Mediastinal adenopathy, lung infiltrates, and hemophagocytosis: unusual manifestation of pediatric anaplastic large cell lymphoma: report of two cases.
Publication/Presentation Date
3-1-2007
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.
Volume
127
Issue
3
First Page
458
Last Page
464
ISSN
0002-9173
Published In/Presented At
Sevilla, D. W., Choi, J. K., & Gong, J. Z. (2007). Mediastinal adenopathy, lung infiltrates, and hemophagocytosis: unusual manifestation of pediatric anaplastic large cell lymphoma: report of two cases. American journal of clinical pathology, 127(3), 458–464. https://doi.org/10.1309/CQGUUF5BUVJE0YU5
Disciplines
Medicine and Health Sciences
PubMedID
17276937
Department(s)
Department of Pathology and Laboratory Medicine
Document Type
Article