1/37. polycythemia vera in an anephric man.The effect of renal failure and bilateral nephrectomy on erythropoiesis and plasma erythropoietic activity was observed in a patient with polycythemia vera. For eight years the patient's hematocrit was maintained between 45 and 50 per cent by phlebotomy and in spite of the development of renal failure the hematocrit did not decline. Following rejection of a renal transplant, the hematocrit fell to 18 per cent but rose to 40 per cent with oral iron therapy. Following bilateral nephrectomy, the hematocrit fell to 29 per cent but subsequently increased to 37 per cent. After an episode of gastrointestinal bleeding the hematocrit was 21 per cent but subsequently rose to 32 per cent. erythropoietin could not be detected in the plasma either before or after nephrectomy. In addition, erythropoietin failed to stimulate 59Fe incorporation into heme in vitro in the patient's marrow cells. The data incidate that, in polycythemia vera, erythropoiesis does not require erythropoietin.- - - - - - - - - - ranking = 1keywords = phlebotomy (Clic here for more details about this article) |
2/37. multiple myeloma on polycythemia vera following radioactive phosphorus therapy.A 74-year-old white man with established polycythemia vera was treated with radioactive phosphorus after phlebotomies alone failed to control his disease. About 2 3/4 years later he died of multiple myeloma. The mutagenic effect of radioactive phosphorus may have caused or possibly accelerated preexisting myeloma. Basic nonmalignant disease deserves careful consideration before radiation or radiomimetic agents are used. One might consider a probably less mutagenic drug such as hydroxyurea in patients with polycythemia vera when phlebotomy alone does not give good control of red cell mass and thrombocytosis.- - - - - - - - - - ranking = 1keywords = phlebotomy (Clic here for more details about this article) |
3/37. Simultaneous occurrence of multiple aetiologies of polycythaemia: renal cell carcinoma, sleep apnoea syndrome, and relative polycythaemia in a smoker with masked polycythaemia rubra vera.A 58 year old male heavy smoker presented with intracranial haemorrhage and erythrocytosis. Four aetiologies of polycythaemia--polycythaemia rubra vera (PRV), renal cell carcinoma, sleep apnoea syndrome, and relative polycythaemia--were found to be associated with the underlying causes of erythrocytosis. He did not fulfill the diagnostic criteria for PRV at initial presentation, but an erythropoietin independent erythroid progenitor assay identified the masked PRV, and the low post-phlebotomy erythropoietin concentration also suggested the likelihood of PRV evolution. This case demonstrates that a search for all the possible causes of erythrocytosis is warranted in patients who already have one aetiology of polycythaemia.- - - - - - - - - - ranking = 1keywords = phlebotomy (Clic here for more details about this article) |
4/37. chorea disclosing deterioration of polycythaemia vera.Neurological manifestations occur frequently in polycythaemia. chorea, however, is a rare complication of the disease. A case of chorea in a patient previously diagnosed with polycythaemia vera is reported. Choreic movements started after measurement of haematological variables showed deterioration. It was considered that this was caused by inappropriate treatment with iron because the chorea was markedly reduced after the two first venesections and normalisation of the packed cell volume and haemoglobin parameters.- - - - - - - - - - ranking = 1.4983784294137keywords = venesection (Clic here for more details about this article) |
5/37. polycythemia vera in a patient with the human immunodeficiency virus: a case report.It has been suggested that hiv plays a role in the generation of myeloproliferative disorders, including polycythemia vera (PV). Seven cases of polycythemia in hiv patients have been described in the literature, but only 3 of these met criteria for determining a primary origin (vera) of polycythemia. We report a case of PV in a patient infected with hiv. A 45 year old non-smoking homosexual male presented with 15.7 g/dl hemoglobin in 1991, and was diagnosed with hiv. After 7 years, he presented with plethora, splenomegaly, an erythrocyte mass of 71 ml/kg, and an oxygen saturation of 93.9% (the latter three constituting the major criteria for the diagnosis of PV). erythrocytes 7.35 x 10(6)/ml hemoglobin, 21.4 g/dl, hematocrit 63%, leukocytes 12,400, erythropoietin < 5 nmoll/ml. These values are all compatible with a diagnosis of PV. The CD4 count was 321 cells/mm(3) and hiv viral load was undetectable. The patient was initially treated with zidovudine. He was then treated with didanosine, lamivudine, and saquinavir, but all of them failed to slow the increase in erythrocyte levels. After a diagnosis of PV, he was treated with hydroxyurea and phlebotomy, which normalized the hemogram. CD4 count rose to 474 cells/mm(3) and hiv viral load remained at undetectable levels. The patient remains in stable condition with combination treatment after 1 year. We suggest that this is a case of hiv infection which may have led to the emergence of polycythemia vera. Treatment of the hiv did not prevent the appearance of the myeloproliferative disorder.- - - - - - - - - - ranking = 1keywords = phlebotomy (Clic here for more details about this article) |
6/37. Peripheral gangrene as the initial manifestation of polycythaemia vera.Peripheral gangrene is an uncommon initial manifestation of polycythaemia vera, especially if it is not associated with occlusive disease of larger vessels of the limbs. A 57-year-old Chinese male with polycythaemia vera presenting with recurrent gangrene of the toes is described. Absence of occlusive arterial disease of larger vessels was shown by the presence of peripheral pulses. The digital gangrene was due most probably to impaired perfusion resulting from hyperviscosity. Correction of hyperviscosity in this case by venesection and treatment with the cytotoxic drug, busulphan, not only corrected the polycythaemia vera, but also cured the digital gangrene.- - - - - - - - - - ranking = 1.4983784294137keywords = venesection (Clic here for more details about this article) |
