López-López JM, Sánchez-Guerra JA, Duany-Almira LF, Pompa-Carrazana SG, Larramendi-Benítez L

Language: Spanish
References: 0
Page: 195-200
PDF size: 416.99 Kb.

ABSTRACT

The hemophagocytic syndrome, also known as macrophage activation syndrome or reactive histiocitosis, is a heterogeneous entity that was described more than 60 years ago. Its etiology is still unknown, but it has been related to a decontrol and exaggerated response of the immunological system. It has a 1-2 incidence for every million children without predominance of age or sex. Average death rate can be 60 %. It was presented a case of a male 7 month old still on milk child who after of presenting several infections, is admitted in the Hospital due to a serious sepsis product of a pneumonia, the complete blood count reveled severe decrease of all the hemopoietic lines being diagnosed with Secondary Hemophagocytic syndrome, it was ordered a genetic study and the doctor acted in consequence of the disease, the patient didn´t respond to the treatment and died days before, arriving postmortem the genetic diagnose of Familiar hemophagocytic lymphohistiocytosis.