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)

A clinicopathological study was performed on 90 patients (39 males - 51 females, age 68 years) with primary (idiopathic) myelofibrosis - osteomyelosclerosis (OMF) in order to correlate laboratory and histomorphological parameters with each other and to calculate factors of prognostic impact on survival. In addition to multiple interactions between various laboratory features, there was a significant correlation between degree of medullary fibrosis and osteosclerotic changes with sizes of spleen and liver, level of LDH and duration of relevant prediagnostic symptoms. In trephine biopsies of the bone marrow, reduction of hematopoietic tissue was assessed by evaluating the amount of fat cells plus the degree of osteosclerotic lesions. This histological parameter did not reveal significant relationships with hepatosplenomegaly, duration of relevant symptoms or length of disease, but was correlated with the clinical findings of bone marrow failure. On univariate analysis, several clinical (age greater than 45 years, presence of relevant prediagnostic symptoms, hemoglobin level less than 9 g/dl, counts of myelo- and normoblasts, thrombocyte count less than 100 and greater than 700 x 10(9)/l, spleen size and LDH level) and histological features (reduction of hematopoiesis, counts for megakaryocytes and lymphoid nodules) were found to exert a predictive value on prognosis. However, on multivariate regression analysis only age remained significant. This result apparently reflects the numerous interactions between the various clinical as well as histological variables tested.
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PMID:Primary myelofibrosis-osteomyelosclerosis (agnogenic myeloid metaplasia): correlation of clinical findings with bone marrow histopathology and prognosis. 275 Dec 68

A case of primary myelofibrosis complicated with pericardial effusion and proteinuria is described. A 66-year-old female was admitted to our hospital because of abdominal fullness and shortness of breath. On admission, hepatosplenomegaly and pericardial effusion were observed. Blood examination revealed leukoerythroblastic anemia and thrombocytosis with tear drop cells and giant platelets. Bone marrow aspiration was dry tap and its biopsy showed remarkable myelofibrosis. Urinalysis indicated severe proteinuria. Although neutrophilic alkaline phosphatase score was low, no signs of acute blastic crisis of chronic myelogenous leukemia was found. The diagnosis of an atypical type of primary myelofibrosis was obtained. Administration of MCNU was started in August 1987. Hepatosplenomegaly, pericardial effusion and proteinuria were gradually improved after the administration. The etiology of the pericardial effusion and proteinuria were not obvious, however, these facts suggest that these abnormal findings might be related to PMF itself and MCNU was effective to PNF.
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PMID:[The use of MCNU to a patient of primary myelofibrosis complicated with pericardial effusion and proteinuria]. 276 70

Nonspherocytic hemolytic anemia, characterized by marked reticulocytosis, hepatosplenomegaly, hemosiderosis of reticuloendothelial organs and bone marrow myelofibrosis, and osteosclerosis, was diagnosed in 5 related Poodles. The unremitting anemia was clinically evident by 1 year of age, and was fatal as early as 3 years of age. Despite intense diagnostic endeavors including RBC fragility studies, RBC enzyme assays, and hemoglobin electrophoresis, the cause of this nonspherocytic hemolytic anemia remains to be determined.
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PMID:Familial nonspherocytic hemolytic anemia in poodles. 396 71

Clinical and morphological findings are described in 6 patients with malignant (acute) myelosclerosis/fibrosis (MMS). Haematological data are characterized by severe anaemia and thrombocytopenia, frequently accompanied by a leucopenia with an increase in myeloblasts and promyelocytes in the peripheral blood count. There is an absence of, or a minimal hepatosplenomegaly and the survival times after onset of clinical symptoms to death range from 4-12 months. The histopathology of the bone marrow shows a conspicuous proliferation of blasts (myeloblasts, promyelocytes and megakaryoblasts) in a variable amount, besides a fibrosclerosis consisting of reticulin and collagen fibrils. A comparison of MMS with ordinary myelofibrosis/osteomyelosclerosis (MF/OMS) of a chronic course implicates two important facts: (a) evolution of fibrosclerosis takes a considerable period of time for manifestation, which ranges between 20-30 months; (b) the histopathology of MMS is identical with those features observable in the rare event of a terminal stage, i.e. blastic transformation of chronic MF/OMS. Consequently MMS should be designated as an accelerated variant of MF/OMS with a rather early occurrence of a blastic crisis. The insidious onset with the dominant clinical finding of anaemia is probably responsible for the relatively late appearance of symptoms, while the progressive course prevents an overt myeloid metaplasia with a massive hepatosplenomegaly.
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PMID:Malignant (acute) myelosclerosis--a clinical and pathological study in 6 patients. 661 83

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 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

The case presented here is an 18-month-old female infant with agnogenic myelofibrosis. Clinically, this case was characterized by pancytopenia, hepatosplenomegaly, and rapidly fatal course, without response to steroids or other chemotherapy. Bone marrow biopsy and postmortem examination, revealed myeloid metaplasia of the liver, spleen, and lymph nodes, and conspicuous proliferation of immature cells, presumably of reticulum cells or precursors of hematopoietic cell lines, associated with prominent proliferation of reticular fibrous tissue. These morphologic features corresponded with those of agnogenic myelofibrosis, classified as a form of myeloproliferative disorder. A review of 15 cases of myelofibrosis in children collected from the literature showed a mean age of 2 years and 9 months with most cases being less than 2 years of age. There was a distinct female preponderance, while male infants with myelofibrosis were associated with Down's syndrome or C monosomy. The mean survival was 6.7 months. Approximately 25 cases of myelofibrosis have been reported in the English literature, while only 5 cases have been described in Japan.
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PMID:Agnogenic myelofibrosis in children--a case report and review of the literature. 723 18

We report on 15 patients affected by myelofibrosis. The main clinical aspects were hepatosplenomegaly, anemia and leukocytosis. Immature cells and anisopoikilocytosis of the erythrocytes were frequently found in the peripheral blood. At bone biopsy, various aspects of panhyperplasia, increase in the reticular fibers, myeloid fibrosis and osteosclerosis were observed. Leukocyte alkaline phosphatase was frequently elevated. The clinical and laboratory course of the disease, causes of death and the possible etiopathogenetic aspects of this disease are considered and discussed.
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PMID:[Laboratory diagnosis of idiopathic myelofibrosis. Review of the literature and presentation of 15 cases]. 729 Apr 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

Histoplasma infections in Europe are rare, and acute disseminated histoplasmosis has only been observed in immunocompromised persons. We describe a case of acute disseminated histoplasmosis in a young, nonimmunocompromised European woman. The probable source of infection was Sri Lanka or the Maldives. At presentation she was severely ill with fever, lymphadenopathy, anemia, thrombocytopenia, hepatosplenomegaly, and polyserositis. Histologically, myelofibrosis and osteosclerosis were observed with extramedullary hematopoiesis. Histoplasma capsulatum yeasts were detected in bone marrow trephine biopsy by methenamine silver staining. Treatment with conventional and liposomal amphotericin B and subsequent itraconazole led to rapid and complete recovery.
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PMID:Disseminated histoplasmosis in a non-immunocompromised host. 789 16


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