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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The study of the evolution of 168 cases of idiopathic myeloid splenomegaly allowed to point out the prognostic value of the clinical hematologic, isotopic and radiological parameters. The correlation of these data with the histological type of the osteomedullary lesions at the time of the diagnosis, allows to confirm the long survival of the hyperplastic formes (type I) and the more reserved prognosis in advanced myelofibrosis (type II and III together). Indeed, median survival is of 82 months for type I, and 60 months for type II and type III together. It seems advice to gather the two last types and so, to compare two anatomoclinical entities : one is characterized by hyperplastic marrow with reticulinic fibrosis, usually associated with enlarged spleen and sometimes even with increase red blood cells volume but without radiological bone lesions; the other one is characterized by a marrow of middle importance or marked marrow depletion, fibrosis, with or not osteosclerosis, and shows moderate or marked radiological lesions (respectively 16,9% of the cases) and, often, pancytopenia.
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PMID:Prognosis of myeloid metaplasia with myelofibrosis. 99 Mar 77

The study of the evolution of 168 cases of idiopathic myeloid splenomegaly allowed to point out the prognostic value of the clinical, hematologic, isotopic and radiological parameters. The correlation of these data with the histological type of the osteomedullary lesions at the time of the diagnosis, allows to confirm the long survival of the hyperplastic formes (type I), and the more reserved prognosis in advanced myelofibroses (type II and type III together) without it being possible to bring out a significant difference between the average presumption of survival of type II and type III considered each separately. Indeed, average survival is of 82 months for type I, and 60 months for type II and type III together. The regrouping of these two types seems to be justified and allows to individualize two anatomoclinical forms. One is characterized by hyperplastic marrow with reticulinic fibrosis, usually associated with enlarged spleen and sometimes even with polyglobulia but without radiological bone lesions. The other form is characterized by a marrow of middle importance or frankly hypoplastic, fibrous, with or without osteosclerosis, and shows moderate or marked radiological changes (respectively 16,9 p. 100 of the cases, and, as a rule, pancytopenia.
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PMID:[Prognostic factors in idiopathic myeloid splenomegaly]. 121 74

The clinical course of 103 patients (50 males, 53 females; median age 59 years) with idiopathic myelofibrosis (IMF) seen at our hospital between 1967 and 1986 was analyzed retrospectively. Common symptoms and signs at the time of diagnosis were: myelofibrosis (96%), splenomegaly (84%), anemia (81%), osteosclerosis (45%), malaise (41%) and leukocytosis (41%). It was possible to follow the majority of patients without treatment or with transfusion therapy only for prolonged periods of time. The use of cytostatic drugs and radiotherapy was restricted as much as possible. Probably due to this treatment strategy the incidence of acute leukemia was low (5%). Major thromboembolic complications were seen in 19% of the patients. Median survival of the patients was 4.3 years. The prognostic influence of several disease parameters determined at the time of diagnosis was tested: age, sex, leukocytes, platelets, hemoglobin, reticulocytes, LDH, ANP-score, spleen size and percentage of peripheral blood blasts + promyelocytes had no significant influence on the length of survival. Osteosclerosis, a presumed sign of advanced disease, was not correlated with survival either.
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PMID:Idiopathic myelofibrosis: a retrospective study of 103 patients. 222 19

Histomorphometry was performed on representative trephine biopsies of the bone marrow on admission of 50 patients (21 male, 29 female - age 67 years) with so-called primary osteomyelofibrosis/-sclerosis (OMF) not preceded by any other subtype of chronic myeloproliferative disorders. This study was firstly aimed at testing correlations between histological features (amount of haematopoiesis, cytological aspects of megakaryocytes, density of reticulin and collagen fibres and degree of osteosclerosis) and laboratory data, as well as spleen size and duration of relevant prediagnostic symptoms. Secondly, we concentrated on a discrimination of OMF patients into two subgroups according to bone marrow morphology and clinical variables. Statistical evaluation of histomorphometric variables and haematological findings disclosed that there was a progressive fibro-osteosclerotic process in the evolution of disease features. Increase in medullary fibrosis was significantly paralleled by an abnormal or pleomorphic megakaryopoiesis in the bone marrow: there was an increase in irregularity of perimeters for megakaryocytes and naked nuclei combined with smaller sizes of these elements including the nuclei. Additionally, there was a greater number of pycnotic bare nuclei. A number of morphometric features (density of fibres, degree of osteosclerosis, amount of haematopoiesis) were associated with corresponding clinical data (spleen size, length of preclinical history). By consideration of a set of basic histomorphometric variables our cohort of 50 patients could be divided into an early hyperplastic subtype with no or minimal medullary reticulin and another group with conspicuous fibrotic and osteosclerotic alterations of the bone marrow. It was noticeable that we found no significant correlation between amount of haematopoiesis or marrow cellularity with splenomegaly. This result suggests that splenic haematopoiesis (myeloid metaplasia) may represent an autonomous or neoplastic process and not only compensation for a failing fibro-osteosclerotic bone marrow.
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PMID:Histomorphometry of bone marrow biopsies in primary osteomyelofibrosis/-sclerosis (agnogenic myeloid metaplasia)--correlations between clinical and morphological features. 250 23

