Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “MYELOFIBROSIS”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 397 records · Page 22Linked to original sources
PubMed2003

Thrombotic thrombocytopenic purpura associated with bone marrow metastasis and secondary myelofibrosis in cancer.

To examine the relationship between cancer and development of thrombotic microangiopathy (TM), the medical records of patients with known TM were examined in one institution from January 1981 to December 2002. Nine out of 93 patients with the established diagnosis of TM had active cancer. All nine of those patients had thrombotic thrombocytopenic purpura (TTP). Among those patients, two patients received chemotherapy prior to the development of TTP. Six of the seven patients who received no chemotherapy had extensive bone marrow metastasis and secondary myelofibrosis. There were two patients each with breast cancer, lung cancer, and stomach cancer. Severe anemia and thrombocytopenia with leukoerythroblastosis were prominent clinical features in all six patients. Four patients had neurological (mental) changes and three developed fever, but none had significant renal dysfunction. Upon establishing the diagnosis of TTP, four patients were treated with exchange plasmapheresis (EP) and two patients were treated with chemotherapy because there were no neurological changes. Three patients achieved complete remission of TTP, one with EP alone and two with chemotherapy. The one patient who achieved remission with EP alone was later treated with chemotherapy and survived for 2 1/2 years. The other three patients treated with EP alone died within 2 months after the diagnosis of TTP. Since TTP occurred in association with bone marrow metastasis and myelofibrosis in six patients among seven chemotherapy-untreated cancer patients, this marrow change was considered to be the possible cause of the development of TTP. It is recommended that all cancer patients with unexplained anemia and thrombocytopenia be evaluated for the coexistence of bone marrow metastasis and TTP.

Adult↗
PubMed2003

Idiopathic myelofibrosis with refractory massive ascites.

A 55-year-old woman presented with massive refractory ascites in the course of idiopathic myelofibrosis. The ascites was exudative, and a cytological examination revealed granulocytes of varying maturity, erythroblasts, and megakaryocytes with trisomy 8. The ascites was assumed to have developed from peritoneal extramedullary hematopoiesis. An abnormal karyotype in the cells in the ascitic fluid, which was the same abnormality as in peripheral blood, helped to prove extramedulary hematopoiesis in this case, which can be an aid in making a differential diagnosis in cases of ascites associated with myelofibrosis.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1985

Essential thrombocythemia terminating in myelofibrosis and myeloblastic transformation.

A 58-year-old man with essential thrombocythemia terminating in myelofibrosis and myeloblastic transformation is described. He was treated with busulfan and lived for 6 years and 6 months. At autopsy, significant osteomyelofibrosis was noted. Moreover, myeloblastic infiltration with proliferation of megakaryocytes and erythroblasts was seen in the bone marrow and spleen. In the lymph nodes, myeloid metaplasia was noted. The past reports on this disease terminating in myelofibrosis and/or blast transformation have been reviewed.

Bone Marrow↗
PubMedDate not supplied

Pachydermoperiostosis with myelofibrosis and anemia: report of a case of anemia of multifactorial causes and its improvement with steroid pulse and iron therapy.

A 26-year-old male patient with pachydermoperiostosis is reported. He had severe anemia with myelofibrosis. Treatment with iron, prednisolone, oxymethorone and 1 alpha (OH)D3 were not satisfactory. But steroid pulse therapy with parenteral iron improved his anemia and pancytopenia, but was not sufficient to relieve the bone marrow fibrosis or splenomegaly. The mechanism of anemia which was considered to be multifactorial including gastro-intestinal bleeding associated with peptic ulcer or erosion and bone marrow failure due to myelofibrosis, is discussed.

Adult↗
PubMed2006

Aberrant collagenase expression in chronic idiopathic myelofibrosis is related to the stage of disease but not to the JAK2 mutation status.

