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At least 217 records · Page 12Linked to original sources
PubMed1990

Esophageal cancer complicated by acute myelofibrosis--report of an autopsy case.

An autopsy case of esophageal cancer complicated by myelofibrosis was studied. A 62-year-old Japanese patient with esophageal cancer, received surgical treatment and then splenectomy. The resected spleen was normal in appearance. Five months later he received radiation therapy for the cancer. After the therapy, his blood profile revealed thrombocytopenia and leukoerythroblastosis, and the bone marrow punctures were dry tap. He died of disseminated fungal infections at eleven months after the first operation. An autopsy of the patient revealed a striking increase in the megakaryocytes and a moderate increase in the reticulin fibers in the bone marrow. Myeloid metaplasia was noted in the lymph nodes, kidneys, and other organs and tissues, although the lymph nodes were not grossly swollen. These findings suggest esophageal cancer complicated by acute myelofibrosis. This it the first case report on esophageal cancer complicated by acute myelofibrosis.

Acute Disease↗
PubMedDate not supplied

[Glomerular disease associated with myelofibrosis (author's transl)].

The authors report the case of a 46 year-old patient presenting with membranous-proliferative glomerular disease, megakaryocytes present in the glomerular capillaries, evolving concomitantly with myelofibrosis. There are two possible explanations for this unusual association: the glomerular disease and the myelofibrosis may both result from the same etiologic and pathogenic factor, or the glomerular disease may be the consequence, thrombocytosis existing, of platelet activation, either direct or after deposition of immune complexes. The formation of immune complexes after antigenic stimulation in myelofibrosis is theoretically compatible with immunitary anomalies found in the evolution of this disorder as described in the literature.

Humans↗
PubMed1990

The production of transforming growth factor-beta in acute megakaryoblastic leukemia and its possible implications in myelofibrosis.

Acute myelofibrosis is often associated with acute megakaryoblastic leukemia (AMKBL). Although the exact mechanism for the progression of myelofibrosis in AMKBL is unclear, certain humoral factors from megakaryoblastic cells, the precursors of platelets, may be involved in the enhancement of collagen synthesis by bone marrow fibroblasts. The present study, therefore, is an investigation of the possible pathogenic role of transforming growth factor-beta (TGF-beta), known to be a very potent collagen-stimulating factor found in platelets in the myelofibrosis of AMKBL. The results obtained were as follows: (1) Conditioned media from peripheral megakaryoblasts taken from an AMKBL patient and from established megakaryoblast cell lines (MEG-01) had much greater stimulatory effects on collagen synthesis in bone marrow fibroblasts than conditioned media from other leukemic cell types. (2) Based on an assessment of soft agar colony formation, there was greater TGF-beta activity in media that had been conditioned from megakaryoblasts than in media from other leukemic cell types. (3) When compared with other leukemic-cell types, megakaryoblasts showed substantially greater expression of TGF-beta mRNA that was hybridized at 2.5 kb with a TGF-beta cDNA probe, and TGF-beta polypeptides were detected at 13 Kd with anti-TGF-beta antibodies. (4) The addition of the anti-TGF-beta antibody inhibited the stimulatory effects of the megakaryoblast conditioned medium on collagen synthesis in bone marrow fibroblasts. These results clearly suggest that megakaryoblasts produce and secrete an active form of TGF-beta and stimulate collagen synthesis in bone marrow fibroblasts in a paracrine manner.

Animals↗
PubMed1989

[Peripheral T-cell lymphoma initially presenting as secondary myelofibrosis].

A case of peripheral T-cell lymphoma presenting with secondary myelofibrosis and meningeal involvement is described. A 65-year-old female was admitted because of remarkable weight loss and pancytopenia. On admission, she was confused and showed tiny cervical lymph nodes but no hepatosplenomegaly. Bone marrow aspiration resulted in dry tap and its biopsy showed remarkable myelofibrosis with marked decrease of hematopoiesis and increase of lymphoid cells. Lymph node biopsy revealed diffuse medium sized cell lymphoma, which was diagnosed as CD3+4+8-peripheral T-cell lymphoma with immunohistochemistry (anti-HTLV-1 antibody negative). The lymphoid cells of bone marrow expressed the markers of T-cell lineage (LCA+ UCHL1+ MT1+ L26- MB1-). The cerebrospinal fluid examination revealed many lymphoma cells. She was treated with CHOP regimen and intrathecal injection of MTX. After three months, bone marrow biopsy showed recovery of hematopoiesis and disappearance of lymphoma cells and reticulin fibers. Immunohistochemical analysis of bone marrow specimen was useful for the diagnosis of atypical myelofibrosis.

