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POLICITEMIA VERA
Y OTROS TRANSTORNOS MIELOPROLIFERATIVOS
Rugani María Florencia
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Crecimiento clonal expansivo de célula progenitora hematopoyética pluripotencial
POLICITEMIA VERA
MIELOFRIBROSIS IDIOPATICA CRONICATROMBOCITOSIS ESENCIALLEUCEMIA MIELOIDE CRONICA (CLM)
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POLICITEMIA VERA
La policitemia vera (PV) es un trastorno mieloproliferativo de etiología desconocida que se caracteriza por una excesiva producción 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.
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Policitemia Vera
1.9/100,000 per year,Trastorno clonal de celula progenitora hematopoyética pluripotencial
Aumento de eritrocitos, granulocitos y plaquetas
Ausencia de estimulo fisiológico conocido
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RELATIVAS VERDADERAS
Masa eritrocitaria normal
Secundarias a disminución del volumen plasmático
Masa eritrocitaria elevada PRIMARIAS (PV)
SECUNDARIAS
HIPOXEMIA CARBOXIHB EPO hepatocellular carcinoma, renal cell carcinoma,
hemangioblastoma, pheochromocytoma
uterine myomata: TRANSPLANTE RENAL SINDROME DE CUSHING
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MANIFESTACIONES CLINICAS
NEUROLOGICAS : cefaleas, mareos, alteraciones visuales
ENFERMEDAD TROMBOEMBOLICA
RUBICUNDEZ FACIAL o eritrosis
ERITROMELALGIA (enrojecimiento doloroso distal en las extremidades)
PRURITO (al bañarse con agua caliente) The cause of pruritus in PV is unclear. It has been suggested that mast cell
degranulation, with release of histamine, fibrinolytic factors, prostaglandins, or interleukin-31 may play a role
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] .
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Polycythemia vera - related clinical and laboratory featuresPersistent leukocytosis Persistent thrombocytosis Microcytosis due to iron deficiency Splenomegaly Generalized pruritus (post-bath) Unusual thrombosis Erythromelalgia (acral dysesthesia and
erythema)
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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.
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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
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Criterios para el diagnostico
Masa eritrocitaria alta
Saturacion normal de oxigeno
Esplenomegalia
Leucocitosis y trombocitosis
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TRATAMIENTO
SANGRIASAAS (eritromelalgia)AntihistaminicosHidroxiureaIFN alfaAnagrelidaFosfato sodico P32
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most common causes of death
Thrombosis (29 percent)
Hematologic malignancies (23 percent)
Non-hematologic malignancies (16 percent) Hemorrhage (7 percent)
Myeloid metaplasia with myelofibrosis (3 percent)