Bone marrow fibrosis in chronic idiopathic myelofibrosis (cIMF) most likely represents an imbalance between synthesis and turnover of collagen fibers. Because the JAK-STAT signaling pathway is involved in the regulation of genes encoding matrix metalloproteinases (MMPs), we examined the expression of MMPs, their tissue inhibitors (TIMPs), and collagen types in relation to the JAK2 status (V617F mutation versus wild-type) in cIMF (n = 64). Whereas no correlation was found between the JAK2 status and MMP gene products, there was an evident association with the stage of disease. Membrane type 1-MMP (MMP-14) was overexpressed by up to 80-fold in advanced stages that progressed to fibrosis (P < 0.001), and megakaryocytes and endothelial cells were unmasked as the major cellular source. By contrast, a significantly higher expression of neutrophil collagenase (MMP-8) was encountered in the prefibrotic stages of cIMF (P < 0.001). Altogether, the stepwise progress of myelofibrosis in cIMF was associated with expression of a defined subset of target genes as shown in sequential trephine biopsies of cIMF patients. We conclude that the expression of matrix-modeling genes in cIMF is not influenced by the JAK2 mutation status but is predominantly related to the stage of disease.

Adult↗
PubMed2000

Acquired delta-storage pool deficiency associated with idiopathic myelofibrosis.

A 73-year-old woman complained of easy bruising, as a consequence of prolonged bleeding time despite normal platelet counts. Platelet aggregation profile, mepacrine fluorescence test, flow cytometry and transmission electron microscopy studies led to the diagnosis of delta-storage pool deficiency (SPD) A few months later, she developed hyperleucocytosis with immature granulocytes and erythroblasts. The presence of bone marrow fibrosis and clonal cytogenetic abnormalities led to the diagnosis of idiopathic myelofibrosis (IM). Association between SPD and IM has never been reported. The pathogenesis of this unusual association remains unclear and may involve proliferation of abnormal monoclonal stem cells with differentiation into activated megakaryocytes associated with impaired dense granule development and increased cytokines release which may be. involved in myelofibrosis.

Aged↗
PubMed2000

Myelofibrosis in systemic lupus erythematosus.

In this study we present a case of coexisting systemic lupus erythematosus (SLE) and myelofibrosis. Literature review supports the fact that the two diseases rarely occur together in the same patient. The young female patient studied was admitted with pancytopenia and a clinical picture which met the criteria of SLE. Histological examination of the bone marrow biopsy revealed severe myelofibrosis with hypocellularity of the myeloid cell lines. Treatment with immunosuppressive and colony stimulating factor led to slow but complete regeneration of the bone marrow and subsequently to an improved haematological status, and the patient was spared bone marrow transplantation.

Adolescent↗
PubMed2003

Acute myelofibrosis in a patient with diffuse large cell non Hodgkin's lymphoma and renal cancer.

Relapse after anthracycline based combination chemotherapy is frequently seen in patients with aggressive non Hodgkin's Lymphomas (NHL), whereas complications such as secondary leukemia or solid tumor rarely occur. We report a patient with diffuse large cell (DLC) NHL and concurrent renal cancer, who developed acute myelofibrosis (AMF) later in the course of her disease. This 60-year-old female patient presented with pancytopenia and a right sided renal mass. Diagnostic work up revealed severe bone marrow infiltration by DLC NHL and renal cancer T1N0M0G2. Cytogenetic and molecular evaluation of bone marow cells showed three distinct clones, (a normal 46XX karyotype, a ringed chromosome 7 and a third clone with an enlarged chromosome 2 as well as several fragments). The patient underwent nephrectomy and eventually received 6 cycles of CHOP 14 chemotherapy. Anemia persisted followed by severe granulocytopenia and thrombocytopenia 6 weeks later. Repeated bone marrow biopsy showed absence of lymphoma and/or cancer metastasis, but massive myelofibrosis with an increased number of atypical megakaryocytes. Considering the short clinical course and the absence of hepatosplenomegaly AMF was diagnosed. The concurrence of three distinctneoplasms within a short period of time as well as the complex cytogenetic aberrations found in her bone marrow cells reflect a strong individual susceptibility to malignant disease in this patient.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1995

The role of alpha-interferon in essential thrombocythaemia, polycythaemia vera and myelofibrosis with myeloid metaplasia (MMM): a concise update.

