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)

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

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

Acute megakaryoblastic leukemia or acute "malignant" myelosclerosis is an acute and rapidly progressive myeloproliferative syndrome characterized by minimal or absent splenomegaly, pancytopenia, diffuse marrow fibrosis, and circulating blasts of megakaryocytic origin. The disease must be differentiated from other hematologic malignancies especially myelofibrosis with myeloid metaplasia. The radiographic changes of osteosclerosis in our patient have not been previously reported in the literature.
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PMID:Acute megakaryoblastic leukemia (acute "malignant" myelofibrosis): an unusual cause of osteosclerosis. 696 30

In 30 consecutive splenectomies for myelofibrosis (MF) with cytopenia(s) and/or massive splenomegaly the operative mortality was 6.7% (2 patients). Postoperative complications were observed in 11 (37%) patients. When surgery was undertaken within one year of the diagnosis of MF the postoperative morbidity rate was 13% (2/16), but significantly (p less than 0.01) higher at 64% (9/14) in patients operated on later. A similar significant difference was observed in the amount of intraoperative blood loss. Of the patients undergoing splenectomy for anaemia and/or thrombocytopenia, 79% gained definite benefit from the operation for 2 to 70 months (median 10 months). It is concluded that, although splenectomy probably does not prolong the life of patients with MF, it gives considerable symptomatic relief in cases with massive splenomegaly, and can improve the patient's haematological status and reduce blood transfusion requirements. To avoid the increased risks of postoperative complications surgery is best undertaken as soon as cytopenia is present.
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PMID:Splenectomy for myelofibrosis. 714 14

In a 54-year-old woman with oesophageal varices there were the unusual morphological findings of portal-vein aneurysm and irregular hepatic fibrosis. The patient had been treated for 8 years for polycythaemia vera with transition into myelofibrosis. Histologically the hepatic tissue showed occlusion of intrahepatic portal-vein branches by organised thrombi, as well as angiomatous vessels in the sclerosed portal areas. the increased pressure as well as medial atrophy with portal-vein sclerosis were the cause of the portal-vein aneurysm. Portal hypertension may have been the result of intrahepatic vascular obstruction and of the increased flow through the enlarged spleen. It is suggested that interaction of several factors, related to the basic disease of myelofibrosis led to these complex anomalies of the liver and portal vascular system.
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PMID:[Portal vein aneurysm and liver fibrosis in myelofibrosis]. 717 59

In a retrospective study of 39 splenectomies, patients with increased blood cell breakdown (13 cases of idiopathic thrombocytopenic purpura (ITP), 5 cases of hereditary spherocytosis, 2 of Felty's syndrome and 2 of autoimmune hemolytic anemia) were compared with those patients also presenting decreased blood cell production [14 cases of myelofibrosis (MF) with splenomegaly and 3 cases of advanced chronic myelogeneous leukemia (CML)]. Platelet regeneration post-operatively was significantly (p less than 0.01) more rapid in the ITP than in the MF group. Only 1/22 patients in the ITP group had major post-operative complications as compared to 10/17 in the MF group. None of the patients in the ITP group died within 25 days of operation as compared to 5/17 in the MF group. Only 3/22 patients in the ITP group lost more than 800 ml of blood during the operation as compared to 8/17 with MF. No statistically significant higher blood loss was found in patients with less than 30 x 10(9) platelets/l preoperatively, compared to those with higher platelet counts. However, correlation between the splenic weights and amount of blood loss was statistically significant (p less than 0.01). Thus, splenectomy seems much better tolerated in patients with ITP, even if platelets are low, than in patients with myelofibrosis.
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PMID:[Splenectomy in idiopathic thrombopenic purpura and in myelofibrosis. A retrospective study of platelet increase, hemorrhagic complications and mortality]. 719 36

