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PubMed1982

Systemic mastocytosis, myelofibrosis and portal hypertension.

A case of systemic mastocytosis is described in which the finding on initial presentation was hepatosplenomegaly. No dermatological abnormality was present, and the bone marrow histology originally caused some confusion with primary myelofibrosis. The clinical course and the importance of distinguishing between these two diseases is discussed. The dermatological manifestation of systemic mastocytosis, in the form of urticaria pigmentosa, is well recognised, and alerts the physician to the underlying disease. In the absence of cutaneous signs, however, the diagnosis is less obvious. The case reported had predominantly marrow and splenic involvement by the disease process, giving rise to portal hypertension, and illustrates the problems of diagnosis which can arise.

Bone Marrow↗
PubMed1984

Chronic lymphocytic leukaemia terminating in acute myelofibrosis.

Acute myelofibrosis developed in a patient with longstanding chronic lymphocytic leukaemia who had been treated with alkylating agents and total body irradiation. The case is discussed in the context of acute non-lymphoid leukaemias occurring in chronic lymphocytic leukaemia with special reference to megakaryoblastic variants.

Acute Disease↗
PubMed1999

Clinicopathological features of seven cases of canine myelofibrosis and the possible relationship between the histological findings and prognosis.

Seven dogs with non-regenerative anaemia were diagnosed as having myelofibrosis on the basis of the presence of collagen and increased deposits of reticulin fibre in the haemopoietic spaces of bone marrow core biopsies. A scoring system was used to assess the cellularity of the marrow and the amounts of collagen, reticulin and haemosiderin present. These scores, together with the haematological findings, were compared with the dogs' responses to treatment and their outcome. Treatment consisted of blood transfusions, where required, and anabolic steroids and corticosteroids. Three dogs deteriorated and were euthanased within three months of diagnosis, but the other four recovered fully. There was no correlation between the collagen and reticulin scores, or the degree of anaemia and the outcome, but the four dogs which recovered all had a macrocytosis when first examined. There was no evidence of an underlying lymphoproliferative or myeloproliferative disease in any of the seven cases.

Anemia↗
PubMed1979

Diffuse pulmonary uptake of Indium-111 chloride in idiopathic myelofibrosis.

Unusual indium-111 accumulation and extramedullary hematopoiesis in the lungs of a patient with idiopathic myelofibrosis are described. The bone marrow scan taken 24 hours after intravenous injection of 111InCl3 faithfully depicted the abnormal distribution of marrow elements as assessed histologically at autopsy, thereby supporting the usefulness of 111InCl3 for marrow imaging.

Aged↗
PubMed2002

Idiopathic myelofibrosis with nodal, serosal and parenchymatous infiltration. Case report and review of the literature.

Idiopathic myelofibrosis (IMF) is a breakpoint cluster region rearrangement-negative chronic myeloproliferative disease with progressive bone marrow fibrosis. We report a female patient (65 years old) who was admitted to our hospital in 1996. Trephine bone marrow biopsy revealed diffuse fibrosis with atypical multilobulated megakaryocytes. A cellular phase of IMF was diagnosed. Three years later, despite being at intermediate risk, the patient developed generalized lymphadenopathy and multiple sclerosing tumors throughout the peritoneum and retroperitoneum. Biopsy specimens from these tumors revealed sclerosing hematopoietic infiltrates. The present case demonstrates that IMF not only can progress to acute leukemia but can also spread uncontrollably, or 'metastasize' with extensive sclerosing hematopoietic tumors throughout the body.

Aged↗
PubMed2004

Severe pulmonary hypertension in chronic idiopathic myelofibrosis.

