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PubMed1993

Progression of essential thrombocythemia to blastic crisis via idiopathic myelofibrosis.

We report a 61-year-old man with essential thrombocythemia (ET) whose clinical course was followed for 12 years. The ET evolved into true idiopathic myelofibrosis (IM) 6 years after the initial diagnosis and progressed to myeloid blastic transformation 6 years later. The cytogenetic analysis showed a normal karyotype during the ET phase but subsequent analysis revealed an abnormal karyotype during the IM phase which evolved clonally at blastic crisis with constant involvement of chromosome 13q and chromosome 7. The close monitoring of essential events, using clinical, morphologic, immunologic and cytogenetic parameters, allowed us to carefully identify the transition from one chronic myeloproliferative disease (MPD) to another. This is only the second case reported showing a clinical evolution of this nature. The clinical and biological aspects of the disease are briefly discussed.

Blast Crisis↗
PubMed1993

Coexistent multiple myeloma and myelofibrosis.

Although the association of bone marrow fibrosis with plasma cell dyscrasias has already been described in several reports, the close relationship between these entities still remains unclear. In this report we describe a patient with clinical and pathologic findings which initially suggested a diagnosis of myelofibrosis, subsequently shown to be coexistent with multiple myeloma. Possible explanations for this association are discussed.

Bone Marrow↗
PubMed1995

Extramedullary megakaryoblastic tumors following an indolent phase of myelofibrosis.

A 59-year-old man developed multiple subcutaneous and bone tumors after 2 years' duration of indolent myelofibrosis. These tumors exhibited a diffuse proliferation of pleomorphic blast cells. The skeletal x-rays showed disseminated osteolysis and osteosclerosis. Blast cells obtained from the pleural effusion did not react with myeloperoxidase, sudan black B or non-specific esterase. Genotypic analysis of DNA samples at autopsy did not demonstrate rearrangements of T-cell receptor beta-chain or immunoglobulin heavy-chain genes. Frozen-section immunohistochemical studies revealed that the neoplastic cells were derived from megakaryocytic lineage. The present case represents a novel and unusual subtype of megakaryocytic neoplasm.

Bone Neoplasms↗
PubMed1995

Leukemic transformation of primary myelofibrosis: immunophenotype, genotype and growth characteristics of blast cells.

Blast cells from six patients with leukemic transformation of primary myelofibrosis (PMF) were studied by morphology, immunophenotype and genotype as well as response to hematopoietic growth factors. The majority of the patients showed granulocytic or granulo-monocytic blasts, and only one had T lymphoid-monocytic blasts. None of the patients showed rearrangement of Ig or TCR genes, or the existence of the bcr-abl fused gene. A prominent growth response to GM-CSF and IL-3 was evident in all of the patients examined in liquid as well as semisolid cultures. The response to G-CSF was observed in four of the six patients in suspension culture, and in two of three patients in the clonogenic assay. Stem cell factor (SCF) was a weak growth stimulant, however the combination of this factor with GM-CSF or IL-3 was synergistically stimulatory. These results suggest that leukemic transformation of PMF occurs mainly at the level of myeloid stem cell, and that GM-CSF, IL-3, G-CSF and SCF are major growth factors for the blast cells in these cases.

Aged↗
PubMed1998

Myelodysplastic syndrome with myelofibrosis and basophilia: detection of trisomy 8 in basophils by fluorescence in-situ hybridization.

We report a case of myelodysplastic syndrome (MDS) with myelofibrosis and pulmonary tuberculosis who had marked basophilia in the peripheral blood. A clonal karyotypic abnormality characterized by trisomy 8 was demonstrated by cytogenetic analysis. By correlation of cell morphology with results of fluorescence in situ hybridization using a chromosome 8 probe, we demonstrated that the basophils were not reactive but belonged to the neoplastic MDS clone.

Adult↗
PubMed2004

Durable responses to thalidomide-based drug therapy for myelofibrosis with myeloid metaplasia.

