title: Concomitant Presentation of Hemophagocytic Lymphohistiocytosis and Posttransplant Lymphoproliferative Disease-Like Lymphoma in a Mildly Immunosuppressed Leukemia Patient: An Unusual Association.
abstract: We describe a 4-year-old female with pre-B-cell acute lymphoblastic leukemia on maintenance chemotherapy, who developed hemophagocytic lymphohistiocytosis (HLH) secondary to Epstein-Barr virus (EBV) infection, complicated by an aggressive lymphoproliferative disorder. Although there was no history of bone marrow transplant or underlying immunodeficiency, EBV triggered a post-transplant lymphoproliferative disease (PTLD)-like lymphoma. Multiple regimens of chemotherapy failed to induce remission and patient developed multiorgan failure. The association of HLH with EBV-related PTLD-like lymphoproliferative disorder is rare. We present this case to highlight this unusual association so that this highly fatal disease can be recognized and promptly addressed.
authors.affiliation: Department of Pediatrics, University of Kansas School of Medicine-Wichita, Wichita, Kansas.
authors.forename: Mohamad G
authors.initials: MG
authors.lastname: Sinno
authors.affiliation: Department of Pediatrics, University of Kansas School of Medicine-Wichita, Wichita, Kansas.
authors.forename: David
authors.initials: D
authors.lastname: Rosen
authors.affiliation: Department of Pediatrics, University of Kansas School of Medicine-Wichita, Wichita, Kansas.
authors.forename: Robert
authors.initials: R
authors.lastname: Wittler
mesh_terms: D000970:Antineoplastic Agents / Q000009:adverse effects / Q000627:therapeutic use*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002675:Child, Preschool; D016572:Cyclosporine / Q000627:therapeutic use; D003907:Dexamethasone / Q000627:therapeutic use; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000188:drug therapy / Q000473:pathology*; D005047:Etoposide / Q000627:therapeutic use; D005260:Female; D006801:Humans; D016867:Immunocompromised Host; D007223:Infant; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications / Q000188:drug therapy*; D008297:Male; D015122:Mercaptopurine / Q000627:therapeutic use; D008727:Methotrexate / Q000627:therapeutic use; D009102:Multiple Organ Failure / Q000150:complications / Q000473:pathology*; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000150:complications / Q000188:drug therapy*; D000069283:Rituximab / Q000627:therapeutic use; D017211:Treatment Failure; D014750:Vincristine / Q000627:therapeutic use; D019562:Viral Load
chemical_list: D000970:Antineoplastic Agents; D000069283:Rituximab; D014750:Vincristine; D005047:Etoposide; D003907:Dexamethasone; D016572:Cyclosporine; D015122:Mercaptopurine; D008727:Methotrexate
keywords: Epstein-Barr virus; HLH; acute leukemias; infections in immunocompromised hosts; lymphoproliferative disease