Cases reported "Polycythemia Vera"

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1/7. Long-term successful coronary artery angioplasty in polycythemia vera.

    In a 65-year-old man with polycythemia vera, invalidating angina pectoris was associated with severe narrowing of the right coronary artery. After percutaneous coronary angioplasty (PTCA) the patient became symptom free and remained so for 12 months, while receiving an antiplatelet agent, a calcium antagonist and nitrate. coronary angiography repeated after a year, because of reappearance of angina, documented good patency of the treated artery and some progression of a narrowing involving another coronary vessel. This is the first reported case of long-term success of PTCA in polycythemia vera, a disease exposed to a high risk of thrombosis and, possibly, of restenosis. It is undefined whether medical treatment contributed to the anatomical and clinical results. As far as a single case can say, polycythemia vera might not represent a prohibitive background for coronary PTCA.
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2/7. Spontaneous cervical hematoma: a report of two cases.

    Cervical hematomas are generally associated with trauma, surgery, and tumors. Although they are rare, they can be life-threatening because they put the patient at risk for great-vessel compression and upper airway obstruction. We describe two cases of spontaneous cervical hematoma--one in an 81-year-old man and the other in a 30-year-old woman. The man reported dysphonia, dysphagia, and neck swelling of 5 hours' duration. He had been taking 100 mg/day of aspirin for a cardiovascular condition. Examination revealed that the man had polycythemia vera. The woman was found to have neck ache, odynophagia, and cervical ecchymosis; portal hypertension, schistosomiasis, and blood dyscrasia were also found. Both patients denied trauma. A suspected diagnosis of cervical hematoma was confirmed by computed tomography, and treatment was instituted. The hematomas resolved in about 2 weeks. The treatment of cervical hematoma is controversial, although it is agreed that the evaluation of upper airway obstruction and its permeability is mandatory. Surgical treatment is generally reserved for complicated cases because of the risk of infection or bleeding.
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3/7. 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.
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4/7. splenic rupture complicating periinterventional glycoprotein IIb/IIIa antagonist therapy for myocardial infarction in polycythemia vera.

    polycythemia vera is a myeloproliferative disorder predisposing to thromboembolic and bleeding complications. We report the case of a patient with polyglobuly, leukocytosis, and thrombocytosis, who suffered from acute ST-segment elevation myocardial infarction due to thrombotic high-grade pre-stent stenosis two months after percutaneous coronary intervention for complex coronary one vessel disease. Following re-PTCA and stent implantation in conjunction with periinterventional GP IIb/IIIa antagonist treatment, the patient was initially symptom free for about two hours before rapidly developing signs of a hemorrhagic shock. An abdominal CT scan showed splenic rupture with massive intraabdominal hemorrhage as a consequence of secondary bleeding into multiple pre-existing splenic infarctions. The patient's condition stabilized after emergency splenectomy. Subsequent bone marrow biopsy revealed the presence of polycythemia vera. Post-operatively, the patient was treated with the anti-platelet agents aspirin and clopidogrel to prevent subacute stent thrombosis. Additionally, cyto-reductive therapy with hydroxyurea was initiated because of a further increase in the platelet count. In patients with polycythemia vera, the indication for treatment with GP IIb/IIIa antagonists should be carefully weighed against the potentially serious bleeding complications. Should treatment be established, a risk stratification using abdominal sonography and bleeding time testing is recommended, while during treatment red blood count, platelet count, coagulation tests, and hemodynamic parameters should be closely monitored.
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5/7. Polycythaemia vera-transformation to myelofibrosis and subsequent reversal.

    A woman aged 67 who developed polycythaemia vera 21 years ago has been seen regularly since the diagnosis was first made. She was treated initially with pyrimethamine and then for 10 years by repeated venesections. After a 4 year period when her myeloproliferative disease was considered to be transitional a complete transformation to myelofibrosis occurred. She was treated by splenic irradiation and later with alkylating agents in an attempt to give her symptomatic relief from massive splenomegaly. Repeated and massive haemorrhage from gastro-oesophageal varices was treated initially by percutaneous transhepatic sclerosis of the gastro-oesophageal collateral vessels. A subsequent dacron mesocaval jump graft operation was successful in preventing further haematemesis and melaena. During the last year transformation back to polycythaemia vera has occurred. The evolution of her myeloproliferative disease has been fully documented by detailed blood counting, bone marrow aspirates and trephine biopsies, blood volume studies and other radioisotope investigations including the quantitation of functional erythropoietic tissue with 52Fe.
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6/7. Lacunar infarcts in polycythaemia with raised packed cell volumes.

    Lacunar (small deep cerebral infarcts) infarction is described in association with raised packed cell volumes. Two patients had polycythaemia vera, one stress polycythaemia. They presented with transient ischaemic episodes and were shown by computed tomography to have lacunes deep in the basal ganglia and internal capsule. Such lesions may be caused by small vessel occlusions related to increased viscosity and impaired oxygen consumption by adjacent tissues. Finding a raised packed cell volume in patients with lacunes and transient ischaemic attacks offers a further possibility of treatment.
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7/7. Coronary vasculopathy in polycythemia vera.

    thrombosis is a common complication in polycythemia often causing death. In coronary artery occlusion, thrombosis due to hyperviscosity and thrombocytosis is mostly discussed as the origin of the infarction. We discuss the case of a 30-year-old male patient, with polycythemia, who died of myocardial infarction. On autopsy the vessels showed neither ateriosclerotic changes nor thrombotic occlusions. Instead, a marked intima proliferation was found leading to multiple occlusions whereas media and adventitia were unchanged. This pattern of a coronary vasculopathy has not been described before, and can be interpreted as an alternative mechanism for vascular occlusion in polycythemia. Similar histopathological changes have already been found in skin lesions in erythromelalgia, a common symptom in polycythemia.
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