221 patients were treated by splenectomy for various hematologic disorders, including immunologic thrombocytopenia (80 cases), hereditary spherocytosis (15 cases), immune hemolytic anemia (25 cases), chronic lymphatic leukemia (27 cases), lymphosarcoma (27 cases), myelofibrosis (11 cases) and pancytopenia/aplastic anemia (14 cases). There were 8 postoperative deaths (3.6%), and 49 patients (22%) had postoperative complications, mainly infections and bleeding. Complications were more frequent in cases of massive splenomegaly (greater than or equal to 1 000 g) (65 cases), severe thrombocytopenia (less than 20 X 10(6)/1) (51 cases), and leukemia. Late postsplenectomy fulminant infection was seen in 8 patients (3.6%) for a mean follow up of 5.3 years. We conclude that splenectomy is sufficiently well tolerated even by severely ill patients to make it applicable as a therapeutic modality in the various hematologic disorders presently studied.
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PMID:Complications after therapeutic splenectomy for hematologic disease in adults. 722 97

Idiopathic myelofibrosis (IM) is a myeloproliferative syndrome characterized by anemia, fibrosis of the bone marrow, variable proliferation of megakaryocytes, myelocytic and erythrocytic precursors, and splenomegaly. Extramedullary hematopoiesis can develop in diverse areas. Unlike in chronic granulocytic leukemia, transformation to acute leukemia is uncommon in IM. Transformation to acute leukemia is usually accompanied by the findings of progressive increase in blasts in peripheral blood or bone marrow [1]. We have recently treated a patient with an unusual transition from IM to acute leukemia in whom leukemic transformation began in the central nervous system.
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PMID:Extramedullary blast transformation in the central nervous system in idiopathic myelofibrosis. 731 44

A 58-year-old male was diagnosed as having paroxysmal nocturnal hemoglobinuria (PNH) with myelofibrosis in 1984. The administration of hydroxyurea and low dose splenic irradiation were initiated for abdominal distention due to splenomegaly in 1987. In May 1990 the patient developed smouldering acute myeloblastic leukemia (AML); and the blasts proliferated in response to G-CSF administered for refractory pneumonia. The patient died of pneumonia and pleural involvement of leukemia in September 1990. FACS analysis of the blasts using anti-decay accelerating factor (DAF) (CD55) and CD59 (membrane attack complex inhibition factor: MACIF) monoclonal antibodies demonstrated that 25.5% and/or 87.3% of the blasts were negative for DAF or CD59 respectively. There is the earlier evidence that about 90% leukemic myeloblasts from non-PNH AML patients are positive for DAF, and nearly 100% of non-PNH neutrophils have been shown to be positive for both DAF and CD59. Our data suggest that the leukemic blasts from this patient may have derived from the PNH clone.
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PMID:Paroxysmal nocturnal hemoglobinuria with myelofibrosis: progression to acute myeloblastic leukemia. 751 53

A patient with chronic myeloproliferative disorder (CMPD) developed Sweet's syndrome during granulocyte colony-stimulating factor (G-CSF) therapy. A 61-year-old man with essential thrombocythemia was treated with busulfan intermittently since April, 1991. In February, 1993, hepatosplenomegaly with leukoerythroblastosis arose and a diagnosis of myelofibrosis with extramedullary hematopoiesis in the spleen was established. For alleviation of left hypochondralgia due to splenomegaly, he received splenic irradiation in September, 1993. Soon after the irradiation, his peripheral blood revealed pancytopenia and then administration of rhG-CSF was begun on the 9th of October, 1993. One week after G-CSF therapy, he became feverish and painful eruptions on the face and the upper extremities appeared and enlarged. Skin biopsy resulted in a diagnosis of Sweet's syndrome. Treatment with oral prednisone, 30 mg daily, was begun, and rapid and significant improvement of the skin lesions was obtained. The pathogenesis of Sweet's syndrome remains obscure, but careful follow up is necessary for patients during G-CSF therapy with respect to development of Sweet's syndrome.
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PMID:[Sweet's syndrome in a patient with chronic myeloproliferative disorder during recombinant human granulocyte colony stimulating factor therapy]. 754 Feb 25


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