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)

Scintigraphy of the reticuloendothelial system (RES) was performed in 19 patients with polycythemia vera (PCV) and in 18 with secondary or relative polycythemia (PS). Bone marrow extension was found in all patients with PCV and in 11 of 18 patients with PS. The patients with PCV had a higher degree of extension than those with PS. Increased pelvic bone marrow activity was found in 16 of 19 PCV patients, but in none with PS. Splenomegaly was found in 9 patients with PCV, and in none with PS. It is concluded that RES scintigraphy in the majority of patients may differentiate between PCV and PS using the parameters pelvic bone marrow activity, bone marrow extension and splenic size.
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PMID:RES scintigraphy in polycythemia vera and secondary or relative polycythemia. 845 27

A 74-year-old man was admitted because of swelling, pain and ecchymosis in the night lower extremity. A blood analysis revealed that Hb was 11.8g/dl, WBC 37,600/microliters, and platelet count 137.1 x 10(4)/microliters. The NAP value was high. Bone marrow examination disclosed marked megakaryocytic hyperplasia. Chromosomal analysis revealed 47, XY, +9. Hemostatic data were within normal ranges, but the 2nd aggregation of platelet by ADP was diminished. The serum beta-TG was 159, PF-4 56ng/ml, B12 1,100, UB12 BC 1,800pg/ml. Gastric fiberoscopy revealed gastric cancer and CT scan disclosed marked splenomegaly. Essential thrombocythemia (ET) coexisting with gastric cancer was diagnosed based on these examinations. He was treated with anti-platelet agents, busulfan and tegafur uracil, however thromboembolic symptoms suggesting central nervous system and peripheral vascular ischemia and gastrointestinal bleeding occurred. Among diagnostic criteria for ET established by the polycythemia vera study group, there is a category "No known cause for reactive thrombocytosis." The case reported here had gastric cancer which may have contributed to the elevated platelet count, however this case could be diagnosed as ET coexisting with gastric cancer because of the above various clinical signs and laboratory results. Although then are few reports of the coexistence of other malignancies in ET, there may be many more similar cases because of the age preponderance in ET. In order to diagnose ET more precisely, more strict diagnostic criteria are needed.
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PMID:[Coexistence of essential thrombocythemia and gastric cancer]. 849 22

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

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

There is no single diagnostic marker for polycythaemia vera (PV). The Polycythemia Vera Study Group established diagnostic criteria more than twenty years ago. Since some new laboratory and clinical investigations have developed, these criteria can now be updated. It is proposed that the overall pattern of major and minor criteria should remain. A raised red cell mass (greater than 25% above the patient's mean normal predicted value based on surface area) and absence of a cause of secondary polycythaemia are essential criteria. Then either palpable splenomegaly or the presence of a marker of clonal haemopoiesis would support the diagnosis of PV. In the absence of both of these latter major criteria, at least two minor criteria must be present to secure the diagnosis. These are: platelet count > 400 x 10(9)/l; neutrophil count > 10 x 10(9)/l; splenomegaly demonstrated by a scanning technique; characteristic BFU-E growth or reduced serum erythropoietin value.
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PMID:The diagnostic criteria of polycythaemia rubra vera. 895 78

Recent studies have shown rIFN alpha to be an effective agent in the management of polycythemia vera. Red cell mass can be controlled within 6 to 12 months, eliminating the need for phlebotomy in up to 70% of cases. In addition, significant improvement in the platelet counts, iron status, pruritus scores and the degree of splenomegaly have been reported. Most patients require between 9 and 25 x 10(6)U/week, although once the disease is under control it may be possible to reduce both the dose and frequency of administration. Side-effects remain a significant problem, occurring in over 30% of patients, and may be related to the high mean age of the patients. Long-term studies are now indicated to determine if the natural history of the disease is altered and whether, in particular, the incidence of myelofibrosis and/or leukaemic transformation is reduced.
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PMID:Interferon treatment in polycythaemia vera. 895 85

