Mieloptisis y Síndrome de Kasabach Merrit como manifestación inicial de angiosarcoma esplénico
DOI:
https://doi.org/10.31053/1853.0605.v73.n4.14429Keywords:
angiosarcoma primario de bazo, reacción leucoeritroblástica, síndrome de Kasabach-MerritAbstract
El angiosarcoma primario de bazo es una enfermedad maligna, inusual y agresiva. La invasión de la medula ósea y las alteraciones en la coagulación han sido descriptos de forma aislada. Presentamos una paciente de 26 años, que consultó por dolor abdominal, y en estudios iniciales anemia severa y trombocitopenia, asociada a la presencia de reacción leucoeritroblástica. Se observaron también signos de coagulopatía por consumo y microangiopatía: esquistocitos, tiempo de protrombina prolongado, fibrinógeno disminuido y dímero D aumentado. Los estudios imagenológicos demostraron hepatoesplenomegalia con lesiones nodulares de diferentes densidades en ambos órganos, además de nodulillos pulmonares y lesiones óseas con neovascularización. El cuadro clínico de Síndrome de Kasabach Merrit y la presencia de tumor vascular llevó a la sospecha de tumor esplénico primario. Se realizó esplenectomía y biopsia de medula ósea, logrando así, el diagnóstico de angiosarcoma primario de bazo en estadio metástasico.
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