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At least 433 records · Page 24Linked to original sources
PubMed1990

[Myelofibrosis associated with immunological abnormalities].

A 74-year-old man was admitted to the National Defense Medical College Hospital because of purpura on the extremities. The blood hemoglobin level was 11.5 g/dl, the white blood cell count 7. 3 x 10(9)/l with 3% blastic cells, and the platelet count 4.0 x 10(9)/l. There was leukoerythroblastosis with only mild teardrop appearance of erythrocytes. The blastic cells consisted of two types, megakaryoblast-like cells and myeloblast-like cells. There was a very mild hepatosplenomegaly, but no lymphadenopathies. The bone marrow aspirations yielded dry taps. The marrow biopsy specimen revealed myelofibrosis associated with trilineage hyperplasia, but no apparent infiltration of leukemic cells. Platelet-associated IgG (PAIgG) had a high titer and the response to platelet transfusions was poor. On prednisolone and colchicine, the blastic cells in the peripheral blood disappeared and the platelet count increased. He died of pneumonia. The autopsy showed myelofibrosis with hyperplasia of myeloid and erythroid lineage in the bone marrow. Megakaryocytes were relatively few and there was no infiltration of leukemic cells.

Aged↗
PubMedDate not supplied

Idiopathic myelofibrosis: a retrospective study of 103 patients.

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.

Adolescent↗
PubMedDate not supplied

[Renal lesions in myelofibrosis (author's transl)].

In 26 cases of myelofibrosis, the authors investigated for possible renal impairment that can be appraised from the usual clinical, laboratory, and roentgenographic signs. No anomalies were demonstrated in 12 of these cases. In 14 (or 53%) of the patients, some anomaly was discovered : essentially proteinuria with minor alteration of renal function, but also, two cases of poorly functioning left kidney evidenced on intravenous urograms, one case of acute anuric renal failure connected with hyperuricemia, one case of hypokalemic tubulo-interstitial nephritis, and one case of glomerulonephritis with, nephrotic syndrome. This study, when compared to the literature, indicates that besides nephropathy specific to myelofibrosis and attributed to myeloid metaplasia in the kidney, serious consideration must be given to lesions due to (1) compression of the left kidney by the enlarged spleen, (2) urate precipitation in the urinary passages, and (3) a possible glomerular disorder whose mechanism remains undefined.

Adult↗
PubMed1990

[Primary myelofibrosis with extramedullary haematopoiesis of the skin transformed into acute megakaryoblastic leukemia].

A 70-year-old man, who had been diagnosed as primary myelofibrosis in 1987 at Tokyo Women's Medical School, was admitted to our hospital because of left hip-joint pain in May 1988. Physical examinations revealed marked hepatosplenomegaly and multiple reddish papules on the skin. The peripheral blood showed marked leukoerythroblastosis and severe anemia with poikilocytosis. Bone marrow aspirations were dry tap. Needle biopsy of iliac crest showed a diffuse fibrosis. Biopsy of the papules showed an extramedullary haematopoiesis. He was treated with low dose Ara-C for seven days. Although hepatosplenomegaly and skin papules were reduced, he died of pneumonia a month later. At autopsy, leukemic cells massively infiltrated into the multiple organs, including bone marrow. The cells were identified with megakaryoblast, as those were positive for factor VIII related antigen. As far as we know, this is the third case of primary myelofibrosis transformed into acute megakaryoblastic leukemia.

Aged↗
PubMed1990

[Myelofibrosis with marked subcapsular bleeding of the spleen--a case report].

A 22-year old male was admitted to Ashikaga Red Cross Hospital because of the progressing abdominal distension in May 1988. The physical examinations disclosed marked hepato-splenomegaly, lymphadenopathy and cachexic state. In the laboratory examinations, anemia, leukocytosis with left shift of nuclei and severe inflammatory reactions were found. Bone marrow aspirations were dry taps, and the biopsy showed myelofibrotic changes. Abdominal computed tomography and ultrasonography revealed a marked hepatomegaly and a giant splenomegaly with subcapsular hematoma. Philadelphia chromosome was negative and neutrophilic alkaline phosphatase score was slightly high. A little amount of peritoneal effusions was obtained and yielded S. aureus. A diagnosis of myelofibrosis with subcapsular hematoma due to spontaneous rupture of spleen and peritonitis of unknown cause was made. Operative indication was considered, but his family did not agree with it. Then conservative therapies for general conditions, those are, administrations of antibiotics and diuretics, and blood transfusions were taken. On the enlarged spleen, total irradiation was done. After the therapies, his spleen got smaller and hematoma was going to be absorbed. A rare case of myelofibrosis with splenic hematoma is reported and the conservative therapies were effective to this case.

Adult↗
PubMed1989

[Interrelation of the osteogenic and hemopoietic tissues of the bone marrow during the development of estrogenic myelofibrosis].

