PubMed Health⌕ Search

SEARCH · PubMed Health

Results for “MYELOFIBROSIS”

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.

At least 19 recordsLinked to original sources
PubMed2004

Analysis of risk factors of the evolution of myelofibrosis in pre-fibrotic chronic idiopathic myelofibrosis: a retrospective study based on follow up biopsies of 70 patients by using the RECPAM method.

The advanced, fibrotic phase of chronic idiopathic myelofibrosis (CIMF) is preceded by a pre-fibrotic stage. However, the factors which may influence or predict the development of myelofibrosis are not well established. Thus we investigated follow up biopsies of 70 patients with CIMF, diagnosed in stages of no or only scant fiber increase, for the development of myelofibrosis. The influence of histopathological (megakaryocytes, initial fiber content), clinical (age, gender, splenomegaly, chemotherapy) and hematological (Hb, leukocyte- and platelet count) parameters on the development of myelofibrosis was evaluated by using the univariate Log Rank method and the multivariate recursive partition and amalgamation (RECPAM) analysis. Surveying a mean observation period of 47 months we found a development of significant myelofibrosis in 30 of the 70 patients. In the univariate analysis, the development of myelofibrosis was associated with increased megakaryocytes, initial fiber content and age of the patient. In the RECPAM analysis, the patients with a high megakaryocytic count and older age showed the highest risk of developing myelofibrosis (mean 58.3 and 60.1 months). The group with the best prognosis comprised the patients under 60 years which have a low content of megakaryocytes (mean 137 months). We found that the development of myelofibrosis in CIMF was best predicted by an increase of megakaryocytes within the bone marrow, possibly reflecting the release of growth factors by these cells. The next important risk factor was the age of the patients.

Adult↗
PubMed2003

Chronic idiopathic myelofibrosis: prognostic impact of myelofibrosis and clinical parameters on event-free survival in 122 patients who presented in prefibrotic and fibrotic stages. A retrospective study identifying subgroups of different prognoses by using the RECPAM method.

In chronic idiopathic myelofibrosis (CIMF) the factors predicting survival in patients who were already in the fibrotic stage have been well documented by numerous studies. Prefibrotic stages were only rarely evaluated so that the prognostic impact of myelofibrosis is currently not well known. Also predictive factors for disease-related events were not included in those studies. Thus, we evaluated the prognostic impact of myelofibrosis and other histopathological (megakaryocytes, blasts) and clinical [age, gender, splenomegaly, chemotherapy, hemoglobin (Hb), leukocyte, and platelet count] parameters in 122 patients in fibrotic and prefibrotic stages of CIMF on event-free survival. The statistical analysis was performed using the univariate log-rank test and the multivariate recursive partition and amalgamation (RECPAM) approach. In 62 patients disease-related events occurred during a mean observation period of 58 months. In univariate analysis they were associated with blast increase in the bone marrow. In RECPAM analysis a shorter event-free survival was found in anemic patients (mean: 9.3 months). In nonanemic patients older than 60 years, advanced myelofibrosis was associated with a shorter event-free mean survival of 23.2 months versus 69.3 months in less advanced cases. A slight or moderate myelofibrosis was not found to have a prognostic impact on event-free survival. The longest event-free survival was found in nonanemic patients who were younger than 60 years (mean: 185 months), regardless of the grade of myelofibrosis. Thus, we found that the most relevant prognostic parameter for event-free survival in CIMF were the Hb value, age, and grade of myelofibrosis.

Adult↗
PubMed1991

Myelodysplastic syndrome with myelofibrosis: myelodysplastic syndrome as a major primary disorder for acute myelofibrosis.

Seven cases of myelodysplastic syndrome with myelofibrosis, which is defined using the following criteria: (1) pancytopenia with less than 5% blasts in the peripheral blood; (2) minimal or no splenomegaly; (3) myelofibrosis with cellular marrow; (4) absence of diffuse proliferation of blasts in the bone marrow; and (5) presence of myelodysplastic features of bone marrow or peripheral blood cells, are presented. They were in the range of 52-82 years old and consisted of 3 males and 4 females. Six out of 7 cases developed into acute leukaemia after 5 to 8 months from the onset and died from between 2 weeks to 8 months from the evolution to leukaemia. The type of leukaemia was acute myeloblastic in 3 patients, and acute myelo-megakaryoblastic in 3 patients. Another patient died of severe hepatic injury after 5 months from the onset of the disease. These findings revealed that the complication of myelofibrosis in the patients with myelodysplastic syndrome was an indicative sign of rapid progression to overt leukaemia or otherwise poor prognosis for survival. In addition myelodysplastic syndrome is thought to be major primary disorder for acute myelofibrosis. Myelodysplastic syndrome with myelofibrosis is closely associated with the neoplastic proliferation of megakaryoblasts in a considerable number of patients.

Acute Disease↗
PubMed2003

Evolution of myelofibrosis in chronic idiopathic myelofibrosis as evidenced in sequential bone marrow biopsy specimens.

