POLICITEMIA VERA

Y OTROS TRANSTORNOS MIELOPROLIFERATIVOS Rugani Mara Florencia

Crecimiento clonal expansivo de clula progenitora hematopoytica pluripotencial

POLICITEMIA VERA

MIELOFRIBROSIS IDIOPATICA CRONICA TROMBOCITOSIS ESENCIAL LEUCEMIA MIELOIDE CRONICA (CLM)

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

1.9/100,000 per year,

Ausencia de estimulo fisiolgico conocido

Policitemia Vera

RELATIVAS

VERDADERAS

Masa eritrocitaria normal

Masa eritrocitaria elevada

PRIMARIAS (PV)
SECUNDARIAS

Secundarias a disminucin del volumen plasmtico

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)
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

PRURITO (al baarse con agua caliente)

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 features

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

Masa eritrocitaria alta

Saturacion normal de oxigeno Esplenomegalia

Leucocitosis y trombocitosis

Criterios para el diagnostico

SANGRIAS AAS (eritromelalgia) Antihistaminicos Hidroxiurea IFN alfa Anagrelida Fosfato sodico P32

TRATAMIENTO

Thrombosis (29 percent) Hematologic malignancies (23 percent)

Non-hematologic malignancies (16 percent)

Hemorrhage (7 percent) Myeloid metaplasia with myelofibrosis (3 percent)

most common causes of death