By means of estrogenic myelofibrosis, using the method of heterotopic transplantation of the bone marrow, interrelations of osteogenic and hemopoietic tissues have been studied. Under estrone effect disorders in stromal microenvironment take place at the level of stem osteogenic cells of the bone marrow. Deficiency in cells of monocytic-macrophagal line, inhibiting proliferation of the bone marrow connective tissue cells, contributes to development of myelofibrosis. The hormone acts mainly in committed hemopoietic cells, singly launching them from G0- into S-period of the cycle, and then--into differentiation, accompanied with an enhanced discharge of cells from the bone marrow. There is neither direct, nor indirect dependence between the osteogenic mass and the cellularity of the hemopoietic tissue of the bone marrow. The changes, that take place in the bone marrow under estrone effect, are reversible.

Animals↗
PubMed1989

Acute myelofibrosis: correlation of radiographic, bone scan, and biopsy findings.

Acute myelofibrosis is a rare hematologic malignancy which usually follows a rapidly progressive, fatal course. A case of acute myelofibrosis and severe focal bone pain is described showing bone scan and plain radiograph findings. A biopsy of one of the abnormal bony areas revealed malignant cells, necrosis and fibrosis, and abnormal, exuberant new bone formation, which may account for what is seen on the radiographic and radionuclide studies. The etiology and optimal management of this rare and often rapidly fatal disease remain to be defined.

Acute Disease↗
PubMed1989

An autopsy case of myelodysplastic syndrome (MDS): diagnostic problems between MDS and the other haematopoietic disorders including acute myelofibrosis.

We report an autopsy case of myelodysplastic syndrome (MDS) in a 35-year-old male, who presented with pancytopenia and bleedings. Bone marrow specimens disclosed myelofibrosis and hypercellular marrow with more than 60% atypical erythroblasts in the bone marrow cells. Type I or type II blasts were less than 10% of the peripheral blood and bone marrow cells during the clinical course. At autopsy, infiltration by myeloid and erythroid cells and megakaryocytes was noted in the liver, spleen and lymph nodes. According to the FAB classification, this case might be classified into refractory anemia with excess of blasts (RAEB) or RAEB in transformation. However, the remarkable neoplastic proliferation of three haematopoietic cell lines also indicates acute myeloproliferative disorder such as acute myelofibrosis or acute panmyelosis.

Acute Disease↗
PubMed1979

[Quantitation of myelofibrosis in blood diseases by electronic image analysis (author's transl)].

Normal and pathologic reticulin networks colored black by silver nitrate can be automatically quantitated by electronic image analysis. By using this technique, different parameters can be obtained, such as the average density, the surface of network meshes, the thickness of the fibers, the complexity of the reticulum, and the heterogeneity of the myelofibrosis distribution. All of these parameters were obtained in 83 osteomedullar biopsies of blood diseases (primary splenomegaly, chronic myeloid leukemia, polycythermia vera, acute leukemia, and aplastic anemia). We have shown that there is no relation between the different parameters obtained and the medullary richness, hematopoietic center, or patient survival. On the other hand, the histomorphometric parameters can be used to distinguish acute leukemia and chronic myeloid leukemia myelofibrosis, while the parameters in primary splenomegaly are shown to be very heterogeneous.

Acute Disease↗
PubMed1988

Evidence for pluripotent stem cell origin of idiopathic myelofibrosis: clonal analysis of a case characterized by a N-ras gene mutation.

Three cases of idiopathic myelofibrosis were screened for the presence of mutations at codon 12, 13, or 61 of the ras gene family by a rapid method based on polymerase chain reaction and hybridization to mutation-specific oligonucleotides. PB cells of one patient showed a point mutation at codon 12 of the N-ras oncogene. This molecular genetic hallmark was used to investigate the clonal relationship of different cell lineages by cell separation analysis. Presence of the N-ras 12 mutation in granulocytes, monocytes, B cells, and T lymphocytes, as well as erythroblasts, indicates that idiopathic myelofibrosis originates from a pluripotent stem cell, at least in this patient.

Clone Cells↗
PubMed1979

Myelofibrosis--pathophysiology and treatment.

A case of myelofibrosis successfully treated by the use of splenectomy has been reported. The feeling of the authors is that splenectomy should be considered at a much earlier stage of the disease, when the patient is a better risk with a longer survival. We see no valid physiological reasoning for leaving a spleen in, which is valueless to the patient in addition to causing multiple side effects, directly and indirectly from its size and overactivity. This is especially true when attempts to reduce the size have potential hazards, none are completely effective and when the physiological necessity for this reticulo endothelial site seems to be unnecessary. Perhaps it would be of greatest value if all cases of myelofibrosis should be seen at the inception and throughout by the hematological and surgical service together.

Bone Marrow↗
PubMed1985

Myelofibrosis, autoimmune haemolytic anaemia and Tn-polyagglutinability.