OBJECTIVE: To present the results of a long-term analysis of 2 sequential phase 2 trials of thalidomide (alone or in combination) for palliation of myelofibrosis with myeloid metaplasia (MMM). PATIENTS AND METHODS: We analyzed (March 1999 to August 2003) initial and long-term outcomes from 36 patients with symptomatic MMM who had enrolled in either our thalidomide single-agent trial (n=15) or our trial of low-dose thalidomide (50 mg/d) combined with prednisone (n=21). RESULTS: Among the 36 study patients, 20 (56%) showed some improvement in their clinical course. Response rates for specific end points included improvements in anemia (15 of 36 [42%]), thrombocytopenia (10 of 13 [77%]), or splenomegaly (5 of 30 [17%]). The combination of low-dose thalidomide and prednisone, as opposed to single-agent thalidomide, was better tolerated and more efficacious. After a median follow-up of 25 months (range, 20-56 months), 10 of 36 patients (28%) showed an ongoing response, including 8 patients in whom protocol treatment has been discontinued for a median of 21 months (range, 16-31 months). Durable treatment responses were documented for only anemia and thrombocytopenia. Treatment response was not affected by the baseline status of bone marrow fibrosis, angiogenesis, osteosclerosis, cytogenetics, or circulating myeloid progenitor (CD34) cell count. Unusual drug effects, all reversible, included leukocytosis (8 patients) and/or thrombocytosis (6 patients). CONCLUSIONS: Thalidomide (alone or combined with prednisone) is an effective first-line treatment of symptomatic anemia or thrombocytopenia in MMM. Thalidomide-based therapy has the potential to produce durable responses in MMM-associated cytopenias, even after discontinuation of the drug.

Adult↗
PubMed2004

Histiocytic sarcoma associated with idiopathic myelofibrosis.

We describe the case of a 39-year-old man with idiopathic myelofibrosis, who developed histiocytic sarcoma (true histiocytic lymphoma) 6 months after diagnosis. The patient developed generalized lymphadenopathy. A lymph node biopsy showed pronounced distension of the sinuses in the medulla and periphery, caused by the accumulation of large tumor cells. The tumor cells had abundant clear or eosinophilic cytoplasm. The nuclei were of various sizes and shapes, with condensed chromatin and prominent nucleoli. Some tumor cells displayed erythrophagocytosis. Immunohistochemically, the tumor cells were positive for CD68, alpha(1)-antitrypsin, CD45, CD45RO, and S100 protein, and were negative for B- and T-cell markers, CD30, CD1a, lysozyme, myeloperoxidase, factor VIII-related antigen, CAM 5.2, and HMB-45. Despite multiagent chemotherapy, the patient died of disease 25 months after diagnosis. Although histiocytic sarcomas are very rare, their recognition may be important for clinical and prognostic reasons.

Adult↗
PubMed2005

Acute lymphoblastic leukemic transformation in a patient with chronic idiopathic myelofibrosis and paroxysmal nocturnal hemoglobinuria: a case report and review of the literature.

Leukemic transformation of chronic idiopathic myelofibrosis (CIMF) to acute lymphoblastic leukemia (ALL) is rare. We report a case of a patient with CIMF who developed paroxysmal nocturnal hemoglobinuria (PNH) 2 years after initial presentation. His disease eventually transformed to ALL of precursor B-cell type. In that CIMF and PNH are clonal stem cell disorders with different pathogeneses, there may be an association between them. However, leukemic transformation is a rare sequel of both disorders. Coexistence of CIMF and PNH and subsequent transformation to ALL have, to our knowledge, never been previously reported in the world literature. The simultaneous presentation of CIMF and PNH, complicated by the rare sequela of leukemic transformation, raises important issues with regard to diagnosis and treatment.

Chronic Disease↗
PubMed2006

Chronic idiopathic myelofibrosis: clinicopathologic features, pathogenesis, and prognosis.

CONTEXT: Chronic idiopathic myelofibrosis (CIMF) is a clonal myeloproliferative disease characterized by panmyelosis with intact maturation, progressive bone marrow fibrosis, and multiorgan extramedullary hematopoiesis. OBJECTIVE: This review article aims to summarize the recent updates regarding the clinicopathologic features, molecular pathogenesis, cytogenetic abnormalities, diagnostic criteria, new diagnostic ancillary tests, and prognostic factors of CIMF. DATA SOURCES: Important relevant articles indexed in PubMed/MEDLINE (National Library of Medicine) through the end of 2005 and referenced medical texts. CONCLUSIONS: Because CIMF has a variety of clinical presentations, diagnosis may be challenging; the prefibrotic stage of CIMF has always been a challenging disease for pathologists to diagnose accurately. The recently proposed European Clinical and Pathological criteria can be helpful in the diagnosis of CIMF, especially in its prefibrotic stage. The enumeration of CD34-positive cells in the peripheral blood and the presence of circulating endothelial progenitor cells are the new important ancillary tests for the diagnosis of a small subset of patients with CIMF with atypical presentation. The recent discovery of the new mutation affecting the Janus tyrosine kinase 2 (JAK2V617F), more frequently observed in patients with polycythemia vera, is seen in approximately 35% to 57% of patients with CIMF. This mutation can serve as another diagnostic tool. Important factors affecting prognosis in CIMF are anemia, age of the patient, white blood cell count, degree of fibrosis, and number of blasts in the peripheral blood.

