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 new abnormal hemoglobin, Hb J Amiens beta 17 (A 14) Lys replaced by Asn, has been discovered during the exploration of a recent polycythemia in a 65-year-old patient of Spanish extraction. Oxygen affinity of washed red blood cells was found to be normal at pH 7.13 (P 50 = 30.0 mmHg, N = 29.5 +/- 1). Cooperativity is unchanged, and no instability was detected. From this study, it is concluded that there is no relation between this functionally silent hemoglobin and the polycythemia. In fact, the recent appearance of the polycythemia, the involvement of the other blood cell lines, particularly the thrombocytosis, the high score of leukocyte alkaline phosphatases, and the results of the bone marrow biopsy led to the diagnosis of polycythemia vera.
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PMID:[Hemoglobin J Amiens beta 17 (A 14) Lys replaced by Asn. Coincidence of a functionally silent new abnormal hemoglobin and a polycythemia vera (author's transl)]. 12 38

32P is effective therapy for polycythemia and primary thrombocytosis. The Polycythemia Vera Study Group is comparing radioactive phosphorus with alkylating agents to determine relative efficacy. Less well investigated is the effectiveness of 32P vs. busulfan in chronic granulocytic leukemia. Endolymphatic administration of radiopharmaceuticals may play a role in the therapy of infradiaphragmatic lymphoma. Among the radionuclides that have at times been used in hematology are 32P, 198Au 24Na, 76As, 89Sr, 52Mb, 54Mn, 91Y, 95Zr, 95Cb, 111Ag, 109Pd, 131I, 185W, and 192Ir. As stated, 32P has proven single most efficacious agent. The hematologic diseases that have been treated include both malignant and benign conditions. Among the malignant conditions are polycythemia vera, agnogenic myeloid metaplasia, thrombocythemia, leukemia, Hodgkin's disease, and multiple myeloma. Hemophilia, and Osler--Weber--Rendu disease are among the benign entities in which the agents have been tried. Polycythemia and thrombocythemia remain those in which the greatest success has been achieved.
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PMID:Radionuclide therapy of hematologic disorders. 48 47

A striking linear relationship was found between the logarithms of the medians of the circulating neutrophil (granulocyte plus metamyelocyte) counts and the monocyte counts in 5 groups of individuals studied. These groups consisted of (1) 100 normal adult males, (2) 100 normal, non-pregnant females, (3) 50 females in the last trimester of pregnancy, (4) 15 patients with a persistent neutrophil leucocytosis and (5) 20 patients with polycythaemia rubra vera, essential thrombocythaemia or myelofibrosis. A different relationship appeared to exist between the circulating neutrophil and monocyte counts in a group of 16 patients with chronic granulocytic leukaemia.
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PMID:Relationship between the concentration of neutrophils and monocytes in venous blood. 81 47

Ten patients with pregangrenous and gangrenous changes of the toes in the presence of normal peripheral pulses are described. In the absence of diabetes this is an uncommon condition and is only rarely reported upon in the literature. Four patients had non occlusive arteriosclerotic changes in large arteries; three suffered from thrombocytosis and one from polycythemia vera; one patient had a monoclonal gamopathy and one was exposed to cold three months before the onset of gangrene. None of these patients smoked regularly. Severe pain usually preceded the gangrene. The process did not progress proximally in any patients, and in those who underwent toe amputations the healing was uneventful. Vasodilators and low-molecular dextran were not effective. Lumbar sympathectomy was performed in three patients, also with no effect on the course of the disease. Treatment of hematological disorders gave relief in three patients. Proximal arteriosclerotic changes should be corrected if possible to eliminate a source of emboli. In two patients anti-platelet aggregation agents provided relief. Toe amputation should be conservative and performed when definite demarcation appears between necrotic and viable tissue. This condition has a benign prognosis.
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PMID:Gangrene of toes with normal peripheral pulses. 84 22

A 74-year-old white man with established polycythemia vera was treated with radioactive phosphorus after phlebotomies alone failed to control his disease. About 2 3/4 years later he died of multiple myeloma. The mutagenic effect of radioactive phosphorus may have caused or possibly accelerated preexisting myeloma. Basic nonmalignant disease deserves careful consideration before radiation or radiomimetic agents are used. One might consider a probably less mutagenic drug such as hydroxyurea in patients with polycythemia vera when phlebotomy alone does not give good control of red cell mass and thrombocytosis.
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PMID:Multiple myeloma on polycythemia vera following radioactive phosphorus therapy. 101 58

