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PubMed1986

Immunohistochemical study of basement membrane proteins and type III procollagen in myelofibrosis.

In this study the distribution of type IV collagen in the marrow is compared with that of laminin, another basement membrane protein. In addition, incompletely processed type III procollagen is identified with specific antibodies. In normal bone marrow the distribution of the type III procollagen antigen closely resembles that of reticulin staining. In all the myelofibrotic samples, representing both early and advanced disease, the fibrous tissue stains heavily for this antigen. Thus type III procollagen which has not completely lost its aminoterminal propeptide is a genuine component of the extracellular matrix fibres in human bone marrow. Laminin is found with type IV collagen in continuous basement membranes in arterial walls, whereas only discontinuous strips of staining are seen along the sinusoids in normal marrow. In myelofibrosis the dilated or obliterated sinusoids have thickened or continuous basement membranes, visible with both stainings. Neovascularization also increases the extent of basement membrane staining in fibrotic marrow. With respect of these antigens, there is no difference between primary and secondary myelofibrosis. These changes warrant the use of serum antigens related to type IV collagen and to type III procollagen as markers for developing myelofibrosis.

Adult↗
PubMed1987

Cytogenetics of bone marrow fibroblastic cells in idiopathic chronic myelofibrosis.

To determine the nature of the fibroblastic proliferation in myelofibrosis, we studied the cytogenetic composition of a pure population of in-vitro bone marrow fibroblastic cells (FC) from a patient with idiopathic chronic myelofibrosis who had trisomy 8 in her unstimulated peripheral blood cells. This cytogenetic abnormality was absent in all the FC studied supporting the premise that in the myeloproliferative disorders the myelofibrosis represents a secondary or non-neoplastic reaction to the clonal proliferation of haematopoietic stem cells.

Aged↗
PubMed1987

Excess of blood group B in primary myelofibrosis.

The distribution of ABO and Rhesus (D) blood groups was studied retrospectively in 40 patients with primary myelofibrosis (PMF). Only patients with a leukoerythroblastic peripheral blood, splenomegaly and marrow fibrosis in whom chronic myeloid leukemia and secondary myelofibrosis was absent were included in the study. In 14 patients (35%), PMF was preceded by another myeloproliferative disorder (polycythemia rubra vera, essential thrombocythemia or unclassified myeloproliferative disorder), while 26 patients (65%) represented agnogenic myeloid metaplasia (AMM). Comparison with Hospital and Irish blood group distribution showed a significant increase in blood group B (p less than 0.01) in PMF. This increase remained statistically significant for both the AMM and the non-AMM subgroup of PMF when each subgroup was considered separately. This finding supports previous suggestions that the various myeloproliferative disorders which proceed to myelofibrosis are a closely related group rather than a heterogeneous collection of diseases.

Humans↗
PubMed1983

Chronic myelocytic leukemia with marked myelofibrosis and osteosclerosis.

An autopsy case of a 50 years old woman who manifested chronic myelocytic leukemia (CML) with secondary myelofibrosis (SMF) is reported. The interesting point of this case was a marked diffuse myelofibrosis (MF) with osteosclerosis, so that it was difficult to make a diagnosis of either CML or idiopathic myelofibrosis (IMF), but both clinical and histological findings supported the diagnosis of CML with SMF. Her long term busulfan therapy was suspected as being the cause of this severe MF. The relationship of CML and IMF was also discussed.

Biopsy↗
PubMed1987

Urinary hydroxyproline excretion in the myelofibrosis-osteomyelosclerosis syndrome and related diseases.

The total urinary hydroxyproline excretion was assessed in 47 patients with chronic myeloproliferative disorders. Urinary hydroxyproline excretion was normal in 16 patients with idiopathic myelofibrosis and in 5 out of 6 patients with acute myelofibrosis. In patients with osteomyelosclerosis (n = 8) values for urinary hydroxyproline excretion were higher (median 202, range 54-652) than those in idiopathic myelofibrosis (median 139, range 84-216). This difference was not significant (p greater than 0.1). Elevated values for urinary hydroxyproline excretion were found in 10 patients (1 AMF patient, 3 OMS patients and 6 patients with CML in the accelerated phase of the disease). All but 1 of these patients had been treated, or were being treated, with cytotoxic agents at the time of investigation. These findings are compatible with impaired degradation of bone marrow collagen which, together with enhanced collagen synthesis from bone marrow fibroblasts, accounts for progressive accumulation of connective tissue in the bone marrow. This process appears to be influenced by cytotoxic treatment as reflected in increased urinary hydroxyproline excretion in those patients receiving cytotoxic agents.

