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)

Quantitative 52Fe scans were performed in 180 patients. Expansion of bone marrow was observed in 70. This bone marrow expansion was a nearly constant feature in haemolytic anaemia and in sideroblastic anaemia. It occurred in a third of the patients with myelofibrosis. In patients with polycythaemia rubra vera, expansion was noticed in only two out of seven. Erythropoiesis in expansion areas occurred despite persistence of fat in the iliac crest bone marrow biopsy. It could exist with a slight increase in erythropoiesis and might develop only after a long period of erythropoietic stimulation. Increased marrow activity can take place without erythropoietic expansion in long bones. The fraction of iron uptake in expansion areas did not exceed a third of total marrow iron uptake. With increasing erythropoiesis, the increase in iron uptake in expansion areas was less marked than the increase in the central areas. Erythropoiesis in expansion areas was usually not of major quantitative importance but could nevertheless reach the erythropoiesis of a normal adult.
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PMID:Quantitative assessment of erythropoiesis in bone marrow expansion areas using 52Fe. 394 48

17 cases of Budd-Chiari syndrome with polycythemia diagnosed by the authors are reviewed, and the primary or secondary etiology of this disorder in these cases, in the other 36 reported in the literature, and in the 8 users of oral contraceptives who have suffered the combined syndrome since 1966, is discussed. The Budd-Chiari syndrome is a primitive thrombosis of all or part of the subhepatic veins, and among 350 known cases, only 36 have also had polycythemia. Contrary to the mostly older patients with polycythemia vera, these 17 cases averaged 36.8 years, and were more often female (10 of 17), 4 had gynecologic antecedents 1 (oral contraceptives, 1 pregnancy, 1 abortion, 1 myomectomy). The clinical findings commonly seen were ascites (82%), hepatosplenomegaly (94%), bromosulphthalein retention (81%), low serum lipids (59%), high serum albumin (81%), low serum iron (75%), and other extensive thrombosis. 2 of these patients died within 2 months, 5 died within 2 years, and 5 are still living. The patient who took oral contraceptives had had 3 induced abortions and 1 pregnancy; took Ovariostat for 5 days, 1 month after first contracting progressive jaundice; was hospitalized 9 months later; and was successfully treated. The authors commented that polycythemia was documented in all cases of Budd-Chiari syndrome in pill users in which tests were made. Among these 17 cases, 2 had true polycythemia, and 8 has secondary polycythemia.
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PMID:[Polycythemia and Budd-Chiari syndrome. Apropos of 17 cases]. 515 23

The 61 observations of primary thrombocythemia described in this report represent approximately 15% of the cases of polycythemia vera recorded by the authors over the same 18-year period. The group includes 35 females and 26 males, with a mean age of 62. The disease is usually discovered on routine blood tests (half of cases), and more rarely because of hemorrhagic or thrombotic manifestations. Splenomegaly is found in one-third of cases. Platelet count is permanently above 800 X 10(9)/l (mean : 1 500 X 10(9)/l); mild hyperleukocytosis (mean : 16 X 10(9)/l) with predominant neutrophil polynuclears is usual but myelemia is not constant (28% of cases) and always very moderate; red cell parameters are normal in three-fourths of cases, while the remaining patients have anemia, either due to iron depletion or not. Reticulinic myelofibrosis, usually minimal, is found in 40% of cases. Medullary karyotype is always normal, without chromosome Ph1. Platelet functional abnormalities are not constant and do not correlate with the magnitude of thrombocythemia. 51 patients (84%) received myelosuppressive therapy, mainly by busulfan or radioactive phosphorus. Most deaths were due to intercurrent causes and only one patient developed acute leukemia. 71% of patients are alive at five years and subsequent decrease in the actuarial survival curve is very gradual.
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PMID:[Essential thrombocythemia. Clinical, biological study and developmental study of 61 cases]. 632 10

Biological data obtained principally with Fe-59 citrate are used with physical data to calculate radiation absorbed doses for ionic or weak chelate forms of Fe-52, Fe-55, and Fe-59, administered by intravenous injection. Doses are calculated for normal subjects, primary hemochromatosis (also called idiopathic or hereditary hemochromatosis), pernicious anemia in relapse, iron-deficiency anemia, and polycythemia vera. The Fe-52 doses include the dose from the Mn-52m daughter generated after injection of Fe-52. Special attention has been given to the dose to the spleen, which has a relatively high concentration of RBCs and therefore of radioiron, and which varies significantly in size in both health and disease.
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PMID:Radiation absorbed doses from iron-52, iron-55, and iron-59 used to study ferrokinetics. 633 90

