Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “MYELOFIBROSIS”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 325 records · Page 18Linked to original sources
PubMed1982

[Portal vein aneurysm and liver fibrosis in myelofibrosis].

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.

Aneurysm↗
PubMed2003

T cell lymphoma associated with myelofibrosis.

Myelofibrosis is most frequently associated with certain primary myeloproliferative disorders,but is rare in lymphoid neoplasms. We report the fourth case associated with T-cell lymphoma, involving bone marrow, lymph nodes and spleen. Marked extramedullary hematopoiesis was present. Myelofibrosis subsided completely with response to standard anti-lymphoma combination chemotherapy. Since lymphomatous splenic and bone marrow involvement was minimal in our patient, fibrotic bone marrows should be carefully evaluated for lymphoma.

Biopsy↗
PubMed2002

Reversible myelofibrosis induced by tuberculosis.

Tuberculosis and myelofibrosis are reported in conjunction often enough to raise the possibility that a relationship exists between the 2 entities. However, whether tuberculosis stimulates a secondary fibrotic reaction or develops in patients who have preexisting myeloproliferative disorders is not clear. We describe the case of a 28-year-old man in whom myelofibrosis disappeared completely after administration of antituberculous treatment, which suggests that a causal relationship exists between the 2 disease entities.

Adult↗
PubMed1999

A pilot study of recombinant human interleukin-4 therapy of myelofibrosis.

Twelve patients with myelofibrosis were treated with recombinant human interleukin-4 (IL-4) administered subcutaneously thrice weekly. Dosage ranged from 1 microg/kg to 4 microg/kg. Median patient age was 65 years (range 36-74). Five patients had transient minor responses, and 5 patients had progressive disease. One patient had a transient minor response, rapidly followed by progressive disease. One patient suffered angioneurotic edema with first injection. Other significant toxicities included fever, flu-like symptoms, peripheral edema, and ascites. IL-4 at this schedule was toxic and had no significant activity in myelofibrosis.

Adult↗
PubMed1975

Mast cells and myelofibrosis.

Autopsy of a patient with well-documented myelofibrosis revealed marked proliferation of mast cells associated with areas of bone-marrow and splenic fibrosis. The findings suggest that the local fibrosis represents the healed phase of an inflammatory reaction mediated by mast cells via the release of histamine into the tissue spaces. Tissue mastocytosis may be the pathogenetic mechanism in some cases of myelofibrosis.

Aged↗
PubMed1980

Acute myelofibrosis terminating as acute myeloblastic leukemia.

The case of an 8-year-old girl in whom acute myelofibrosis developed is described. The patient was managed conservatively. A year later evolution to acute myeloblastic leukemia occurred, accompanied by myeloid and erythroid dyspoiesis and an elevated fetal hemoglobin level. A remarkable reversal of the marrow fibrosis occurred when the patient was treated with combination chemotherapy. Intensive chemotherapy combined with extensive transfusion support, and possibly bone marrow transplantation, may offer similar patients hope for a prolonged survival and possible cure. The nature of acute myelofibrosis and its differential diagnosis are discussed.

Antineoplastic Agents↗
PubMed1982

Acute myelofibrosis terminating in erythroleukemic state.

A patient with acute myelofibrosis associated with erythroleukemia is described. The terminal course of the patient was marked by the erythroblast population in the peripheral blood increasing to a level of 21.8 x 10(3)/mm3, 18% of which were PAS-positive. Possible transformation of acute myelofibrosis into the erythroleukemic state is discussed.

Acute Disease↗
PubMed1990

Scintigraphic evaluation of secondary myelofibrosis associated with prostatic cancer before and after hormone therapy.

A patient with secondary myelofibrosis associated with prostatic cancer gained hematologic remission after hormone therapy. Before treatment, a bone scan with Tc-99m MDP showed diffuse, increased uptake in the axial skeleton without visualization of the appendicular skeleton; a bone marrow scan with In-111 chloride revealed decreased uptake in the central marrow. Following hormone therapy, a bone scan showed an almost normal distribution with visualization of the appendicular skeleton and bone marrow scan indicating improved uptake of the central marrow. Radionuclide bone and bone marrow imaging was thus useful not only in diagnosing secondary myelofibrosis but also in evaluating the effects of therapy.

