真性红细胞增多症继发局灶节段性肾小球硬化症1 例

1临床资料 患者,男,78岁,因间断头痛、头晕伴双下肢水肿4年,加重3个月入院。患者4年前开始间断出现头痛、头晕、复视,伴双下肢水肿,无恶心、呕吐、言语不利及肢体活动障碍,收入某院,测血压210／80mmHg;     

广泛门静脉血栓伴红细胞血红蛋白逐渐增高1例报道

该病例影像学检查示门静脉系统广泛栓子,经系统检查未明确病因.4个月后出现红细胞、血红蛋白升高,考虑真性红细胞增多症.该疾病合并血栓原因为血液黏滞度增加,此患者在红细胞、血红蛋白升高前已形成广泛血栓,有无其他原因有待进一步研究.     

Pulmonary vein thrombosis in a patient with polycythemia vera

Pulmonary vein thrombosis(PVT) is a rarely encountered disease entity with varied clinical presentations. It is usually associated with lung carcinoma, lung surgeries and as a complication of the radiofrequency catheter ablation procedure for atrial fibrillation. Its clinical manifestations can vary from mild hemoptysis to lung infarction with hemodynamic compromise. A 76-year-old male presented with a 2-d history of pleuritic left sided chest pain. His past medical history included polycythemia vera, atrial fibrillation, coronary artery disease, pulmonary embolism and pulmonary hypertension. Chest radiograph was normal, troponins were normal and the 12-lead electrocardiogram did not show any ischemic changes. A computerized tomography pulmonary angiogram revealed a filling defect in the left lower lobe pulmonary vein. He was treated with subcutaneous enoxaparin and his symptoms improved. This case highlights a rare etiology of chest pain and the first reported case of the association of polycythemia vera and pulmonary vein thrombosis. A high index of suspicion is required for appropriate diagnostic work up. PVT can mimic pulmonary embolism. The diagnostic work up and treatment strategies depend on acuity of presentation.     

Life threatening chylothorax in a patient with congenital thrombophilia: case report

We reported here a case of bilateral chylothorax as a result of widespread thrombi formation in a patient who was heterozygote for factor V leiden gene mutation and who had antithrombin III deficiency. We performed bilateral chest tubes, thrombolytic and oral anticoagulant therapy. The patient responded to the therapy. She has been in follow up without symptoms for 18 months.     

Aspirin responsive platelet thrombophilia in essential thrombocythemia and polycythemia vera

Essential thrombocythemia (ET) and polycythemia vera (PV) frequently present with erythromelalgia and acrocyanotic complications, migraine-like microvascular cerebral and ocular transient ischemic attacks (MIAs) and/or acute coronary disease. The spectrum of MIAs in ET range from poorly localized symptoms of transient unsteadiness, dysarthria and scintillating scotoma to focal symptoms of transient monocular blindness, transient mono- or hemiparesis or both. The attacks all have a sudden onset, occur sequentially rather than simultaneously, last for a few seconds to several minutes and are usually associated with a dull, pulsatile or migraine-like headache. Increased hematocrit and blood viscosity in PV patients aggravate the microvascular ischemic syndrome of thrombocythemia to major arterial and venous thrombotic complications. Phlebotomy to correct hematocrit to normal in PV significantly reduces major arterial and venous thrombotic complications, but fails to prevent the platelet-mediated erythromelalgia and MIAs. Complete long-term relief of the erythromelalgic microvascular disturbances, MIAs and major thrombosis in ET and PV patients can be obtained with low dose aspirin and platelet reduction to normal, but not with anticoagulation. Skin punch biopsies from the erythromelalgic area show fibromuscular intimal proliferation of arterioles complicated by occlusive platelet-rich thrombi leading to acrocyanotic ischemia. Symptomatic ET patients with erythromelalgic microvascular disturbances have shortened platelet survival, increased platelet activation markers β-thromboglobulin (β-TG), platelet factor 4 (PF4) and thrombomoduline (TM), increased urinary thromboxane B2 (TXB2) excretion, and no activation of the coagulation markers thrombin fragments F1+2 and fibrin degradation products. Inhibition of platelet cyclooxygenase (COX1) by aspirin is followed by the disappearance and no recurrence of microvascular disturbances, increase in platelet number, correction of the shortened platelet survival times to normal, and reduction of increased plasma levels of β-TG, PF4, TM and urinary TXB2 excretion to normal. These results indicate that platelet-mediated fibromuscular intimal proliferation and platelet-rich thrombi in the peripheral, cerebral and coronary end-arterial microvasculature are responsible for the erythromelalgic ischemic complications, MIAs and splanchnic vein thrombosis. Baseline platelet P-selectin levels and arachidonic acid induced COX1 mediated platelet activation showed a highly significant increase of platelet P-selectin expression (not seen in ADP and collagen stimulated platelets), which was significantly higher in JAK2^V617F mutated compared to JAK2 wild type ET.     

