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 polycythemia vera with an inhibitor against factor XII was reported. A 60-year-old female was admitted to Hokkaido University School Hospital because of erythrocytosis and hepatosplenomegaly. The hemoglobin was 22.5 g/dl and white cell count was 9,500/microliters without immature cells. The platelet count was 484,000/microliters. Bone marrow specimens showed marked hypercellularity. Philadelphia chromosome was not found on chromosome analysis. She was diagnosed as polycythemia vera according to the criteria of polycythemia Vera Study Group. Activity of factor XII was found to be decreased on the initial examination, but she had no personal and familial history of bleeding. In order to clarify the cause of decreased activity of factor XII, her plasma was mixed with normal plasma, and then examined PTT using factor XII deficient plasma. Her plasma mixed with equivalent normal plasma did not show the correction of prolonged PTT. It was suggested that an inhibitor of her plasma was included in the IgG fraction using gel chromatography. The patient was treated with phlebotomy and administration of N4-palmitoyl (1- -D-arabinofurasyl) cytosine (derivative of cytosine arabinoside; PLAC) 200 mg/day and Busulfan (1 mg/day). Factor XII was not corrected by phlebotomy, but corrected gradually by administration of PLAC and Busulfan.
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PMID:[Polycythemia vera with an inhibitor against factor XII]. 260 Oct 45

The ability of erythroid cultures to distinguish among myeloproliferative disorders was examined. We studied 14 patients with polycythemia vera (PV), 11 with chronic myelogenous leukemia (CML), four with non-PV erythrocytosis, two with agnogenic myeloid metaplasia, as well as three normal fetuses and greater than 25 normal adults. Endogenous, i.e. grew without added erythropoietin, bone marrow CFU-E-derived colonies were observed in all but one PV patient. However, endogenous blood BFU-E-derived bursts were observed in only eight of 14 PV patients. Endogenous erythroid colonies were not seen in cultures from any normal adults or fetuses, or patients with CML, erythrocytosis, or myeloid metaplasia. In PV, relative HbF synthesis was always greater in cultures without erythropoietin, while in cultures from all other patients relative HbF synthesis was similar to that observed in cultures from normal individuals. We conclude that PV and CML are distinguishable in culture since CML patients do not have endogenous growth. Most important, endogenous bone marrow CFU-E-derived colonies are the only consistently unique observation in patients with PV, and endogenous CFU-E- and BFU-E-derived colonies and bursts are not uniformly observed in PV blood cultures. In-vitro studies of erythropoiesis to confirm the diagnosis of PV, therefore, require marrow when endogenous colonies and bursts are absent from blood cultures.
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PMID:Comparison of erythroid progenitor cell growth in vitro in polycythemia vera and chronic myelogenous leukemia: only polycythemia vera has endogenous colonies. 271 48

Clinico-laboratory and radionuclide (radio-hepatography with 131I-rose bengal and liver scanning with 198Au colloid solution) investigation of liver function was performed in 110 patients with polycythemia vera during exacerbation, in 16 patients with symptomatic erythrocytosis (10--chronic pulmonary disease, 3--polycystic kidney, 2--obesity, 1--peptic ulcer), and in 11 patients with a polycythemic form of myelofibrosis. The results of the radionuclide method showed disturbed liver function in 51 (46.3%) patients with polycythemia, in 4 patients with myelofibrosis and in 3 with erythrocytosis which were characterized by a decrease in hepatocytic absorptive-excretory function and a decrease in the activity of the reticulo-histiocytic stroma. Liver changes depended on a stage of disease and were detected earlier than with the use of biochemical methods. As distinct from erythrocytes, one-type disorders were noted in the patients with myelofibrosis. Correlation in the lever of RP accumulation in the spleen, its sizes and stage of disease was established. Liver function returned to normal after cytostatic therapy in the patients with stage IIA polycythemia, partially IIB stage, during remission.
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PMID:[Radionuclide study of liver function in polycythemia vera and other types of polycythemia]. 282 15