Aged↗
PubMed1989

[The use of MCNU to a patient of primary myelofibrosis complicated with pericardial effusion and proteinuria].

A case of primary myelofibrosis complicated with pericardial effusion and proteinuria is described. A 66-year-old female was admitted to our hospital because of abdominal fullness and shortness of breath. On admission, hepatosplenomegaly and pericardial effusion were observed. Blood examination revealed leukoerythroblastic anemia and thrombocytosis with tear drop cells and giant platelets. Bone marrow aspiration was dry tap and its biopsy showed remarkable myelofibrosis. Urinalysis indicated severe proteinuria. Although neutrophilic alkaline phosphatase score was low, no signs of acute blastic crisis of chronic myelogenous leukemia was found. The diagnosis of an atypical type of primary myelofibrosis was obtained. Administration of MCNU was started in August 1987. Hepatosplenomegaly, pericardial effusion and proteinuria were gradually improved after the administration. The etiology of the pericardial effusion and proteinuria were not obvious, however, these facts suggest that these abnormal findings might be related to PMF itself and MCNU was effective to PNF.

Aged↗
PubMed1988

[Idiopathic myelofibrosis. Radiological aspects of bone changes].

Idiopathic myelofibrosis is a chronic myeloproliferative disease characterized by skeletal lesions (30 to 70% of cases). We considered 49 patients with idiopathic myelofibrosis treated between 1972 and 1986 at the Institutes of Hematology and Radiotherapy, University of Bologna. Only 19 of these patients underwent roentgenographic skeletal surveys, associated with whole body bone scintigraphy in 4 cases, and with CT in 1 case. The most common bone change, as seen in 12 patients, was osteosclerosis, following two distinct patterns: pure, and mixed. Other types of bone involvement (osteoporosis and pure osteolysis) were seen in 2 cases only; in 5 patients radiological skeletal examinations did not show meaningful lesions. Conventional radiology is hardly ever conclusive in the diagnosis of idiopathic myelofibrosis. This is due partly to the often moderate degree of bone involvement, partly to the scanty specificity of the findings. However, a careful examination of the plain roentgenographs, completed when possible by other more recent imaging techniques, may be very important towards a more precise definition of the disease and, in some cases, for a correct diagnosis.

Adult↗
PubMed1987

Plasma fibronectin in idiopathic myelofibrosis and related chronic myeloproliferative disorders.

Plasma fibronectin concentrations were measured in 49 patients with chronic myeloproliferative disorders and compared to sex- and age-matched controls. A significantly lower plasma fibronectin concentration was observed in patients with idiopathic myelofibrosis as compared with the control group (p less than 0.01). In addition, plasma fibronectin concentrations in myelofibrosis patients differed significantly, when compared with patients with polycythaemia vera (p less than 0.01), whereas no significant difference was found between myelofibrosis patients and those with a transitional myeloproliferative disorder or chronic myelogenous leukaemia (p greater than 0.05). An inverse relationship was demonstrated between plasma fibronectin and spleen size, the lowest plasma fibronectin levels being found in patients with large spleens. It is supposed that low plasma fibronectin concentrations in splenomegalic patients may be due to enhanced consumption of the opsonin in the expanded splenic mononuclear-macrophage system.

Adult↗
PubMed1987

Amyloidosis complicating idiopathic myelofibrosis.

Three cases of amyloidosis occurring in the later course of idiopathic myelofibrosis were studied at autopsy. In these cases, the amyloid deposition was seen only in the renal glomerulus, which had caused proteinuria. Although their exact nature could not be determined by immunohistochemistry, the amyloid deposits in the three cases were permanganate resistant and exhibited the same organ distribution, indicating a similarity in their character. These cases suggest that amyloidosis might be a complication of idiopathic myelofibrosis, implying that idiopathic myelofibrosis could be a disorder underlying amyloidosis.