Bone Marrow↗
PubMed1989

Development of polycythaemia vera in a patient with myelofibrosis.

In March 1981, a 53-year-old man presented with itching and was diagnosed as having myelofibrosis. There was gradual enlargement of the spleen over the following 5 yr. His spleen had to be removed in February 1986 because of physical discomfort. 3 months post-splenectomy he became polycythaemic. Bone marrow examination was consistent with severe myelofibrosis. It was possible to demonstrate erythropoietin-independent BFU-E from peripheral blood, and ferrokinetic studies showed that erythropoiesis was localised to the liver with little bone marrow activity. Thus, despite severe marrow fibrosis, liver erythropoiesis was now polycythaemic, suggesting the coexistence of myelofibrosis and polycythaemia vera.

Bone Marrow↗
PubMed1990

Aggressive polychemotherapy for acute myelofibrosis.

Acute myelofibrosis is a rare and still ill-defined disease. Based on morphological observation, immunophenotyping and ultrastructural analysis, we support the assumption that acute myelofibrosis is a malignant disorder mainly of the megakaryocytic lineage and is closely related to acute megakaryocytic/blastic leukaemia. Consequently, the 11 patients reported here were treated with aggressive polychemotherapy with combinations including daunorubicin and cytosine arabinoside and 6-thioguanin or VP16-213. 4 complete remissions, 2 partial remissions and 1 minor response were observed. Duration of aplasia was not significantly prolonged. These findings indicate that the use of aggressive polychemotherapy is feasible in acute myelofibrosis and results in a significant number of remissions.

Acute Disease↗
PubMedDate not supplied

Idiopathic myelofibrosis: dental treatment considerations.

Idiopathic myelofibrosis is a myeloproliferative disorder of unknown origin. The bone marrow becomes fibrotic with an associated decrease in hematopoiesis resulting in anemia, bleeding problems, splenomegaly, and other secondary abnormalities. Although idiopathic myelofibrosis is usually diagnosed in middle age, there have been a few reports of the disorder in the pediatric population. This case report documents dental treatment considerations in a 6-year-old female with idiopathic myelofibrosis, severe anemia, and abnormal blood coagulation studies. The patient was successfully treated in a hospital after medical consultation, transfusion of packed red blood cells, and administration of prophylactic antibiotics. Local hemostatic measures following multiple extractions of carious teeth controlled bleeding. No postoperative sequelae occurred.

Anemia↗
PubMed2004

Prefibrotic chronic idiopathic myelofibrosis--a diagnostic enigma?

To analyze precursor lesions of chronic idiopathic myelofibrosis (CIMF) 21 patients were recruited who developed manifest myelofibrosis after about 70 months, preceded by a prefibrotic stage but without interference by cytoreductive therapy. Prodromal bone marrow lesions included a prominent granulocytic and megakaryocytic proliferation with megakaryocytes showing conspicuous abnormalities associated with a borderline to slight anemia, thrombocytosis, and minimal splenomegaly. Comparison of this cohort with 211 additional patients at this precursor stage revealed no differences concerning clinical findings and bone marrow histopathology. Relative incidence of prefibrotic CIMF was 24% and median survival 143 months, thus contrasting overt CIMF (82 months). In CIMF progressive myelofibrosis occurred in more than 50% of patients according to the last biopsy specimen; however, this figure increased according to relevant clinical data in the follow-up period.

Bone Marrow Examination↗
PubMed1996

Myelofibrosis secondary to renal osteodystrophy.

