Fabiola Morales-Vias, MD,
Second Year Resident, Pediatric Residency Program
San Juan City Hospital, Puerto Rico, United States
Disclosure information not submitted.
Carlos Ocasio Lopez, MD
Pediatric Critical Care Associate Professor
University Pediatric Hospital, Puerto Rico, United States
Disclosure information not submitted.
Hilda Diaz, MD
Pediatric Critical Care Associate Professor
University of Puerto Rico School of Medicine, Puerto Rico, United States
Disclosure information not submitted.
Leslie Soto, MD
Pediatric Hematology/Oncology Associate Professor
University of Puerto Rico, Puerto Rico, United States
Disclosure information not submitted.
Sanet Torres, MD
Program Director, Pediatric Residency Program San Juan City Hospital
n/a, Puerto Rico, United States
Disclosure information not submitted.
Title: Aplastic Anemia presenting as HLH in a 5-year-old admitted to the ICU
Introduction:
Hemophagocytic lymphohistiocytosis (HLH) is an acquired or inherited disease that can be potentially fatal. It involves an altered response of the immune system that can be triggered by vaccinations, infections, or other underlying diseases (e.g. rheumatological, malignancy). HLH is known to be able to cause an aplastic bone marrow if untreated for a prolonged time, but aplastic anemia (AA) and HLH are rarely seen simultaneously in pediatric patients admitted to the PICU for new onset HLH management.
Description:
This is the case of a 5-year-old girl without history of systemic illness; with 1-week evolution of a non-febrile erythematous skin rash with a cephalocaudal pattern at first, followed by multiple non-pruritic painless generalized petechiae and easy bruising. Initial evaluation was remarkable now for fever, jaundice, petechiae, hepatomegaly, severe pancytopenia, elevated AST and ALT (1641 and 3965), increased LDH (464), and elevated ferritin levels. Lipid and coagulation profiles were within normal parameters. The patient was admitted to the pediatric ICU with initial assessment of Hepatitis associated Aplastic Anemia. Liver biopsy performed with nonspecific inflammatory changes. Bone marrow biopsy revealed hemophagocytes and acellular bone marrow. This was confirmed by positive IL-2R ALPHA (CD25 soluble). With an emergent steroid in combination with etoposide therapy, fevers disappeared, and liver function quickly improved; yet pancytopenia persisted warranting initiation of ATGAM therapy.
Discussion:
Aplastic Anemia associated with HLH has been rarely reported in the pediatric population. This case highlights the importance of a high index of suspicion for the condition and problems encountered during its diagnostic evaluation; due to the fast-deteriorating clinical course it carries and its lethality if treatment initiation is delayed.