Biomarkers↗
PubMed1998

[Extranodal non-Hodgkin's lymphoma associated with systemic bone metastasis and secondary myelofibrosis].

A 68-year-old woman was admitted in March 1997 because of lumbago, fever, vomiting, and general malaise. Laboratory data disclosed anemia and severe hypercalcemia (7.7 mEq/l). Multiple osteolytic lesions were detected in the patient's vertebra, pelvis, and bilateral tibia by x-ray films and 99mTc bone scintigrams. Bone marrow aspiration sample was not obtained due to dry tap. Marked myelofibrosis and proliferation of lymphoid cells were revealed by a bone marrow biopsy specimen. Immunohistochemical analysis showed that cells in the biopsy specimen were positive for L-26 and LCA, but not for UCHL-1. Gastrointestinal endoscopic examination found multiple polypoid lesions in the stomach; biopsy specimens of the lesion tissue disclosed invasion by B lymphoid cells. A diagnosis of diffuse large B cell lymphoma was thus made. THP-COP chemotherapy was performed, but only minimal response was obtained. Lymphoma cells subsequently invaded the brain stem, and the patient eventually died of respiratory failure.

Aged↗
PubMed1999

[Allogeneic peripheral blood stem cell transplantation in an elderly patient with myelodysplatic syndrome with myelofibrosis].

Allogeneic peripheral blood stem cell transplantation (Allo-PBSCT) has in recent years become an alternative to allogeneic bone marrow transplantation because it facilitates rapid hematopoietic reconstitution without an increase in the incidence of severe graft-versus-host disease (GVHD). We report on a 61-year-old man with myelodysplastic syndrome (MDS) and myelofibrosis who received an allo-PBSCT from his HLA-matched 68-year-old brother. The preparative regimen consisted of busulfan and cyclophosphamide. Cyclosporin A and methotrexate were administered for GVHD prophylaxis. The donor was treated with granulocyte colony-stimulating factor (G-CSF) at a dose of 10 micrograms/kg/day subcutaneously for 4 consecutive days. A preparation of 4.04 x 10(6) CD34+ cells/kg recipient weight was collected in a single apheresis and infused immediately. Engraftment times to a neutrophil count greater than 500/microliter and platelet count greater than 2.0 x 10(4)/microliter were 15 days each. Acute GVHD of grade II developed, but was resolved with methylprednisolone. However, the patient died of thrombotic microangiopathy 97 days after his allo-PBSCT. Administration of G-CSF and apheresis in the donor were feasible and well tolerated. Allo-PBSCT may result in earlier engraftment and be especially beneficial to elderly patients with MDS.

Hematopoietic Stem Cell Transplantation↗
PubMed1976

Colony forming activity, density distribution and morphological features of the stem cell population in primary myelofibrosis.

In four patients with untreated primary myelofibrosis, the peripheral-blood cell growth in agar culture (frequency of clusters and colonies) was studied. As cell sources, were used either the buffy coat or the fractions obtained from an albumin discontinuous density gradient, prepared according to Dicke's technique. Besides the agar culture, also morphological features of the blasts of the granulocytic cell series of the gradient fractions were investigated, in order to relate the above-mentioned cell parameters. The results, taken as a whole, pointed out: a) increase in circulating CFUc's (expressed as the number/10(5) nucleated cells) in the patients, when compared to the controls; b) high percentage of CFUc's with an abnormal specific density (below 1.062 in 3 out of 4 patients); c) presence in 3 out of 4 patients, of two populations of blasts: one showing low specific density, small size, high N/C rate, high plating efficiency and no nucleoli; the other with a higher specific density and low plating efficiency, showing the typical morphological features of the myeloblasts.

Adult↗
PubMed1999

[Primary myelofibrosis with fatal mesenteric arterial thromboembolism caused by antiphospholipid syndrome].

A 60-year-old woman was admitted to our hospital in February 1993 due to dizziness, dyspnea, abdominal pain, and high susceptibility to bleeding. Physical examination revealed livedo reticularis of the foot, but did not detect hepatosplenomegaly. Examination of the peripheral blood detected pancytopenia, leukoerythroblastosis, and tear-drop erythrocytes. Primary myelofibrosis (PMF) was diagnosed on the basis of bone marrow biopsy findings. Antiphospholipid syndrome (APS) was confirmed by positive response to anti-cardiolipin antibody and recurrent splenic infarction. Because of factor XIII deficiency, the patient experienced severe gingival bleeding after tooth extraction. Her condition was complicated by mesenteric arterial thromboembolism and she died of sepsis 5 years after onset. Although the incidence of immunopathy in PMF patients is high, few studies to date have focused on APS patients presenting with a variety of severe embolic symptoms. Our patient required careful monitoring due to bleeding tendency and thromboemboli.