Treatment of essential thrombocythaemia (ET), polycythaemia vera (PV), and myelofibrosis with myeloid metaplasia (MMM) patients is frequently a difficult issue. To date, there is no generally accepted treatment for these diseases which can reduce the risk of thromboembolism and/or haemorrhagic events, avoid any increase in the frequency of secondary myelofibrosis and terminal blast transformation and decrease the reticulin content in the bone marrow of MMM patients. The most frequently used myelosuppressive agent is hydroxyurea (HU), but widespread application has failed to demonstrate that is not leukaemogenic. In patients with MMM, conflicting results have been obtained following alpha-IFN treatment. Haematological responses have been seen in 50% of the patients. Usually the patients showing good responses had a hyperproliferative type of disease. In only one case was a reduction of reticulin content of the bone marrow observed. Thus, these findings do not indicate alpha-IFN as a first-line therapy. On the other hand, the results of several reports in ET and PV patients have shown a reduction in the abnormal proliferation of megakaryocytes and erythroid elements, following alpha-IFN treatment. A reduction in spleen size has also frequently been seen. Together with the improvement of haematological parameters, clinical symptoms have also responded positively. Long term control of these diseases can be obtained with a well-tolerated low dose of alpha-IFN. However, PV and ET are not usually characterized by cytogenetic abnormalities, making it very difficult to demonstrate the disappearance of clonal haemopoiesis following alpha-IFN therapy, even if this does occasionally occur, as evident from the two cytogenetic convertions described in the literature. As compared to myelosuppressive drugs or phlebotomy, alpha-IFN thus represents an attractive new treatment, able to exert a fundamental influence on these diseases, presumably without any untoward leukaemogenic or gonadotoxic activity.

Humans↗
PubMed1996

Risk of leukaemia, carcinoma, and myelofibrosis in 32P- or chemotherapy-treated patients with polycythaemia vera: a prospective analysis of 682 cases. The "French Cooperative Group for the Study of Polycythaemias".

An analysis of the risk of progression towards leukemia, carcinoma and myelofibrosis was performed in 93 patients treated by 32P alone (PVSG protocols) since 1970-1979, 395 patients over the age of 65 years treated by 32P with or without maintenance therapy using hydroxyurea (French protocol) since 1980-1994, and 202 patients under the age of 65 treated by either hydroxyurea or pipobroman since 1980. The risk of leukemia, or myelodysplasia, or lymphoma in the 32P-treated patients was 10% at the 10th year, but increase after that time to reach a value of about 30% at the 20th year, in the surviving case. This risk was not dose-related. Despite a marked reduction of the cumulative 32P dose in the patients maintained by hydroxyurea, the actuarial risk was 19% at the 10th year. In the patients treated exclusively by non radio-mimetic agents (hydroxyurea or pipobroman) a risk of 10% at the 10th year was observed. The risk of carcinoma (excluding skin cancers) was about 15% at the 10th year in the 32P-treated cases, a value similar to that generally reported by the French statistics. There was no prevalence of digestive carcinomas. In contrast, the patients receiving 32P and hydroxyurea as maintenance had an excess risk: 29% at the 10th year. In the relatively young cases treated by non radio-mimetic agents, the risk was similar in both arms: 9% at the 10th year, similar to the expected incidence at this age. The risk of myelofibrosis with myeloid metaplasia was still relatively low at the 10th year, about 15% in all arms, but increased towards a value higher than 30% in the patients surviving at the 20th year. At the present time, but in only a few cases with long-term following, no myelo-fibrosis with splenic metaplasia has been observed in the pipobroman-treated cases. The present results, which need to be confirmed (the present analysis has been done in spring 95) suggest that:-the use of non radio-mimetic agents does not protect against leukemic transformation, which may be a consequence of the disease; rather than of the treatment,-maintenance therapy after initial use of 32P increases the risk of both leukemia and carcinoma,-and hydroxyurea does not delay the risk of developing myelo-fibrosis, in comparison with 32P alone.

Actuarial Analysis↗
PubMed1997

Lymphoid myelofibrosis associated with high grade B cell lymphoma of the liver: morphological, cytogenetic, and clinical features.

