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

A 70-year-old female patient is described in whom classical hairy cell leukemia developed following a 15-year course of polycythemia vera continued by myelofibrosis. This association has not been described previously.
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PMID:Hairy cell leukemia in a patient with polycythemia vera. 643 33

In these last years the use of alpha-interferon (alpha-IFN) has received increasing attention especially in the onco-haematological field. alpha-IFN is particularly useful in the treatment of hairy cell leukemia, cryoglobulinemia, multiple myeloma and myeloproliferative syndromes (SMP). Among these latter conditions alpha-IFN must be considered as the treatment of choice of the early chronic phase of chronic myelogenous leukemia (LMC) in patients not eligible for allogenic bone marrow transplantation because its ability to induce a greater number of clinical remission and cytogenetic responses when compared to the classical chemotherapeutic agents. A myelosuppressive, non-leukemogenic effect and a more selective activity on the neoplastic hemopoiesis appear to be the most important advantages of alpha-IFN therapy. Based on the results obtained in LMC the use of alpha-IFN has been extended to the other SMP, essential thrombocytemia (TE), polycythemia vera (PV), idiopathic myelofibrosis with myeloid metaplasia (MMM). alpha-IFN is able to control thrombocytosis which often characterize the SMP so it appears to be particularly effective in TE. Actually a relatively limited literature is available about the alpha-IFN treatment of PV and MMM and so it is difficult to draw a final conclusion about the effectiveness of the treatment in these disorders. However, especially in PV, the use of this cytokine appears to be promising. The latest reports of the literature are here summarized and discussed.
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PMID:[Interferon-alpha in the treatment of myeloproliferative syndromes]. 785 89

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 a case of hairy cell leukemia variant developing in a background of polycythemia vera in a 77-year-old man who presented with lymphocytosis and splenomegaly. Classic hairy cell leukemia in a patient with polycythemia vera has been reported previously, but hairy cell leukemia variant arising in a patient with polycythemia vera has never been described to the best of our knowledge. Initial testing of the peripheral blood showed circulating medium to large leukemic cells with large, centrally placed nuclei, each containing a prominent nucleolus, and some cells showed cytoplasmic projections. A bone marrow biopsy had marked myeloid and erythroid hyperplasia and interstitially distributed cells with a fried-egg appearance. We verified a monoclonal B-cell population by flow cytometric analysis, which revealed expression of bright CD11c, CD22, and CD103 expression, and a lack of CD25 expression. The patient received a 5-day course of cladribine and subsequently had a complete remission. Approximately 2 months later, he had a relapse and was treated with pentostatin; however, he had no clinical response and died.
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PMID:Hairy cell leukemia variant developing in a background of polycythemia vera. 1268 4

Interferon (IFN) has been associated with development of thrombotic microangiopathy including thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). We reviewed literature from the earliest reported association in 1993, to July 2016 and found 68 cases. Analysis of this data shows: (1) Mean age at diagnosis was 47 years (95% CI, 44-50). (2) Majority of cases were seen where IFN was used for the treatment of chronic myelogenous leukemia (CML), multiple sclerosis (MS), chronic hepatitis C virus infection (HCV) and one case each for hairy cell leukemia (HCL) and Sezary syndrome. (3) There were no cases reported for polycythemia vera (PV) or lymphoma. (4) Sex distribution was nearly equivalent with the exception in patients with multiple sclerosis where there was female predominance (12 of 16 with reported data). (5) For pooled analysis, the average duration of treatment with IFN before TMA was diagnosed was 40.4 months. (6) Comparative analysis showed that patients with MS required the highest cumulative dose exposure before developing TMA (MS 68.6 months, CML 35.5 months, HCV 30.4 months). (7) Cases of confirmed TTP (where A disintegrin and Metalloprotease with thrombospondin type 1 motif 13: ADAMTS 13 level was measured) showed presence of an inhibitor. (8) In all cases of confirmed TTP, moderate to severe thrombocytopenia was a striking clinical feature at presentation while this was not a consistent finding in all other cases of TMA. (9) Outcome analysis revealed complete remission in 27 (40%), persistent chronic kidney disease (CKD) in 28 (42%) and fatality in 12 patients (18%). (10) Treatment with corticosteroids, plasma exchange and rituximab resulted in durable responses.
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PMID:Interferon induced thrombotic microangiopathy (TMA): Analysis and concise review. 2832 51