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At least 883 records · Page 49Linked to original sources
PubMed1990

[Successful high-dose methylprednisolone therapy in a patient with primary myelofibrosis].

We are presenting a patient with primary myelofibrosis who responded to the High-Dose methylprednisolone therapy (1 g/day for 3 days). Three and a half years ago, a 55-year-old male was admitted to our hospital because of severe erythroblastic anemia, thrombocytopenia, splenomegaly and "dry tap" of bone marrow aspiration. Bone marrow biopsy revealed a marked fibrosis without any blastoid cell proliferations. Since a thrombocytopenia was progressive and refractory to the ordinary therapy, high-dose methylprednisolone therapy was performed which was followed by an administration of activated vitamin D3. After the therapy, hematologic improvements were achieved within a month (RBC: 284 x 10(4)/microliters----413 x 10(4)/microliters, WBC: 3,000/microliters----11,500/microliters, Plat.: 7,000/microliters----20,000/microliters). Three months after the therapy, the bone marrow biopsy and 113In scintigraphy were performed. These tests also proved marked improvement of histological features of the bone marrow and a decrease of uptake of 113In to the spleen, respectively. The patient continues to be in good condition and he is free from any medications at present time.

Bone Marrow↗
PubMed1990

[Idiopathic myelofibrosis: clinical course, survival, and causes of death in a series of 60 patients].

The evolutive patterns, actuarial survival and causes of death of idiopathic myelofibrosis were analysed in a series of 60 patients. The median age of the patients was 64 years; 41 were males and 19 females. In the initial bone-marrow biopsy studies 25 patients were in stage MF/C, 17 in MF/O- and 18 in MF/O+. When performing this analysis, 32 patients were dead, 19 were still alive and 9 had been lost after a median follow-up of 6 months. The median survival of the whole series was 57 months. Four major evolutive patterns were recorded: 1) blastic crisis (7 cases), 2) portal vein hypertension (4 cases), 3) liver insufficiency due to massive myeloid metaplasia of the liver without signs of portal vein hypertension (5 cases); in 2 of them this pattern followed splenectomy), and 4) heart failure ascribable, at least partially, to post-transfusion haemochromatosis (3 cases). The blastic crisis appearing in 7 patients presented after a median follow-up of 19 months, and 6 of these patients have died after a median of 5 months since the diagnosis of the blastic crisis. In the 16 patients who died without any characteristic evolutive pattern, the following causes of death could be registered: septic shock (6 cases), intracranial haemorrhage, haemoperitoneum and acute renal failure (1 case each), whereas the cause of the deceased was unclear in the remaining 7 patients.

Aged↗
PubMedDate not supplied

1,25-Dihydroxyvitamin D3 in the treatment of idiopathic thrombocythemia and myelofibrosis.

The effect of treatment with 1,25-dihydroxyvitamin D3 administered at the dose of 1.50-3.00 ug/day for at least 12 months was evaluated in three patients with idiopathic myelofibrosis and in five patients with idiopathic thrombocythemia. This treatment did not cause any significant change in the hematological values or the clinical course of the myeloproliferative diseases in any of the patients. Based on these data, treatment with 1,25-dihydroxyvitamin D3 in non toxic doses seems to be of doubtful benefit in patients with these disorders.

Adult↗
PubMed1990

Extramedullary hematopoiesis involving the esophagus in myelofibrosis.

In this report, we describe a case of myelofibrosis with myeloid metaplasia; a 53-yr-old man was splenectomized for a massively enlarged spleen in which multiple foci of myeloid metaplasia were histologically demonstrated. The patient was referred to us for endoscopic examination, following the repeated occurrence of melena. Upper gastrointestinal endoscopy revealed two active ulcerative lesions in the bulb, and only a moderate erythema in the lower third of the esophagus, which showed no varices. There was no endoscopic evidence of active or recent bleeding. Subsequent histologic examination of biopsies taken from the esophageal lesion surprisingly revealed the presence of hematopoietic tissue.

Biopsy↗
PubMed1990

Clinical and hematological improvement in a patient receiving danazol therapy for myelofibrosis with myeloid metaplasia.

We report the positive effect of danazol on clinical and hematological data in a patient with myelofibrosis and myeloid metaplasia. Severe cytopenia was corrected within 2 months and was maintained for an additional 2-month period. Myeloid metaplasia (lymph nodes and skin lesions) was greatly reduced. The patient experienced no side effects. We propose further trials to confirm the utility of this agent in this disease.

Danazol↗
PubMed1989

[Acute myelofibrosis: apropos of a case with immunocytochemical and ultrastructural studies].

