventional cytotoxic drugs (6-mercaptopurine, hydroxyurea), the prognosis of CMML appears poor (median survival 475 days). None of the clinical hematologic or biologic parameters tested had a significant effect on prognosis. As other chemotherapy trials seemed necessary, we recently administered small doses of cytosine-arabinoside (ARA-C) to six patients over several consecutive days and obtained a complete remission in four. These preliminary results must be confirmed by larger series using the diagnostic criteria proposed by the FAB cooperative group.
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PMID:Chronic myelomonocytic leukemia according to FAB classification: analysis of 35 cases. 658 39

The clinical, radiologic, ultrastructural, and histopathologic findings in 14 patients with systemic mastocytosis were evaluated. Seven patients had evidence of urticaria pigmentosa (UP) and seven patients presented with no recognizable cutaneous lesions. There were no major clinical differences between patients with or without UP except for splenomegaly, which was present in one/seven patients with UP and five/seven patients without UP and the median age, 44 in patients with UP, and 75 in patients without UP. Bone marrow involvement was present in 13/13 specimens studied. Involvement was both focal and diffuse. The focal involvement occurred frequently in a perivascular and paratrabecular location. The diffuse involvement resembled myelofibrosis. Involved lymph nodes exhibited prominent sinusoidal and paracortical infiltration by mast cells. Splenic involvement was characterized by fibrosis occurring both focally and diffusely. The focal splenic involvement was perivascular and involved both the red and white pulp in a nonpreferential manner. Liver specimens showed prominent portal fibrosis. The morphology of the mast cells in the different lesions varied considerably; some were typical, others were spindle-shaped, and some resembled histocytes. The mast cells reacted positively with toluidine blue and chloroacetate esterase. Six patients had radiologic changes: three were osteoblastic, two osteolytic, and one osteoblastic and osteolytic. Two patients developed a poorly differentiated lymphoreticular tumor and one a myeloproliferative disorder after the diagnosis of mastocytosis.
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PMID:Systemic mastocytosis. Extracutaneous manifestations. 661 8

The authors report 15 cases of nodular regenerative hyperplasia (NRH) of the liver observed in 10 women and 5 men during a 9 year period. Gastrointestinal bleeding due to ruptured esophageal varices revealed the liver disease in 11 cases. Hepatomegaly and splenomegaly were noted in 9 cases and ascites in 7. Anicteric cholestasis was demonstrated in 10 cases. Another disease, e. g. myelofibrosis and monoclonal gammapathy, was present in 11 patients. In 10 patients, portal diversion was performed; outcome being favorable with a follow-up of one to six years. The analysis of these cases and of the 113 previously published reports calls for the following comments: 1) In most cases, NRH is characterized by small-sized hepatocytic nodules scattered throughout the entire liver with no surrounding fibrosis; however this histological pattern may vary somewhat, with adjacent normal zones being found adjacent to typical cirrhotic fibrosis; although a precise morphometric study was not performed in our patients, obstruction of the tiny branches of intrahepatic portal veins was not observed. 2) Histological diagnosis of NRH is difficult and in most cases requires surgical biopsy specimens and specific coloration of the reticulin network. 3) NRH must be considered as a new cause of intrahepatic (sinusoidal or presinusoidal) portal hypertension and/or of chronic anicteric cholestasis. 4) A number of various conditions may be associated with NRH, the most frequent being Felty's syndrome and myeloproliferative disorders. 5) The pathogenesis of NRH remains unknown. 6) Portal diversion generally has a favorable outcome in this disease.
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PMID:[Nodular regenerative hyperplasia of the liver. Study of 15 cases and review of the literature]. 662 10