The relation between whole blood viscosity and iron status was studied in 11 patients with polycythemia vera (PV) who were treated with venesection without iron supplementation. Six were already iron deficient at the start of the study, five were followed from normal iron status to deficiency. Iron status was investigated with serum ferritin, erythrocyte protoporphyrin, mean cell volume and mean cell hemoglobin. There was no correlation between whole blood viscosity at a fixed erythrocyte volume fraction of 44% and any of these variables. The mean whole blood viscosity during iron deficiency and during normal iron state did not differ. Even after several months of iron deficiency there was no increase in whole blood viscosity. It is concluded that iron deficiency in treated PV does not give increased whole blood viscosity.
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PMID:Does iron deficiency in treated polycythemia vera affect whole blood viscosity? 649 76

Recent studies from this department have suggested that the level of 2,3-DPG may be determined in part by the volume of the erythrocyte; this conclusion was reached on the basis of the finding of significantly elevated values of 2,3-DPG in heterozygous beta-thalassemia, even in the absence of anemia. In order to test the role of microcytosis in the formation of 2,3-DPG levels, a study was undertaken on a different patient material characterized by microcytosis without anemia or hypoxia, namely on cases of polycythemia vera (PV) rendered microcytic through therapeutic venesection (or blood loss) and on appropriate controls. Five cases of untreated PV (mean HB 18.36 +/- 1.53 g/dl, mean MCV 94.4 +/- 3.9 fl) had 2,3-DPG levels slightly lower than normal controls (13.67 +/- 0,75 mumoles/g Hb vs 14.18 +/- 1.41 mumoles/g Hb). Six microcytic iron deficient PV's (mean Hb 17.42 +/- 2.34 g/dl, mean MCV 74.5 +/- 6.2 fl) had very significantly increased 2,3-DPG levels (17.73 +/- 1.75 mumoles/g Hb). Similar high levels were obtained in five cases venesected in the past and maintained with cytostasis (mean Hb 15.22 +/- 0.67 g/dl, MCV 82.5 +/- 7.5 fl, 2,3-DPG 17.04 +/- 2.44 mumoles/g Hb). A strong linear negative correlation was obtained between 2,3-DPG values and the MCV (r = -0.736, P less than 0.001). It is concluded that microcytosis of other etiology and not only of beta-thalassemia may also lead, per se, to increased levels of 2,3-DPG. The different levels of 2,3-DPG in PV undergoing venesection vs untreated patients may explain some discrepant reports on the behavior of this metabolite in PV.
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PMID:[Erythrocyte volume and 2,3-DPG level. A study on a primary polycythemia model]. 662 51

An autopsy case of erythremia with sideroblastic tumor cell proliferation is described. A 60-year-old man was admitted to the hospital due to general fatigue and anorexia. Bone marrow aspiration revealed abnormalities in erythropoiesis (megaloblasts, 4%; sideroblasts, 84%; ring-formed, 39%, and PAS-positive, 5%). Therapy was directed to pulmonary tuberculosis. Anemia was not improved despite repeated whole blood and platelet transfusions. Serum iron and percentage saturation of the total iron-binding capacity rose during the course. Administration of vitamin B12, B6 or folic acid was inefffective. INAH was replaced by its derivative, IHMS, during the course, but the population of sideroblasts especially of ring-sideroblasts was invariably large (78%-100% and 39%-65% for total sideroblasts and ring-sideroblasts, respectively). He died with increasing abdominal pain and jaundice after three months' hospitalization. Main autopsy findings were: diffuse proliferation of atypical erythroblasts in the bone marrow, systemic lymph nodes, liver, spleen and kidneys. Most of the cells positively stained with iron. Tuberculosis of lungs with cavity formation. Discussion is focussed on the relationship between erythremia and sideroblastic anemia.
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PMID:Erythremia with special reference to sideroblastic anemia. 693 66

