1/35. diagnosis of polycythemia vera in an anemic patient.Criteria proposed by the polycythemia vera Study Group (PVSG) as well as several derived algorithms are currently used for the diagnosis of polycythemia vera. Although these guidelines have significantly enhanced diagnostic accuracy, they uniformly consider erythrocytosis as the requisite premise for instigating the subsequent workup. We describe the unusual presentation of a patient with microcytic anemia in whom the diagnosis of polycythemia vera was reached using the PVSG criteria and confirmed by in vitro culture assay of erythroid progenitor cells. This case highlights the usefulness of the PVSG criteria, including the red cell mass determination, for the diagnosis of polycythemia vera even in anemic patients. The roles of spleen red cell pooling and plasma volume expansion as major determinants of this unusual presentation are discussed.- - - - - - - - - - ranking = 1keywords = plasma (Clic here for more details about this article) |
2/35. Transient polycythemia and multi-organ failure due to acute alcohol ingestion.Relative polycythemia is characterized by elevated hematocrit with normal red cell mass and results from decreased plasma volume. We present a case of a 39-year-old man who had at least two episodes of severe relative polycythemia and multi-organ failure following acute alcohol ingestion. Although the acute dehydrating effects of alcohol are well known, they usually result in an indolent course. This is the first report of recurrent severe polycythemia and multi-organ failure following acute alcohol consumption.- - - - - - - - - - ranking = 1keywords = plasma (Clic here for more details about this article) |
3/35. Spurious polycythemia.Spurious polycythemia is not a primary disease process. It sometimes may be nothing more than an unusual, but normal, physiologic state. In other instances, however, it is associated with a true abnormality of plasma volume. Although there is probably overlap between these extremes, differentiation of these subclasses may be of prognostic significance. The elevation in hematocrit bears no relation to morbidity, and, because there is no evidence of abnormal erythroid proliferation, reduction of red cell volume via phlebotomy or myelosuppression is inappropriate. Nonhematologic parameters, particularly hypertension, are the major factors of significance in the substantial cardiovascular morbidity in spurious polycythemia, and they demand attentive and aggressive management.- - - - - - - - - - ranking = 1keywords = plasma (Clic here for more details about this article) |
4/35. Haemoglobin Rahere (beta Lys-Thr): A new high affinity haemoglobin associated with decreased 2, 3-diphosphoglycerate binding and relative polycythaemia.A new haemoglobin with increased oxygen affinity, beta82 (EF6) lysine leads to threonine (Hb Rahere), was found during the investigation of a patient who was found to have a raised haemoglobin concentration after a routine blood count. The substitution affects one of the 2, 3-diphosphoglycerate binding sites, resulting in an increased affinity for oxygen, but both the haem-haem interaction and the alkaline Bohr effect are normal in the haemolysate. This variant had the same mobility as haemoglobin A on electrophoresis at alkaline pH but was detected by measuring the whole blood oxygen affinity; it could be separated from haemoglobin A, however, by electrophoresis in agar at acid pH. The raised haemoglobin concentration was mainly due to a reduction in plasma volume (a relative polycythaemia) and was associated with a persistently raised white blood count. This case emphasises the need to measure the oxygen affinity of haemoglobin in all patients with absolute or relative polycythaemia when some obvious cause is not evident.- - - - - - - - - - ranking = 1keywords = plasma (Clic here for more details about this article) |
5/35. Polycythemia in the newborn.Neonatal polycythemia, a venous hematocrit >65%, occurs in 1% to 5% of the total newborn population. Polycythemia can result from an excess production of red blood cells (active form) or from an increase in fetal blood volume (passive form). Clinical manifestations of polycythemia are caused by an increase in whole blood viscosity with a subsequent decrease in blood flow to organ systems. However, little information exists in the nursing literature concerning neonatal polycythemia. This article addresses the two categories of polycythemia and their etiologies; the involved pathophysiology; clinical manifestations of affected organ systems; supportive and specific therapies that can be used to treat polycythemic infants; the prognosis for polycythemic infants; and the difficulty healthcare providers face in deciding whether to treat this disorder. In addition, a case of a symptomatic infant who was treated with a partial exchange transfusion is presented.- - - - - - - - - - ranking = 0.025051937476966keywords = flow (Clic here for more details about this article) |
