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PubMedDate not supplied

Long-lasting effect of cyclosporin-A on anemia associated with idiopathic myelofibrosis.

Some reports suggest that immunologic mechanisms may play a role in the pathogenesis of anemia in idiopathic myelofibrosis (IMF). Herein we report the case of a transfusion dependent IMF patient with psoriasis in whom cyclosporin-A (CyA) treatment for skin lesions (200 mg/day) was associated with long-lasting correction of anemia. After 2 months of CyA therapy the patient's Hb level increased and he became transfusion free in 4 months. After 12 months immunosuppressive therapy was discontinued due to renal toxicity, yet the Hb level remained stable for an additional 12 months. The patient is currently being administered CyA at a reduced dosage because of mild renal impairment along with transfusional support consisting of a median of 2 red cell units/month. Altogether the patient received no transfusional support for 36 months. This case, as well as other reports, suggests that the issue of immunosuppressive treatment in IMF anemia deserves further investigation.

Aged↗
PubMedDate not supplied

Idiopathic myelofibrosis with neutrophil myeloperoxidase deficiency: a case report.

We describe a case of idiopathic myelofibrosis with total neutrophil myeloperoxidase deficiency. The combination of this enzymatic defect with myelofibrotic changes in the nuclear shape of neutrophils confers a peculiar appearance on leukograms produced by a Technicon H*1. The clinical course of the disease was shortened by recurrent infections that may be ascribed, at least in part, to reduced leukocyte microbicidal ability.

Bone Marrow↗
PubMed1997

Acute myelofibrosis.

Acute myelofibrosis is a rare disorder. Five such cases have been diagnosed out of 3,149 Bone Marrow (B.M) Aspirations and Trephine Biopsies studied over a period of eight and a half years. Four out of five patients were males and the other female. Their age ranged from 14-71 years. Neither splenomegaly not red cell poikilocytosis (tear drop cells) were prominant features. B.M. trephine biopsy showed increase of atypical megakaryocytes/megakaryoblasts and marked fibrosis. There was rapid downhill clinical course of the disease in all cases.

Acute Disease↗
PubMed1997

[Idiopathic myelofibrosis: initial features, evolutive patterns and survival in a series of 106 patients].

BACKGROUND: Idiopathic myelofibrosis (IM) is an infrequent myeloproliferative disorder with few large series published in the medical literature. Information on the characteristics of more recently diagnosed patients with IM is scarce. PATIENTS AND METHODS: The initial features, evolutive patterns and survival from 106 patients diagnosed with IM in a single institution between 1975 and 1996 were analyzed. RESULTS: Median age of the series was 64 years (range: 17-89); there were 61 males and 55 females. One third of the patients were asymptomatic at IM diagnosis. The most common presenting symptoms were related to hypermetabolism, anemia and splenomegaly. A palpable spleen was noted in 85% of patients, and 66% had hepatomegaly. Anemia was the most frequent hematologic abnormality (50% of cases). Bone marrow biopsy showed cellular phase IM in 50 patients, IM without osteosclerosis in 39, and osteosclerotic IM in the remaining 17. Evolution into acute leukemia was seen in 14 patients (actuarial probability at 7 years: 20%, 95% CI: 0-40%), 8 patients developed portal hypertension, 5 liver failure without portal hypertension and 14 heart failure. With 62 patients having died, the series' median survival was 59 months (95% CI: 41-75). CONCLUSION: IM is usually diagnosed in the old age. From the histologic point of view, a predominance of the cellular phase is observed at disease presentation. In recent years an increased proportion of patients asymptomatic at diagnosis is observed.

Adolescent↗
PubMed1998

Retinal manifestations of idiopathic myelofibrosis, a hematologic disorder.

BACKGROUND: Idiopathic myelofibrosis (IMF) is a bone marrow and hematologic disorder. Retinal and systemic manifestations are observed and result from inefficient blood cell production and blood cell irregularities. CASE REPORT: A review of IMF as a disease process is presented by way of case report. A 70-year-old woman came to us with specific systemic symptoms. Further ocular investigation revealed bilateral retinal hemorrhages. The hemorrhages were consistent with the results of subsequently ordered blood tests. An understanding of the underlying systemic condition and recognition of the retinal manifestations of IMF aid in the diagnosis. Recognizing differential diagnoses is important as well because IMF produces retinal findings similar to other blood disorders with comparable blood counts and morphologies. There are certain systemic signs the optometrist may look for to assist in the recognition of IMF as opposed to the other diseases. CONCLUSION: This is an important disease process to recognize, since the systemic and retinal manifestations are within optometry's scope to co-manage.

Aged↗
PubMed1998

[Occurrence of myelofibrosis in individual types of chronic myeloproliferative diseases].

