Síndrome hemofagocítico asociado a tuberculosis en una paciente con inmunodeficiencia adquirida / Hemophagocytic syndrome associated with tuberculosis in a patient with acquired immunodeficiency

RESUMEN

El síndrome hemofagocítico secundario es infrecuente en pediatría y, más aun, asociado a tuberculosis. Presentamos el caso de una paciente con síndrome de inmunodeficiencia adquirida, tuberculosis diseminada y síndrome hemofagocítico. Niña de 8 años, condiagnóstico de síndrome deinmunodeficiencia adquirida, que se internó por fiebre, vómitos y dolor abdominal. Presentaba distensión abdominal, deshidratación, taquipnea, rales subcrepitantes y sibilancias en ambos pulmones, anemia, plaquetopenia y alteración de la coagulación. Recibió antibióticos de amplio espectro y se realizó una laparotomía exploradora con apendicectomia y biopsia ganglionar. A las 72 horas, presentó convulsión tónico clónica, deterioro del sensorio, fiebre, hipoxemia, hepatoesplenomegalia, ascitis y edema periférico. Presentaba bicitopenia, hiperferritinemia y examen microscópico de médula ósea con hemofagocitosis. Recibió gammaglobulina intravenosa, corticoides y transfusiones sanguíneas. Se aisló Mycobacterium tuberculosis del aspirado gástrico, médula ósea y biopsia ganglionar abdominal. Se trató con isoniacida, rifampicina, estreptomicina y etambutol, y presentó franca mejoría.

ABSTRACT

The secondary hemophagocytic syndrome is rare in children and even rarer associated with tuberculosis. e report the case of a patient with acquired immunodeficiency syndrome, disseminated tuberculosis and hemophagocytic syndrome. An 8-year-old girl, diagnosed with acquired immunodeficiency syndrome, was admitted due to fever, vomiting and abdominal pain. She presented abdominal distension, dehydration, tachypnea, crackles and wheezing in both lungs, anemia, thrombocytopenia and coagulopathy. She received broad-spectrum antibiotics and exploratory laparotomy was performed with appendectomy and lymph node biopsy. After 72 hours the patient presented tonic clonic seizure, impaired sensory, fever, hypoxemia, hepatosplenomegaly, ascites and peripheral edema. She developed bicytopenia, hyperferritinemia and bone marrow microscopic examination with hemophagocytosis. She received intravenous gammaglobulin, steroids and blood transfusions. Mycobacterium tuberculosis was cultured in gastric aspirate, bone marrow and abdominal lymph node biopsy. She was treated with isoniazid, rifampicin, streptomycin and ethambutol, showing marked improvement.