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PubMed1995

An isochromosome of the long arm of chromosome 18 in a patient with myelodysplastic syndrome with myelofibrosis.

A case of myelodysplastic syndrome (MDS) with myelofibrosis and i(18q) is reported. The patient, a 29-year-old Chinese man, was noted to be anemic over a 10-year period. Recently, his spleen became progressively massive and bone marrow aspirates yielded "dry taps" on several occasions. Hematologic investigation disclosed pancytopenia, numerous nucleated red cells, and slightly increased myeloblasts (8%) in the peripheral blood. Bone marrow aspirate and biopsy revealed hypercellular marrow, trilineage dysplasia, and significant reticulin fibrosis, but without collagen formation. Bone marrow karyotypic analysis with R-banding showed an isochromosome 18q as a sole abnormality in 20 of 24 metaphases analysed. The patient died of severe anemia and bleeding due to bone marrow failure. We believe that i(18q) and myelofibrosis may be related to his poor prognosis.

Adult↗
PubMed1990

Cutaneous extramedullary hematopoiesis in myelofibrosis.

Erythematous papulonodules that resembled a malignant lymphoma developed in a 62-year-old man. Further examination revealed that he had primary myelofibrosis with cutaneous extramedullary hematopoiesis. All three marrow elements (myeloid, erythroid, and megakaryocytic series) were present in the skin lesions. Although extramedullary hematopoiesis of the skin is a rare complication of myelofibrosis, 13 similar cases have been reported. In nine cases, including ours, all three marrow elements were present in the cutaneous lesions.

Aged↗
PubMed1988

Hypothesis: a possible role for interferon in the treatment of idiopathic myelofibrosis.

It is proposed that interferon may be an active agent in the treatment of patients with idiopathic myelofibrosis. In this disorder the megakaryocyte cell lineage plays a major role in the deposition of bone marrow collagen by the release of growth promoting factors, including platelet-derived growth factor, which are mitogenic for fibroblast proliferation. Interferon reduces collagen deposition in the bone marrow by suppressing the activity of the proto-oncogene, which is involved in the production of growth factors from abnormal megakaryocytes and platelets. A direct myeloid cytoreductive effect of interferon upon the megakaryocyte proliferation contributes to reducing growth factor activity in the bone marrow. Finally, interferon induces monocytoid differentiation, thereby increasing bone marrow collagenase-activity. Thus, interferon has several actions, which in concert might reduce bone marrow collagen in myelofibrosis.

Bone Marrow↗
PubMedDate not supplied

Myelofibrosis associated with prominent periosteal bone apposition. Report of two cases.

Myelofibrosis is a myeloproliferative disorder that is characterized by splenomegaly and bone marrow replacement by fibrous tissue. The predominant radiographic feature is osteosclerosis; however, in rare instances, periosteal bone apposition or periostitis is apparent in the metaphysis of the distal femura and proximal tibiae. It has been suggested that periostitis, when associated with fever and bone pain, is indicative of more aggressive disease. We report this unusual radiographic finding and its similar appearance to hypertrophic osteoarthropathy in two patients with myelofibrosis. In our patients, the presence of periosteal bone apposition did not correlate with increased disease aggressiveness.

Aged↗
PubMed2005

Hairy cell leukemia (HCL) with extensive myelofibrosis responds to thalidomide.

We present a 75-year-old man who was admitted to our hospital because of splenomegaly, transfusion-dependent anemia and thrombocytopenia. The diagnosis of idiopathic myelofibrosis was suggested by the bone marrow trephine biopsy, which was hypocellular with myelofibrosis. Thalidomide was started at a daily dose of 100 mg/d and increased to 400 mg/d. Within 12 weeks, thrombocytes increased to 100.000/microl, hemoglobin normalized, lasting for about 11 months. Then, thalidomide had to be discontinued because of mild polyneuropathy. A second bone marrow biopsy showed fibrosis as well as a diffuse infiltration of the bone marrow (80%) by lymphocytes with expression of CD11c, CD19, CD20, CD103. Tartrate resistant acid phosphatase test (TRAP) was also positive. Hairy cell leukemia was diagnosed and he was administered 2-chlorodeoxyadenosine (2-CDA) achieving a complete remission.

Aged↗
PubMed2004

Impaired GATA-1 expression and myelofibrosis in an animal model.

