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PubMed2001

Leukemic transformation of polycythemia vera after treatment with hydroxyurea with abnormalities of chromosome 17.

The leukemogenic risk attributed to therapy of polycythemia vera with radiophosphorus and alkylating drugs has led, over the last 20 years, to the increased use of myelosupressive nonmutagenic drugs, especially hydroxyurea. But there exist reports, which showed the development of polycythemia vera into acute leukemia not only in patients treated with alkylating agents and radiophosphorus but also with single hydroxyurea. In this article we present two cases of polycythemia vera, in which the development to acute myeloblastic leukemia occurred after long-term treatment with hydroxyurea. Significant is the fact, that in both presented cases cytogenetic and FISH analysis showed abnormalities of chromosome 17, in the one of case fullfilled criteria for "17p-syndrome". Due to the possibility of leukemogenic potential in the time of hydroxyurea treatment, it is necessary to be careful especially in young patients. The dynamic follow up of cytogenetic analysis is necessary, especially, in those, where long-term hydroxyurea therapy is supposed.

Antineoplastic Agents↗

PubMed1976

Activation of the coagulation system in polycythemia vera.

Thrombosis is one of the major complications of polycythemia vera. Seventeen patients with polycythemia vera in good hematologic control were evaluated for abnormalities of the coagulation system. Activation of the intrinsic coagulation cascade was suggested by low levels of factor XII, prekallikrein, and kallikrein inhibitors in 12 of 17 patients. The group also demonstrated a significant increase in soluble fibrin complexes using plasma gel filtration on 4% agarose. Fibrin degradation products were normal and antithrombin III levels were slightly elevated. It appears that patients with polycythemia vera have chronic activation of the coagulation system, probably initiated by activation of factor XII. No correlation between the degree of coagulation abnormalities and thromboembolic complications was evident in this group of patients.

Antithrombins↗

PubMed2003

[Gastrointestinal bleeding revealing polycythemia. A case report].

PURPOSE: Gastrointestinal bleeding, an uncommon complication of polycythemia can be the inaugural sign in exceptional cases. CASE REPORT: A 35-year-old patient was hospitalized for upper gastrointestinal bleeding. Physical examination and laboratory tests led to the diagnosis of polycythemia (Vaquez disease). Gastroscopy showed an hemorrhagic bulber ulcer. Outcome was favorable after chemotherapy and antinuclear treatment. CONCLUSION: Based on this observation and data in the literature we propose that this unusual presentation would suggest a relationship between polycythemia and ulcer disease.

Adult↗

PubMed1992

Increased thromboxane biosynthesis in patients with polycythemia vera: evidence for aspirin-suppressible platelet activation in vivo.

Increased thromboxane (TX) production and modified aspirin sensitivity has been detected in vitro in platelets isolated from patients with polycythemia vera. To verify the relevance of these capacity-related measurements to the actual rate of TXA2 biosynthesis in vivo and its suppression by oral aspirin, we have investigated the urinary excretion of major enzymatic metabolites of TXB2 in 17 patients with polycythemia vera and 23 gender- and age-matched controls. Urinary 11-dehydro-TXB2 and 2,3-dinor-TXB2 were measured by previously validated radioimmunoassays. In addition, urinary immunoreactive leukotriene (LT) E4 was measured to explore the 5-lipoxygenase pathway of arachidonate metabolism. Polycythemic patients had significantly (P < .001) higher excretion rates of both 11-dehydro-TXB2 (1,033 +/- 1,050 v 117 +/- 45 pmol/mmol creatinine; mean +/- SD) and 2,3-dinor-TXB2 (725 +/- 676 v 82 +/- 43 pmol/mmol creatinine) than controls. In contrast, urinary LTE4 was not significantly different. Enhanced metabolite excretion did not correlate with the platelet count or with the hematocrit value, and was not related to the current treatment or to a clinical history of thrombotic complications. Platelet TX receptor studies did not show any significant changes in the binding characteristics of two different ligands. A platelet-selective regimen of aspirin therapy (50 mg/d for 7 to 14 days) was associated with greater than 80% suppression in metabolite excretion in nine patients. These results are consistent with abnormal stimuli operating in polycythemia vera to induce a selective enhancement in the platelet biosynthesis of TXA2 without changes in receptor binding. This in vivo abnormality in platelet biochemistry can be largely suppressed by low doses of aspirin.