The absolute polycythaemias include all patients who have a raised red cell mass. They may be divided by clinical and laboratory investigation into: primary proliferative polycythaemia (polycythaemia rubra vera), secondary polycythaemias, and idiopathic erythrocytosis. In vitro, the PCV is the single most important determinant of whole blood viscosity with the most marked effects at low shear rates. Yield stress is also dominantly influenced by PCV. Thus in the absolute polycythaemias, from whatever cause, whole blood viscosity and yield stress are high. However, flow conditions in vivo are much more complex than those usually used in in-vitro measurements. Under normal physiological conditions, whole blood viscosity probably does not determine flow in vivo. The observed in-vitro changes are only of relevance in vivo when local or general abnormalities of flow occur. In primary proliferative polycythaemia (PPP) and idiopathic erythrocytosis, presentation with ischaemia or thrombosis (either arterial or venous) is common, and the cerebral circulation is at particular risk. The incidence of vascular occlusion is positively correlated with the PCV. There is an inverse correlation between PCV and cerebral blood flow (CBF), with untreated patients having low CBF values. This is probably a 'physiological' change related to the increased oxygen carrying capacity at high PCV values. However, in many patients, cerebral oxygen carriage actually increases on reduction of the PCV to normal. This explains the improvement in cerebral ischaemic symptoms and mental performance which may be observed following treatment. Platelet contact and adhesion to the vessel wall are increased at high PCV values and, combined with the lower blood flow in both the arteries and veins, tend to favour thrombus formation. Ischaemia causes vessel dilatation but when this is maximal the high whole-blood viscosity demonstrated in vitro is a major determinant of flow. Hence in man and experimental animals, as demonstrated in the brain, the area of ischaemia or non-perfusion following arterial occlusion is greatest at high PCV values. Patients with polycythaemia due to hypoxic lung disease have a poor prognosis. The hypoxic pulmonary vasoconstriction combined with the high blood viscosity causes an increase in pulmonary vessel resistance. PCV reduction of 0.50-0.52 leads to an improvement in work performance and mental alertness, and reduction in pulmonary vascular resistance.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Rheology of the absolute polycythaemias. 332 60

An autosomal erythrocytosis, inherited as a dominant, occurred in seven members of a family. The propositus was first diagnosed as having erythrocytosis at 26 years of age. He had headaches and marked plethora. Polycythaemia vera and secondary erythrocytosis of known cause were excluded. Erythropoietin level was not elevated. Two of his three children were also found to have erythrocytosis. As in this family, the disease is characterized in middle age by hypertension, cardiovascular and thromboembolic phenomena, as well as abnormal bleeding. For over eight years the propositus has been successfully treated with repeated venous blood lettings.
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PMID:[Idiopathic familial erythrocytosis. Report on a family with autosomal dominant inheritance]. 337 Dec 13

In polycythemia vera, idiopathic myelofibrosis, and essential thrombocytosis, hematopoietic cell proliferation is increased in the absence of a recognizable stimulus, suggesting the autonomous production of growth factors in these disorders. Sonicates of peripheral blood mononuclear cells (PBMNC) from patients with polycythemia vera, idiopathic myelofibrosis, and essential thrombocytosis contained soluble factors that stimulated the proliferation of quiescent-confluent 3T3 cells. PBMNC sonicates from normal individuals; from patients with secondary erythrocytosis, chronic myelogenous leukemia, B-cell chronic lymphocytic leukemia, and acute myelogenous leukemia; and from K-562 and HL-60 cells did not stimulate proliferation. Polycythemia vera PBMNC sonicates also induced anchorage-independent colony formation in soft agar by normal rat kidney fibroblasts. Both the mitogenic and transforming activities of the polycythemia vera PBMNC sonicates resided in the T-lymphocyte-depleted mononuclear fraction of the PBMNC and were not secreted. By gel filtration, reversed-phase HPLC and NaDodSO4/PAGE, the mitogenic and transforming activities in the polycythemia vera PBMNC were localized to three proteins with molecular masses of 13-, 17-, and 65-kDa. The 13-kDa protein was only mitogenic, and the 17-kDa protein was only transforming, whereas the 65-kDa protein had both mitogenic and transforming activity. These proteins may be involved in the autonomous hematopoiesis that characterizes polycythemia vera, idiopathic myelofibrosis, and essential thrombocytosis.
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PMID:Intracellular growth factors in polycythemia vera and other myeloproliferative disorders. 346 72

