SEARCH · PubMed Health
Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
Using an enzyme-linked immunosorbent assay (ELISA), elevated RBC-Ig of the IgG and IgM class were found in 8 of 14 patients with idiopathic myelofibrosis. In 2 patients with high levels of RBC-Ig the direct Coombs' test was positive. It is supposed that immune haemolysis may contribute to the anaemia in some patients with idiopathic myelofibrosis.
Elevated levels of platelet-associated IgG and/or IgM were found in 15 of 18 patients with myelofibrosis (83%). All but 5 patients with elevated PAIg had active disease. The amounts of PAIg were not correlated to either S-Ig, platelet count or spleen size. Levels of PAIgG well above the normal range were especially found in patients with short duration of disease and/or a transitional myeloproliferative state. It is debated whether immune-mediated platelet dysfunction may be of importance for the development of bone marrow fibrosis, mediated by the release of platelet-derived growth factors in the bone marrow. Elevated PAIg may also contribute to abnormal haemostasis and thrombocytopenia in myelofibrosis.
19 patients with myelofibrosis, primary or following polycythaemia vera were studied cytogenetically. Bone marrow cells, unstimulated and stimulated cells from peripheral blood were investigated. 7 patients were found to have clonal aberrations, 3 of whom had a structural rearrangement of chromosome 13. In 2 additional cases single mitoses with 13q- were found. Reviewing the files on patients previously studied in our laboratory, 2 more patients with 13q- markers were noted. Both had had haematologic disorders in which fibrosis of the bone marrow can be found, but this feature could not be evaluated retrospectively, because no biopsies had been taken. Our data and those found in the literature suggest that rearrangements of 13q12----q22 are often associated with myelofibrosis, both in its primary form or following polycythaemia vera.
The circulating GM-CFC density-distribution profile was studied in 19 cases of myelofibrosis with myeloid metaplasia. The distribution profile for the population studied appeared to be similar to that of normal bone marrow GM-CFC's but shifted toward lower densities in comparison with normal circulating GM-CFC's. For individual patients, it appeared that the greater the circulating blood GM-CFC's concentration, the lower the mean density. It appears likely that the premature release into blood of the GM-CFC's in myelofibrosis is a property of the neoplastic haematopoietic stem cell clone at the core of the disease.
A 52-year-old male with idiopathic myelofibrosis of 8 years' duration developed pericardial tamponade during recovery from acute tubular interstitial nephropathia following septicaemia. Splenectomy had been performed 7 yr previously. The tamponade was relieved by pericardiocentesis and its recurrence was prevented by a minor pericardiectomy. Pathological examination, including staining for factor VIII-positive cells, demonstrated extramedullary haematopoiesis in the pericardium. In patients with myelofibrosis and increased silhouette on X-ray film, with or without clinical heart failure, echocardiographic examination is recommended in order to identify a possible pericardial effusion.
A patient with polycythaemia vera developed typical myelofibrosis after 15 yr. After a further 8 months, during which time she was pancytopenic and transfusion-dependent, a slow spontaneous recovery in haemopoiesis occurred and the full blood count became normal. 6 months later pancytopenia recurred and soon afterwards the patient developed acute myeloblastic leukaemia from which she died. The evolution of bone marrow morphology and isotopic studies. Only 2 previous reports of this kind of transformation exist in the literature, although restoration of normal or polycythaemic haemopoiesis has been reported in 8 patients with myelofibrosis. It is likely that these transformations occur because of alterations in stem cell behaviour rather than as a result of therapy.
A total of 59 symptomatic patients with myeloproliferative disorders (polycythaemia vera 24 pats., essential thrombocythaemia 25 pats., myelofibrosis 10 pats.) and elevated platelet counts were studied during 1653 months of continuous treatment with hydroxyurea. Reduction of the platelet level to less than 500 x 10(9)/l was achieved within 8 weeks in 86% of polycythaemia pats., 80% of thrombocythaemia pats., 60% of myelofibrosis pats. Control of disease-related symptoms was achieved within 1 year in 78% of the patients. There was no instance of severe bone marrow toxicity. Side-effects of hydroxyurea were modest. Survival was excellent, with 86% probability of survival after 5 yr of therapy. We consider hydroxyurea a first-choice alternative in the treatment of patients with polycythaemia vera, essential thrombocythaemia and mylofibrosis with thrombocytosis.
Bone marrow stroma was investigated immunohistochemically in 31 patients with haematological diseases, mainly idiopathic myelofibrosis (n = 8) and related chronic myeloproliferative disorders (n = 14). The bone marrow from patients with idiopathic myelofibrosis and some CML patients showed marked staining reactions with antibodies against type III procollagen (pN collagen), type IV collagen, fragment P1 of laminin and factor VIII. Patients with osteomyelosclerosis had particularly increased collagen content, including both newly deposited type III collagen (pN collagen) and mature collagen fibres. As in normal bone marrow, argyrophilic fibres and type III collagen displayed a close co-distribution, which was also demonstrated for type IV collagen and laminin. While normal bone marrow sinusoids had discontinuous basement membranes, fibrosing bone marrow was characterized by endothelial cell proliferation and capillarization, with the development of continuous sheets of basement membrane material beneath endothelial cells.