Aged↗
PubMed1986

[Hypocalcemia and myelofibrosis: an unrecognized association].

Observation of a case of myelofibrosis with myeloid metaplasia of liver and spleen associated with hypocalcemia led us to investigate whether this association was fortuitous. We retrospectively analyzed the data of 30 patients with myelofibrosis and identified 9 patients with hypocalcemia out of 22 in whom the plasma calcium level corrected for serum protein could be obtained, i.e. a prevalence of hypocalcemia of 41%. The spleen was significantly larger in the patients with hypocalcemia than in those with normal plasma calcium level, suggesting that hypocalcemia was related to the duration of the disease. In 5 patients with hypocalcemia, biological and/or morphological evidence of osteomalacia was found. It is concluded that an association exists between myelofibrosis and hypocalcemia, possibly as a consequence of a disturbed vitamin D metabolism.

Aged↗
PubMed1984

Therapeutic implications of collagen metabolism in myelofibrosis.

Myelofibrosis is an idiopathic condition of man associated with increased deposition of bone marrow collagen. This is directly seen on bone marrow reticulin or collagen staining, and reflected by increased levels of serum procollagen III aminoterminal peptide (28). It is but one of a number of diseases in which collagen, and an aberration of the connective tissue matrix, influence normal anatomy and physiology. Animal experiments have shown that the therapeutic manipulation of the intra- and extracellular processing of collagen with various agents can influence its deposition and the degree of end-organ damage. Several of these drugs have been used therapeutically for various clinical conditions and deserve clinical trials to evaluate their effectiveness in myelofibrosis. Bone marrow fibrosis, and peripheral serum markers which reflect collagen metabolism could be followed as experimental parameters. Development of an effective and safe antifibroblastic therapy is awaited eagerly. An attempt to prevent the fibrosis found in myelofibrosis, as opposed to managing its complications, would revolutinize our approach to managing these patients.

Aminopropionitrile↗
PubMed1983

The syndrome of idiopathic myelofibrosis. A clinicopathologic review with emphasis on the prognostic variables predicting survival.

We describe here a series of 88 consecutive patients with bone marrow fibrosis. Primary causes for the fibrosis were discovered in 26% of the cases shortly after the initial diagnosis. Pathology review of the remaining cases revealed an 8% incidence of "hairy cell leukemia" that had escaped detection originally. The remaining cases, characterized as having "unexplained bone marrow fibrosis" or "idiopathic myelofibrosis," are the subject of this study. The clinical and laboratory findings are compared to those reported in previous series of selected cases with similar features in which patients were diagnosed as having "agnogenic myeloid metaplasia," "myelosclerosis," or "myelofibrosis." A brief summary of the treatment modalities used, and the clinical course and outcome of these patients are also presented. There was a marked variability in the clinical severity of the disease and in the survival of these patients. A detailed statistical analysis of 40 variables at the time of initial diagnosis showed that the factors that best predicted a poor survival were unexplained fever, weight loss, night sweats, anemia and thrombocytopenia. On the other hand, the size of the spleen or of the liver, the degree of immaturity of the peripheral blood white cells, and the degree of fibrosis or cellularity in the bone marrow biopsy were of no detectable prognostic significance. These findings suggest that in patients with unexplained fibrosis of the bone marrow (the syndrome of idiopathic myelofibrosis) a poor prognosis is not a direct consequence of the marrow fibrosis or the associated extramedullary hematopoiesis, but rather is related to the presence and/or the severity of some unexplained primary marrow defect, which is also often associated with the nonspecific symptoms of a systemic illness.

Adolescent↗
PubMed1994

[Hematologic abnormalities in a patient with chronic myelogenous leukemia with advanced myelofibrosis were improved by G-CSF].