Severe pancytopenia associated with moderate hepatosplenomegaly, increased serum lactic dehydrogenase (LDH) levels, and hypogammaglobulinemia were found in a young male patient. Bone marrow histology showed extensive fibrosis, hypoplasia of erythro- and granulocytopoiesis, and hyperplasia of megakaryocytopoiesis associated with histiocytic fat cell phagocytosis and infiltration of abnormal lymphocytes, compatible with lymphoid myelofibrosis. Striking chromosomal aberrations indicating karyotype evolution were also demonstrated by cytogenetic analyses (47, XY, +3 / 47, XY, +3, 1p+ / 46, XO, +3, 1p+, -Y). The clinical course was characterized by cyclic febrile episodes accompanied by excessive increase of serum LDH levels and leukocyte counts, and decrease of platelet counts, followed by spontaneous regression. Further diagnostic procedures, including two liver biopsies and computed tomography, did not detect any manifestation of lymphoma. Eventually, the patient developed rapidly progressive, lethal pulmonary aspergillosis. At autopsy, high grade B cell lymphoma of the liver was found. In this case, the lymphoid myelofibrosis seen on bone marrow biopsy may be considered as a manifestation of "discordant" bone marrow histology related to high grade lymphoma. With respect to the cyclic clinical course, a possible role of apoptotic mechanisms in the physiopathology of this disorder is reviewed.

Adult↗
PubMed1999

[A case of elderly-onset systemic lupus erythematosus (SLE) complicated with severe liver dysfunction and pancytopenia due to myelofibrosis].

A 67-year-old woman was admitted to our hospital with a fever. She had been experiencing arthralgia for about one month. On admission, she had a fever of 38.5 degrees C, was anemic and was experiencing tenderness in the joints of both hands, elbows and feet. Laboratory data revealed proteinuria, urinary cylinders, pancytopenia (WBC 900/mm3, Hb 9.5 g/dl, Plt 7.8 x 10(4)/mm3), liver dysfunction (GOT 414 IU/l, GPT 140 IU/l), and hyper-gamma globulinemia. Antibiotics and granulocyte-colony stimulating factor were administered intravenously. Bone marrow aspiration was unsuccessful, but a bone marrow biopsy revealed bone marrow fibrosis. Immunological examinations were positive for antinuclear antibodies, anti-deoxyribonucleic acid (DNA) antibodies, anti-double stranded anti- DNA antibodies, as well as a decreased level of serum complement and an increased level of serum immune complexes. Tests for viral antigens and antibodies known to cause hepatitis were negative. Based on these findings, a diagnosis of SLE accompanied by liver dysfunction and bone marrow fibrosis was made. Steroid pulse therapy was initiated, but her liver function deteriorated on the first day of steroid therapy, and she died three days later. SLE accompanied by myelofibrosis is extremely rare, and only 17 and cases have been reported to date. Among these reports, the present case is the second oldest subject and the first SLE patient to suffer from both myelofibrosis and severe liver dysfunction.

Aged↗
PubMed1976

Myelofibrosis in Western Australia: an epidemiological study of 29 cases.

Survival figures and other epidemiological features are described for 29 patients with myelofibrosis living in Western Australia during the period 1959 to 1963. These patients form part of a larger series of 509 patients with leukaemia and allied disorders. The diagnostic incidence rate was found to be 0.5 per 100,000 population, the mortality rate was 0.4 per 100,000 and the prevalence 1.8 per 100,000. Comparison with related myeloproliferative disorders shows that myelofibrosis occurs somewhat less frequently than chronic granulocytic leukaemia and considerably less so than polycythaemia vera. The sex ratio indicates a small male preponderance and the age distribution is similar to that in other descriptions. Survival times for all patients accord with those reported in the literature, but this series showed a marked female advantage in survival. Findings in other series are described.

Age Factors↗
PubMed1999

[Hypereosinophilic syndrome complicated by myelofibrosis].

Hypereosinophilic syndrome (HES) with myelofibrosis was diagnosed in a 36-year-old man on the basis of bone marrow biopsy findings and clinical features. Although the patient was treated with steroid (1 mg/kg), hydroxyurea, and immunosuppressive therapy, eosinophilia persisted. Patients with HES and myelofibrosis are usually unresponsive to antineoplastic agents and/or immunosuppressants. However, cyclosporin may be an effective alternative for such patients.

Adult↗
PubMed1999

Laparoscopically assisted splenectomy following preoperative splenic artery embolization using contour emboli for myelofibrosis with massive splenomegaly.

