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PubMed1979

[Polycythemias of young subjects (author's transl)].

During the last 12 years, 86 apparently primary polycythemias have been observed in subjects less than 40 years old. In twelve of them a non-suspected cause has been found (seven abnormalities of the hemoglobin function). In 43 cases, all males, the erythrocytosis (red cell volume more than 36 ml/kg) was pure and stable, without any complication; this "benign erythrocytosis" probably depicts extreme values of the normal distribution of the red cell mass. In 31 cases, the disease had criteria similar to those of polycythemia vera observed in older patients; in 12 of these cases, severe thrombo-embolic complications have been observed at the very beginning of the disease; however no further complication has been seen, when the patients were actively treated (phlebotomies or 32P); at the present time, no one of these cases did show transformation into acute leukemia, but the long-term prognosis (beyond 15 years) cannot yet been precisely drawn.

Adolescent↗
PubMed1980

Neonatal polycythemia causing multiple cerebral infarcts.

A child had neonatal polycythemia, severe seizures beginning at 6 weeks of age, and a computerized tomography scan showing multiple cerebral infarcts. Follow-up showed substantial developmental delay with spastic quadriplegia. This case illustrates that neonatal polycythemia is a rare but preventable cause of multiple cerebral infarctions, subsequent seizures, and developmental delay.

Cerebral Infarction↗
PubMed1986

Polycythemia, hypomagnesemia, and hypocalcemia in infants of diabetic mothers.

Hypomagnesemia (serum magnesium concentration, less than 1.5 mg/dL [0.62 mmol/L]) and hypocalcemia (serum calcium concentration, less than 8 mg/dL [2.00 mmol/L]) have been reported in polycythemic infants, as well as in infants of diabetic mothers (IDMs). These latter infants are at risk for neonatal polycythemia (venous hematocrit, greater than or equal to 65% [0.65]). We tested the hypothesis that neonatal polycythemia in IDMs is associated with increased serum calcitonin concentration, hypomagnesemia, and hypocalcemia. Serum magnesium and calcium concentrations were measured at 24 and 72 hours of age in 76 IDMs; serum calcitonin concentration was measured at 24 hours of age. Peripheral venous spun hematocrit was measured between 2 and 4 hours of age. The rates of hypomagnesemia and hypocalcemia were similar in polycythemic and nonpolycythemic IDMs (0% vs 9% and 56% vs 49%, respectively). The serum calcitonin concentration was similar in both groups. There was no correlation between hematocrit and the serum magnesium or calcium concentration; a significant association existed between hypocalcemia and hypomagnesemia.

Female↗
PubMed1997

Improved method for diagnosis of polycythemia vera based on flow cytometric analysis of autonomous growth of erythroid precursors in liquid culture.

"Autonomous" development of erythroid colonies in erythropoietin (EPO)-free semi-solid culture has been used as an in vitro assay for diagnosis of polycythemia vera (PV). These colonies, however, are small and poorly hemoglobinized, rendering the assay in many cases unreliable. We report here on the use of a novel assay; it combines a modified culture procedure that maximizes the growth of EPO-independent erythroid cells, and immunofluorescence flow cytometry for their detection and quantitation. Peripheral blood mononuclear cells are cultured for 2-5 days in the presence of a combination of growth factors. During this phase, early erythroid committed progenitors, burst forming units (BFUe), proliferate and differentiate into colony forming units (CFUe)-like progenitors. In the second phase, the latter cells, in the presence of stem cell factor, hemin, and iron-saturated transferrin, continue to proliferate and mature into hemoglobin (Hb)-containing orthochromatic normoblasts. Neither phases contained EPO. The culture produced large, pure, and synchronized erythroid cell populations. The cells were then dually labeled with fluorescent probes, nuclear DNA with thiazole orange and intracellular hemoglobin (Hb) with phycoerythrin-conjugated monoclonal antibodies against human Hb. Cells positive for both labels were assigned as Hb-containing nucleated precursors. The presence of such cells in EPO-free cultures indicated "autonomous growth." None of the EPO-free cultures derived from normal donors or patients with secondary polycythemia contained such cells. Cultures derived from PV patients contained from 5 to 92% "autonomously grown" cells. These culture and analysis methods should minimize false negative results with PV patients and provide objective and quantitative data.