Adult↗

PubMed2005

[Preterm delivery by cesarean section in patient with polycythemia vera].

Polycythemia vera is a myeloproliferative disease most commonly diagnosed in male 60-70 years old patients. It is associated with a higher risk of thrombosis and bleeding. It is very rarely diagnosed in young patients, and thus only few cases of pregnant women have been reported in the literature. These reports show that polycythemia vera may have a detrimental effect on outcome of the pregnancy (PIH, spontaneous miscarriages, preterm delivery, and stillbirth). We report a 33-year-old woman with polycythemia vera who underwent caesarean delivery in 34 weeks gestation. This case demonstrates fully successful result of our intensive perinatal care.

Adult↗

PubMedDate not supplied

Packed cell volume reference intervals to aid in the diagnosis of anemia and polycythemia in young broiler chickens.

Packed cell volume (PCV) reference intervals were established for use in diagnosing anemia and polycythemia in young broiler chickens. Blood samples were collected from one hundred twenty 3-to-49-day-old clinically healthy conventionally reared broiler chickens. PCVs were determined and analyzed. PCVs regressed significantly (P less than 0.025) on age, which shows that neonatal physiologic anemia occurs in broiler chickens, as well as in mammals and specific-pathogen-free (SPF) chickens. The definition for anemia in broiler chicks varies with age. We defined anemia as a PCV less than or equal to 26%, 29%, 33%, 32%, 33%, 33%, 31%, and 28% for broiler chickens, 3, 7, 14, 21, 28, 35, 42, and 49 days old, respectively. We define polycythemia as a PCV greater than or equal to 42%, 43%, 41%, 40%, 42%, 42%, 41%, and 40% for broiler chickens 3, 7, 14, 21, 28, 35, 42, and 49 days old, respectively. Otherwise, broiler chickens have PCVs within established reference intervals. Results provide guidelines for detecting anemia and polycythemia in young broiler chickens of various ages.

Analysis of Variance↗

PubMedDate not supplied

Packed cell volume reference intervals to aid in the diagnosis of anemia and polycythemia in young leghorn chickens.

Packed cell volume (PCV) reference intervals were established for use in diagnosing anemia and polycythemia in young chickens. Blood samples were collected from 3-to-35-day-old specific-pathogen-free leghorn chicks. PCVs were determined and analyzed. PCVs regressed significantly (P less than 0.0001) on age, which shows that neonatal physiologic anemia occurs in chicks as well as in mammals. The definition for anemia in chicks varies with age. We defined anemia as a PCV less than or equal to 23%, 25%, 26%, 28%, 31%, and 30%, respectively, for chicks 3, 7, 14, 21, 28 and 35 days old. We defined polycythemia as a PCV greater than or equal to 36%, 37%, 38%, 37%, 41%, and 40%, respectively, for chicks 3, 7, 14, 21, 28, and 35 days old. Healthy chicks have PCVs within established reference intervals. Results provide guidelines to detect anemia and polycythemia in young leghorn chicks of various ages.

Anemia↗

PubMed1991

[The role of polycythemia in the pathogenesis of hypoxic myocardial hypertrophy].

Either chronic hypoxia or polycythemia induced by CoCl2 injection resulted in an increase of RBC, Hb, Hct and blood viscosity at different shear rate. There was an increase of right ventricular systolic and end diastolic pressure, +/- dp/dtmax, Vmax and right weight index, as compared with the control rats. Chronic hypoxia combined with polycythemia induced by CoCl2 injection further augmented the above parameters except Vmax of RV, and caused an increase of left ventricular weight index. The positive correlation was seen between the RV weight index and Hct. The above results prove that polycythemia might play important role in pathogenesis of hypoxic myocardial hypertrophy.