Laparoscopically assisted splenectomy with an 8- to 10-cm left upper paramedian laparotomy was performed following preoperative splenic artery embolization using painless contour emboli (super absorbent polymer microsphere) with early successful results in two men (46 and 37 years old) with myelofibrosis accompanied by massive splenomegaly. Dissection around the lower part of the spleen and the hilum initially was performed intracorporeally with the usual laparoscopic view under 12 mm Hg pneumoperitoneum. The alternating changes of viewpoints between the direct view through an 8- to 10-cm incision and the usual laparoscopic view with or without application of a retraction method were effective for safe hilar devascularization. Preoperative splenic artery embolization at the distal site was effective for safe dissection around the enlarged spleen. The patients did not complain of pain before operation. Preoperative painless embolization and laparoscopically assisted splenectomy with small laparotomy promotes the feasibility and safety of minimally invasive splenectomy for myelofibrosis with massive splenomegaly.

Adult↗
PubMed2000

Relevance of bone marrow features in the differential diagnosis between essential thrombocythemia and early stage idiopathic myelofibrosis.

BACKGROUND AND OBJECTIVES: Diagnosis of essential thrombocythemia (ET) remains a challenging problem and has been predominantly established by exclusion of other thrombocythemic disorders. In this context the updated diagnostic criteria of the Polycythemia Vera Study Group (PVSG) are generally accepted, although histopathologic features of the bone marrow were only marginally considered. DESIGN AND METHODS: A retrospective evaluation was performed of 168 patients presenting with ET in accordance with the criteria of the PVSG. Analysis was focused on the discriminating impact of bone marrow morphology. RESULTS: Histopathology revealed that our cohort of patients could be divided into three distinct groups (true ET, questionable ET and false ET). These groups were characterized by certain diagnostic constellations of clinical data on admission. True ET was found in 53 patients presenting with no or a borderline splenomegaly and no relevant anemia or leuko-erythroblastic blood picture. The other patients showed clinical signs and symptoms which were more compatible with initial-prefibrotic (52 patients) or early (68 patients) idiopathic-primary myelofibrosis (IMF) with severe thrombocythemia. In true ET no significant hypercellularity of the bone marrow including myeloid precursors or an increase in reticulin fibers was detectable. Most prominent were changes of megakaryopoiesis which revealed large to giant-sized cells lacking a definite maturation defect. Their appearance in true ET contrasted with the clusters of abnormally differentiated, often bizarre elements of this lineage in patients with initial and early IMF (questionable or false ET). Calculation of survival disclosed a relevant disparity with a non-significant loss in life expectancy of 10.9% in true ET compared to 29.6% in questionable and 51.3% in false ET. Follow-up studies and repeated bone marrow biopsies revealed no transition into myelofibrosis in true ET, whereas this did occur in 22 of 27 patients with questionable and false ET. In the latter cohort bone marrow changes were accompanied by increasing anemia, splenomegaly, tear-drop poikilocytosis and reduction of the platelet count consistent with IMF. INTERPRETATION AND CONCLUSIONS: A detailed evaluation of bone marrow features, in particular megakaryopoiesis is recommended to establish positive criteria for the diagnosis of ET and thus to accomplish a significant improvement of the PVSG postulates. In this context ongoing clinical trials on ET must regard pretreatment bone marrow biopsies as a major clue to diagnosis.

Adult↗
PubMed2001

Idiopathic myelofibrosis complicated by portal hypertension treated with a transjugular intrahepatic portosystemic shunt (TIPS).

Idiopathic myelofibrosis may be accompanied by portal hypertension. The authors report a 56-year-old man with idiopathic myelofibrosis and splenomegaly complicated by hepatopathy, severe portal hypertension and recurrent variceal bleeding. A transjugular intrahepatic porto-systemic shunt (TIPS) was inserted. Variceal bleeding never recurred. A short episode of encephalopathy, which is a known complication of porto-systemic shunting, ceased promptly after conservative treatment. The patient eventually died six months later due to metabolic deterioration and hepatic failure related to his underlying hematological disease. TIPS is a promising treatment modality for alleviating symptomatic portal hypertension in hematological disorders.

Biopsy↗
PubMed2002

Intraspinal extramedullary haematopoiesis in a patient with myelofibrosis.

A 54-year-old patient with myelofibrosis developed paresis of the legs, and bladder dysfunction due to extramedullary haematopoiesis in the spinal channel. He was given palliative radiotherapy but died shortly afterwards. Although rare, the possibility of extramedullary haematopoiesis in the central nervous system should be considered when neurological symptoms appear in a patient with myelofibrosis, because good palliation is possible with timely radiotherapy.

Hematopoiesis, Extramedullary↗