The genetic lesion(s) underlying chronic idiopathic myelofibrosis, as well as the mechanisms leading to the typical fibro-osteosclerotic changes of the bone marrow microenvironment, are still undefined. Recently, animal models of the disease have been described. We will briefly review the characteristics of these models, the thrombopoietin-overexpressing mice and the GATA-1(low) mice (mice deficient for GATA-1 expression in megakaryocytes), and illustrate how they provided insights into pathogenetic mechanisms of myelofibrosis, with special regard to the role of abnormal megakaryocyte proliferation and maturation.

Animals↗
PubMed1992

Idiopathic myelofibrosis in pregnancy: a case report and review of the literature.

Idiopathic myelofibrosis is a rare myeloproliferative disorder characterized by excessive accumulation of connective tissue in the bone marrow in association with anemia, splenomegaly, and extramedullary hematopoiesis. The cause of this disease is unknown, and the prognosis is generally poor. To our knowledge, this is the first case report of a patient with idiopathic myelofibrosis who carried a term pregnancy. In spite of the increased perinatal risks, a favorable outcome was possible with close antepartum surveillance.

Adult↗
PubMed2005

Abnormalities of GATA-1 in megakaryocytes from patients with idiopathic myelofibrosis.

The abnormal megakaryocytopoiesis associated with idiopathic myelofibrosis (IM) plays a role in its pathogenesis. Because mice with defective expression of transcription factor GATA-1 (GATA-1(low) mutants) eventually develop myelofibrosis, we investigated the occurrence of GATA-1 abnormalities in IM patients. CD 34(+) cells were purified from 12 IM patients and 8 controls; erythroblasts and megakaryocytes were then obtained from unilineage cultures of CD 34(+) cells. Purified CD 61(+), GPA(+), and CD 34(+) cells from IM patients contained levels of GATA-1, GATA-2, and FOG-1 mRNA, as well as of GATA-2 protein, that were similar to controls. In contrast, CD 61(+) cells from IM patients contained significantly reduced GATA-1 protein. Furthermore, 45% of megakaryocytes in biopsies from IM patients did not stain with anti-GATA-1 antibody, as compared to controls (2%), essential thrombocythemia (4%), or polycythemia vera (11%) patients. Abnormalities in immunoreactivity for FOG-1 were not found, and no mutations in GATA-1 coding sequences were found. The presence of GATA-1(neg) megakaryocytes in bone marrow biopsies was independent of the Val 617 Phe JAK 2 mutation, making it unlikely that a downstream functional relationship exists. We conclude that megakaryocytes from IM patients have reduced GATA-1 content, possibly contributing to disease pathogenesis as in the GATA-1(low) mice and also representing a novel IM-associated marker.

Antibodies↗
PubMed1998

Molecular evidence of bone marrow involvement in advanced case ot Tgammadelta lymphoma with secondary myelofibrosis.

We describe the case of a middle-aged man with long indolent course of generalized Tgammadelta lymphoma. The onset of secondary myelofibrosis made cytological monitoring of the bone marrow infiltrates impossible. As during progression of the disease splenectomy revealed typical histological features of a high-grade hepatosplenic Tgammadelta lymphoma, the low-grade bone infiltrate was considered a secondary lymphoma. The use of the polymerase chain reaction helped to detect a constant and identical monoclonal rearrangement pattern of the T-cell receptor gamma-chain gene in both bone marrow and splenic T-cell infiltrates. The notion of a secondary spread of malignant T-cells to the bone marrow was thereby confirmed despite striking cytological differences between bone marrow and splenic infiltrates. This is the first report of a diagnostic DNA-based molecular approach using fixed decalcified bone marrow. This method may provide a major tool when dealing with myelofibrosis, which normally hampers sampling of cytological specimens.

Adult↗
PubMed2003

Karyotypic abnormalities in myelofibrosis following polycythemia vera.

Polycythemia vera (PV) is a chronic myeloproliferative disease characterized by an increase of total red cell volume; in 10% to 15% of cases, bone marrow fibrosis complicates the course of the disease after several years, resulting in a hematologic picture mimicking myelofibrosis with myelocytic metaplasia (MMM). This condition is known as post polycythemic myelofibrosis (PPMF). Among 30 patients with PPMF followed in Northern France, 27 (90%) expressed one or two abnormal clones in myelocytic cell cultures. Of these, 19 (70%) had partial or complete trisomy 1q. This common anomaly either resulted from unbalanced translocations with acrocentric chromosomes, that is, 13, 14, and 15, or other chromosomes, that is, 1, 6, 7, 9, 16, 19, and Y, or from partial or total duplication of long arm of chromosome 1. A single patient had an isochromosome 1q leading to tetrasomy 1q. In all cases, a common trisomic region spanning 1q21 to 1q32 has been identified. Given that most patients had previously received chemotherapy or radio-phosphorus to control the polycythemic phase of their disease, this study illustrates the increased frequency of cytogenetic abnormalities after such treatments: 90% versus 50% in de novo MMM. Moreover, karyotype can be used to distinguish PPMF-where trisomy 1q is the main anomaly-from primary MMM where trisomy 1q is rare and deletions 13q or 20q are far more common. Whether trisomy 1q is or is not a secondary event remains a matter of debate, as well as the role of cytotoxic treatments.

