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Idiopathic myeloproliferative disease that is rare in childhood. Manifestations are caused by occlusive vascular lesions.

Vaquez Disease; Osler-Vaquez Disease; Vaquez Polycythemia; Polycythemia Vera.

5 to 6:1,000,000 in general population; affects males more often than females.

Evocated accidentally on routine blood examination. Diagnosis is usually made by the clinical findings of erythrocytosis, leukocytosis, thrombocytosis, and splenomegaly. Also, the association of blood examination and several signs, such as headaches, weakness, dyspnea, dizziness, or tinnitus, might confirm the diagnosis. However, only two or three of these criteria may be present. Direct determination of red cell mass has also been suggested, but is of uncertain value because of the difficulties in standardization and because of expense. Occasionally reported in childhood, it occurs mostly in middle-age males. Diagnosis can be confirmed by the association of splenomegaly, arterial saturation >92%, and an increased red blood cell (RBC) mass (>36 mL/kg in men and 32 mL/kg in women). Treatment includes phlebotomy and myelosuppression.

Insidious onset usually in the sixth decade of life. Erythrocytosis, neutrophilia, thrombocytosis, and splenomegaly are present. Thrombosis (10% with Budd-Chiari syndrome; 50% of patients develop at least one thrombotic complication, which includes cardiovascular accident, myocardial infarction [MI], deep venous thrombosis, and pulmonary embolism). Bleeding and bruising, although usually minor. Pruritus, peptic ulcer disease, gastric varices, angina, MI, congestive heart failure, dizziness. Reports of spinal cord compression from extramedullary hemopoiesis. Gout, secondary to increased urate turnover. Increased perioperative bleeding and thrombosis. Associated with lymphocytic lymphomas. Clinical features can also include headache, mental clouding, facial plethora pruritus, hepatomegaly, high blood pressure, and gout. More severe manifestations, caused by occlusive vascular lesions, can also be observed: transient ischemic attack, digital ischemia, stroke, bleeding (including gastrointestinal tract). Death occurs within 18 months without treatment and within 15 years with appropriate treatment, but there is a 20% incidence of transformation to myelofibrosis and approximately a 5% incidence of transformation to acute leukemia. Vaquez disease is a myeloproliferative disease. Hyperplasia involves all marrow elements and replaces marrow fat. There is increased production and turnover of RBCs, neutrophils, and platelets.

Hematology consultation for recommendations regarding phlebotomy. Check complete cell blood count and coagulation status. Postpone elective surgery in presence of myocardial angina and congestive heart failure. Evaluate cardiac function in cases of high blood pressure (clinical, chest radiographs, ECG, echocardiography). Determine toxicity secondary to chemotherapeutics. Evaluate vasoocclusive risk (full history, platelet count, hematocrit, leukocytes) and hepatic function (echocardiography, CT, laboratory investigations, including serum glutamic-oxaloacetic transaminase [SGOT], serum glutamic-pyruvic transaminase [SGPT], bilirubin). Elective surgery should be postponed until the hematocrit is reduced to <42% and platelets to <600,000/μL. Perioperative antithrombotic therapy should be considered.

Because surgical procedures may be hazardous, elective surgery should be postponed until the hematocrit is reduced to <42% and platelets to <600,000/μL. Normovolemic hemodilution can be useful. Careful intraoperative positioning is necessary. Pulsating boots or elastic stockings placed on the patient's legs can be used to reduce venous blood stasis. Avoid, if possible, the placement of a nasogastric tube in case of bleeding. Avoid regional anesthesia in presence of bleeding disorder. Maintain hydration to decrease viscosity. Avoid esophageal instrumentation in the presence of varices. Deep venous thrombosis prophylaxis is highly recommended.

The full regimen of perioperative antithrombotic therapy, including intravenous heparin, should be reevaluated in patients with recent bleeding episode.

Michiels JJ, Thiele J: Clinical and pathological criteria for the diagnosis of essential thrombocythemia, polycythemia vera, ...

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