BACKGROUND: During the course of chronic myeloproliferative disorders (CMPD), myelofibrosis (MF) represents a negative prognostic factor. The data concerning the incidence and progression of MF are rather heterogenous. OBJECTIVES: The aim of the study was to evaluate the incidence and progression of MF in cases of CMPD registered in the Consultation Center for Haematopathology Biopsies in Martin Faculty Hospital. METHODS: Fibrotic changes involving bone marrow were evaluated histologically semiquantitatively using reticulin fiber impregnation (method of Gomori). The study included 77 cases of chronic myelocytic leukemia (CML), 99 cases of polycythaemia vera (PV), 38 cases of essential thrombocythaemia (ET), and 126 cases of the fourth type of CMPD, mostly known as myelofibrosis/osteomyelofibrosis type (MF/OMF). The occurrence and degree of MF were evaluated at the time of all primary biopsies; in 52 of cases also in rebiopsy material. RESULTS: At the time of primary diagnosis, MF occurred in 37/77 (48%) cases of CML, in 27/99 (27.3%) cases of PV, in 8/38 (21%) cases of ET, and in 119/126 (94.4%) cases of MF/OMF. In repeated (secondary) biopsies, the progression of MF or evolution to MF was most common in CML and MF/OMF types. CONCLUSIONS: At the time of the CMPD diagnosis, more than 50% of cases showed the presence of MF. During the course of CMPD, the MF seems to represent a dynamic process evolving the underlying disease. The early diagnosis of MF is important for the selection of the appropriate therapeutic regimen. (Tab. 3, Fig. 2, Ref. 23.)

Bone Marrow↗
PubMed1998

Cyclosporin-A in severe refractory anemia of myelofibrosis with myeloid metaplasia: a preliminary report.

BACKGROUND AND OBJECTIVE: Severe anemia is the outstanding problem in approximately 50 percent of patients with myelofibrosis with myeloid metaplasia (MMM). The present trial was based on the considerations that abnormal immune responses are frequently associated with MMM and that cyclosporin A (Cy-A) has proven to be effective in improving anemia in autoimmune disorders. The aim of this study was to evaluate the effect of Cy-A on anemia of MMM. DESIGN AND METHODS: We studied 10 patients with MMM and severe anemia who were not responsive to corticosteroids. Eight of them showed evidence of immune defects (direct or indirect Coombs' test, antinuclear or antimitochondrial antibodies, circulating immune complexes). Cy-A was delivered orally in two refracted doses of 5 mg per kilogram bw every day and the serum level of the drug was maintained between 100 and 200 ng/mL for at least 6 months. Clinical effects were measured by calculating a normalized transfusional need (NTN), and response was defined as about a 30% reduction in the initial transfusion requirement. Hematologic parameters, s-Epo, s-TfR, s-IL2R and lymphocyte flow cytometric analysis were also evaluated. The results were analyzed with the Student's t-test. RESULTS: Only 6 patients completed the entire 6 months of planned therapy. Three of these responded, with one no longer needing transfusions. A high CD4/CD8 ratio was predictive of response (mean value 4.7 +/- 3.5 in responders versus 0.9 +/- 0.4 in non-responders, p = 0.06). INTERPRETATION AND CONCLUSIONS: An immunomediated mechanism negatively affects erythropoiesis in MMM. Cy-A may be effective for patients with severe refractory anemia in this disease.

Adult↗
PubMed1998

Effect of cyclosporin-A on anemia in idiopathic myelofibrosis.

The recent report of Pietrasanta et al., describe an improvement of anemia in a case of idiopathic myelofibrosis (IMF) treated with cyclosporin-A (CyA) for Psoriatic skin lesions. We have also used CyA in four IMF patients, all with transfusion dependent anemia . We report briefly the clinical aspects, hematological parameters and responses to the treatment for every patient.

Aged↗
PubMed1998

Systemic lupus erythematosus presenting as myelofibrosis.

Myelofibrosis is not frequent in systemic lupus erythematosus (SLE). A review in the literature reveals that the co-incidence is rather rare since there are only a few papers reporting this combination. The female patient described hereby, presented with thrombocytopenia; following investigation, the diagnosis of SLE was established and bone marrow examination revealed an increase of marrow reticulin. Treatment with steroids reversed both thrombocytopenia and bone marrow fibrosis.

Adult↗
PubMed1998

[Transformation of myelofibrosis into acute myelogenous leukemia (M0) with multiple tumor emboli].

A 60-year-old woman was admitted in August 1995 complaining of abdominal pain. A diagnosis of essential thrombocythemia had been made in 1987, and myelofibrosis developed in the patient thereafter. Physical examination revealed massive hepatosplenomegaly, and the peripheral blood showed leukoerythroblastosis with chromosomal abnormalities in peripheral blood cells. In May, 1996, blastic transformation occurred. Based on the findings of surface marker analysis, the blasts met the diagnostic criteria for acete myelogenous leukemia because they were negative for peroxidase and positive for CD13. In June, the patient died of multiple organ failure. Postmortem examination revealed multiple tumor emboli.

Cell Transformation, Neoplastic↗
PubMed1976

Prognosis of myeloid metaplasia with myelofibrosis.