Forty-three operative procedures were performed on a population of 250 patients with myeloproliferative disorders, including polycythemia vera, myeloid metaplasia (MM) and chronic myelogenous leukemia (CML). The overall operative mortality was approximately 7% and the incidence of excessive bleeding which could be related to coagulopathy was 5%. Twenty-one patients with MM or CML underwent splenectomy for palliation of symptoms related to the enlarged spleen or hematologic problems. Eighty-four percent of the latter group were improved. Adverse hematologic effects which could be attributed to splenectomy in these patients were confined to two patients who developed marked thrombocytosis. Among the 23 patients with MM, 9 had portal hypertension. Three underwent portacaval shunt and one a splenorenal shunt for bleeding varices. One of the patients died of hepatic necrosis. Estimated hepatic blood flow determinations (EHBF) in 4 patients with portal hypertension demonstrated a marked absolute increase and an increase in the ratio of EHBF/Cardiac Index. Absence of any evidence of intrahepatic or extrahepatic obstruction in these patients and the demonstration that splenectomy relieved portal hypertension defined at surgery in 4 patients, suggests that augmented adhepatic flow contributes to portal hypertension in some cases. The review leads to the conclusions that: 1) Operative procedures in prepared patients with myeloproliferative disorders are not associated with prohibitive mortality and morbidity rates. 2) Splenectomy is indicated for patients with increasing transfusion requirements and symptomatic splenomegaly or hypersplenism and should be performed early in the course of disease. 3) When associated portal hypertension and bleeding varices are present, hemodynamic studies should be carried out to define if splenectomy alone, or a portal systemic decompressive procedure is indicated.
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PMID:Myeloproliferative disorders. 105 48

Over a 19-year period, a patient with polycythemia vera who had undergone a splenectomy received six courses of busulfan for recurrent thrombocytosis. The total dose of busulfan given for the sixth course was greater than that used for the previous ones. Severe pancytopenia followed, which persisted for 4 months. During this period there was marked erythroid hyperplasia in the bone marrow with striking dyserythropoiesis; PAS-positive red cell precursors, as well as moderate numbers of circulating normoblasts and evidence of chronic and acute hemolysis, were present. All of these findings reverted to normal without therapy, and the polycythemic state eventually recurred. These events are interpreted as an unusual marrow reaction following busulfan overdosage rather than a transient erythroleukemia.
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PMID:Erythroleukemia-like syndrome due to busulfan toxicity in polycythemia vera. 106 2

A patient with polycythemia rubra vera developed marked thrombocytosis and hemorrhage after splenectomy. Plateletpheresis with a blood processor proved to be a safe, efficient, and rapid method for the mechanical removal of large quantities of platelets prior to adequate control with chemotherapy.
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PMID:Successful control of life-threatening thrombocytosis with a blood processor. 119 93

Over a seven year period 52 patients having a clinical diagnosis of spontaneous peripheral arterial microembolization were identified. Sixty-one percent of patients were female, 15% were diabetic, and 73% used tobacco chronically. A striking finding was the very high incidence of associated systemic disorders such as thrombocytosis (8), polycythemia vera (3), metastatic adenocarcinoma (3), or collagen disease requiring steroid therapy (4). Forty-nine patients had significant proximal arterial lesions as the origin of their emboli. Three patients had digital ischemia as a result of increased platelet aggregation without arterial obstruction. Forty-eight patients underwent surgical therapy. Operative mortality was 4% and overall limb salvage in survivors was 96%. The clinical syndrome of arterial microembolization may result from several pathophysiologic mechanisms including cholesterol embolization from ulcerated plaques, fibrino-platelet aggregation in patients with hematologic disorders, or dislodgement of mural thrombus in those with aneurysmal disease. We observed aortoiliac disease to be more frequent than femoral-popliteal disease, and both were amenable to surgical correction. We conclude that the genesis of arterial microembolization is multifactorial and that a variety of systemic diseases may work in concert with atherosclerotic arterial disease to produce this clinical syndrome. Prompt recognition and appropriate treatment of this disorder can yield high rates of limb salvage.
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PMID:Spontaneous peripheral arterial microembolization. 139 20

A decrease in platelet serotonin content is well recognized in myeloproliferative disorders, although the basis for this is unclear. The relationship between decreased platelet serotonin and the number of serotonin transport channels in the platelet membrane was explored indirectly by measuring the number of the closely associated imipramine binding sites. Imipramine binding parameters were determined in 12 patients suffering from either essential thrombocythaemia or polycythaemia rubra vera and in 10 healthy controls. The maximal number of imipramine binding sites (Bmax) was increased in both patient groups, significantly so in those suffering from essential thrombocythaemia. This increase contrasts with a decrease in binding in other conditions known to be associated with decreased platelet serotonin. This study thus demonstrates a discrepancy in terms of reduced platelet serotonin content and increased imipramine binding on platelets from patients suffering from essential thrombocythaemia. It is suggested that further investigation of the imipramine binding site in these disorders, in parallel with measures of platelet serotonin content and uptake, is warranted.
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PMID:Platelet imipramine binding is increased in essential thrombocythaemia. 145 Mar 37


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