Antiphospholipid Syndrome↗
PubMed1999

Successful low-dose cytosine arabinoside treatment in a patient with acute leukemia from idiopathic myelofibrosis.

We report a 63-year-old male with acute leukemia developed from idiopathic myelofibrosis. Cytogenetic analysis revealed chromosomal abnormalities; 46,XY,t(3;21)(q21;q22) del(20q). Intensive chemotherapies [combination of adriamycine/vincristine/prednisolone, or idarubicine/cytosine arabinoside (Ara-C)] were unsuccessful. The patient was then treated with continuous intravenous low-dose Ara-C because of his poor physical condition and showed marked hematological improvement. Leukemic cells disappeared from the peripheral blood and the segmented neutrophil count recovered. The dysplastic morphology observed in the segmented neutrophils suggests that the recovery of the neutrophils has resulted mainly from the differentiation activity of Ara-C.

Acute Disease↗
PubMed1999

Idiopathic myelofibrosis with extramedullary hematopoiesis in the kidneys.

Extramedullary hematopoiesis is a common finding in idiopathic myelofibrosis and is usually found in liver and spleen. We report on a patient with biopsy-proven myeloid metaplasia and fibrosis of the renal parenchyma as a rare cause of chronic renal failure. The renal biopsy specimen showed numerous infiltrates of hematopoietic cells expressing growth factors like M-CSF, GM-CSF, IL-1beta and PDGF while TGF-beta was not elevated. These findings suggest that hematopoietic growth factors play a key role in the pathogenesis of this condition causing proliferating fibrosis and enlargement of the kidneys.

Aged↗
PubMed1976

A preliminary study on humoral control of granulopoiesis in primary myelofibrosis and chronic granulocytic leukaemia.

The extent of the urine Colony Stimulating Factor (CSF/u), as well as the Colony Stimulating Activity (CSA) of peripheral-white blood cells (WBC) were evaluated in two groups of patients with myeloproliferative disorders, at the disease onset. The first patient group deals with Primary Myelofibrosis (PM), whereas the second group deals with Chronic Granulocytic Leukaemia (CGL). The Stanley technique was used for the above-mentioned assay, and in each group of patients either human or murine bone-marrow cell-suspensions were cultivated. In all patients with PM, a low CSF/u was detected, while patients with CGL showed a high CSF/u, regardless whether Ph1-positive or not. CSA of WBC in the PM-group was normal. In contrast, a significant low CSA in the CGL-group was noted. This fact could be of some importance in differentiating the two disorders at their onset.

Animals↗
PubMed2000

Splenectomy in myelofibrosis with myeloid metaplasia: a single-institution experience with 223 patients.

In a 20-year period, 223 patients (median age, 64.8 years) with myelofibrosis with myeloid metaplasia (MMM) had therapeutic splenectomy at our institution. Primary indications for surgery were transfusion-dependent anemia (45.3%), symptomatic splenomegaly (39. 0%), portal hypertension (10.8%), and severe thrombocytopenia (4.9%). Operative mortality and morbidity rates were 9% and 31%, respectively. The 203 survivors of surgery had a median postsplenectomy survival time (PSS) of 27 months (range, 0-155). Among preoperative variables, thrombocytopenia (platelet count less than 100 x 10(9/)L) and nonhypercellular bone marrow were identified as independent risk factors for decreased PSS. Durable remissions in constitutional symptoms, transfusion-dependent anemia, portal hypertension, and severe thrombocytopenia were achieved in 67%, 23%, 50%, and 0% of the patients, respectively. Histologic or cytogenetic features of bone marrow obtained before splenectomy did not predict a response in cytopenias. After splenectomy, substantial enlargement of the liver and marked thrombocytosis occurred in 16.1% and 22.0% of the patients, respectively. The thrombocytosis was associated with an increased risk of perioperative thrombosis and decreased PSS. The rate of blast transformation (BT) was 16.3%, and the risk of BT was higher in the presence of increased spleen mass and preoperative thrombocytopenia. However, the PSS of patients with BT was not significantly different from that of patients without BT. We conclude that presplenectomy thrombocytopenia in MMM may be a surrogate for advanced disease and is associated with an increased risk of BT and inferior PSS. However, the development of BT after splenectomy may not affect overall survival and does not undermine the palliative role of the procedure for the other indications.

Adolescent↗