Childhood non-Hodgkin lymphoma presenting as hemophagocytic syndrome.
An 8-year-old girl with a progressive systemic hemopagocytic syndrome was found to have non-Hodgkin lymphoma (NHL) after multiple nondiagnostic biopsies. Routine histochemistry and flow cytometry demonstrated this to be a peripheral T-cell process and cytogenetics identified a t(2;5)(p23;q35). An extensive evaluation for an infectious agent failed to identify a pathogen. Treatment according to a standard lymphoma protocol produced a rapid response and the girl remains in remission without evidence of hemophagocytic syndrome 18 months from diagnosis. In children with systenic hemophagocytosis, a diagnosis of NHL should be aggressively pursued.
Blatt
Julie
J
Division of Pediatric Hematolotv-Oncology, University of North Carolina, Chapel Hill 27599, USA. jblat@med.unc.edu
Weston
Brent
B
Belhorn
Tom
T
Hamrick
Harvey
H
Maia
Diane
D
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D003937:Diagnosis, Differential; D005260:Female; D015616:Histiocytosis, Non-Langerhans-Cell / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D008198:Lymph Nodes / Q000473:pathology; D008228:Lymphoma, Non-Hodgkin / Q000175:diagnosis* / Q000188:drug therapy; D011379:Prognosis; D012698:Serologic Tests; D013577:Syndrome