Although the new World Health Organization (WHO) classification acknowledges "prefibrotic" phases, progression of myelofibrosis in chronic idiopathic myelofibrosis (cIMF) is controversial because there are only a few studies about sequential biopsy specimens, and they yield conflicting results. The conflicting results might be due to a mixture of different degrees of myelofibrosis and therapy regimens within the respective groups studied. To prove this hypothesis, we studied sequential bone marrow biopsy specimens from patients with cIMF and compared 3 groups with different degrees of myelofibrosis at initial diagnosis with a group of patients with primarily unfibrosed disease who met the WHO criteria for prefibrotic cIMF. Patients receiving chemotherapy were considered separately from patients without treatment. Our results favor a steady progression of myelofibrosis unrelated to therapy modalities, whereas confusing literature data can be explained: fibrosis may remain static or lessen, especially in more advanced stages of cIMF.

Antineoplastic Agents↗
PubMed1997

Allogeneic marrow transplantation for primary myelofibrosis and myelofibrosis secondary to polycythaemia vera or essential thrombocytosis.

Primary myelofibrosis is a clonal haemopoietic disorder, incurable with conventional therapy, and associated with a median survival of 4-5 years. Patients with polycythaemia vera and essential thrombocytosis who progress into a myelofibrotic picture also have a poor prognosis. Between 1980 and 1996, 13 patients with myelofibrosis due to one of these three myeloproliferative disorders (primary myelofibrosis [n=8], essential thrombocytosis [n=3], polycythaemia vera [n=2]) underwent allogeneic marrow transplantation in Seattle. The median age was 40 years (range 18-49). The median time from myeloproliferative diagnosis to transplantation was 39 months (range 5-192). Three patients received preparative regimens containing total body irradiation and 10 received busulphan-cyclophosphamide regimens. Nine patients received marrow from HLA-matched related donors, one from a one antigen mismatched related donor, and three from HLA-matched unrelated donors. The median time to both granulocyte and platelet engraftment was 21 d. Nine patients survive between 1.2 and 7.1 years post-transplant. Two patients relapsed at 1 year post-transplant, both of whom survive in a chronic myeloproliferative state. Four patients died of transplant-related complications between 43 d and 2.2 years post-transplant. At 1 year post-transplant the majority of the disease-free survivors have normal peripheral blood counts and none-to-minimal marrow fibrosis. These preliminary results are encouraging, and suggest that stem cell transplantation can be curative therapy for selected patients with myelofibrosis.

Adolescent↗
PubMed2001

Fluorescence in situ hybridization analysis of 25 cases of idiopathic myelofibrosis and two cases of secondary myelofibrosis: monoallelic loss of RB1, D13S319 and D13S25 loci associated with cytogenetic deletion and translocation involving 13q14.

To identify a commonly deleted region of 13q14 in idiopathic myelofibrosis (IMF), we used fluorescence in situ hybridization analysis to test for deletion of the RB1 and BRCA2 genes, and the microsatellite loci D13S319 and D13S25, in a series of 25 patients. A further two patients with myelofibrosis secondary to polycythaemia vera and essential thrombocythaemia with reciprocal 13q translocations were studied in an attempt to further define the CDR. Twenty out of 21 patients with a cytogenetically normal chromosome 13 failed to show allelic loss with any of the four probes. In contrast, all four cases with cytogenetic deletion of 13q14 and both cases with 13q translocations involving 13q14 exhibited loss of RB1, D13S319 and D13S25. Loss of the BRCA2 locus was present in a single case only. Our results indicate that cryptic deletions of the 13q14 in myelofibrosis are rare. In addition, the genetic loss associated with cytogenetic 13q14 deletions or reciprocal translocations involving 13q14 is large and encompasses the gene-rich region around RB1, D13S319 and D13S25.

Adult↗
PubMed1995

Evans' syndrome, myelofibrosis and systemic lupus erythematosus: role of procollagens in myelofibrosis.

We describe an 18 yr old female with systemic lupus erythematosus presenting with Evans' syndrome (autoimmune hemolytic anemia and immune thrombocytopenia) and dense myelofibrosis. Her clinical course and response to treatment were monitored with regular blood counts, serial measurements of serum procollagen I and III and periodic bone marrow examinations. This report brings to 9 the number of well-documented cases of systemic lupus erythematosis and myelofibrosis in the literature; we discuss the pathogenesis and management of patients with this apparently rare disorder, with particular reference to the role of serum procollagen measurements as markers of myelofibrosis.

Adolescent↗
PubMedDate not supplied

Multiple myeloma with coexistent myelofibrosis: improvement of myelofibrosis following recovery from multiple myeloma after treatment with melphalan and prednisolone.

We describe a case of multiple myeloma associated with myelofibrosis. This patient had hepatosplenomegaly, moderate anemia with anisocytosis and nucleated red blood cells, and Bence-Jones protein (kappa) in the urine. A bone marrow biopsy showed extensive marrow fibrosis and proliferation of numerous immature plasma cells containing kappa light chain in the cytoplasm. Melphalan-prednisolone therapy not only facilitated the disappearance of the immature plasma cells but also resulted in an improvement of myelofibrosis in the bone marrow. The immature plasma cell proliferation and marrow fibrosis in the bone marrow were seen again after interruption of chemotherapy. Therefore, this myelofibrosis may be secondary to the coexistent multiple myeloma.

