Çocuk Sağlığı ve Hastalıkları Dergisi
2010 , Vol 53 , Num 3
Glikojen depo hastalığı ve hemofagositik lenfohistiyositoz birlikteliği:Bir vaka takdimi
Hacettepe Üniversitesi Tıp Fakültesi 1Pediatri Uzmanı, 2Pediatri Profesörü, 3Pediatri Doçenti
Hemophagocytic lymphohistiocytosis is a syndrome characterized by fever,
hepatosplenomegaly, hypofibrinogenemia, hypertriglyceridemia, and cytopenia,
with hemophagocytosis in bone marrow, spleen or lymph nodes. The
glycogen storage disease type Ia case we present had the parameters specific
for hemophagocytic lymphohistiocytosis, and the bone marrow aspiration
revealed histiocytic proliferation and erythrophagocytosis. The association of
hemophagocytic lymphohistiocytosis and metabolic diseases has been reported
before. Our case and other hemophagocytic lymphohistiocytosis cases associated
with metabolic diseases show that the activation of the reticuloendothelial
system may be triggered by metabolic decompensation.
Anahtar Kelimeler :
hemofagositoz, histiyositoz, glikojen depo hastalığı, metabolik
hastalıklar.