Adult↗
PubMed1999

Amyloidosis complicating idiopathic myelofibrosis.

An 84-year-old man presented with ankle edema, significant proteinuria, and mild impairment of renal function soon after treatment was started for idiopathic myelofibrosis. Renal amyloidosis was found on biopsy. The amyloid deposit was resistant to potassium permanganate treatment and showed no immunoreactivity to immunoglobulin light chains, beta-amyloid protein, or amyloid A component. A review of the literature showed that the occurrence of amyloidosis in idiopathic myelofibrosis is very rare, and the chemical nature of the amyloid involved remains unclear.

Aged↗
PubMed2003

SU6668 in idiopathic myelofibrosis--a rational therapeutic approach targeting several tyrosine kinases of importance for the myeloproliferation and the development of bone marrow fibrosis and angiogenesis.

Idiopathic myelofibrosis is a chronic myeloproliferative disorder being featured by progressive accumulation of connective tissue in concert with marked neovascularization (angiogenesis) of the bone marrow. Both fibrogenesis and angiogenesis are considered to develop consequent to the intramedullary release of various growth-promoting factors from rapidly proliferating and dysplastic megakaryocytes. Among these growth factors are platelet-derived growth factor (PDGF), basic fibroblast growth factor (bFGF), transforming growth factor beta (TGF-beta) and vascular endothelial growth factor (VEGF). The protein kinase inhibitor SU6668 is a potent antiangiogenic inhibitor of receptor tyrosine kinases, including those of VEGFR, PDGFR, bFGFR, and c-kit. The hypothesis is that SU6668 may be an effective agent in the treatment of idiopathic myelofibrosis. This compound has an inhibitory target profile on several tyrosine kinases involved in the myeloproliferation, the development of myeloid metaplasia (bFGFR, PDGFR, VEGFR, and c-kit) and the development of the major stromal changes in the bone marrow - fibrosis and angiogenesis (bFGFR, PDGFR, and VEGFR).

Bone Marrow↗
PubMed2003

Myelofibrosis with myeloid metaplasia.

MMM is a chronic myeloproliferative disorder characterized by bone marrow fibrosis and neoangiogenesis, constitutive release ofa high number of CD34+ stem cells from the bone marrow, and extramedullary hematopoiesis. It presents with heterogeneous clinical features in which anemia and progression to symptomatic splenomegaly dominate. The pathogenesis is undefined, but the dual action of deregulation of the bFGF pathway may influence myeloproliferation, myelofibrosis, and neoangiogenesis. Animal models suggest that chronic exposure to high doses of thrombopoietin or impairment of the capacity of megakaryocytes to differentiate into platelets, as occurs in the GATA-1(low) mice, is a necessary event for myelofibrosis. Allogeneic stem cell transplantation offers a chance of cure, and low conditioning regimens may extend the age of transplantable patients with lower mortality. Autologus stem cell transplantation and splenectomy are risky procedures that may be considered in patients with advanced disease when conventional therapies for correcting anemia (danazol, recombinant human erythropoietin, or cyclosporine) or chemotherapy for splenomegaly and myeloproliferation (hydroxyurea or interferon alfa) have failed. Thalidomide has been tested in numerous series, and its capacity to improve anemia and thrombocytopenia while reducing splenomegaly has been documented.

Diagnosis, Differential↗
PubMed1987

Acute myelofibrosis and multiple chloromas of the mandible and skin.

Acute myelofibrosis is a rare clinical entity characterized by severe marrow fibrosis and peripheral blood pancytopenia. It may be very closely related to and has been considered synonymous with megakaryocytic leukemia. Chloroma is also a rare entity composed of a localized collection of immature myeloid cells and is associated with a known or covert leukemia. A patient presenting with a rare combination of acute myelofibrosis, megakaryocytic leukemia and multiple chloromas of the mandible and skin, are described. Although presenting a diagnostic challenge, their combination in one patient aids in the understanding of the myeloproliferative process. To our knowledge, this is the 3rd case of chloroma involving the oral osseous structures.

