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

Disciplines

Medicine and Health Sciences

PubMedID

17276937

Department(s)

Department of Pathology and Laboratory Medicine

Document Type

Article

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