Animals↗

PubMed1990

Neurologic complications of polycythemia and their impact on therapy.

Polycythemia vera, a clonal stem cell disorder, produces neurologic problems in 50-80% of patients. Some symptoms, such as headache and dizziness, are related to hyperviscosity, and respond immediately to reduction of cell counts. Others seem to result from an associated coagulopathy. Patients with polycythemia tend to develop both arterial and venous thrombosis and are prone to hemorrhages. Treatments for polycythemia include phlebotomy, chlorambucil supplemented with phlebotomy, and 32P plus phlebotomy. Whatever treatment is chosen, the aim of therapy should be to reduce the hematocrit to approximately 40-45%.

Bloodletting↗

PubMed1990

Tumor cells are the site of erythropoietin synthesis in human renal cancers associated with polycythemia.

One to five percent of human renal cell carcinomas are associated with polycythemia. It is generally assumed that polycythemia results from the secretion of erythropoietin (Epo) by the malignant cells. However, there is no direct proof supporting this hypothesis. Three patients with typical renal adenocarcinoma and polycythemia were studied. All three exhibited high Epo serum levels as measured by radioimmunoassay (RIA). A strong Epo signal was observed on Northern blot analysis of total RNA extracted from the renal tumors. The Epo message seemed to be of normal size and no Epo gene rearrangement was observed with the restriction enzymes tested. Using the in situ hybridization technique, a significant labeling was constantly observed on the tumor cells. Immunohistochemical studies showed that these tumor cells, known to be of tubular origin, were labeled by an anti-cytokeratin antibody and therefore were of epithelial nature. Thus, this study demonstrated that malignant cells of tubular origin were able to produce Epo constitutively, whereas in the mouse hypoxic kidney, peritubular cells (probably capillary endothelial cells) were the major site of Epo synthesis.

Adenocarcinoma↗

PubMed1990

Polycythemia in the newborn: do asymptomatic babies need exchange transfusion?

A total of 2288 infants were screened for hematocrit over a period of 15 months. Polycythemia was diagnosed in 27 cases (1.2%). Preterm and term babies had more or less equal risk to develop polycythemia (1.5% and 1.1% respectively) while postterm infants had at least three times increased risk (3.4%) when compared to their term counterparts. Large-for-dates (LFDs) and small-for-dates (SFDs) infants had increased risk of manifesting polycythemia which was nearly four times (2.2%) and twenty-five times (13.2%) respectively, as compared to appropriate-for-dates (AFDs) babies (0.5%). About one-third of polycythemic infants had one or more symptoms. The most common symptoms observed was jitteriness (25.9%) followed by respiratory distress (14.8%) and lethargy (11.1%). The mean (+/- SD) hematocrit of symptomatic newborns (76.0 +/- 4.04) was found to be significantly higher (p less than 0.001) as compared to asymptomatic babies (70.84 +/- 2.73). Partial exchange transfusion with plasma was performed in all the symptomatic cases within 8 hours of onset of symptoms. No such intervention was performed in asymptomatic cases. On neurodevelopment follow-up, the development indices (MDI and PDI) of both asymptomatic and symptomatic cases were found to be comparable.

Growth↗

PubMed1989

[Polycythemia vera with an inhibitor against factor XII].

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.

Factor XII↗

PubMed1989

Rheological study on vascular occlusion and cellular hyperviscosity syndrome in polycythemia vera.

Whole blood viscosity was determined in 10 patients with polycythemia vera and 129 normal controls using a cone-plate type rotational viscometer at 37 degrees C. Three of the patients with polycythemia vera exhibited vascular occlusions including cerebral and myocardial infarction. The remaining patients had transient complications such as headache, short of breath and hot flush probably due to the circulatory disturbance. All patients had a blood viscosity higher than normal determined at a shear rate between 37.5 and 375 sec-1. This indicated that the patient might be in the imminent danger of occlusion when the viscosity is above twice the normal mean value. Clinical symptoms appeared in all patients at least over the value of the mean plus 3 standard deviations of the normal controls in the viscosity at a shear rate between 37.5 and 375 sec-1. The present study represented a close relationship between the occurrence of occlusive and/or reversible lesions and the elevated values of whole blood viscosity in polycythemia vera. The phlebotomy and fluid infusion therapy was valuable in most cases because the viscosity was decreased at once to the normal level. In addition an extended classification of hyperviscosity syndrome consisting of cell, plasma and combined hyperviscosity is proposed.