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.     

Heparin-induced thrombocytopenia and thrombosis in a patient with polycythemia vera

A 75-year-old Japanese woman with polycythemia vera was admitted to our hospital in January 2003 with suspected pulmonary thromboembolism. After administration of heparin, platelet count decreased from 1,694 x 10(9)/l on admission to 60 x 10(9)/l on hospital day 14. The patient developed acute limb embolism and transient cerebral ischemic attack on days 17 and 25, respectively. Signs and symptoms mimicked those of disseminated intravascular coagulation. Antibodies against heparin and platelet factor 4 complexes were detected in serum, and a diagnosis of heparin-induced thrombocytopenia and thrombosis was made. Argatroban treatment improved thrombocytopenia and hypercoagulable state.     

Systemic mastocytosis in a patient with polycythemia vera treated with radioactive phosphorus.

Systemic mastocytosis occurred as a fatal event in a patient with long-standing polycythemia vera. The patient had been treated over the course of 21 yr with radioactive phosphorus. Possible relationships between mastocytosis and polycythemia vera, and also between mastocytosis and treatment with ionizing radiation, are discussed. Histopathologic and electron microscopic findings are illustrated. Difficulties in establishing the diagnosis of mast cell disease in this setting are also described.     

Huge post-operative ulcer following hydroxyurea therapy in a patient with polycythemia vera

A 75-year old woman was referred to our hospital due to a worsening post-operative cutaneous ulcer. She had a history of polycythemia vera and had been treated with a daily dose of 500mg hydroxyurea (HU) for six years. She underwent a right hernioplasty in another hospital in September 2002. Two days after the hernioplasty, the skin around the wound became erythematous and necrosis was observed. Even with the administration of antibiotics and debridment was performed three times, her condition deteriorated and she was referred to our hospital. On admission, a 22x15cm ulcer was observed on the lower abdomen. With termination of HU, the cutaneous ulcer became smaller and with the withdrawal of other medication, the ulcer size continued to decrease. Although post-operative cutaneous ulcer complicated by HU administration has rarely been reported, our case clearly indicates that early termination of HU should be considered when patients develop symptoms preceding cutaneous ulcer.     

Sequential development of chronic lymphocytic leukemia in a patient with polycythemia vera

A patient with a seven-year history of polycythemia vera treated by repeated phlebotomies and intermittent busulfan administration developed gradually lymphocytosis accompanied by thrombocytopenia in peripheral blood and in the bone marrow. A marked pathological monoclonal proliferation of the B-cell population was detected. The sequential development of chronic lymphocytic leukemia in the patient with polycythemia vera could be considered as a coincidence because there is no reliable explanation of this event at present.     

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 vera, 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.     

Thrombocytopenic purpura complicating radioactive phosphorus treatment in a patient with polycythemia vera

A patient who had polycythemia vera was given radioactive phosphorus and developed severe thrombocytopenic purpura associated with prominent changes in the appearance of the megakaryocytes.