A 54-year-old woman with leukocytosis, was referred to our clinic in February 1982. Based on findings of pancytosis, high NAP score, high serum vitamin B12, increase in total red cell volume and splenomegaly, she was diagnosed as having polycythemia vera (PV). Since then, she has been treated with pipobroman, hydroxycarbamide and phlebotomy. Leukocytosis with increase in blastic cells and thrombocytopenia was noted in August 1995, and she was admitted to our hospital. Since the blastic cells were CD10(+)19(+)20(+), she was diagnosed as having acute lymphoblastic leukemia and treated with vincristine and prednisolone, resulting in remission. This case suggests that PV is a disease of multipotent stem cells including those with a lymphoid lineage.
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PMID:[Polycythemia vera progressing to acute lymphoblastic leukemia after 13 years]. 899 30

In addition to the criteria of the Polycythemia Vera Study Group, positive markers for essential thrombocythemia (ET) include spontaneous BFU-E, splenomegaly, and megakaryocyte morphology in bone marrow smears and biopsy material. The hematologic features of 11 reported cases of ET in childhood showed platelet counts in excess of 1000 x 10(9)/L in all, slight leukocytosis in 8, and splenomegaly in 9. The presenting thrombohemorrhagic manifestations in 8 symptomatic cases were microcirculatory disturbances and transient neurologic ischemic attacks in 2, recurrent mucocutaneous bleedings in 6, and priapism in 1. There are no reports of ET in childhood complicated by microcirculatory disturbances at platelet counts below 1000 x 10(9)/L. Anagrelide and alpha-interferon, which are non-leukemogenic agents for the reduction of platelet counts, may become the treatment of choice in childhood ET. Anagrelide is tolerated better than alpha-interferon. The potential leukemogenic drugs hydroxyurea and busulfan should be used cautiously and withheld as long as possible.
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PMID:Essential thrombocythemia in childhood. 925 10

We reported a 72-year-old female patient who developed acute leukemia following a long course of polycythemia vera (PV). For 12 years she had been treated with phlebotomy, nimustine, busulfan, hydroxyurea and irradiation on splenomegaly. In November 1995, her peripheral blood smear showed blast of 30%. Bone marrow blasts were microscopically as well as electromicroscopically peroxidase-negative and CD7 and HLA-DR positive. Six months later, the blasts were positive for CD7, CD34 and HLA-DR. On the basis of morphologic, biochemical and immunophenotypic features, the patient was diagnosed acute leukemia, probably arising at a primitive multipotential stem cell level. She failed to respond to the various combination therapy including prednisolone, vincristine, cytarabine, daunorubicin and etoposide. The stem-cell-leukemia transformation in PV occurs rarely and may be refractory to chemotherapy.
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PMID:[CD7+, CD34+, electronmicroscopically peroxidase-negative acute leukemia transformed from polycythemia vera after 12 years follow-up]. 936 71

Essential thrombocythemia (ET) and polycythemia vera (PV) are chronic clonal myeloid disorders that originate from the multipotential hematopoietic stem cell. They are characterized, respectively, by excessive thrombocytosis and erythrocytosis, a high incidence of thrombohemorrhagic events, vasomotor symptoms, and an inherent tendency to undergo leukemic transformation. Current standard therapies to control the excess accumulation of myeloid cells and to provide symptomatic relief carry either a persistent risk of thrombosis, as in the case of phlebotomy, or, in the case of hydroxyurea, the potential for inducing leukemia. None alter the natural history of these diseases. Interferon-alpha has been shown to have potent antiproliferative effects on the hematopoietic stem cells and bone marrow fibroblasts and, as a result, has received much attention as a therapeutic agent for chronic myeloproliferative disorders. The ability of interferon-alpha to induce hematologic and cytogenetic remission in chronic phase chronic granulocytic leukemia has further increased interest in this agent. Interferon-alpha has shown therapeutic activity in PV and ET, as demonstrated in multiple small studies and single-arm trials reviewed in this article. Reported beneficial effects include the ability to control excessive erythrocytosis and thrombocytosis and such disease-related features as vasomotor symptoms, pruritus, and splenomegaly. Recent reports of cytogenetic remission and reversal of bone marrow fibrosis after interferon therapy are of interest. Advantages over current therapeutic standards include lack of known leukemogenic and teratogenic effects and the potential to alter the underlying course of disease. Nevertheless, none of the information available allows definite therapeutic recommendations for the use of interferon-alpha in PV or ET. The available data support the need for randomized controlled trials comparing interferon-alpha with standard therapy.
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PMID:Interferon-alpha therapy in polycythemia vera and essential thrombocythemia. 938 5


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