A 56-year-old woman was admitted with pyrexia, cough, and dyspnea on August 21, 1991. Physical examination revealed anemia in the palpebral conjunctivas and moist rales at the right lower lung field. Neither the Liver nor spleen was enlarged. Examination of the peripheral blood showed a hemoglobin level of 8.1 g/dl, a platelet count of 14.8 x 10(4)/microliters, and a white blood cell count of 2,800/microliters, with 7% blasts and 8% megakaryocytes. Tear drop-like erythrocytes, agranular neutrophils, and erythroblasts were also seen in the peripheral blood. Examination of the bone marrow showed 15% peroxidase positive blasts, and many micromegakaryocytes. Cytogenetic studies for bone marrow cells revealed the existence of the Philadelphia (Ph1) chromosome. Bone marrow biopsy showed normal cellularity with increase of megakaryocytes and advanced myelofibrosis. Breakpoint cluster region (bcr) rearrangement analysis using the peripheral blood mononuclear cells revealed M-bcr rearrangement. According to the Hannover classification for myeloproliferative disease, she was diagnosed as having CML with advanced myelofibrosis followed by CML with megakaryocytic increase. Since she had neutrocytopenia and severe infectious disease, she received a subcutaneous injection of 125 micrograms of G-CSF. Not only increase of the white blood cell count, but also disappearance of blasts, improvement of anemia, increase of the platelet count, and improvement of myelofibrosis were observed.

Blood Cell Count↗
PubMed1995

Multiple myeloma with bone marrow biopsy features simulating concomitant chronic idiopathic myelofibrosis.

Multiple myelomas occasionally exhibit bone marrow lesions simulating a concomitant chronic idiopathic myelofibrosis. In the present study, trephine biopsy histologies of such "myelofibrotic" myelomas are described and compared to those from a case of true chronic idiopathic myelofibrosis which developed in the course of chronic lymphocytic leukaemia. "Myelofibrotic" myeloma are characterized by osteosclerosis, marrow fibrosis and focal megakaryocytic hyperplasia in the presence of plasma cell infiltration of the bone marrow. These myelomas are to be distinguished from the more commonly occurring multiple myeloma with simple marrow fibrosis and/or osteosclerosis. Furthermore, "myelofibrotic" myelomas are not identical to myelomas coexisting with true chronic idiopathic myelofibrosis, a condition which would appear to be extremely rare and should only be diagnosed if focal megakaryocytic hyperplasia with atypia can be unequivocally demonstrated. Avoidance of misinterpretation of "myelofibrotic" myeloma requires a knowledge of these different myeloma variants.

Adult↗
PubMed1995

Increased proliferation of bone marrow-derived fibroblasts in primitive hypertrophic osteoarthropathy with severe myelofibrosis.

Pachydermoperiostosis or primary hypertrophic osteoarthropathy (HOA) is a rare congenital growth disorder of connective tissue. We report a case of severe myelofibrosis in a patient with HOA. When cultured in vitro, patient bone marrow-derived fibroblasts displayed a high proliferative potential with a shortened doubling time (24 hours v 36 to 48 hours for normal fibroblasts). The role of platelet-derived growth factor (PDGF), previously implicated in the pathogenesis of secondary acquired myelofibrosis, was studied. HOA fibroblasts expressed an increased number of PDGF-BB binding sites (300,000 sites/cell v 200,000 sites/cell for normal fibroblasts) without any modification of affinity. The increased expression of PDGF-R beta appeared to result from an accelerated rate of PDGF-R beta resynthesis with normal kinetics of endocytosis. As a consequence, a several-fold increase of PDGF-R beta tyrosine kinase activity was observed. No autocrine mechanism of growth was suspected as neither spontaneous PDGF-R beta autophosphorylation nor mitogenic activity in HOA fibroblast-conditioned medium was detected. Patient serum and platelet lysate were less potent than controls in inducing [3H]thymidine incorporation into HOA fibroblasts. This was inconsistent with a paracrine mechanism of growth. In vitro, human serum or PDGF-BB were not more mitogenic for HOA than normal fibroblasts. High levels of cyclin D1, a putative oncogene, were detected in serum-deprived HOA fibroblasts. Cyclin D1 overexpression could be implicated in the accelerated growth of these cells. Our results suggest that the mechanism of fibroblastic proliferation observed in this case of myelofibrosis might differ from those reported in other acquired myeloproliferative syndromes and could be associated with an intrinsic abnormality of HOA fibroblast growth.

Anemia↗
PubMed1995

[Transformation into chronic myelomonocytic leukemia in a patient with primary myelofibrosis associated with severe hypoplasia: report of an autopsy case].