A patient with myelofibrosis was studied for about four years. During this period he developed autoimmune haemolytic anaemia, a rare complication of myelofibrosis. Furthermore, about two years after the investigation started, his red blood cells, previously normal, became Tn-polyagglutinable, a change known to be due to somatic mutation in haemopoietic stem cells.

Anemia, Hemolytic, Autoimmune↗
PubMed1985

[Development of polycythemia vera during chronic idiopathic myelofibrosis].

A case of typical idiopathic myelofibrosis turning into polycythaemia vera 6 years after diagnosis is reported. This transformation occurred after treatment with busulphan. The present case is added to similar previous observations to give further support to the contention that the transition of chronic idiopathic myelofibrosis to polycythaemia vera may be a consequence, though rare, of therapy rather than a spontaneous event occurring during the course of the disease.

Adult↗
PubMed1979

[Malignant myelofibrosis. One case (author's transl)].

In a 75-year-old man, the rapid development of a pancytopenia as a result to total marrow failure, in the absence of tumour or extramedullary myelopoiesis, but with a histological appearance of the marrow identical to that seen in agnogenic myeloid metaplasia led to a diagnosis of malignant myelofibrosis. The patient died 7 months after the apparent onset of the disease. The 48 other published cases are discussed. Only 17 are considered to be true cases of malignant myelofibrosis.

Acute Disease↗
PubMed1978

Acute myelofibrosis with peripheral myeloblastosis: an acute myeloproliferative disorder.

Acute myelofibrosis is an uncommon fulminant disorder characterized by pancytopenia, premature myeloid elements in the peripheral blood, and bone marrow fibrosis. We report the case of a 59-year-old man who had acute myelofibrosis and peripheral myeloblastosis clinically suggesting the diagnosis of acute granulocytic leukemia. The disease was unresponsive to cytotoxic drugs or androgens and the patient died five months later. The association of bone marrow fibrosis with large numbers of myeloblasts in the peripheral blood has rarely been reported and suggests a spectrum of morphological changes in acute myeloproliferative disorders, analogous to the merging of chronic myeloproliferative disorders into one another and into leukemic blast crisis.

Bone Marrow↗
PubMed1982

Pathophysiological mechanisms operating in the development of myelofibrosis: role of megakaryocytes.

In this article current concepts on the regulation of bone marrow collagen are reviewed and a hypothesis regarding the mechanisms leading to marrow fibrosis associated with Primary Myelofibrosis (PMF) is presented. Type I and type III collagen, products of marrow fibroblasts, are the main constituents of myelofibrotic tissue and megakaryocytes are the predominant cells proliferating in PMF. There is evidence for the clonal nature of the hematopoietic cell proliferation and the secondary origin of myelofibrosis. Also, evidence exists indicating that defective megakaryocyte maturation, i.e. ineffective megakaryocytopoiesis occurs in patients with PMF. It is postulated that ineffective megakaryocytopoiesis leads to an excessive concentration of megakaryocyte components in the marrow intercellular space and that the development of marrow fibrosis involves mainly 2 megakaryocytic products: growth factor and factor 4. The growth factor stimulates fibroblast proliferation and collagen secretion. Factor 4 inhibits the activity of the enzyme collagenase. Thus, the imbalance between increased collagen production and decreased collagen degradation leads to an excessive deposition of collagens in bone marrow matrix.

Bone Marrow↗
PubMed1983

The reversal of myelofibrosis associated with chronic myelogenous leukemia after allogeneic bone marrow transplantation.

A 20-year-old male with chronic myelogenous leukemia (CML) and severe bone marrow fibrosis underwent splenectomy, then ablative chemotherapy, followed by bone marrow transplantation from a histocompatible sister. The myelofibrosis completely resolved. Prompt marrow engraftment and disappearance of Philadelphia chromosome positive cells were documented. Therefore, the presence of marrow fibrosis which frequently accompanies CML is rapidly reversible following high dose chemotherapy and is not indicative of a hostile microenvironment which could preclude marrow transplantation for patients with CML and myelofibrosis.

Adult↗
PubMed1984

Acute myelofibrosis. A report of four cases and review of the literature.

Four new cases of acute myelofibrosis are reported, and 63 cases reported in the literature are reviewed. The typical features of this disease include a rapidly progressive clinical course; nonspecific symptoms such as weight loss, anorexia, fatigue and weakness; the absence of organomegaly; pancytopenia; circulating blast cells; and mild abnormalities in the red blood cell morphology. The bone marrow aspirates are usually "dry." The bone marrow biopsies are essential for the diagnosis and show four consistent features: hypercellularity, reticulin fibrosis, proliferation of blast cells and bizarre, atypical megakaryocytes. In 16 cases, the blast cells in peripheral blood and bone marrow, which are unclassifiable by conventional morphology, could be identified as megakaryoblasts by ultrastructural and immunocytochemical techniques. It is concluded that acute myelofibrosis is a definite clinicopathologic entity, which may be related to acute megakaryoblastic leukemia.

Acute Disease↗