A hemodialyzed women with secondary hyperparathyroidism who recovered well from myelofibrosis after a total parathyroidectomy with autotransplantation of parathyroid tissue to the forearm (PTx) is described. Before the operation, she had received regular transfusions to maintain an adequate hematocrit even under recombinant human erythropoietin (rhEpo) therapy. She showed splenomegaly and leukoerythroblastosis was present in her peripheral blood. A bone marrow biopsy and bone marrow scintigraphy confirmed the diagnosis of myelofibrosis. After PTx, her hematocrit gradually increased without any transfusion. It has been maintained around 35% now 16 months since the operation. Her spleen has also gradually decreased in size. In addition, no leukoerythroblastosis has been found in the peripheral blood. Serial follow-up scintigraphy of bone marrow revealed a decline in extramedullary hematopoiesis. These findings indicated that her myelofibrosis was the result of secondary hyperparathyroidism, and that this complication is potentially reversible if accurate treatment is given. Physicians dealing with the end-stage renal disease should be aware of this complication to avoid additional transfusions.

Alkaline Phosphatase↗
PubMed1997

A case of interstitial pneumonitis associated with natural alpha-interferon therapy for myelofibrosis.

A 55-year-old man with myelofibrosis was treated with natural alpha-interferon with a good hematologic response. Initially, he had anemia, leukocytosis, thrombocytosis and hepatospleomegaly. A bone marrow biopsy showed replacement with fibrosis with an increase in megakaryocytes. Natural alpha-interferon (alpha-IFN) was started at a dose of 3 x 10(6) units/day. The leukocyte and platelet counts gradually normalized, and the liver and spleen decreased in size. However, the patient complained of a dry cough and dyspnea on the 61st treatment day, when the accumulated dose of alpha-IFN treatment had reached 1.8 x 10(8) units. He subsequently developed acute respiratory failure (PaO2 < 60 mm Hg) with bilateral lung infiltrations, suggesting the occurrence of interstitial pneumonitis associated with alpha-interferon therapy. Immediately, the alpha-interferon was discontinued and high-dose methylprednisolone (1.5 g/day) was administered for 3 days. This treatment was followed by oral prednisone therapy. Steroid therapy brought about gradual improvement as suggested by a repeat radiograph. Since high levels of fibrogenic cytokines, such as PDGF and TGF-beta, have been reported in patients with myelofibrosis, it is necessary to pay attention to interstitial pneumonia as a complication in alpha-IFN therapy for myelofibrosis.

Anti-Inflammatory Agents↗
PubMed1996

Leukemia with megakaryocytic differentiation following essential thrombocythemia and myelofibrosis. Case report and review of the literature.

Leukemias of megakaryocytic lineage are rare and heterogeneous clinical entities. The nomenclature published in the literature is confusing and perhaps inappropriate to designate these primary myeloproliferative disorders. We describe a patient with essential thrombocythemia who evolved through myelofibrosis and myeloid metaplasia to a final picture of leukemia with megakaryocytic differentiation in the peripheral blood. This case illustrates different aspects of a chronic myeloproliferative disorder where myelofibrosis and myeloid metaplasia are frequent but secondary events. We have reviewed the literature focusing on the role of clonal megakaryocytic proliferation in myelofibrosis and on the clinical characterization of leukemia with megakaryocytic phenotype. We also present our interpretation of the literature which indicates that a formal review of the nomenclature is urgently needed.

Female↗
PubMed1993

Neutropenia associated with myelofibrosis in systemic lupus erythematosus.

Although myelofibrosis has been described in systemic lupus erythematosus (SLE), this coexistence must be rare since there are few reports showing this combination. The possible relationship between hematologic abnormalities and SLE remains unresolved. The authors describe a 39-year-old woman with persistent neutropenia and SLE in whom myelofibrosis was found. Unlike previously reported cases, myelofibrosis did not resolve with steroid therapy. In this report, the clinical course of the patient is compared with others described in the literature.

Adult↗
PubMed1988

Myelofibrosis and pancytopenia in systemic lupus erythematosus.

A 13-year-old Saudi girl presented with severe clinical features of systemic lupus erythematosus of 3 months' duration. In addition to laboratory evidence of the disease, the patient was found to have pancytopenia and myelofibrosis. While pancytopenia was considerably improved by steroids, myelofibrosis was not reversed. The coexistence of myelofibrosis and pancytopenia in patients with systemic lupus erythematosus has been reported, but their possible causal relationship remains unresolved.