We report a case of acute myelofibrosis. This is a rare myeloproliferative disorder characterized by pancytopenia, minimal or absent anisopoikilocytosis, bone marrow fibrosis with hyperplasia and immaturity of the three main cellular lines with megakaryocyte predominance, absence of splenomegaly and rapidly fatal course. We discuss its relationship with acute megakaryocytic leukemia, as its blast elements correspond to megakaryocytes when ultrastructural and antifactor VIII immunoperoxidase techniques are used; these techniques disclose alpha granules and cell demarcating membranes.

Aged↗
PubMed1989

[Acute myelofibrosis terminating in acute myelomegakaryoblastic leukemia].

We report a case of acute myelofibrosis (AMF) developing into acute myelomegakaryoblastic leukemia. A 33-year-old woman was admitted to our hospital because of fever and chest pain. On physical examination, hepatosplenomegaly was not noticed. Pancytopenia and a small number of blast cells were observed in the peripheral blood. Poikilocytosis was not detected. Bone marrow examination revealed dry tap on aspiration, and moderate increase in reticulin fiber on biopsy. The diagnosis of AMF was made. Eight months later, blast cells markedly increased. Surface marker was investigated and MCS-2 (CD13), C17 (CDw41) and P2 (CDw41) were found to be positive. Electron microscopic examination revealed that blast cells were composed of PPO-positive cells and MPO-positive cells. Based on these findings, it was considered that the patient developed acute myelomegakaryoblastic leukemia. Recently AMF is thought to be a state to have the ability to develop into various types of acute leukemia. Adequate therapy may be required before the development of leukemia.

Acute Disease↗
PubMed1989

Antigenic characteristics of circulating granulocyte-macrophage progenitors (CFU-GM) in idiopathic myelofibrosis and polycythaemia vera defined by monoclonal antibodies.

The antigenic characteristics of "early" (type 1) and "late" (type 2) granulocyte-macrophage progenitor cells (CFU-GM) from patients with polycythaemia vera (PV) and idiopathic myelofibrosis (MF) were studied using antimyeloid murine monoclonal antibodies (McAbs) and anti-HLA-DR McAb (L243) in a complement dependent cytotoxic assay followed by culture in methyl cellulose. The results were compared with the previously published data on the antigen phenotype of CFU-GM from normal bone marrow and peripheral blood. McAbs L243, MY9, S3-13 and S17-25 reacted with lower percentage of CFU-GM from patients with PV and MF than with their normal counterparts MCAbs R1.B19 and WGHS 29.1, which recognized the antigens on "late" CFU-GM (type 2), reacted with a high proportion of day 7 CFU-GM from MF peripheral blood and normal marrow but did not react with CFU-GM from PV and normal peripheral blood. The antigens recognized by two other McAbs (53/10 and PM81) were present on some of normal CFU-GM and were not found on CFU-GM from MF and PV peripheral blood. Our data suggest that CFU-GM in the circulation in MF differ from those in normal blood, perhaps because they are released from the spleen which in this disease has haemopoietic function.

Adult↗
PubMedDate not supplied

1 alpha(OH)D3 treatment and procollagen III (PC III) studies in idiopathic myelofibrosis.

Eight patients with idiopathic myelofibrosis (IMF) were treated with escalating doses (0.5 microgram/day to 4.0 micrograms/day) of 1 alpha(OH)D3 for periods up to 6 months. The treatment did not improve the disease parameters in any of the patients under study. No patient demonstrated a decrease in bone marrow fibrosis as determined by serial procollagen (PC III) serum level analysis. Although 1 alpha(OH)D3 was well tolerated at the administered doses, the therapeutic value of this treatment for IMF requires further evaluation.

Aged↗
PubMed1989

Acute myelofibrosis: report of a case and review of current literature.

Acute myelofibrosis is a rare hematologic disorder characterized by pancytopenia, normal erythrocyte morphology, extensive marrow fibrosis, and a rapidly fatal clinical course. The etiology of this disease is unknown, but the malignant cell may be of megakaryocytic lineage. Although allogeneic bone marrow transplantation may be effective in achieving complete hematologic remission, optimal management of this rare and often rapidly fatal disease remains to be defined.

Acute Disease↗
PubMedDate not supplied

Primary myelofibrosis-osteomyelosclerosis (agnogenic myeloid metaplasia): correlation of clinical findings with bone marrow histopathology and prognosis.

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.

Bone Marrow↗
PubMed1989

[Allogeneic bone marrow transplantation as primary treatment in acute myelofibrosis].

A 20-year-old man with acute myelofibrosis received ablative chemotherapy consisting of busulfan, Ara-C and cyclophosphamide followed by bone marrow transplantation from his histocompatible sister. There was a prompt engraftment with a rising WBC and platelet count and with donor karyotype in specimen of marrow. A bone marrow examination on day 29 showed a prominent decrease in marrow fibrosis. However, fibrosis was again exacerbated with leukemic relapse as overt acute megakaryoblastic leukemia on day 110, The patient died on day 126. Marrow fibrosis was found to be reversible by ablation of malignant cells and repopulation of normal hemopoietic cells after bone marrow transplantation.