Cells, Cultured↗
PubMed1979

Polycythemia vera in a child with acute lymphocytic leukemia.

A 10-year-old boy, who had been in an uninterrupted remission of acute lymphocytic leukemia (ALL) for six years, developed polycythemia vera (PV). One and a half months after detection of PV, he was found to have active leukemia. Both the polycythemia and leukemia receded with anti-leukemia therapy. Three possible explanations for the development of PV in a child with ALL are discussed: 1) PV was a part of his original ALL and recurred whtn patient relapsed. The PV phase was detected only during relapse because the patient was under close observation. 2) PV was a second neoplasm independent of ALL. 3) PV was part of a second leukemia which was different from the original leukemia; this new ALL was derived from a pluripotential cell line involving both erythroid and lymphoid elements. A precedent for this explanation has been observed in chronic myelogenous leukemia.

Antineoplastic Agents↗
PubMed1982

Secondary treatment of polycythemia rubra vera with 6-thioguanine.

6-thioguanine, an antimetabolite not potentiated by allopurinol, has been used in the treatment of 29 patients with polycythemia vera. All but two of the patients had been treated previously by venesection and/or radioactive phosphorus (32p) and/or alkylating agents. The usual dose was 40-160 mg daily alternate weeks according to individual response. The treatment period varied from 6-66 months (mean, 31 months). Of 27 evaluable patients 24 (89%) responded to treatment and at one year there was a significant fall in all blood count parameters. Seven patients relapsed while on treatment and four were withdrawn because of side effects. 6-thioguanine merits further evaluation in the management of polycythemia vera, particularly in those patients who have received large cumulative doses of 32p and/or alkylating agents and in whom an alternative mode of therapy is desirable.

Aged↗
PubMed1989

POEMS syndrome associated with polycythemia vera.

POEMS syndrome is a multisystem disorder associated with polyneuropathy, organomegaly, endocrinopathy, a monoclonal protein (M-protein), and skin changes. The authors describe a patient with POEMS syndrome who has polyneuropathy of the upper and lower extremities, splenomegaly, impotence, IgA-lambda monoclonal protein (M-protein), and marked thickening of his skin. In addition, he has polycythemia vera. Although myeloproliferative disorders have been reported to occur in association with multiple myeloma, they have not been described with POEMS syndrome. The possible etiology of this association is discussed. This patient was successfully treated with melphalan and prednisone at the time of his initial diagnosis, but relapsed 10 years later. The relapse was treated with pulse doses of prednisone alone with complete resolution of his polyneuropathy and skin changes. This was accompanied by a fall in his IgA levels and improvement of his polycythemia.

Adult↗
PubMed1976

Pathogenesis of polycythemia vera--new concepts.

Data presently available suggest that polycythemia vera is an acquired clonal disorder of the pluripotent stem cell resulting in expansion of committed stem cell pools, most prominently in the erythroid line. In vitro and in vivo studies suggest that erythropoiesis in polycythemia vera is at least partially modulated by erythropoietin.

Erythropoiesis↗
PubMed1982

Mixed myeloid-Lymphoid colonies in a patient with polycythemia vera.

Peripheral blood mononuclear cells from a patient with polycythemia vera were cultured in a methylcellulose system employing human serum. Electron microscopy documented the appearance of mixed colonies containing lymphocytes, granulocytes, megakaryocytes, and erythrocytes. In vitro culture characteristics were similar to those seen for other patients with polycythemia ver, ie, colonies grew in the absence of added erythropoietin or other pathway-specific regulators. Plating efficiency was linearly related to the number of cells plated, which supports the concept that each colony arose from a single cell. The appearance of mixed myeloid-lymphoid colonies points to the existence of a primitive stem cell capable of giving rise to multiple hematopoietic cell lines.

Aged↗
PubMed1986

Trisomy 1q in polycythemia vera and its relation to disease transition.