Aged↗
PubMed1998

Acute myelofibrosis mimicking multiple bone metastases on Tc-99m MDP bone imaging.

PURPOSE: Tc-99m bone scintigraphy is widely used for evaluation of osseous spread of malignant tumors. PATIENT: A 60-year-old man had multiple areas of increased uptake on a bone scan in a pattern considered characteristic for extensive metastatic disease. However, a primary neoplasm could not be identified. Finally, acute myelofibrosis--a rare, fatal myeloproliferative syndrome--was diagnosed. CONCLUSION: To avoid delays and extensive diagnostic procedures, acute myelofibrosis should be considered in the differential diagnosis of bone scans showing multiple hot spots. In such cases, a diagnosis can be made on an a tissue sample.

Acute Disease↗
PubMed1988

Acute monoblastic leukemia with osteosclerosis and extensive myelofibrosis.

A 4-month-old infant was admitted with a monoblastic infiltration of the skin associated with osteosclerosis. Both lesions spontaneously disappeared within a few months, but 2 years later, a monoblastic leukemia occurred that was associated with marked skin erythema and myelofibrosis. Skin and bone marrow specimens showed a monoblastic infiltration with numerous intermingled mast cells of normal appearance. Whether myelofibrosis was a feature of a systemic mastocytosis or of the leukemic process is discussed in this case.

Humans↗
PubMed2003

Anemia and hepatosplenomegaly as presenting features in a child with rickets and secondary myelofibrosis.

Anemia and hepatosplenomegaly are common reasons for referring a child to a pediatric hematologist or oncologist. Among the many causes for these findings is severe rickets, which has been shown to be associated with secondary myelofibrosis and myeloid metaplasia. The authors present the case of an infant with severe rickets and secondary myelofibrosis and review the differential diagnosis of hepatosplenomegaly from the viewpoint of the pediatric hematologist/oncologist.

Anemia↗
PubMed2004

Pulmonary hypertension complicating bone marrow transplantation for idiopathic myelofibrosis.

Idiopathic myelofibrosis is a rare hematologic disorder that is occasionally associated with pulmonary hypertension and has been cured with bone marrow transplantation (BMT). Most cases occur in older adults, but children with similar clinical and pathologic findings have been described. The authors describe a critically ill male infant with idiopathic myelofibrosis and subtle findings suggestive of pulmonary hypertension who was treated with BMT after failing to respond to chemotherapy. After BMT, the patient's clinical course improved in all respects, but he ultimately died of progressive pulmonary hypertension.

Bone Marrow Transplantation↗
PubMed2005

Myelofibrosis on F-18 FDG PET Imaging.

A 60-year-old male was referred for a positron emission tomography (PET) scan using F-18 fluoro-2-deoxyglucose (F-18 FDG) for evaluation of a right lung opacity identified on a computed tomography (CT) scan. The patient also had a history of idiopathic myelofibrosis. The PET scan revealed markedly increased uptake throughout the spleen and liver, which were massively enlarged. There was also significantly increased uptake diffusely throughout the bone marrow. These findings are a reflection of the patient's myelofibrosis.

Fluorodeoxyglucose F18↗
PubMed1976

Quantitative studies of splenic erythropoiesis in polycythaemia vera and myelofibrosis.

A quantitative scanning method employing cyclotron-produced 52Fe has been developed to assess splenic erythropoiesis in patients with myeloproliferative disorders. In 12 patients with myelofibrosis splenic uptake of 52Fe was from 5.0% to 48% of the injected dose. Although a single patient with classical polycythaemia vera had a minor uptake of 2.8% of six other patients with this diagnosis showed no concentration of isotope in the splenic area. The fraction of 52Fe in the spleen of four patients with 'transitional' myeloproliferative disorders characterized by a high red cell mass, hypercellular bone marrow and a leucoerythroblastic blood film varied from 5% to 41%. No clear relationship was noted between the degree of splenic erythropoiesis as defined by this technique and the level of haemoglobin, the degree of splenomegaly, the effectiveness of erythropoiesis of traditional 59Fe surface counting. If splenectomy is considered in patients with myelofibrosis splenic 52Fe quantitation will provide more precise data on the contribution of splenic erythropoiesis than 59Fe surface counting alone.