Red cell ferritin (rFt) values were measured in 18 patients with iron deficiency anemia (IDA), 7 patients with hereditary spherocytosis (HS), 8 patients with megaloblastic anemia (MA), and 6 patients with polycythemia vera (PV). Furthermore, rFt was analyzed by column isoelectric focusing (IEF). The rFt values (Mean +/- SD) for healthy males and females are 14.3 +/- 10.3 and 7.5 +/- 3.6 ag/cell (ag = 10(-18)g), respectively, and a significant difference was noted between them by Student's t-test (p < 0.01). The mean rFt values in IDA, HS, MA, and PV patients were 1.9, 115.5, 198.3, and 3.3 ag/cell, respectively. The rFt values in IDA and PV patients were significantly lower than normal. However, there was no significant difference between levels presented in the two disease states. The rFt levels in HS and MA were significantly higher than those in healthy subjects. The isoelectric point (pI) of rFt determined by IEF in healthy subjects ranged from 5.1 to 5.7. The pI ranges for IDA patients showed a slight basic shift compared with those of normal subjects, and so did the ranges for PV patients. Marked increase in acidic red cell ferritin was detected by IEF in patients with IDA after iron therapy. The pI ranges for HS patients were diverse, and were considered to depend on the severity or clinical stage of the disease. The rFt of MA patients showed pI ranges similar to healthy subjects and the ranges differed little after Vitamin B12 therapy, irrespective of a drastic decrease in rFt concentration.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Red cell ferritin in non-leukaemic various erythropoietic disorders]. 792 80

With the very long life expectancy of Polycythaemia Vera late complications are often observed: progressive resistance to treatment, bad tolerance to maintenance by phlebotomy, progression towards myelofibrosis. Resistance to phosphorus 32 is reflected by a progressive reduction in the duration of remission and by a gradually decreasing remission rate. A complete resistance appears after a mean duration of disease of 7 years. In the maintenance treatment by hydroxyurea, there is a secondary resistance in one third of cases with a poor control of the excess platelets. Resistance to Pipobroman is less frequent (10%). The phosphorus resistance could be delayed by addition of maintenance treatment by Hydroxyurea. In the presence of resistance to 32 P, Hydroxyurea and Pipobroman often remain effective. In the case of resistance to Hydroxyurea or Pipobroman, we have several possibilities: inversion of chemotherapy, other chemotherapy as Busulfan, 32 phosphorus. Intolerance of phlebotomy as baseline treatment is almost constant. Three complications lead to discontinuation of phlebotomy: development of cardiovascular complications, raised platelet count to above 800,000 and often more than 1 million, progressive increase in the size of the spleen with appearance of signs of myeloid splenomegaly. Exclusive phlebotomies are not indicated as baseline treatment of Polycythaemia Vera. The progression towards myelofibrosis (spent phase, post polycythaemia myeloid splenomegaly) increases with the duration of the disease and the frequency of transformation differs according to the type of treatment. The time to transformation is much shorter in the patients treated by phlebotomy. The transformation towards myelofibrosis is demonstrated by bone marrow biopsy and isotope investigations (bone marrow scintigraphy and kinetic by iron 59 and chromium 51).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Clinical and laboratory assessment and therapeutic problems in longstanding polycythaemia vera. 803 42

We conducted a prospective study on 81 consecutive patients who had a kidney transplant with graft function for over 3 months to evaluate the prevalence of erythrocytosis following renal transplantation (PTE) and its potential risk factors. True PTE was defined as a RBC mass > 120% of the theoretical value allowing for sex, weight and height. 18 patients (22.2%) developed PTE (RBC mass = 157 +/- 21%) with no evidence of polycythemia vera (PV), or secondary polycythemia due to reduced arterial oxygen, kidney or hepatic tumors. PTE was more common in males (p = 0.041) and less common in patients treated with recombinant erythropoietin (rHEPO) prior to transplantation. 18 non-polycythemic patients (Hb 12.6 +/- 1.3 g/dl) matched for sex, age and renal function were used as case controls. Fewer PTE patients were transfused post-transplantation (p = 0.026). At the time of diagnosis, mean serum EPO was normal and similar to that of controls. PTE patients had lower serum ferritin (p = 0.005) and more commonly received iron supplementation when PTE occurred (p = 0.003). Other clinical factors did not differ significantly between the two groups. Two patients had a thrombotic event, 6 recovered spontaneously and 11 were successfully treated with angiotensin-converting enzyme inhibitors (ACEI). The normalization of Hb, hematocrit and RBC mass in ACEI treated patients was accompanied by a decline in serum EPO (p = 0.008). We conclude that true erythrocytosis is prevalent in cyclosporine-treated renal transplant patients. PTE seems to be an idiopathic erythrocytosis. Pretransplant rHEPO treatment may limit PTE by blunting the increased sensitivity of erythroid precursors to EPO and iron supplementation, which stimulates the development of PTE. ACEI treatment is effective and safe.
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PMID:Factors predisposing to post-renal transplant erythrocytosis. A prospective matched-pair control study. 912 2


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