The study of the evolution of 168 cases of idiopathic myeloid splenomegaly allowed to point out the prognostic value of the clinical hematologic, isotopic and radiological parameters. The correlation of these data with the histological type of the osteomedullary lesions at the time of the diagnosis, allows to confirm the long survival of the hyperplastic formes (type I) and the more reserved prognosis in advanced myelofibrosis (type II and III together). Indeed, median survival is of 82 months for type I, and 60 months for type II and type III together. It seems advice to gather the two last types and so, to compare two anatomoclinical entities : one is characterized by hyperplastic marrow with reticulinic fibrosis, usually associated with enlarged spleen and sometimes even with increase red blood cells volume but without radiological bone lesions; the other one is characterized by a marrow of middle importance or marked marrow depletion, fibrosis, with or not osteosclerosis, and shows moderate or marked radiological lesions (respectively 16,9% of the cases) and, often, pancytopenia.

Adult↗
PubMed1998

Reversal of bone marrow fibrosis in idiopathic myelofibrosis after treatment with alpha-interferon.

A 14-year-old boy presenting with primary myelofibrosis (MMM) was treated with alpha-interferon (IFN). After 6 months his spleen was no longer palpable and blood counts had returned to normal. A reversal of bone marrow fibrosis was histologically documented. Although improvement in symptoms and blood counts has been reported, this is only the second description of reversal of bone marrow fibrosis in MMM after treatment.

Adolescent↗
PubMed2006

FLT3/D835 mutation and inversion of chromosome 16 in leukemic transformation of myelofibrosis.

We present an atypical case of myelofibrosis developing into secondary leukemia FAB subtype M4, with inversion of chromosome 16, FLT3/D835 point mutation and diffuse osteolytic lesions accompanied by elevated TNF-alpha. The simultaneous occurrence of these mutations reflects the progressive association of genetic lesions developing into secondary leukemia with a relatively benign course.

Journal Article↗
PubMed2004

Fatal hepatic failure associated with graft rejection following reduced-intensity stem-cell transplantation for chronic idiopathic myelofibrosis (CIMF).

A 54-year-old man with chronic idiopathic myelofibrosis (CIMF) underwent RIST. His clinical course had been uneventful until day 60, when splenomegaly reappeared. Hepatic dysfunction developed on day 75. Recipient-type hematopoiesis increased to 51% on day 90. After rapid tapering of cyclosporin, serum levels of AST and ALP normalized in parallel with recovery of complete chimerism on day 134. Yet, jaundice progressed. He died of liver failure on day 176. Postmortem examination revealed neither GVHD nor VOD. Graft rejection following RIST for CIMF may lead to fatal hepatic damage through extramedullary hematopoiesis in the liver or cytokine-mediated immune dysregulations.

Chronic Disease↗
PubMedDate not supplied

Bone marrow fibrosis (pseudo-myelofibrosis) in human kala-azar.

Thirty cases of human kala-azar were diagnosed by iliac crest biopsy and myeloculture. Histological analysis of 12 patients showed diffuse thickening of reticulin fibers. To the best of our knowledge, this is the third report describing secondary bone marrow fibrosis (myelofibrosis-like) associated with kala-azar. Patients with positive bone marrow fibrosis (pbmf = 12) were compared to patients without detectable bone marrow fibrosis (wbmf = 18). There were no significant differences in clinical and blood parameters following treatment. All patients showed regression of hepatosplenomegaly. Our findings suggest that associated bone marrow fibrosis is transient and did not interfere in the evolution of treated patients.

Biopsy↗
PubMed2005

A patient with TP53 germline mutation developed Bowen's disease and myelodysplastic syndrome with myelofibrosis after chemotherapy against ovarian cancer.

Here we report a case of myelodysplastic syndrome (MDS) with myelofibrosis associated with Bowen's disease. A female patient had undergone an operation and chemotherapy for ovarian cancer when she was 65 years old, and she developed MDS at the age of 70 years old. PCR-single strand conformation polymorphism (SSCP) analysis of peripheral blood mononuclear cells, a Bowen's disease lesion, and normal skin showed an abnormal peak in TP53 exon5. Direct sequencing revealed that they all had missense mutation in codon 175 (G to A) of arginine switched to histidine, suggesting a germline mutation of TP53. It was speculated that p53 function was lost by TP53 germline mutation with the loss of a wild type allele induced by the chemotherapy against ovarian cancer, leading to the development of MDS. No therapeutic effects of low dose melphalan or cyclosporine A on MDS were observed, however one month of 30 mg/day prednisolone administration induced a hematological response.

Aged↗
PubMed1977

[Lymphoid myelofibrosis or hairy cell leukemia].

By lymphoid myelofibrosis or hairy cell leukaemia or tricholeukaemia is meant an unusual haemopathic condition known only for the past few years. It is characterized pathognomonically by the presence of lymphocyte type cells with villous extroflexions, hence the name "hairy cell". Clinically the disease presents as an involutive myelopathy associated with splenomegaly, generally without any particular lymph gland involvement. The attention of students today is concentrated on the nature of the hairy cells; while some are inclined to admit their monocyte or histiocyte derivation, others consider that they derive from B lymphocytes. Therapeutically, almost everybody agrees that splenectomy is the only valid step. A case of H.C.L., which was typical from the clinical and laboratory viewpoints is reported. It is probable that certain haemopathic pictures once classified among atypical leucoses and lymphomas, would today be more correctly classed as hairy cell leukaemia.

Alkaline Phosphatase↗