7/37. polycythemia vera presenting as complex regional pain syndrome of the lower limbs.CASE REPORT: A 73-year-old woman with bilateral severe pain in her legs and feet was admitted to the hospital. The pain had started spontaneously without any known trauma a year earlier and had progressively worsened to the point that she was unable to walk. The patient complained of pain, discoloration, and sweating of the lower extremities. physical examination revealed swollen, cold, wet, and blue extremities, with both allodynia and hyperalgesia, fulfilling criteria for a diagnosis of complex regional pain syndrome (CRPS) type 1. Laboratory findings demonstrated a hemoglobin level of 180 g/L(-1) and a hematocrit of 47%, leading to a diagnosis of polycythemia vera (PCV). Her condition slowly improved after repeated venesection, mild analgesics, and amitriptyline. CONCLUSIONS: polycythemia vera has not been described previously as a contributing factor in CRPS. In the present case, the diagnosis of PCV was important, because its treatment also improved the CRPS.- - - - - - - - - - ranking = 1.4983784294137keywords = venesection (Clic here for more details about this article) |
8/37. Qualitative platelet defect and thrombohaemorrhagic complications in a patient with polycythaemia vera. Case 10.We describe a 67-year-old patient with polycythaemia vera and pathological functional platelet studies. He not only suffered a transient ischaemic attack despite taking of antiplatelet agents, but also showed bleeding diathesis with cerebral bleeding and spontaneous suffusions. Platelet function studies and clinical findings improved after phlebotomy and cytoreductive treatment with hydroxyurea. thrombosis and haemorrhage account predominantly for morbidity and mortality in patients with polycythaemia vera. The pathophysiological mechanisms to explain thrombosis and bleeding in patients with myeloproliferative disorders including polycythaemia vera were intensively studied. However, up to now no clear correlation of laboratory findings in relation to clinical history of thrombosis and bleeding was demonstrated. In this report the most important pathophysiological mechanisms and therapy with antiplatelet agents are discussed.- - - - - - - - - - ranking = 1keywords = phlebotomy (Clic here for more details about this article) |
9/37. A case of transition of polycythemia vera to chronic neutrophilic leukemia.Chronic Neutrophilic leukemia (CNL) is a rare myeloproliferative disorder characterized by a persistent increase of mature peripheral neutrophils, myeloid hyperplasia in bone marrow, hepatosplenomegaly, elevated neutrophil alkaline phosphatase (NAP) and absence of philadelphia chromosome, with no evidence of infection or malignancy sufficient to mimic a leukemoid reaction. CNL has been associated with multiple myelomas in many reported cases, but transition of polycythemia vera (PV) to CNL is very rare. An 81-year-old female patient, who had undergone intermittent phlebotomy following the diagnosis of PV 8 years previously, was admitted to our hospital due to lower back pain. A physical examination showed a splenomegaly 2 cm below the costal margin, with tenderness of the thoracic and lumbar spine area. A peripheral blood examination showed a WBC count of 91,800/microL (neutrophil 88%) with a rare immature form, hemoglobin of 9.1 g/dL and a platelet count of 1,661,000/microL. Her NAP score was 58. The bone marrow examination showed 95% cellularity, with an M:E ratio of 10:1, increased megakaryocytes with normal morphology and the absence of myelofibrosis. Chromosomal studies showed no philadelphia chromosome. A radiological examination showed compression fractures of the vertebrae and spinal cord compression. No underlying disease causing a leukemoid reaction was detected. With iron replacement, the hemoglobin level failed to increase over 12 g/dL. Therefore, it was concluded to be a transition of PV to CNL. After administration of hydroxyurea and vertebroplasty, the symptom improved and the WBC count was sustained below 40,000/microL.- - - - - - - - - - ranking = 1keywords = phlebotomy (Clic here for more details about this article) |
10/37. carcinoid tumor of the duodenum and accessory papilla associated with polycythemia vera.Carcinoid tumors have been reported in a wide range of organs but most frequently involve the gastrointestinal tract; however, duodenal carcinoid tumors are rare. We report a 50-year-old male patient complaining of multiple melenas for 3 wk. The panendoscopy and endoscopic retrograde cholangiopancreaticography revealed swelling accessory papilla with an ulcer. The biopsy taken showed a carcinoid tumor. The lesion was removed by wide resection. Patient was found to have an abnormal blood cell count during the follow-up period with elevated levels of hemoglobin and hematocrit of 21.2 g/dL and 63.5%, respectively, thrombocytosis of 501,000/microL, and leukocytosis of 20,410/microL. He was diagnosed as a polycythemia vera by a hematologist after further evaluation. He received periodic phlebotomy and hydroxyurea treatment. The response was good and his hematocrit was stabilized by periodic phlebotomy in the range of 44-49% during the last 2 years. The possible origin of UGI bleeding by a duodenal carcinoid tumor, although rare, should be considered. There has been one case report of a duodenal carcinoid tumor that involved accessory papilla of the pancreas divisum and one case report of metastatic carcinoid tumor associated with polycythemia vera. It is different in our patient as compared with the latter report, which mentioned a polycythemia vera patient who was found to have a metastatic carcinoid in the 17 years follow-up period. Chemotherapy had been given before the carcinoid tumor was revealed. Our patient had no previous chemotherapy for polycythemia vera before he was found to have duodenal carcinoid tumor; this excludes the possibility of chemotherapy induced carcinoid tumor, although it had been suspected in the previous report. In our patient, the existence of both diseases may be by predisposition of each other since both diseases have an increased incidence of other neoplasm, or they may be coexistent incidentally.- - - - - - - - - - ranking = 2keywords = phlebotomy (Clic here for more details about this article) |
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