Dados do Trabalho

Título

HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS IN FIRES:CASE REPORT

Apresentação do caso único

Male patient, six-year-old, previously healthy. The patient initially presented with persistent high fever, which progressed to frequent seizures, followed by super-refractory status epilepticus (SRSE). Normal liquor result. He had periods of SRSE remission at the expense of high doses of midazolam and ketamine in continuous infusion, in addition to intermittent anticonvulsants in target doses (Valproic Acid, Phenobarbital, Lacosamide and Topiramate). He received pulse therapy with Methylprednisolone for five days and two doses of intravenous immunoglobulin, associated with a period of ketogenic diet. A sustained increase in ferritin, triglycerides, hepatic transaminases was noted, associated with anemia, thrombocytopenia, hepatomegaly and cholestasis, a bone marrow biopsy was performed with signs of hemaphagocytosis. Thus, the diagnosis of Hemophagocytic Lymphohistiocytosis was confirmed. Treatment with intravenous Etoposide associated with Dexamethasone and intrathecal Methotrexate was initiated according to the HLH-2004 protocol.

Discussão

Unexplained high fever concomitant with multisystem dysfunction is an association that should be considered as a differential diagnosis for hemophagocytic lymphohistiocytosis (HLH). There is permission in the literature for the coexistence between Febrile Infection-Related Epilepsy Syndrome (FIRES) and HLH in the pediatric age group - which may have a cause and consequence relationship. Patients with splenomegaly, cholestasis, cytopenia in peripheral blood, hypertriglyceridemia and marked increase in ferritin are candidates for investigation to HLH. The treatment is based on the 2004 protocol, with rapid resolution of seizures with marked improvement in encephalopathy when performed the standard therapy early. Central nervous system involvement is an important factor associated with poor prognosis and may precede the characteristic signs and symptoms of HLH.

Comentários finais

Although rare, the simultaneity between FIRES syndrome and HLH is a diagnostic challenge that must be considered in neurological evaluation. Early treatment can ensure patient survival and reduce morbidity.

Referências

1- Farias moller et al. Fueling the Fires :hemophagocytic lymphohistiocytosis in febrile infection-related epilpesy syndrome. Epilepsia
. 2018 Sep;59(9):1753-1763. doi: 10.1111/epi.14524. Epub 2018 Aug 22.

2. Yun‑Ze Zhao et al. Central Nervous System Involvement in 179 Chinese Children with Hemophagocytic Lymphohistiocytosis.Chin Med J (Engl). 2018 Aug 5;131(15):1786-1792. doi: 10.4103/0366-6999.237409.

Palavras Chave

HEMOPHAGOCYTIC; LYMPHOHISTIOCYTOSIS; FIRES