title: Mediastinal adenopathy, lung infiltrates, and hemophagocytosis: unusual manifestation of pediatric anaplastic large cell lymphoma: report of two cases.
abstract: To date, only 1 report describes an anaplastic large cell lymphoma (ALCL) associated with hemophagocytosis in the pediatric population. To better characterize this unusual manifestation of ALCL, we identified 2 additional cases. Both patients had fever, cytopenia, decreased fibrinogen level, mediastinal or hilar adenopathy, minimal to no peripheral adenopathy, and lung infiltrates. Bone marrow biopsies and aspirates revealed striking hemophagocytosis but no ALCL. One patient fulfilled the criteria for hemophagocytic syndrome, but the other lacked 1 criterion. Both patients were initially given a misdiagnosis of infection-associated hemophagocytosis. Definitive diagnosis required lymph node biopsies that showed CD30+, anaplastic lymphoma kinase-l+ ALCL. Both tumors responded to standard lymphoma chemotherapy. One patient achieved complete remission, whereas the other patient died of complications after 2 cycles of therapy. These findings are similar to the first reported case and indicate that pediatric ALCL can manifest with an unusual constellation of symptoms consisting of hemophagocytosis, mediastinal or hilar adenopathy, and lung infiltrates.
authors.affiliation: Department of Pathology, Duke University Medical Center, Durham, NC 27710, USA.
authors.forename: Deborah W
authors.initials: DW
authors.lastname: Sevilla
authors.forename: John K
authors.initials: JK
authors.lastname: Choi
authors.forename: Jerald Z
authors.initials: JZ
authors.lastname: Gong
mesh_terms: D030301:Activin Receptors, Type II / Q000235:genetics; D016827:CD8 Antigens / Q000032:analysis; D002648:Child; D002675:Child, Preschool; D002889:Chromosomes, Human, Pair 2; D002895:Chromosomes, Human, Pair 5; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D017730:Ki-1 Antigen / Q000032:analysis; D008171:Lung Diseases / Q000473:pathology*; D008206:Lymphatic Diseases / Q000473:pathology*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000473:pathology*; D017728:Lymphoma, Large-Cell, Anaplastic / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D008482:Mediastinum; D014178:Translocation, Genetic
chemical_list: D016827:CD8 Antigens; D017730:Ki-1 Antigen; C425364:ACVRL1 protein, human; D030301:Activin Receptors, Type II