POLICITEMIA VERA

Y OTROS TRANSTORNOS MIELOPROLIFERATIVOS

Rugani María Florencia

Crecimiento clonal expansivo de célula progenitora hematopoyética pluripotencial

POLICITEMIA VERA

MIELOFRIBROSIS IDIOPATICA CRONICATROMBOCITOSIS ESENCIALLEUCEMIA MIELOIDE CRONICA (CLM)

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.

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

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

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] .

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)

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

Criterios para el diagnostico

Masa eritrocitaria alta

Saturacion normal de oxigeno

Esplenomegalia

Leucocitosis y trombocitosis

TRATAMIENTO

SANGRIASAAS (eritromelalgia)AntihistaminicosHidroxiureaIFN alfaAnagrelidaFosfato sodico P32

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)