The clinical and hematological profile of 53 patients in whom primary myelofibrosis (PMF) had been diagnosed during the last 15 years was evaluated. Median age was 64 years (range 17-86). Thirty-five patients were males and 18 females. The most frequent symptoms were associated with the hypermetabolic state, anemia and splenomegaly. The latter was found in 96% of patients, while 83% had hepatomegaly and 9% had lymphadenopathy. Thirty-three patients had anemia at the time of diagnosis. The leukocyte and platelet counts were normal or moderately high in most cases. Myelemia was found in 83% of patients, with circulating erythroblasts in 72%. The most common biochemical abnormalities were the increased serum LDH (84%) and hypocholesterolemia (62%). Bone marrow aspirate was not analyzable in most cases. Bone marrow biopsy showed myelofibrosis in hypercellular phase in 22 patients, myelofibrosis without osteosclerosis in 17, and myelofibrosis with osteosclerosis in 14. The median survival of the series was 3.8 years; 34 patients had died at the time of the analysis. The major causes of death were infection, cardiovascular complications and hemorrhage. In 4 patients the evolution from PMF to acute leukemia was observed.
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PMID:[Primary myelofibrosis: description of a series of 53 patients]. 266 65

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

The effect of ES upon hematopoiesis was studied following 4 to 24 weeks of administration in adult female mice. ES produced osteosclerosis, hepatomegaly, splenomegaly with an increase in splenic erythropoiesis mild anemai, and a relatively stable, moderately severe neutropenia. Intact and splenectomized mice failed to develop hepatic hematopoiesis to compensate for these blood changes. The neutropenia was characterized by a proportionally normal-sized marginal granulocyte pool and a reduced marrow granulocyte reserve in the marrow, cellularity, peroxidase-positive cells. CFU-S, and CFU-GM declined during 4 to 12 weeks of study in the same study period, splenic granulocytopoiesis increased as measured by these perameters, but it only partially compensated for the neutropenia. CSA was present in serum, and no inhibitors of in vitro granulocytopoiesis were detected. The direct addition of E3S to normal murine marrow cells in vitro failed to inhibit CFU=GM proliferation. Daily ES administration failed to inhibit in vivo granulocytopoiesis in diffusion chambers. These studies suggest that ES-induced neutropenia is not due to direct inhibition of CFU-S or CFU-GM proliferation or differentiation to mature granulocytes and by implication, suggest that it may be mediated through effects on the hematopoietic microenvironment.
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PMID:Estrogens and hematopoiesis: characterization and studies on the mechanism of neutropenia. 696 70

Myelofibrosis is a myeloproliferative disorder that is characterized by splenomegaly and bone marrow replacement by fibrous tissue. The predominant radiographic feature is osteosclerosis; however, in rare instances, periosteal bone apposition or periostitis is apparent in the metaphysis of the distal femura and proximal tibiae. It has been suggested that periostitis, when associated with fever and bone pain, is indicative of more aggressive disease. We report this unusual radiographic finding and its similar appearance to hypertrophic osteoarthropathy in two patients with myelofibrosis. In our patients, the presence of periosteal bone apposition did not correlate with increased disease aggressiveness.
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PMID:Myelofibrosis associated with prominent periosteal bone apposition. Report of two cases. 803 11

A 74-year-old woman with severe, but asymptomatic weight loss was found to have splenomegaly, thrombocytosis, and neutrocytosis. A trephine biopsy showed a hypercellular bone marrow with a proliferation of abnormal megakaryocytes, mild reticulin fibrosis, and osteosclerosis, i.e., features of an early stage of idiopathic myelofibrosis. In contrast to this predominant pattern, the marrow also contained a distinct hypocellular focus of gelatinous transformation (GMT) in which hematopoietic and fat cells were replaced by gelatinous substances that were characterized as hyaluronic acid mucopolysaccharides histochemically. GMT is a rare disorder of unknown pathogenesis, and is an unspecific indicator of severe illness which most often occurs in patients with a background of weight loss. This is the first reported case of gelatinous changes in an otherwise fibrotic bone marrow, a constellation which a priori seemed to be incompatible.
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PMID:Gelatinous bone marrow transformation in a case of idiopathic myelofibrosis: a morphological paradox. 1118 74

Diagnosis of essential thrombocythemia (ET) has been usually established by regarding the criteria of the Polycythemia Vera Study Group. Accordingly, a retrospective clinicopathological study was performed on 120 patients with a follow-up ranging between 5 and 13 years and repeated bone marrow trephine examinations. Following the new WHO classification, at presentation patients revealed three distinctive patterns of bone marrow (BM) features: (true) ET in 43 patients, prefibrotic idiopathic myelofibrosis (IMF) in 50 patients, and early IMF in 27 patients. Heterogeneity of morphological features was associated with correspondingly expressed laboratory data. Contrasting initial and early IMF, patients with true ET displayed an about 80% probability to lack splenomegaly, anemia, and increase in the LDH and LAP values and also failed to show any myeloblasts or erythroblasts on the peripheral blood films. Follow-up examinations including sequential BM biopsies (mean interval 39 +/- 31 months) disclosed that of the 43 patients with true ET only one developed an increase in reticulin. On the other hand, 65 of 77 patients with prefibrotic and early IMF evolved into overt myelofibrosis-osteosclerosis. Moreover, survival analysis demonstrated significant differences in our patients. A neglectable proportion of life loss according to a sex- and age-matched general population was found in true ET (less than 11%) opposed to IMF without or mild fibrosis (range 21% to 32%).
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PMID:Follow-up examinations including sequential bone marrow biopsies in essential thrombocythemia (ET): a retrospective clinicopathological study of 120 patients. 1221 Aug 9


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