6/35. Erythrocytosis in hypophosphatemic rickets: irreversible complication due to nephrocalcinosis after vitamin d and phosphate therapy.A patient with hypophosphatemic vitamin d-resistant rickets developed secondary erythrocytosis during treatment with large doses of vitamin D2 and phosphate. Erythrocytosis was accompanied by a fall in circulating plasma volume and appeared to have developed as a consequence of nephrocalcinosis because it occurred after the appearance of nephrocalcinosis following several episodes of hypercalcemia and hyperphosphatemia. nephrocalcinosis and erythrocytosis did not disappear even after recovery of renal function. Thus, the present observations point to the importance of preventing these irreversible complications that could cause renal failure, erythrocytosis, and thrombotic events during the management of hypophosphatemic vitamin d-resistant rickets.- - - - - - - - - - ranking = 1keywords = plasma (Clic here for more details about this article) |
7/35. Systemic capillary leak syndrome associated with compartment syndrome and rhabdomyolysis.Systemic capillary leak syndrome (SCLS) is a rare disorder characterized by recurrent spontaneous episodes of hypovolaemic shock due to marked plasma shifts from the intravascular to the extravascular space. This presents as the characteristic triad of hypotension, haemoconcentration and hypoalbuminemia often with an associated monoclonal gammopathy. We describe a patient with SCLS who required aggressive fluid resuscitation and emergency fasciotomies for compartment syndrome with rhabdomyolysis. At presentation the patient was considered to have severe erythrocytosis and was therefore initially referred to a haematologist, which appears to be a frequent sequence of presentation for patients with SCLS. This patient also highlights the importance of muscle compartment pressure monitoring during volume resuscitation in patients with SCLS.- - - - - - - - - - ranking = 1keywords = plasma (Clic here for more details about this article) |
8/35. Polycythemia in diabetic patients on CAPD.Polycythemia in CAPD patients has been rarely described. Over an eight year period, 4 out of 123 CAPD patients (3%) were identified as having Hct values exceeding 50% for 1 month or longer. All of the 4 patients were insulin dependent diabetics (4/47 diabetic patients, 8.5%). charts were reviewed on 3 of these 4 patients. Polycythemia developed after a mean of 21 /- 7 months on peritoneal dialysis. Prior to the development of polycythemia, ferritin levels were low and ferrous sulfate therapy was begun at a time the Hct values were 36 to 40%. erythropoietin levels were obtained in 2 patients, and were 22 U/L (Hct 51%) and less than 5 U/L (Hct 55%). Renal ultrasound failed to show renal masses or cysts. One patient had a plasma volume of 2.1 L (normal 2.4-3.2 L); another patient was clinically volume depleted. Complications during the period of polycythemia included gangrenous feet requiring amputation in 2 patients, CVA in 2 patients, and splenic infarct in 1 patient. One patient died of cerebral thrombosis. We conclude that polycythemia is uncommon in CAPD patients and occurs most often in diabetic patients. Volume depletion and iron therapy may play a role in its etiology. In this high risk group of patients polycythemia may contribute to vascular complications and should be avoided.- - - - - - - - - - ranking = 1keywords = plasma (Clic here for more details about this article) |
9/35. Polycythemia and steroid overproduction in a gonadotropin-secreting seminoma of the testis.While investigating the cause of mild polycythemia in a young man, a testicular seminoma was discovered with unusual and tumor-dependent features: an absolute polycythemia with high plasma erythropoietin (EPO) levels, an overproduction of estradiol and testosterone, and a dramatic Leydig cell hyperplasia surrounding the tumor tissue. The authors attempted to gain insight into the relationship between this testicular tumor and the hormonal overproduction, i.e., of EPO, estradiol, and testosterone. Their results favored the conclusion that the high EPO levels and the polycythemia were an indirect effect secondary to the steroid overproduction rather than a direct EPO-producing activity. Moreover, the steroid overproduction by the testis could be caused by a paracrine mechanism through human chorionic gonadotropin activity on the leydig cells.- - - - - - - - - - ranking = 1keywords = plasma (Clic here for more details about this article) |
10/35. Patent ductus arteriosus with Eisenmenger's syndrome detected by first-pass study.The case of a 20-year-old man with patent ductus arteriosus complicated with Eisenmenger's syndrome is described. Radionuclide angiocardiography characterized the abnormal pattern of blood flow through the central circulation to establish a diagnosis of patent ductus arteriosus with a significant right-to-left shunt, which had not been indicated by other clinical findings.- - - - - - - - - - ranking = 0.025051937476966keywords = flow (Clic here for more details about this article) |
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