Clinical and cytogenetic details of 12 patients with polycythemia vera and complete or partial trisomy of the long arm of chromosome 1 are reported. All patients had trisomy for at least the segments 1q22 to 1qter. The 1q or material from 1q was translocated to another chromosome in eight patients. This was chromosome 9 in four patients, and those cases all had trisomy also for 9p. The trisomy 1q was found at the time of diagnosis in three patients, later during the polycythemic phase in five, and in four patients when they were first examined during a late stage of the disease. Acute leukemia or a myelodysplastic syndrome developed in eight of the 12 patients. Signs of advanced disease, eg, myeloid metaplasia or myelofibrosis, preceded the leukemia in four cases and was noted in one more patient. Trisomy 1q was the most frequent structural chromosome abnormality in patients with polycythemia vera. It is thus one of several nonrandom abnormalities that can appear at any stage of the disease. It seems to occur with higher frequency in patients with myelofibrosis and/or leukemia, but it is not a specific characteristic of these complications.

Adult↗
PubMed1987

Acquired von Willebrand disease in patients with polycythemia rubra vera.

Platelet function and factor VIII complex were evaluated in ten patients with polycythemia rubra vera. Seven patients showed abnormal epinephrine-induced aggregation. The intracellular concentrations of adenosine diphosphate (ADP) were below normal, and the ratio of adenosine triphosphate (ATP)/ADP was greater than normal. In four of eight cases, there was a decrease in ristocetin cofactor activity and a reduction in the slowly migrating forms of vWF:Ag on crossed immunoelectrophoresis. Defect of large multimers of vWF:Ag was also observed. The ratio of vWF:Ag to ristocetin cofactor was elevated in these patients. Plasma from the patients had no effect on normal plasma except in one case, in which isolated IgG appeared to cause inactivation of ristocetin cofactor. Treatment with 1-deamino-8-arginine vasopressin caused correction of the vWF abnormalities with rapid return of ristocetin cofactor to baseline in some patients. The present study shows that the alterations of multimeric structure of vWF occur in more than 50% of patients with polycythemia rubra vera and are in some part due to the inhibitor specific for vWF.

Adenosine Diphosphate↗
PubMed1988

Recurrent spontaneous bacterial peritonitis in a patient with polycythemia vera.

Spontaneous bacterial peritonitis (SBP) is an infectious process that usually occurs in patients with cirrhosis. There are few reports of SBP in patients with other pathologies such as nephrotic syndrome, acute and chronic hepatitis, cardiac ascites, and ascites secondary to neoplastic disease. We report a patient with polycythemia vera in whom recurrent episodes of SBP occurred 8 months following a portacaval shunt operation for Budd-Chiari syndrome. Conceivably, the polycythemia vera (PV) complicated by hepatic vein thrombosis and portacaval shunt resulted in significant loss of hepatic reticuloendothelial system function and predisposed the patient to bacterial peritonitis.

Budd-Chiari Syndrome↗
PubMed1989

Reversal of bone marrow fibrosis and subsequent development of polycythemia in patients with myeloproliferative disorders.

Bone marrow fibrosis is a characteristic finding in agnogenic myeloid metaplasia and in the spent phase of polycythemia vera. It is commonly believed that the reticulin deposition is irreversible. However, we report four patients who demonstrated clinical and laboratory evidence of transition from myelofibrosis to polycythemia. The transition was documented by improvement in the hemoglobin concentration and by determination of the Cr51 red blood cell mass, accompanied by a resolution of the fibrosis on serial bone marrow biopsies. Two of the patients had been treated with alkylating agents and splenectomy, one with myelosuppressive therapy without splenectomy, and one with splenectomy alone. These findings indicate that bone marrow fibrosis in the chronic myeloproliferative disorders is not always an irreversible phenomenon. Pathogenetic implications will be discussed.

Aged↗
PubMed1989

Myelodysplastic syndrome with trisomy 11 associated with polycythemia vera.

A 52-year-old male with myelodysplastic syndrome (MDS) who had a prior history of polycythemia vera is reported. Chromosome analysis revealed that the bone marrow and blood cells at the MDS phase contained trisomy of chromosome 11 as the sole cytogenetic change. Trisomy 11 is rarely found in hematologic neoplasia, and all of the reported cases with trisomy 11 were diagnosed as having nonlymphocytic neoplasia. In this report, a correlation between the chromosome change and leukemia/MDS developed in polycythemia vera is discussed.