The amino-terminal fragment of procollagen III was assayed by RIA in 186 patients with myeloproliferative disorders, at intervals of 6 to 24 months. The present results suggest that this assay may be useful for discriminating primary and secondary erythrocytosis, for evaluating the evolution of polycythemia vera towards spent phase and/or myelofibrosis, and for estimating the degree of myelofibrosis in patients with myeloid metaplasia of the spleen. However the methodology of the assay still needs technical improvements. An assay of procollagen I is needed. Excessive values of serum procollagen III may be due to hepatic or pulmonary fibrosis. Finally we don't have as yet a follow-up delay long enough to ascertain the prognostic value of the assay in polycythemia vera and in primary myelofibrosis.
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PMID:[Clinical value of determining the amino terminal fragment of procollagen III in myeloproliferative disorders]. 361 94

Zinc and iron status have been measured in three groups of polycythaemic patients managed by venesection: polycythaemia rubra vera, secondary polycythaemia and idiopathic erythrocytosis. Zinc status was assessed from the concentration of zinc in polymorphonuclear cells (PMN) and plasma. The mean PMN zinc value was lower than that of age-matched controls (polycythaemics 57.5 +/- 2.3 micrograms/10(10) PMN, n = 26; controls 68.9 +/- 5.2 micrograms/10(10) PMN, n = 9; means +/- SEM, p less than 0.05). The difference was most marked in polycythaemia rubra vera; the patients were iron-deficient. The reduced PMN zinc levels may indicate that polycythaemic patients managed by repeated venesection are zinc-deficient. This may contribute to some nonspecific symptoms of polycythaemia and to recurrent infections in the hypoxic patients.
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PMID:Zinc status in polycythaemia. 365 65

The definite differential diagnosis between spurious polycythemia (SP) and pure erythrocytosis (PE) was tested. Serum erythropoietin (EPO) levels in 6 patients with PE were 12.8 +/- 3.7 mU/ml and were significantly lower than those of both 19 normal controls (28.5 +/- 15.0 mU/ml) and 9 patients with SP (21.3 +/- 10.2 mU/ml). Three of 11 patients with SP and 1 of 3 patients with PE had significantly higher marrow erythroid progenitor cells (CFU-Es) than those of the normal controls. Spontaneous CFU-E growth (CFU-E growth in the absence of added EPO) was found in 4 of 11 patients with SP, 1 of 3 patients with PE, and all patients with polycythemia vera. However, the number of spontaneous CFU-E was low in SP and PE. The measurements of serum EPO levels and CFU-E growth did not provide differentiation between PE and SP. Even if some patients, whose total red cell volumes are either higher than 12.5% above the mean predicted values or their CFU-E growth is great, are diagnosed as SP, consideration should be made that they might, in fact, have PE.
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PMID:Differential polycythemia: a comparative study between spurious polycythemia and pure erythrocytosis. 368 23

Eighty-one consecutive repeat male blood donors with hematocrit levels ranging from 0.49 to 0.54 underwent hematologic investigation, blood gas analysis, and respiratory function tests in order to ascertain whether they represent the upper end of the reference distribution or have underlying causes of erythrocytosis. Three of the 81 (3.7%) proved to have early-stage polycythemia rubra vera, nine (11.1%) to have erythrocytosis secondary to respiratory failure, and two (2.4%) to have relative erythrocytosis due to reduced plasma volume. Detection and evaluation of blood donors with high hematocrit values are ways in which blood banks could contribute to health screening and become involved in preventive medicine.
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PMID:Screening and evaluation of blood donors with upper-limit hematocrit levels. 368 58


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