BACKGROUND: Chronic myeloproliferative disorders (CMPD) seem to be associated with an increased risk for pulmonary hypertension (PH). CASE REPORT: A patient with history of chronic idiopathic myelofibrosis (CIMF) presented with progressive dyspnea (New York Heart Association class III). Until this time he had not received specific treatment for CIMF. Echocardiography and rightheart catheterization confirmed PH. Further diagnostic procedures excluded a specific cause of PH. Therefore, primary PH was assumed. 2 years later he presented again with progressive dyspnea due to a progress of PH. A few days later the patient died from acute posterior myocardial infarction. Pathologic examination of the lung showed an obstruction of the small vessels by conglomerates of megakaryocytes. DISCUSSION: We conclude that PH developed secondarily due to CMPD. PH should be suspected in patients with CMPD and should influence the decision for treatment of CMPD.

Chronic Disease↗
PubMed2005

Extramedullary hematopoiesis in chronic myelofibrosis encasing the pelvicaliceal system and perirenal spaces: CT findings.

We examined a 46-year-old male patient with idiopathic myelofibrosis diagnosed 28 months earlier. After chemotherapy and irradiation, CT showed nonnodular, soft tissue masses in the renal hilum, pelvicaliceal system, perirenal and periureteral spaces. Ultrasound-guided biopsy showed extramedullary hematopoiesis (EMH). The mass, which caused delayed nephrographic enhancement, without hydroureteronephrosis, was the only site of EMH. We report the CT findings of this uncommon EMH localization.

Chronic Disease↗
PubMed2006

Trisomy 8 in prefibrotic early stages of chronic idiopathic myelofibrosis: a fluorescence in situ study of bone marrow biopsies.

Repeatedly performed bone marrow biopsies were studied in 30 patients with chronic idiopathic myelofibrosis (CIMF) by fluorescence in situ hybridization to detect and quantify trisomy 8 anomaly during the evolution of disease. For the establishment of threshold values we used negative and positive control specimens. At least 500 cells were evaluated in each specimen and only nuclei with three distinctive signals were regarded as positive. According to the controls, 27 patients revealed false-positive signals ranging from 0 to 1.2% (0.88 +/- 1.12). On the other hand, 3 patients showed an incidence of more than 6.5% (up to 10.1%) in the initial prefibrotic as well as advanced fibro-osteosclerotic stages of CIMF. In conclusion, trisomy 8 has been demonstrated already in the prodromal stages of CIMF and therefore is not limited to classical fibro-osteosclerotic manifestations.

Biopsy↗
PubMed2006

Recombinant human erythropoietin for the treatment of anaemia in patients with chronic idiopathic myelofibrosis.

BACKGROUND: Patients with chronic idiopathic myelofibrosis (CIMF) usually present with anaemia. Treatment is often palliative and the majority of patients receive regular red blood cell (RBC) transfusions. Although recombinant human erythropoietin (rhu-EPO) has been proved effective for the treatment of anaemia in several chronic diseases, including haematological malignancies, its role in the treatment of the anaemia in CIMF is not well established. We report the beneficial effect of rhu-EPO administration in 20 patients with CIMF and discuss the parameters predicting favourable response. PATIENTS: Twenty patients with CIMF (9 women and 11 men) regularly treated with supportive RBC transfusions were included in the study. The median age was 70 years (range 45-81 years). Rhu-EPO, 10,000 U, was given subcutaneously 3 times a week. The median duration of therapy was 83 months, ranging from 13 to 87 months. RESULTS: Treatment was considered effective if haemoglobin levels increased over 2 g/dl within 12 weeks after enrolment or the RBC transfusion requirements were reduced by 50% within the same interval. Twelve patients (60%) responded to therapy. Responders were mainly female, had smaller spleen size (p = 0.024), low RBC transfusion requirements (< or = 1-2 units per month), and significantly lower endogenous serum erythropoietin (EPO) and beta2-microglobulin (beta2-M) levels when compared with non-responders (p < 0.0001 and 0.00001, respectively). Treatment was well tolerated and none of the patients was withdrawn from the treatment protocol because of side effects. CONCLUSIONS: Rhu-EPO administration is an effective, safe and well-tolerated treatment for patients with CIMF and anaemia leading to a significant reduction in RBC transfusion requirements. Factors predicting favourable response are low endogenous EPO and beta2-M serum levels and slight to moderate splenomegaly.