Adult↗

PubMed1989

Segmental portal hypertension and polycythemia vera.

A patient known to have polycythemia vera developed recurrent melena and was found to have bleeding gastroesophageal varices. Selective superior mesenteric angiography suggested splenic vein thrombosis. Splenectomy led to the disappearance of the varices with no subsequent recurrence of bleeding. We believe this to be the first fully described case of polycythemia vera associated with segmental portal hypertension, and propose that polycythemia vera patients may develop "silent" segmental portal hypertension and that this should be taken into consideration when treating them.

Humans↗

PubMedDate not supplied

The association between neonatal polycythemia, hypocalcemia, and hypoglycemia.

A study about the association between neonatal polycythemia, hypoglycemia, and hypocalcemia was carried out at the Hasan Sadikin General Hospital Bandung, Indonesia, between November 1986 and August 1987. Forty-six neonates with polycythemia were enrolled in the study and 92 neonates born during the same period formed the control subjects. The control subject neonates were sampled among those with equal birth weight and gestational age compared to the polycythemia subjects. Blood glucose levels were examined more than three hours after birth and before the first feeding by using the God-PAP method and blood calcium levels at less than 24 hours of age by using calorimetric method. The cut-off point for hypoglycemia and hypocalcemia were 30 mg% and 7 mg%, respectively. The results showed that there was a significant difference for hypoglycemia (p less than 0.01) and hypocalcemia (p less than 0.05) between the exposed subjects and the control subjects.

Humans↗

PubMed1988

Hepatocellular carcinoma associated with a rare combination of polycythemia and chronic thyroiditis.

A case of hepatocellular carcinoma associated with polycythemia and chronic thyroiditis, is reported in a 76-year-old female. At autopsy, the liver tumor was shown to be co-existent with liver cirrhosis. A series of hematological studies including the determination of plasma erythropoietin levels, led to the conclusion that this patient's polycythemia was most likely due to an excessive production of erythropoietin by the liver tumor. Chronic thyroiditis, another combined disease, might be related to liver cirrhosis, if the formerly advocated "hepato-thyroidal syndrome" is accepted. The author reports on this patient, since prior to this case there has been no documented case report of hepatocellular carcinoma accompanying both polycythemia and chronic thyroiditis.

Aged↗

PubMed1978

[Complications of antithrombotic prophylaxis with acetylsalicylic acid (ASS) in polycythemia (author's transl)].

Eight patients with polycythemia whose main complaints resulted from vascular complications were prophylactically treated with ASS. 4 patients tolerated the treatment without any side effects, 4 patients had an acute upper gastrointestinal hemorrhage, which was fatal in 2 cases. Correlations between the thrombohemorrhagical syndrome in polycythemia on one hand and the side effects of ASS on the other hand are discussed. As a result of this antithrombotic prophylaxis with ASS is not indicated in polycythemia even in predominance of vascular complications and absence of bleeding tendency. In life-threatening situations antithrombotic therapy with heparine should be preferred.

Aged↗

PubMed1986

[Scintigraphy of the bone marrow with 111In-citrin in polycythemia vera].

Scintigraphy of the bone marrow with 111In-citrin was performed in 32 patients with polycythemia vera and 8 controls. The method ensured an objective assessment of bone marrow function in patients with polycythemia vera. A characteristic RP scintigraphic picture corresponded to different clinical stages of the disease. A degree of a decrease in the blood radioactivity within 24 h after i. v. administration of 111In-citrin reflected the summary erythropoietic activity of the bone marrow and could serve as a differential diagnostic criterion in determining a stage of polycythemia vera.

Adult↗