Primary thrombocythemia is a myeloproliferative disease characterized by a sustained and marked increase in platelet count. The platelet number rises in excess of 1000 X 10(9)/l. Adult patients present with recurrent hemorrhages, thrombotic episodes and microvascular disturbances. It is a relatively benign disease and rarely reported in children. We describe two patients with a thrombocythaemia. The first, a 13 1/2 year old boy, had no complaints. A thrombocythemia (2167 X 10(9)/l) and splenomegaly were found by chance. He was treated with melfalan. Acetylsalicilic acid and dipyridamol were added as anti-coagulants. The second patient, a 12 year old girl, was submitted to a splenectomy at the age of 10, because of a traumatic rupture. Sixteen months later she was admitted to our hospital with symptoms of thrombotic as well as bleeding tendency. Periferal microvascular disturbances and symptoms on the basis of impaired cerebro-vascular bloodflow were found. The number of platelets was 5083 X 10(9)/l. She was treated in the same way as the first patient. Symptoms disappeared as soon as the number of platelets decreased. In both patients periferal blood and bonemarrow examination as well as platelet function, measured by aggregation studies, were abnormal. Consideration of the literature on children and our own case I indicate that this disease in the younger patient may exist without undue risk of complications. One might consider treatment with anti-aggregating agents only.
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PMID:[Primary thrombocythemia in childhood]. 668 55

Ingestion rate of granulocytes in osteomyelofibrosis with splenomegaly, which is still a matter of controversy, was measured in 32 patients. The mean ingestion rate in patients' granulocytes was similar to that of the controls; the results, however, were more dispersed in the patients than in the controls, with very high (three patients) and very low (three patients) ingestion rates. Ingestion alterations were serum-independent. Neutrophil glycolytic enzymes and adenylate-kinase were measured in order to assess: (1) if they could be responsible for the observed abnormalities and (2) if enzyme abnormalities, previously described in red blood cells, also occur in the neutrophils. Major increases in phosphofructoaldolase and in 3-phosphoglycerate kinase activities, contrasting with a decrease in pyruvate kinase activity were observed. These, however, did not correlate with ingestion alterations. In conclusion, we showed that the granulocyte ingestion rate is altered in a few patients only, that the alterations are unrelated to the serum, to adenylate kinase or to glycolytic enzyme abnormalities. The latter, however, are important. The mechanisms of their occurrence are unknown and hypotheses such as those proposed for red blood cells enzyme modifications in myeloproliferative disorders could be applicable.
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PMID:Ingestion rate and glycolytic enzymes in neutrophils of patients with agnogenic osteomyelofibrosis and splenomegaly. 671 65

A total of 134 cases of erythroleukemia (119 from the literature and 15 of the authors' patients) were reviewed in an attempt to correlate survival with age, sex, hepatomegaly, splenomegaly, lymphadenopathy, infection, or hemorrhagic complications at initial presentation; hemoglobin (Hgb), white blood cell (WBC) count, and platelet count; percent myeloblasts in the marrow at diagnosis; and treatment regimens employed. Statistical methods included single classification analysis of variance, nonparametric analysis of variance (Kruskal-Wallis method), contingency table analysis, and correlation coefficient determination for numerical data. No significant correlation between survival and age, sex, hepatomegaly, lymphadenopathy, infection, or hemorrhagic phenomena was found. Improved survival was noted in patients without splenomegaly, and in daunorubicin-treated cases in contrast to those treated with other chemotherapeutic agents. A positive correlation between survival and initial Hgb and WBC was also noted. Erythroleukemia complicating a chronic myeloproliferative disorder had a worse prognosis than de novo erythroleukemia.
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PMID:Prognostic factors and treatment effects on survival in erythroleukemia: a retrospective study of 134 cases. 694 45

Between 1976 and 1979 a myeloproliferative disease associated with cells monosomic for chromosome number 7 in the bone marrow was seen in six boys aged 5 1/2 months to 8 years (median 10 months). Presenting features included hepatosplenomegaly (5/6), respiratory infections (4/6), pallor (2/6) and skin infections (1/6). Haematological features included a leucoerythroblastic anaemia with leucocytosis and thrombocytopenia, and a hyperplastic marrow with a slight excess of blasts. Fetal haemoglobin was normal in four patients and mildly raised in the other two. Neutrophil function tests showed defective chemotaxis with reduced killing, despite a normal NBT test. Cytogenetic analysis of the marrow showed a preponderance of cells with monosomy 7; the blood lymphocytes were cytogenetically normal. In three patients the disease progressed to acute myeloid leukaemia (AML) after 3 weeks to 23 months; the only patient who remitted did so in response to 6-mercaptopurine and prednisolone, but relapsed 16 months later. A fourth child developed massive splenomegaly which initially responded to 6-mercaptopurine and prednisolone, but progressed to myelofibrosis 11 months later. A fifth child died from anaemia and respiratory infection without progression to leukaemia and the sixth patient has not yet developed leukaemia. Monosomy 7 is the diagnostic criterion of one of the more common myeloproliferative states in childhood and carries a high risk of progression to AML. The acute phase is usually resistant to chemotherapy, but even in responsive cases treatment does not result in elimination of the abnormal clone. Allogeneic bone marrow transplantation should be considered in cases with a suitable donor.
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PMID:Monosomy 7 in childhood: a myeloproliferative disorder. 694 67