Aged↗
PubMed1989

A new glucose 6-phosphate dehydrogenase variant (G6PD Thessaloniki) in a patient with idiopathic myelofibrosis.

A new deficient glucose 6-phosphate dehydrogenase (G6PD) variant, G6PD Thessaloniki, which was found in the red blood cells of a 70-year-old woman who had idiopathic myelofibrosis, is described. G6PD Thessaloniki had a low Michaelis constant (Km) for G6P (20 microM), high Km for NADP (10.1 microM), normal pH optimum, reduced heat stability, decreased electrophoretic mobility (96-98% of the normal), increased 2-deoxy-G6P and decreased galactose 6-phosphate utilization. Several other enzymatic activities measured in the patient's red blood cells were normal. Studies of red blood cell survival and glucose utilization gave evidence of haemolysis caused by defective glucose utilization by the pentose phosphate pathway. The only son of the patient had normal G6PD in his red blood cells. In an attempt to investigate the origin of G6PD Thessaloniki, heat stability tests of G6PD extracted from the patient's skin have been performed.

Aged↗
PubMed1994

Myelofibrosis in severe combined immunodeficiency due to vitamin D deficiency rickets.

An infant with severe combined immunodeficiency is described in whom a refractory anemia and thrombocytopenia developed after the age of 6 months, associated with poor growth and frequent episodes of upper respiratory tract infections and diarrhea. He required frequent blood and platelet transfusions. Bone marrow biopsy provided evidence that the anemia and thrombocytopenia were the results of myelofibrosis which was secondary to vitamin D deficiency. Therapy with 1,25-dihydroxycholecalciferol resulted in resolution of the hematologic and skeletal abnormalities, but he remained susceptible to recurrent serious infections and died at the age of 13 months.

Biopsy, Needle↗
PubMed1993

Recombinant human erythropoietin in patients with myelodysplastic syndrome and myelofibrosis.

In a dose titration study we tested the efficacy and tolerance of recombinant human erythropoietin (rhEPO) in 10 patients with myelodysplasia (MDS) and 2 patients with idiopathic myelofibrosis. Patients with a haemoglobin level < 100 g/l were treated as out-patients for 12 weeks with daily doses ranging from 30 U/kg body weight (BW) up to 240 U/kg BW in non-responders. Of the 10 patients with MDS, 6 were suffering from refractory anaemia with sideroblasts (RAS) and 4 from refractory anaemia with an excess of blasts. The median age was 73 years (range 41-81). Two patients with RAS responded with a rise in haemoglobin concentration to > 130 g/l. They had not been transfusion-dependent prior to treatment. Both patients had relatively low serum concentrations of immunoreactive EPO. There was neither a rise in haemoglobin nor a reduction in transfusion dependence in any of the other patients. It may be concluded that rhEPO is possibly effective in a subgroup of MDS patients where the disease is less advanced. None of the transfusion-dependent patients benefited.

Adult↗
PubMed1991

Liver dysfunction following splenectomy in idiopathic myelofibrosis: a study of 10 patients.

The hepatic abnormalities that developed after the splenectomy in 10 subjects with idiopathic myelofibrosis were analyzed. In all patients in whom a liver biopsy was performed during the splenectomy, extramedullary hematopoiesis was demonstrated, consisting of dysmorphic megakaryocytes primarily localized in the sinusoids, often accompanied by erythroid precursors. Following splenectomy, a significant increase in both the liver size and serum levels of alkaline phosphatase, bilirubin or gamma-glutamyl transpeptidase was found within 6 months, whereas no such increase was observed in the serum aspartate transaminase and alanine transaminase concentrations. In addition, 2 patients developed acute liver failure leading to death at 3 and 4 weeks from splenectomy, respectively. In contrast with these findings, no hepatic alterations were observed in 10 chronic myeloid leukemia patients who were also submitted to splenectomy.

Adult↗