Giant lymphadenopathy infiltrated by gaucher cells mimicking lymphoma.
Gaucher disease (GD) is a lysosomal storage disease characterized by deficiency of beta-glucocerebrosidase, which results in accumulation of glucocerebroside in reticuloendothelial system, bone marrow infiltration, progressive hepatosplenomegaly, and skeletal complications. Herein we report a 5-year-old female with GD receiving enzyme replacement therapy who had giant mesenteric lymphadenopathies.
Yağci
Begül
B
Department of Pediatric Oncology, Hacettepe University Institute of Oncology, Ankara, Turkey. drbegul@yahoo.com
Salor
Ozge
O
Yalçin
Bilgehan
B
Gürakan
Figen
F
Güçer
Safak
S
Büyükpamukçu
Münevver
M
D003937:Diagnosis, Differential; D005260:Female; D005776:Gaucher Disease / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy; D005962:Glucosylceramidase / Q000172:deficiency / Q000235:genetics* / Q000627:therapeutic use; D006801:Humans; D007223:Infant; D008206:Lymphatic Diseases / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy; D008223:Lymphoma / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy; D014057:Tomography, X-Ray Computed
D005962:Glucosylceramidase