Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0032463 (polycythemia vera)
 3,374 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of pyoderma gangrenosum of the lip occurring in association with paroxysmal nocturnal haemoglobinuria is described. This is an extremely rare association, which has been documented in the literature on only two previous occasions. Pyoderma gangrenosum (PG) is an uncommon ulcerative skin disorder of unknown aetiology. Its clinical appearance is often distinctive, with established lesions consisting of a necrotic ulcer surrounded by a ragged undermined violaceous edge. Lesions are usually painful and are most often found on the lower limbs but can occur on the trunk, head and neck. The diagnosis is essentially clinical as there are no characteristic histopathological changes. Since its original description in 1930, PG has been frequently associated with a number of underlying systemic diseases. Foremost among these are inflammatory bowel disease and inflammatory polyarthritis. The association with haematological disorders is also well recognized, and includes acute and chronic lymphocytic and myeloid leukaemias, polycythaemia rubra vera, myelofibrosis, myelodysplastic syndrome, essential thrombocythaemia, hypogammaglobinaemia, monoclonal gammopathy, multiple myeloma and non-Hodgkin's lymphoma. We report a case of PG occurring on the lower lip of a 26-year-old man recently diagnosed as having paroxysmal nocturnal haemoglobinuria (PNH).
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PMID:Pyoderma gangrenosum associated with paroxysmal nocturnal haemoglobinuria. 803 97

Since early eighties there have been several studies evaluating the effectiveness of biotherapy with natural and recombinant interferon alpha (IFN-alpha) in patients with proliferative hemocytopathies of both lymphoid and myeloid origin. In patients with hairy cell leukemia (HCL) interferon induces clinical and hematological remission and was considered for several years as the treatment of choice in the management of this disease. Subsequently it was found that IFN reveals myelosuppressive effect in patients with chronic myelogenous leukemia (CML) leading in majority of them to clinical and hematological remission and in part--cytogenetic one. Interferon decreases also thrombocytosis and its thrombotic-haemorrhagic complications in patients with essential thrombocythemia (ET) and decreases erythrocyte count in patients with polycythemia rubra vera (PRV).
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PMID:[Use of interferon in patients with hairy cell leukemia and myeloproliferative disorders]. 806 97

We report the disease characteristics and therapeutic results for 25 patients suffering from essential thrombocythaemia (ET), treated with recombinant interferon-alpha-2b (IFN-alpha 2b). ET was diagnosed according to the criteria of the Polycythaemia Vera Study Group. All patients were programmed to receive a subcutaneous induction treatment consisting of 3 MU of IFN-alpha 2b daily for 6 months. In responding patients, treatment was continued for a further 6 months with 3 MU of IFN-alpha 2b three times a week. Complete response was achieved in 13 of 25 patients, partial response in 10 of 25. In 2 cases, therapy was unsuccessful. Side effects were usually mild, consisting of flu-like symptoms in most cases, and were easily controlled by paracetamol. After a median follow-up of 14 months after discontinuation of the treatment, most patients retained the therapeutic response in the absence of toxicity.
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PMID:Recombinant interferon alpha-2b as treatment of essential thrombocythaemia. 809 33

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

Interferon-alpha (IFN-alpha) exhibits a clear platelet reductive effect in patients with essential thrombocythemia as well as in other chronic myeloproliferative disorders with thrombocytosis. In a total of 51 patients with chronic myeloproliferative disorders with thrombocytosis we analyzed the effect of IFN-alpha in respect to platelet reduction, remission rates, induction- and maintenance dosage, long term tolerance and side effects. According to our classification CML 6, chronic mega-karyocytic granulocytic myelosis 5, essential thrombocythemia 26 and polycythemia vera 15 patients were treated. Treatment consisted of induction with 3 or 5 MU IFN-alpha daily followed by a maintenance therapy with 3 or 5 MU thrice weekly. Platelet reduction was found in all patients, CR (platelets < 450 G/l) in 78%. Within 2 months of induction therapy, CR in patients treated with 5 MU IFN daily was found in 75% compared to 52% in patients treated with 3 MU IFN daily. Dosage reduction in maintenance periode caused an increase of platelets to more than 450 G/l in 39% of patients. Out of 40 Philadelphia-negative chronic myeloproliferative disorders treated for more than 3 months in 10 patients treatment was disrupted after 5 to 18 months because of the following side effects: nausea, fatigue, vertigo, fever, headache, diarrhea, anorexia, heartburn, hairloss, myalgia, and thrombocytopenia. Due to the mutagenic effect of alkylating cytostatics and Radiophosphorus, IFN-alpha treatment represents a first line strategy for chronic myeloproliferative disorders with thrombocytosis especially in younger patients who are symptomatic and in those who suffered from episodes of bleeding or thrombosis.
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PMID:[Interferon therapy in essential thrombocythemia]. 827 65