Acute Disease↗
PubMed1987

Effects of recombinant alpha and gamma interferons on the in vitro growth of circulating hematopoietic progenitor cells (CFU-GEMM, CFU-Mk, BFU-E, and CFU-GM) from patients with myelofibrosis with myeloid metaplasia.

Myelofibrosis with myeloid metaplasia (MMM) is a chronic myeloproliferative disorder due to clonal expansion of a pluripotent hematopoietic progenitor cell with secondary marrow fibrosis. No definitive treatment has as yet been devised for this condition, which shows a marked variability in clinical course. To evaluate whether excessive hematopoietic progenitor cell proliferation could be controlled by recombinant human interferon alpha (rIFN-alpha) and gamma (rIFN-gamma), we studied the effects of these agents on the in vitro growth of pluripotent and lineage-restricted circulating hematopoietic progenitor cells in 18 patients with MMM. A significant increase in the growth (mean +/- 1 SEM) per milliliter of peripheral blood of CFU-GEMM (594 +/- 253), CFU-Mk (1,033 +/- 410), BFU-E (4,799 +/- 2,020) and CFU-GM (5,438 +/- 2,505) was found in patients as compared with normal controls. Both rIFN-alpha and rIFN-gamma (10 to 10(4) U/mL) produced a significant dose-dependent suppression of CFU-GEMM, CFU-Mk, BFU-E, and CFU-GM growth. Concentrations of rIFN-alpha and rIFN-gamma causing 50% inhibition of colony formation were 37 and 163 U/mL for CFU-GEMM, 16 and 69 U/mL for CFU-Mk, 53 and 146 U/mL for BFU-E, and 36 and 187 U/mL for CFU-GM, respectively. A marked synergistic effect was found between rIFN-alpha and rIFN-gamma: combination of the two agents produced inhibitory effects greater than or equivalent to those of 10- to 100-fold higher concentrations of single agents. These studies (a) confirm that circulating hematopoietic progenitors are markedly increased in MMM, (b) indicate that these presumably abnormal progenitors are normally responsive to rIFNs in vitro, and (c) show that IFNs act in a synergistic manner when used in combination. Because rIFN-gamma can downregulate collagen synthesis in vivo, this lymphokine could be particularly useful in the treatment of patients with MMM.

Antibodies, Monoclonal↗
PubMed1988

Antigenic characteristics of erythropoietin dependent and independent erythroid progenitors (BFU-E and CFU-E) in polycythaemia vera and idiopathic myelofibrosis defined by monoclonal antibodies.

The antigenic characteristics of erythropoietin (Ep) dependent and independent ("endogenous") erythroid progenitor cells (BFU-E and CFU-E) from patients with polycythaemia vera (PV) and idiopathic myelofibrosis (MF) were studied using 9 selected murine monoclonal antibodies (McAbs) in a complement dependent cytotoxicity assay followed by culture in methyl cellulose. McAbs L343 (HLA-DR antigen) S3-13 and S17-25 reacted with about 50% of BFU-E in MF and PV. In contrast HLA-DR antigens were not presented on CFU-E. McAbs R1.B19, WHGS29.1, PMN29 and PM81 recognized the antigens on less than 25% of BFU-E and CFU-E. The reactivity of Ep-dependent and Ep-independent BFU-E and CFU-E was similar with the majority of McAbs. However, McAbs S4-7 and VIM-2 reacted with a higher proportion of Ep-independent BFU-E than of Ep-dependent BFU-E. These results indicate that Ep independent, neoplastic BFU-E in PV and MF are more mature than their Ep dependent counterparts. The antigens expressed on erythroid progenitor cells from patients with PV and MF were earlier found on BFU-E and CFU-E from normal bone marrow and peripheral blood, usually on a higher proportion of cells, than in myeloproliferative disorders.

Aged↗
PubMed1987

[Myelofibrosis. Pathology of the microenvironment].

Myelofibrosis is the most representative model of the association between the hemopoiesis failure end the abnormalities of the myeloid microenvironment. Long term culture in Dexter's liquid system is a good model for the in vitro study of myeloid differentiation. An experimental in vitro system using an irradiated under layer and aplastic patients plasma has yielded some preliminary results allowing the production of megakaryocytic colonies. Close contact between these colonies and the fibroblasts or stromal cells in the adherent layer could probably be used for the study of the interrelation between the stromal abnormalities and myeloid pathology.

Hematopoietic Stem Cells↗