Adolescent↗
PubMed1978

'Classic' and 'acute' myelofibrosis. A retrospective study.

In a retrospective study of 38 patients with histologically proven myelofibrosis, 5 parameters (recorded on the first admission) were investigated as to their usefulness to predict the course of the disease. In 9 patients the development of 'acute' myelofibrosis could be predicted by the finding of pancytopenia, low reticulocyte counts (median 20,500/mul), extremely elevated SRE (median 125 mm) and normal serum levels of LDH and uric acid on the first admission. In 28 patients the development of classic fibrosis with splenomegaly could be predicted by the finding of high reticulocyte counts (median 133,200/mul) and increased levels of serum LDH (median 547 U) and uric acid (median 8 mg/dl in males and 6.8 mg/dl in females) on the first admission, even when splenomegaly was initially absent. The relationship between classic and acute myelofibrosis and the significance of the mentioned parameters is discussed.

Acute Disease↗
PubMed1977

Chronic idiopathic myelofibrosis. A reversible disease?.

A patient with chronic idiopathic myelofibrosis was subjected to splenectomy 1 year after diagnosis. As a clinically unexpected finding, lymph node biopsy suggested the presence of non-Hodgkin lymphoma. The patient was subjected to intensive combined cytostatic therapy. In the following months, signs and symptoms of myelofibrosis regressed remarkably. The patient died 31 months after splenectomy in massive gastrointestinal bleeding. At post-mortem, myelofibrosis could not be detected in three bone marrow areas and a regular, fat-containing, hypercellular marrow was present. The nature of the previous lymph noede pathology was reconsidered, and angioimmunoblastic lymphadenopathy was diagnosed.

Antineoplastic Agents↗
PubMedDate not supplied

Myeloid metaplasia in aspirates from enlarged spleens: a clue in the absence of peripheral blood findings characteristic of myelofibrosis.

OBJECTIVE: To assess the significance of finding myeloid metaplasia in splenic aspirates from patients presenting with mild to moderate firm splenomegaly, in the absence of characteristic peripheral blood findings, in diagnosing idiopathic myelofibrosis. STUDY DESIGN: Archival records pertaining to 14 patients diagnosed as having myeloid metaplasia on splenic aspirates performed between September 2000 and April 2004 were analyzed. RESULTS: The relevant findings in these 9 women and 5 men were: splenic enlargement 17-21 cm with homogeneous echotexture on ultrasonography, hemoglobin 4-10 g/dL, variable pattern of anemia, total leukocyte count 6,300-28,800/ mm3 with neutrophilia and a few late myeloid precursors on the differential count, normal platelet counts, dry bone marrow tap in 10 patients and cellular marrow aspirate with prominence of megakaryocytes dispersed in a maturing cell population of myeloid and erythroid series in 4 patients. Splenic aspirates yielded foci of trilineage hematopoiesis suggestive of myeloid metaplasia, possibly due to myelofibrosis of idiopathic type, as confirmed on trephine biopsy in all cases. CONCLUSION: Splenic aspirates may be a useful tool for detecting myeloid metaplasia suggesting myelofibrosis when peripheral blood findings are not yet characteristic of the same. The procedure was not associated with any complications.

Adult↗
PubMed1985

Myelofibrosis and true histiocytic lymphoma.

A 60-year-old female presented with a history of progressive shortness of breath, fever, and weight loss of 55 pounds. The work-up consisting of computerized axial tomography (CT) scan of thorax and abdomen, mediastinoscopy, and bilateral bone marrow aspiration and biopsy revealed a large-cell or histiocytic lymphoma involving bone marrow with myelofibrosis. Further immunologic and ultrastructural investigation confirmed the true histiocytic origin of the tumor. The patient was treated with 12 courses of intravenous cyclophosphamide, onconvin, doxorubicin, and prednisone and achieved a complete remission with disappearance of clinical symptoms, normal CT scan of thorax and abdomen, and normal bone marrow with disappearance of myelofibrosis from the same site as the previous bone marrow test. At present the patient is in complete remission. We present this case because of the previously unreported association between histiocytic lymphoma and myelofibrosis, and the unusually good response to chemotherapy and the disappearance of fibrosis from the marrow.

Female↗