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

We used the methylcellulose-culture technique to determine the utility of the erythroid progenitor growth in vitro from nonadherent T-depleted bone marrow and peripheral blood cells in distinguishing polycythemia vera (PV) from essential thrombocythemia. Thirty patients with PV (group A) and 30 patients who presented with idiopathic marked thrombocytosis with platelet count greater than 1,000 x 10(9)/L and a normal or reduced hemoglobin (Hb) level (group B) were studied at initial presentation. Endogenous (erythropoietin-independent) erythroid colonies (EEC) were found in all patients in group A and 13 in group B. The numbers of EEC were comparable between patients in group A and the 13 patients with EEC in group B, 11 of whom with initial Hb levels ranging between 6.4 g/dL and 12.6 g/dL were found to have PV 2 to 45 months after initial evaluation. The number of EEC did not correlate with the time to the progression of polycythemia, whereas myelosuppression delayed the subsequent development of PV. Of the two patients with EEC in group B who did not develop PV, both received chemotherapy soon after presentation, which might preclude the evidence of polycythemia evolution. None of the other patients in group B who did not form EEC developed PV with a median follow-up of 24 months. This study indicates that the assessment of EEC in bone marrow or blood is helpful in early identification of PV or prediction of polycythemia evolution in patients with marked thrombocytosis in whom polycythemia has been initially masked or anemia is present.
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PMID:Identification of masked polycythemia vera from patients with idiopathic marked thrombocytosis by endogenous erythroid colony assay. 829 36

Interferon alpha (INF alpha) is the drug of choice for patients where the interval between the diagnosis and INF alpha administration is not longer than one year. The usual dosage is 5 x 10(6) i.u./m2 INF-alpha s.c. per day. Haematological and cytogenic criteria are applied to evaluate treatment. INF alpha prolongs the life of patients with a cytogenic response. In these patients treatment should be continued till signs of relapse develop. In patients without a cytogenic response INF alpha does not substantially prolong the survival period. INF alpha is indicated in all myeloproliferative diseases associated with thrombocytosis. It holds its place in the treatment of patients with primary myelofibrosis. In both these indications it is recommended to start INF alpha treatment-3 x 10(6) i.u. INF alpha per day. In polycythemia vera INF alpha reduces the number of erythrocytes. It is not clear, however, whether it prolongs life.
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PMID:[Interferon-alfa in the treatment of myeloproliferative diseases]. 831 Jun 73

Chronic myeloproliferative disorders (CMPD) consisted of the disease with chronic myelogenous leukemia (CML), polycythemia vera (PV), essential thrombocythemia (ET), myelofibrosis (MF) and other proliferative diseases have commonly developed increased megakaryocytes. Recently, clonal assay for megakaryocytic progenitors, identification of young megakaryocytes and isolation of megakaryocytes have been greatly facilitated an analysis of megakaryopoeisis. It is still difficult quantitatively and serially to estimate megakaryopoiesis in CMPD. However, number of CFU-Meg, number of megakaryocytes (PGPIIb/IIIb) in the clot section, cytoplasmic area and DNA content of megakaryocytes were measurable in the clinical materials. The present lecture has been focused to following points; 1) megakaryopoiesis in CML, 2) differences of megakaryopoiesis between ET and PV, 3) megakaryopoiesis in Ph1 positive ET, and 4) megakaryopoiesis in rective thrombocytosis by our data and previous studies.
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PMID:[Megakaryopoiesis in chronic myeloproliferative disorders]. 831 26

By using gamma camera imaging the spleen size was determined in 33 consecutive patients with essential thrombocythaemia (ET) and in 33 consecutive patients with reactive thrombocytosis (RT). All ET patients were newly diagnosed and had not received myelosuppressive treatment prior to study; they all fulfilled the criteria for ET as established by the Polycythemia Vera Study Group. In both posterior and lateral projections, the spleen area in the group of ET patients was significantly larger than in the RT patients. The present study has shown that 39% of ET patients at diagnosis have splenic enlargement. Evaluation of spleen size is therefore a useful diagnostic test in patients presenting with unexplained thrombocytosis.
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PMID:Measurement of spleen size using gamma camera scintigraphy in essential thrombocythaemia. 840 28


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