Blood Cell Count↗
PubMed1991

Myelodysplastic transformation of polycythemia vera: case report and review of the literature.

We report a case of refractory anemia with excess blasts (RAEB) developing in a 67-year old man with a history of polycythemia vera; results of cytogenetic and immunophenotyping studies are described. In this report the clinical, cytogenetic and hematologic features of myelodysplasia complicating polycythemia vera are reviewed. Results of immunophenotyping and cytogenetic studies, and the preponderance of cases developing after myelosuppressive therapy suggest that in the majority of cases myelodysplasia is treatment-related.

Aged↗
PubMed1993

Polycythemia associated with primary systemic amyloidosis: elevated levels of hemopoietic factors and cytokines.

A 60-year-old man was diagnosed to have primary systemic amyloidosis (AL type) and polycythemia (Hgb 19.1 g/dl, WBC 14,600/microliters, and platelets 60.5 x 10(4)/microliters). In vitro colony assay for hemopoietic stem cells showed increased numbers of colony-forming units (CFU)-G, CFU-GM, CFU-E, and CFU-Meg. Serum levels of erythropoietin (Epo), granulocyte colony-stimulating factor (G-CSF), interleukin (IL)-3 and IL-6 were elevated. The infiltration of amyloid fibrils into the hemopoietic factor-producing tissues may stimulate abnormal secretion of hemopoietic factors and cytokines, resulting in increased numbers of hemopoietic stem cells and polycythemia in the peripheral blood.

Amyloidosis↗
PubMed1993

Megakaryoblastic transformation of polycythemia vera associated with hypercalcemia.

Leukemic transformation is not uncommon in polycythemia vera, particularly after treatment with chemotherapeutic agents. The leukemias that supervene are mostly of myeloid type but megakaryoblastic transformation is distinctly uncommon. We report a case of polycythemia vera terminating in megakaryoblastic leukemia with associated hypercalcemia.

Cell Division↗
PubMed2006

A phase II trial of pegylated interferon alpha-2b therapy for polycythemia vera and essential thrombocythemia: feasibility, clinical and biologic effects, and impact on quality of life.

BACKGROUND: Conventional interferon-alpha (IFN) is an effective treatment for patients with myeloproliferative disorders. However, many patients discontinue therapy because of side effects. METHODS: In this 24-month, Phase II feasibility study of pegylated interferon alpha-2b (PEG-IFN) treatment, a starting dose of 0.5 microg/kg per week was received by 21 patients with polycythemia vera (PV) and 21 patients with essential thrombocythemia (ET). The treatment objective, a complete platelet response (CR), was a platelet count<400x10(9)/L in symptomatic patients and <600 in asymptomatic patients. Neutrophil polycythemia rubra vera-1 (PRV-1) messenger RNA expression was analyzed prior to and during therapy. Quality of life (QoL) was investigated by using the European Organization for Research and Treatment of Cancer QLQ-C30 questionnaire. RESULTS: At 6 months, 29 of 42 patients (69%) had achieved a CR after a median of 83 days. The CR rate was not related to diagnosis, gender, or previous therapy. Nineteen patients completed the planned 2-year treatment in CR. No thromboembolic or bleeding complications were observed. Phlebotomy requirements were reduced in the majority of patients with PV. Five of 14 patients (36%) who initially were positive for PRV-1 achieved normalized PRV-1 expression under PEG-IFN treatment. Side effects were the cause of therapy failure in 16 of 23 patients. However, only 8 of 19 patients reported any side effects at 2 years. The QLQ-C30 revealed clinically significant impairments in several aspects of QoL at 6 months; however, at 2 years, QoL measurements were not different from baseline. CONCLUSIONS: PEG-IFN effectively reduced platelet counts in 29 of 42 patients, but only 19 patients maintained a CR at 2 years. The reversal of PRV-1 positivity noted in a subset of patients suggested that PEG-IFN may have an effect on the malignant clone. PEG-IFN is a valuable therapeutic alternative for patients who tolerate its initial side effects.

Adult↗