Erythropoiesis↗
PubMed1979

Myelofibrosis in chronic granulocytic leukaemia.

Serial trephine biopsies were performed in 45 cases of chronic granulocytic leukaemia (CGL) in order to determine the frequency and significance of secondary myelofibrosis in the evolution of the disease. Histological changes were graded 1-5b, ranging from no increase in reticulin to dense osteomyelosclerosis. Many cases showed a progressive increase from Grade 1 to Grade 3, and accelerated disease, or blast crisis, often supervened when Grade 3 changes were present. However, a significant number of cases showed Grade 4 and 5 changes, which were indistinguishable histologically from those found in agnogenic myeloid metaplasia (AMM) (idiopathic myelofibrosis), at the time of diagnosis. These patients did not always show a rapidly fatal course and may be considered as an example of 'transitional myeloproliferative disorder', with features intermediate between CGL and AMM.

Adult↗
PubMed1980

An identical translocation between chromosome 1 and 7 in three patients with myelofibrosis and myeloid metaplasia.

An identical chromosome abnormality was observed in three unrelated patients with meylofibrosis and myeloid metaplasia, two of the patients showing a history of polycythaemia vera (PV) before development of the myelofibrosis. Unstimulated peripheral blood cultures showed a translocation between chromosomes 1 and 7 replacing a homologue of pair 7. It was identified by G- and C-banding as t(1;7)(7pter leads to 7p11::1p1? leads to 1qter). While the first patient also showed trisomy 21 and the third patient had some extra material on the short arm of chromosome 17, all three had trisomy 1q and monosomy 7q. Although each of these abnormalities is frequently observed separately in various haematological disorders, the combination of the two in the form of an identical translocation in three patients is an example of induced non-random cytogenetic change in myelofibrosis.

Adult↗
PubMed1983

Immune disorders in agnogenic myeloid metaplasia: relations to myelofibrosis.

Tests for a dysimmune state were done in an unselected group of 67 patients with agnogenic myeloid metaplasia (AMM). The results were compared to those of 56 patients with polycythaemia vera (PV). 75% of AMM patients versus 32% of PV patients had various abnormalities. The most frequent disorders among AMM patients were serum antinuclear and anti smooth muscle autoantibodies (10.3% each), a positive test for rheumatoid factor (21.7%), a polyclonal increase in serum immunoglobulin levels (46.8%) or a serum monoclonal component (9.7%), a positive direct Coombs' test (19%), an anti I autoantibody (30%). In AMM patients there was no relationship between age, sex, importance of splenic enlargement, time from diagnosis or treatment and the present of a dysimmunity. Furthermore, in AMM patients, but also in PV patients, it seems that the more frequent and numerous these abnormalities the more severe is the myelofibrosis. Like other previous studies, these results suggest a lymphoid cell involvement in AMM and a role for these immune disorders in the pathogenesis of myelofibrosis.

Adult↗
PubMed1983

Anaemia in myelofibrosis: its value in prognosis.

Forty-four patients with myelofibrosis were investigated in our hospital in the period 1971-81. Their clinical, laboratory and radioisotope parameters were analysed. The direct correlation between plasma volume and splenic red cell pool has highlighted the role of the spleen in the dilutional anaemia seen in myelofibrosis. 52Fe quantitation enabled us to show that the bone marrow contributes relatively more to effective erythropoiesis than the extramedullary sites. The prognostic value of changes in plasma volume and bone marrow 52Fe activity has been demonstrated. We have shown that the Hb: reticulocyte relationship at diagnosis can be used to recognize probable stages of the disease and provides a useful prognostic determinant.

Adult↗