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Query: UMLS:C0032463 (polycythemia vera)
 3,374 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Familial chronic myeloproliferative syndrome (CMS) was observed in five members from two different generations of the same kindred. Diagnosis included agnogenic myeloid metaplasia (case 1), polycythemia vera (case 2), and essential thrombocythemia (cases 3-5). Cases 1-3 were siblings, case 5 was the daughter of case 1, and case 4 was the cousin of cases 1, 3. Age at diagnosis ranged from 28 to 75 years, cases 1 and 3 were male, and the others were female. The diagnosis was made after an episode of cerebral thrombosis in one patient, during a study for headache and dizziness in another, and fortuitously in the three remainders. All patients had splenomegaly and varying degrees of thrombocytosis. The cytogenetic exam was normal in all four cases. A woman patient was treated with interferon during a pregnancy. Fetal growth was retarded, and the newborn showed bone and genital malformations. No environmental leukemogen factor was found. This familial case strengthens Dameshek's theory of a common pathogenesis of CMS and suggests a genetic and hereditary etiology.
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PMID:Familial myeloproliferative syndrome. 819 53

We studied the effects of recombinant alpha 2-b interferon (alpha 2-b IFN) in a dose of 3 x 10(6)U intramuscularly three times a week for 1 year in 13 patients affected by polycythemia vera (PV) previously treated with phlebotomy only. Response to treatment was evaluated by reduction of the number of phlebotomies required to retain normal hematocrit value. Ten out of 13 patients (77%) responded to treatment; in 4 of them the exigency of phlebotomy was completely eliminated. In all responders a concomitant decrease of platelet count and splenomegaly was obtained. Treatment was well tolerated and side effects were easily controlled. We conclude that alpha-IFN may represent an attractive therapeutic option in the management of the proliferative stages of PV.
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PMID:Recombinant interferon alpha-2b in the treatment of polycythemia vera. 821 63

Patients with polycythaemia and normal controls have been studied to establish and subsequently test non-conventional criteria for the diagnosis of primary polycythaemia (primary proliferative polycythaemia, polycythaemia vera) as compared with conventional Polycythaemia Vera Study Group (PVSG) assessment. One criterion was erythroid colony formation from peripheral blood in a serum-free system, assayed alone and with the addition of recombinant human erythropoietin (Epo), interleukin 3 (IL3), or alpha interferon (alpha-IFN) (Dudley et al. 1990). The remaining criteria were non-culture associated and comprised platelet distribution width (PDW), platelet nucleotide ratio (ATP:ADP), serum erythropoietin and clinical evidence of ischaemic vascular disease. The combination of culture associated and non-culture associated variables, by use of a simple additive scoring system, gave no false positive and only 6% false negative results in distinguishing primary polycythaemia from other polycythaemias and normal controls in those (34 patients Group A) used in its derivation. Testing the scoring system in a newly presenting group (25 patients Group B) was highly satisfactory with no false positives and only a few false negative results (14%). Use of these non-conventional criteria should allow more confident diagnosis of primary polycythaemia, where conventional clinical and laboratory assessment is inconclusive.
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PMID:Primary polycythaemia: diagnosis by non-conventional positive criteria. 824 12

The authors report the case of a 60-year-old white man with a previous history of pulmonary tuberculosis, smoking habits, hypertension, intermittent claudication and erythromelalgia, admitted to our ward with an ischemic cerebral event. Initial laboratory evaluation documented thrombocytosis (platelet-950000/mm3) and discrete anemia. Additional studies confirmed the diagnosis of essential thrombocythemia, meeting all the criteria proposed by the Polycythemia Vera Study Group in 1986, after exclusion of the possible causes of reactive thrombocytosis. Therapy was initiated with alpha-2b interferon (3 MU/m2 subcutaneously three times a week) and aspirin. Platelet count control was obtained and the patient remained asymptomatic. Nine months later cutaneous toxicity obliged the discontinuance of alpha-interferon. Due to a continuous increase of platelet count, hydroxyurea was introduced. The patient is asymptomatic, with platelet counts < 500000/mm3, without toxicity manifestations, two years after diagnosis. The contribution of cardiovascular risk factors versus thrombocythemia in the pathogenesis of the ischemic cerebral event and the benefit of platelet count control are discussed.
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PMID:[Essential thrombocythemia. Apropos a case of cerebrovascular stroke]. 828 17

Following reports that recombinant interferon alfa (rIFN alpha), besides inducing clinical and hematologic remission in polycythemia vera (PV), can also resolve intractable pruritus, we used rIFN alpha to treat 13 PV patients complaining of severe pruritus refractory to conventional treatment (venesection and/or cytostatics). rIFN alpha was administered intramuscularly three times a week at a dosage of 3.0 x 10(6) U. Eight patients (61.5%) reported a > or = 50% reduction of pruritus, which occurred within 2-8 weeks from the start of rIFN alpha treatment, leading to a substantial improvement in their quality of life. Three patients had to stop rIFN alpha within the first month of therapy because of unacceptable side effects. Thus rIFN alpha seems to be capable of providing considerable relief of otherwise intractable pruritus in a good proportion of PV patients.
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PMID:Relief of intractable pruritus in polycythemia vera with recombinant interferon alfa. 837 16