A 70-year-old male was admitted because of anemia in September 1989, and primary myelofibrosis was diagnosed based on the presence of leukoerythroblastosis, a normal chromosomal analysis and pathological findings of fibrosis in bone marrow. Although he was anemic, he did not require any treatment for two years. Then his hematological status deteriorated to severe pancytopenia, and the marrow biopsy revealed marked hypoplasia with fatty replacement and scattered fibrosis. He was treated with metenolon without success and frequent transfusion of packed red cell was required. This hypoplastic status continued for seven months. In May 1992 his WBC count increased gradually with monocytosis. The marrow was filled with various stages of monocytes, with almost no fibrosis remaining. The chromosomal analysis was repeated but disclosed no abnormalities, consistent with the negative result of BCR-ABL rearrangement investigated by the RT-PCR method. One month later, when the patient died of multiple cerebral bleeding and infection, the leukocyte count exceed 90,000/microliters. It is known that major causes of death for patients with primary myelofibrosis are infection, bleeding, cardiac trouble and transformation to leukemia. We describe a case of myelofibrosis who developed to chronic myelomonocytic leukemia following severe aplastic phase.

Aged↗
PubMed1994

[Hypocholesterolemia and other lipoprotein disorders in myelofibrosis].

In the recent years more and more data suggest a significant relationship between malignant diseases and cholesterol, respectively lipoprotein metabolism. It is a significant decrease of cholesterol in primary myelofibrosis (agnogenic myeloid metaplasia) and in secondary myelofibrosis. Similarly, the HDL and LDL cholesterol levels are also decreased in these diseases. On the contrary, the triglyceride level is significantly higher in these groups, 41 patients with agnogenic myeloid metaplasia and 16 patients with myelofibrosis following polycythaemia vera were examined. Decrease of serum cholesterol level was significant: 3.7 and 3.3 mmol/l and 5.2 mmol/l in 76 healthy controls, as the mean values.

Aged↗
PubMed1994

[Mycobacterium kansasii lung infection associated with myelofibrosis--a case refractory to treatment with antitubercular agents].

A case of Mycobacterium kanasasii lung infection associated with myelofibrosis is reported. A 32-year-old woman was admitted to Keio University Hospital because of fever. One year before admission, a diagnosis of myelofibrosis was made at another hospital. The initial chest X-ray film showed bilateral diffuse infiltrative shadows and right hilar enlargement. Sputum cultures yielded Mycobacterium kansasii on 3 occasions. A fever of 38 degrees C or higher persisted for about 4 months despite the use of antitubercular agents, including INH, RFP, SM, and PZA. During the course of treatment, the hilar and mediastinal lymph node enlargement became more severe, and this was followed by calcification of the nodes. The fever and chest X-ray findings improved in response to the addition of treatment with EB, Ofloxacin, TH, and an increase in the doses of INH (from 300 to 500 mg/day) and RFP (from 450 to 600 mg/day). The patient was discharged 14 months after admission. It is rare especially in Japan for a Mycobacterium kansasii lung infection (not disseminated) to persist in spite of treatment with antitubercular drugs, including RFP or TH. The response to treatment may have been impaired by an immunological disorder associated with the myelofibrosis.

Adult↗
PubMed1996

Acute myelofibrosis in children: report on two cases.

In our report, myelofibrosis in children is discussed and two cases of acutely developing myelofibrosis in association with acute megakaryoblastic leukaemia (M7) are presented. In the first case (girl, 34 months), it was acute myelofibrosis of hypocellular marrow. Diagnosis of M7 was confirmed by positive reaction of blasts from peripheral blood with CD42 and CD61 monoclonal antibodies. In the other child (girl, 5 years old), Ph1(+) chronic myeloid leukaemia diagnosed 22 months earlier transformed to M7. Similar to the first case, no marrow aspirate could be obtained and the diagnosis of M7 was made by the bone marrow histology that showed the presence of grossly fibrosed, hypercellular marrow with a large number of dysplastic, maturing megakaryocytes. Neither of the children had Down's syndrome. Although according to FAB classification both cases represent the same haematological entity, their clinical and histopathological presentations are very different.

Child, Preschool↗