Bleeding owing to portal hypertensive colopathy, a form of large bowel angiodysplasia, as a cause of increased blood transfusion requirement is described in a 74 year old man with idiopathic myelofibrosis. The proposed mechanism and the potential therapeutic options for this rare complication of myelofibrosis are discussed.
A 28-year-old man with Philadelphia chromosome positive chronic granulocytic leukaemia developed extensive bone marrow and bone infarction which was associated with anaemia and thrombocytopenia. He survived 20 months from the first symptoms of bone marrow infarction; during this time he developed myelofibrosis and osteosclerosis followed by blastic transformation. Extensive bone marrow infarction is a possible pathogenetic mechanism when chronic granulocytic leukaemia is followed by myelofibrosis.
The iron kinetic model described by Ricketts et al was used to study haemopoiesis in chronic myelofibrosis. The clearance of 59Fe-labelled transferrin from the plasma was analysed to quantify total, effective, and ineffective erythropoiesis, denoted by the terms marrow iron turnover (MIT), red cell iron turnover (RCIT), and per cent ineffective iron turnover (IIT%), respectively, in 12 cases of this disease. The patterns obtained were variable: values for MIT ranged from 24.4 to 510 mumol/l blood/day; those for RCIT from 0.4 to 119 mumol/l blood/day; and those for IIT% from 67 to 98%. One noteworthy feature was the presence in two cases of functional erythroid hypoplasia; these were characterised by severely reduced values for MIT (24.4 and 28 mumol/l blood/day) and RCIT (0.4 and 8 mumol/l blood/day.) A systematic study of the erythrokinetic features of myelofibrosis may indicate that erythroid hypoplasia is a more common cause of anaemia in this disease than has been previously recognised.
A 59 year old man with longstanding myelofibrosis and previous splenectomy was incidently found to have a large lytic lesion in his left femur which required operative fixation. He had undergone right upper lobectomy for squamous carcinoma of the bronchus five years earlier. Histological analysis of bone reamings showed no evidence of metastatic carcinoma. Osteosclerosis is frequently noted in patients with myelofibrosis but osteolytic lesions are uncommon and may be confused with metastatic malignancy.
An unusual case of small cell variant of Ki-1 non-Hodgkin's lymphoma diagnosed one year after an original diagnosis of idiopathic myelofibrosis is reported. On the second occasion, the patient presented with fever, lymphadenopathy and hepatosplenomegaly. A lymph node biopsy specimen confirmed a diagnosis of small cell variant of Ki-1 lymphoma. A repeat bone marrow biopsy specimen showed myelofibrosis with no evidence of lymphomatous infiltration, but cytogenetic studies on blood, bone marrow and skin fibroblasts revealed a novel chromosomal translocation t(3,4)(q13;q12).
A 61 year old man presented with abdominal pain typical of chronic cholecystitis of one month's duration. Pallor was noted on examination and investigation uncovered myelofibrosis and a small gallstone. Cholecystectomy relieved the pain and pathological examination of the gall bladder showed widespread myeloid metaplasia. This is the first reported case of myelofibrosis presenting as chronic cholecystitis.
A case of myelofibrosis related to thorotrast administration is described. Current concepts of acute myelofibrosis are discussed, as are the varied complications of thorotrast.
A 50-year-old man with a 20-year history of myelofibrosis developed mild impairment of dorsal column sensation and ataxia of gait. A myelogram and subsequent peroperative biopsy demonstrated spinal cord compression due to extramedullary haematopoiesis. There was an excellent clinical response to surgery and radiotherapy. The characteristic clinical features and the pathogenesis of this unusual complication of myelofibrosis and extramedullary haematopoiesis are discussed.
A granulocytic sarcoma expanded the malar region in a patient with proven myelofibrosis over a 22 month period before undergoing rapid increase in size concomitantly with transformation to acute granulocytic leukaemia in the marrow and the widespread appearance of subcutaneous tumour deposits. Rapid response was obtained with local radiotherapy, and the systemic disease manifestations were controlled on combination courses of oral 4'-demethoxydaunorubicin and the epipodophyllotoxin VP16-213. This appears to be the first example of a granulocytic sarcoma occurring in a patient with myelofibrosis.
A 68 year old man developed cranial diabetes insipidus 3 years after the diagnosis of myelofibrosis, coincident with a marked increase in nucleated cell count. No mass lesion was demonstrable on computed tomographic or magnetic resonance imaging. It is suggested that hypothalamic damage was caused by local infiltration or infarction, a complication of myelofibrosis which has not previously been reported.