The efficacy and tolerability of rIFN-alpha has been evaluated in 17 selected patients with symptomatic polycythaemia vera, diagnosed according to the PRV Study Group criteria. Complete disease control (CR) was achieved, after 1-12 months, in nine patients, with partial control in a further five cases. Three patients failed to respond. Pruritus significantly improved in 83% (10/12) of cases, following 1-28 weeks of treatment. Six patients (35%), however, were unable to tolerate rIFN-alpha, on account of weight loss, myalgia and mental changes. Overall, alpha-interferon therapy significantly improved venesection requirements, MCV and PCV values, platelet counts, pruritus scores and the degree of splenomegaly. Analysis of pooled published data (100 evaluable patients, including the present study) revealed an overall CR of 60%, a PR of 27%, and a failure rate of 13%. Significant pruritus control (> 50% improvement) occurred in 77% of cases. rIFN-alpha appears to be an effective therapy for PV-associated myeloproliferation and/or pruritus, although side-effects remain a concern. Long-term studies are now indicated to determine if the natural history of the disease is altered, in particular whether the incidence of myelofibrosis and/or leukaemic transformation is reduced.
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PMID:Efficacy of recombinant interferon-alpha (rIFN-alpha) in polycythaemia vera: a study of 17 patients and an analysis of published data. 856 11

Procoagulant activity (PCA) of peripheral mononuclear cells (PMC) was evaluated in patients with primary thrombocythemia (PT, group A), polycythemia vera (PV), idiopathic myelofibrosis (IM) and myelodysplastic syndromes (group B), and in 15 healthy subjects as control group. PCA of PMC was assayed under basal conditions and after agonist-induced stimulation: bacterial lipopolysaccharide, glycosylated granulocyte-macrophage colony-stimulating factor, recombinant alpha-interferon. PCA was similar in the control group and group A when no stimulation was used, while PCA was found significantly higher in group B patients in the same conditions. In group A patients and in the control group, but not in group B patients, a lower PCA expression was found when PMC were simultaneously coincubated with LPS and alpha-interferon with respect to LPS incubation alone.
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PMID:Procoagulant activity of mononuclear cells is increased in myeloproliferative and myelodysplastic diseases. 873 90

The authors present a rare case of polycythemia vera (PV) in a 13-year-old girl initially treated with phlebotomy only and then also with interferon alpha at a dose of 3 x 10(6) U three times per week subcutaneously. The treatment outcome measure was the reduction of the number of phlebotomies required to retain a normal hematocrit value. Treatment with interferon enabled a substantial reduction of phlebotomies and caused a decrease in platelet count. These results confirm that interferon alpha may be regarded as a promising therapeutic approach in the management of PV.
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PMID:[Interferon alpha in the treatment of polycythemia vera in a 13-year-old girl]. 892 76

According to strict morphological, biochemical and cytogenetic criteria Philadelphia chromosome positive essential thrombocythemia and chronic granulocytic leukemia constitute a separate malignant and individual disease entity, whereas Philadelphia chromosome negative essential thrombocythemia, polycythemia vera and agnogenic or megakaryocytic myeloid metaplasia form a chronic proliferation of three hematopoietic cell lines. Histopathology from bone marrow biopsies permits the characterization and diagnostic differention of the various myeloproliferative disorders and appears to be a main and specific diagostic criterion for polycythemia vera and essential thrombocythemia. Hemorrhagic thrombocythemia is a clinical syndrome of recurrent spontaneous mucocutaneous and secondary hemorrhages often preceded by thromboses, extremely high platelet counts, pseudohyperkalemia, increased bone marrow cellularity and frequently splenomegaly. The diagnostic criteria of essential thrombocythemia with paradoxical occurrence of thrombotic events and hemorrhagic manifestations are a platelet count in excess of 1000 x 10(9)/L and increased bone marrow cellularity in the majority of the cases. Erythromelalgia and other microcirculatory ischemic or thrombotic events or accidents in essential thrombocythemia and polycythemia vera already occur at platelet counts in excess of the upper limit of normal. First line treatment options in essential thrombocythemia and polycythemia vera are control of platelet function with low-dose aspirin and reductive control of platelet count and erythrocytes by bloodletting, interferon and busulfan or hydroxyurea monochemotherapy.
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PMID:The myeloproliferative disorders. An historical appraisal and personal experiences. 895 68

Serum soluble interleukin-2 receptor (sIL-2R) levels were determined in patients with chronic myeloproliferative disorders (CMPD): 18 with chronic myelogenous leukemia in chronic phase (CML in CP), seven with CML in accelerated phase (AP) or blastic crisis (BC), six with polycythemia vera (PV), eight with essential thrombocythemia (ET), one with primary myelofibrosis (PMF), and 50 controls. The mean (+/-S.E.M.) levels were higher in CMPD than in controls (CML in AP or BC, 2693 +/- 694 U/ml, P < 0.0001; CML in CP, 792 +/- 63 U/ml, P < 0.0001; PV 553 +/- 89 U/ml, P < 0.05; ET, 449 +/- 56 U/ml; PMF, 628 U/ml vs. controls, 395 +/- 25 U/ml). Patients with CML in CP had significantly higher serum sIL-2R levels than patients with ET (P < 0.005), and levels were markedly elevated in AP and BC (P < 0.001). Serum sIL-2R levels were positively correlated with WBC count and lactic dehydrogenase in CMPD, and in CML in CP. Serum sIL-2R levels in CMPD were negatively correlated with RBC and platelet counts. Serum sIL-2R levels were significantly lower in patients with CML in CP who showed a cytogenetic response after interferon (IFN) therapy than in those who showed no response (P < 0.05). These findings suggest that a high serum sIL-2R level reflects the leukocyte growth in CMPD and is useful both for differentiating CML from other CMPD and for predicting the response to IFN therapy in CML.
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PMID:Clinical significance of serum soluble interleukin-2 receptor in chronic myeloproliferative disorders. 907 16


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