TY - JOUR
T1 - Hemophagocytic syndrome with atypical presentation in an adolescent
AU - Pinto, Marta Valente
AU - Esteves, Isabel
AU - Bryceson, Yenan
AU - Ferrão, Anabela
PY - 2013/9/11
Y1 - 2013/9/11
N2 - A 14-year-old adolescent presented with a prolonged fever, abnormal liver function, anaemia, thrombocytopaenia, but a good general status. Diagnosis of hemophagocytic lymphohistiocytosis (HLH) was suspected, in spite of the initial indolent course. Secondary causes were excluded, but no specific mutation indicative of primary HLH was found. The patient started with specific therapy, but progressed with reactivations and later with persistently active disease. Haematopoietic stem cell transplantation was not successful and the adolescent died 7 months after diagnosis.
AB - A 14-year-old adolescent presented with a prolonged fever, abnormal liver function, anaemia, thrombocytopaenia, but a good general status. Diagnosis of hemophagocytic lymphohistiocytosis (HLH) was suspected, in spite of the initial indolent course. Secondary causes were excluded, but no specific mutation indicative of primary HLH was found. The patient started with specific therapy, but progressed with reactivations and later with persistently active disease. Haematopoietic stem cell transplantation was not successful and the adolescent died 7 months after diagnosis.
UR - http://www.scopus.com/inward/record.url?scp=84885627217&partnerID=8YFLogxK
U2 - 10.1136/bcr-2013-200929
DO - 10.1136/bcr-2013-200929
M3 - Article
C2 - 24027258
AN - SCOPUS:84885627217
SN - 1757-790X
JO - BMJ Case Reports
JF - BMJ Case Reports
M1 - 200929
ER -
