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POLICITEMIA VERA
La policitemia vera (PV) es un trastorno mieloproliferativo de etiologa desconocida que se caracteriza por una excesiva produccin de eritrocitos normales, de leucocitos y de plaquetas2.
Polycythemia is considered to be present when the hematocrit is >48 or >52 percent in women and men, respectively, or when the hemoglobin concentration is >16.5 or >18.5 g/dL in women and men, respectively.
POLICITEMIA VERA
Trastorno clonal de celula progenitora hematopoytica pluripotencial Aumento de eritrocitos, granulocitos y plaquetas
Policitemia Vera
RELATIVAS
VERDADERAS
PRIMARIAS (PV)
SECUNDARIAS
HIPOXEMIA CARBOXIHB EPO hepatocellular carcinoma, renal cell carcinoma, hemangioblastoma, pheochromocytoma uterine myomata: TRANSPLANTE RENAL SINDROME DE CUSHING
MANIFESTACIONES CLINICAS
SINTOMAS GASTROINTESTINALES
alterations in gastric mucosal blood flow due to altered blood viscosity, and/or increased histamine release from tissue basophils, although one study has indicated a high incidence of positivity for infection with Helicobacter pylori [25] .
Persistent leukocytosis Persistent thrombocytosis Microcytosis due to iron deficiency Splenomegaly Generalized pruritus (post-bath) Unusual thrombosis Erythromelalgia (acral dysesthesia and erythema)
Erythromelalgia involving the hands . This condition is associated with burning pain in the feet or hands accompanied by erythema, pallor, or cyanosis, in the presence of palpable pulses, and is common in both essential thrombocythemia and polycythemia vera.
ENFOQUE DIAGNOSTICO
MEDICION DE LA MASA GLOBULAR (>36 ml/k H o >32 ml/k M) insufficient to establish the diagnosis, since this is also observed in conditions associated with chronic hypoxia and with erythropoietin-secreting tumors DESCARTAR HIPOXIA (PO2 y saturacion) Carboxihemoglobina (TBQ) Curva de disociacion de la Hb EPO Vit B12 SERICA Y FAL LEUCOCITARIA PAMO: hiperplasia de las 3 series
Leucocitosis y trombocitosis
SANGRIAS AAS (eritromelalgia) Antihistaminicos Hidroxiurea IFN alfa Anagrelida Fosfato sodico P32
TRATAMIENTO