Myelomonocytic myeloproliferative disease in a horse was diagnosed on the basis of hematologic, enzymatic, and histopathologic findings. It was characterized clinically by depression, weight loss splenomegaly, lymphadenopathy, coagulopathy, and bacteremia. Hematologic findings included severe refractory anemia, thrombocytopenia, monocytosis, and pleomorphic leukocytes, with a left shift of the myeloid series. The serum lysozyme concentration was 14.5 microgram/ml (normal, less than 5 microgram/ml). The bone marrow contained many immature cells of the myeloid series and had a myeloid-to-erythroid ratio of 30.5 to 1. The horse died after brief hospitalization. Necropsy revealed generalized lymphadenopathy and hemorrhages throughout the body. Histopathologically, primitive cells were seen in several tissues. Cells that proliferated in the bone marrow were primarily myeloblastic, with some additional erythropoietic cells. Myeloblastic cells with evidence of normal erythropoiesis were seen in numerous lymph nodes and in the spleen, whereas primarily normal erythropoietic cells proliferated in the adrenal glands. Myeloid blast-type cells predominated in the lungs, myocardium, liver, and kidneys.
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PMID:Myelomonocytic myeloproliferative diseases in a horse. 705 85

Only a fraction of the patients with myeloid metaplasia have indications for splenectomy develop. Palliation for symptomatic splenomegaly is highly successful. Significant relief from hypersplenism, anemia or thrombocytopenia was achieved, however, in less than half of the patients. Since patients with severe thrombocytopenia are at continuing risk of spontaneous hemorrhage and of bleeding after minor trauma, splenectomy appears to be justified in these patients, provided that studies of peripheral blood and bone marrow have excluded those with leukemic conversion. The benefit obtained from splenectomy in patients with chronic anemia requiring frequent transfusions in less certain, particularly in men and in patients converting to myeloid metaplasia from polycythemia vera. In some instances, the continuation of transfusions may result in less morbidity and better palliation than operation. Although the course of this myeloproliferative disorder from first symptoms to death frequently extends for more than a decade, the manifestations which may provide an indication for splenectomy usually occur late in the course of the disease. The element as well as the morbidity of the operation makes a thorough evaluation of each patients essential before splenectomy is considered.
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PMID:Splenectomy for agnogenic myeloid metaplasia. 706 91

Increased numbers of bone marrow mast cells were found in 45 (2.2%) of 2,000 bone marrow specimens obtained from patients who had hematologic disorders. Mast cells were most frequently seen in the marrows of patients who had preleukemic syndromes, lymphoproliferative disorders, and acute leukemia. The 16 patients who had preleukemic syndromes included those with refractory sideroblastic and megaloblastic anemia (with or without an excess of blasts), idiopathic pancytopenia or pure erythrocytic aplasia, paroxysmal nocturnal hemoglobinuria, idiopathic refractory neutropenia, agranulocytosis or thrombocytopenia, and persistent eosinophilia. Five of the seven patients who had acute leukemia had nonlymphoblastic leukemia; two had blastic crisis of chronic granulocytic leukemia. Of the 13 patients who had lymphoproliferative disorders, eight had chronic lymphocytic leukemia, three had macroglobulinemia, and two had non-Hodgkin's lymphoma. Three patients who had chronic renal failure associated with severe anemia and two who had chronic liver disease, splenomegaly, or hypersplenism were also encountered. In this study there appeared to be a consistent relationship between the presence of increased numbers of mast cells and the lymphocyte and plasma cell counts in the bone marrow. The significance of the presence of secondary mastocytosis in premalignant lesions, neoplasia, and, in particular, lympho- and myeloproliferative disorders, is still unclear.
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PMID:Increased bone marrow mast cells in preleukemic syndromes, acute leukemia, and lymphoproliferative disorders. 745 27


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