Agammaglobulinemia↗
PubMed1984

Myelofibrosis revisited: characterization and classification of myelofibrosis in the setting of myeloproliferative disease.

In describing the various forms of myeloproliferative disease in which myelofibrosis develops, it becomes clear that certain cellular and biochemical abnormalities are shared by all forms of reactive myelofibrosis. Of possible etiologic significance are megakaryocytosis, monocyte-macrophage hyperplasia and activation, altered immunologic status, and proliferation of mast cells and basophils. The mechanisms by which these abnormalities could mediate fibrosis and the methodology that makes assessment and monitoring of fibrosis possible during the course of myeloproliferative disease are discussed in the subsequent contributions to this volume. Opportunities for further study and elucidation of the pathogenesis of myelofibrosis abound and should stimulate the generation of new knowledge that has direct relevance to the management of myeloproliferative disease.

Bone Marrow↗
PubMed2000

Induction of remission after donor leucocyte infusion for the treatment of relapsed chronic idiopathic myelofibrosis following allogeneic transplantation: evidence for a 'graft vs. myelofibrosis' effect.

A 54-year-old man showed evidence of disease progression and a reduction in donor chimaerism by molecular microsatellite analysis 6 months after an allogeneic peripheral blood stem cell transplant for chronic idiopathic myelofibrosis. He was treated with a single infusion of donor leucocyte infusions (DLI), which led to the development of mild acute graft versus host disease (GVHD) and the rapid restoration of full donor haemopoiesis. This subsequently led to a progressive reduction in marrow fibrosis from grade IV to grade I over the following 6 months. We believe that this is the first report to provide clear evidence for the efficacy of DLI in this setting, which also provides evidence for the existence of a T-cell-mediated 'graft vs. myelofibrosis' effect similar to that seen against other haematological malignancies.

Chronic Disease↗
PubMed1981

[Acute leukaemias with myelofibrosis. Clinical, cytohistological and evolutive aspects. Their place among malignant blood diseases with myelofibrosis (author's transl)].

In a series of 154 patients with acute leukaemia (AL), 31 had myelofibrosis. The authors report in detail on 9 of these and demonstrate the distinctive features of this form of AL: pancytopenia is severe, circulating blast cells are rare, myelograms are difficult to read, and bone-marrow biopsies are necessary to the diagnosis, severity assessment and prognosis of the disease. Owing to the small number of cells, it is uneasy to distinguish these AL from acute myelofibrosis (a nosological entity which needs to be more precisely defined) and from megakaryoblastic leukaemias, recently individualized.

Acute Disease↗
PubMed2000

Complete remission of idiopathic myelofibrosis following donor lymphocyte infusion after failure of allogeneic transplantation: demonstration of a graft-versus-myelofibrosis effect.

A patient with idiopathic myelofibrosis (IM) in the osteosclerotic phase received an allogeneic stem cell transplant. Hemopoietic engraftment was rapid, and full donor chimerism was observed on day +70. However, a few months later, replacement of donor hemopoiesis by the patient's 20q- cell clone was observed, followed by reappearance of the blood IM features, marrow fibrosis and osteosclerosis. At 8 months from transplant donor lymphocytes were infused, which induced chronic GVHD. This resulted in normalization of the blood, with disappearance of the fibrosis and osteosclerosis, effects which persisted 20 months later. This case provides evidence for a graft-versus-disease effect in IM.

Female↗
PubMed1990

Histo- and immunomorphometry of megakaryopoiesis in chronic myeloid leukemia with myelofibrosis and so-called primary (idiopathic) osteo-myelofibrosis/-sclerosis.

A morphometric study was performed on trephine biopsies of bone marrow in patients with chronic myeloid leukemia (CML) accompanied by myelofibrosis and in so-called primary (idiopathic) osteomyelofibrosis/-sclerosis (OMF) to evaluate distinctive features of megakaryopoiesis. The periodic acid Schiff reaction (PAS) and a monoclonal antibody against glycoprotein IIIa were employed for the identification of megakaryocytes including precursor cells and Gomori's silver impregnation to determine the density of argyrophilic fibers. All patients with CML revealed a slight to moderate degree of medullary fibrosis and were compared with early hyperplastic stages of OMF showing an identical fiber count. Statistical analysis disclosed that distinctive features existed between these two subgroups. Amongst these variables were sizes of megakaryocytes and corresponding nuclei, frequency of bare nuclei, emperipolesis and numbers of isolated nuclear fragments as well as the circular deviation of cell and nuclear perimeters. Immunomorphometry also included immature elements (pro- and megakaryoblasts) of the megakaryocyte series. Consequently higher cell counts were calculable in both groups combined with smaller sizes and a more rounded aspect of nuclei. However, following immunostaining, significant differences in several megakaryocytic parameters (frequency, size, shape of nuclei) were still demonstrable between CML and OMF cases.

Adult↗