Acute Disease↗
PubMed1996

[Myelofibrosis regressing under corticotherapy and intravenous immunoglobulins in an infant].

BACKGROUND: Primary myelofibrosis is rare in infants and children; its association with auto-immune markers has only been reported in adults. CASE REPORT: An 8 month-old girl was admitted because of severe anemia and neutropenia. The marrow aspirate showed dysgranulopoiesis and partial interruption of maturation after the myelocyte level. The bone marrow biopsy revealed reticulinic myelofibrosis. The condition worsened with development of agranulocytosis and thrombocytopenia. Investigations ruled out malignant hemopathy, metastatic infiltration of the marrow and osteopathy. A myelodysplastic syndrome was discussed, but presence of anti-granulocyte auto-antibodies and positive Coombs test led to consider an autoimmune etiology. A corticosteroid therapy was attempted, effective only on the platelet lineage. Addition of intravenous gammaglobulin therapy corrected the problem. After a 24 month-course of the disease, it was necessary to prolong therapy. CONCLUSION: The efficacy of gammaglobulins may be an additional argument for auto-immunity, although no other auto-immune pattern has been observed in our patient, contrary to reported cases in adults.

Combined Modality Therapy↗
PubMed1997

Spontaneous myelofibrosis in castrated and ovariectomized NMRI mice.

The histopathological appearance of myelofibrosis of the bone marrow is described in aged castrated males, ovariectomized females and in male and female control NMRI mice. The highest incidence of the lesion was observed in ovariectomized and female control mice where more than 90% of the animals were affected. The presence of myelofibrosis in the bone marrow of ovariectomized females and castrated males indicates that estrogens may not play a major role in the development of the lesion and other hormonal disturbances must also be considered.

Age Factors↗
PubMed2001

Megakaryopoiesis and myelofibrosis in chronic myeloid leukemia after allogeneic bone marrow transplantation: an immunohistochemical study of 127 patients.

An immunohistochemical and morphometric study was performed on 363 trephine biopsies of the bone marrow derived from 127 patients with chronic myeloid leukemia at standardized end points before and after allogeneic bone marrow transplantation (BMT). The purpose of this investigation was to evaluate features of CD61+ megakaryopoiesis related to successful engraftment. Further, we tried to elucidate possible associations of this lineage, including precursor cells, with the platelet count and reticulin fibrosis during the pretransplant and, specifically, post-transplant periods. A significant correlation was recognizable between the quantity of CD61+ megakaryocytes and the platelet values before BMT and also after completed hematopoietic recovery. In the very early post-transplant period, which is associated with severe thrombocytopenia, patchy regeneration of disarranged hematopoiesis occurred, including dysplastic megakaryocytes. According to planimetric measurements after BMT, the atypical micromegakaryocytes characteristic for chronic myeloid leukemia disappeared, and the engrafted donor bone marrow revealed a prevalence of normal-size cells of this lineage. On the other hand, normalization of megakaryocyte size was absent in sequential examinations of the few patients with a leukemic relapse who had a predominance of atypical dwarf forms comparable with chronic myeloid leukemia. Before BMT occurred, reticulin fiber density was significantly correlated with the number of CD61+ megakaryocytes and its precursor cell population. In 34 patients with myelofibrosis that occurred after myelo-ablative therapy and BMT, an initial regression was followed by an insidious recurrence of fibers concentrated in the areas of regenerating hematopoiesis. This postgraft reappearance of reticulin fibrosis was significantly associated with the quantity of megakaryocytes. Regarding engraftment parameters, pretransplant presence of (reticulin) myelofibrosis exerted a distinctive impact because of a delayed hematopoietic reconstitution according to standard clinical criteria. In line with this finding, slowed engraftment was also significantly related with higher pretransplant megakaryocyte and platelet counts.

Adult↗
PubMed2000

Allogeneic peripheral blood cell transplantation for hypereosinophilic syndrome with myelofibrosis.

Patients with hypereosinophilic syndrome (HES) display a very heterogeneous clinical picture ranging from asymptomatic cases to very aggressive forms. We report a 38-year-old woman with progressive HES who developed severe myelofibrosis and was treated by allogeneic stem cell transplantation, using peripheral blood (PBSCT) instead of bone marrow as the source of progenitor cells, after conditioning with cytoxan and busulphan. To the best of our knowledge, this is the first case of HES with myelofibrosis treated with PBSCT. The patient remains alive 8 months post-PBSCT, and bone marrow fibrosis has significantly decreased following transplantation. Bone Marrow Transplantation (2000) 25, 217-218.

Adult↗