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Pyogenic Variant of Primary Cutaneous Anaplastic Large-Cell Lymphoma: A Lymphoproliferative Disorder With a Predilection for the Immunocompromized and the Young

Papalas, John A MD*; Van Mater, David MD, PhD; Wang, Endi MD, PhD*

The American Journal of Dermatopathology: December 2010 - Volume 32 - Issue 8 - p 821-827
doi: 10.1097/DAD.0b013e3181d81dc3
Original Study

Cutaneous anaplastic large-cell lymphoma belongs to the class of primary cutaneous CD30-positive lymphoproliferative disorders. The pyogenic variant is marked by a neutrophil rich inflammatory background. We describe 2 cases (one which clinically presented as cellulitis, and another arising in a patient with Hodgkin lymphoma) and review the clinicopathologic features of cases reported in the literature. In all cases, the male to female ratio is 1.2:1. The average age at presentation for patients with this variant is 47 years old with 15% of patients being 25 years old or younger. Thirteen percent of patients are immunocompromized. Ten percent of patients experience extracutaneous disease progression and 18% of patients are dead at 10 months. Immunophenotypically, the anaplastic large cells demonstrate loss of pan-T cell antigens, CD2, CD3, CD5, and CD7, with 65% of cases expressing CD4 and 29% of cases expressing CD8. Epithelial membrane antigen expression is reported in over half of the cases. In the clinical context of a progressive ulcerating lesion in younger or immunocompromized patients, it is important for the pathologist when presented with a skin specimen demonstrating a neutrophil-rich inflammatory background to include the pyogenic variant of anaplastic large-cell lymphoma. We hope to increase awareness of this rare CD30-positive lymphoproliferative disorder subtype by better defining the clinical spectrum in which this entity can present.

From the *Department of Pathology; and †Department of Pediatrics, Duke University Medical Center, Durham, NC.

Reprints: John A. Papalas, MD, Department of Pathology, Box 3712, Duke University Medical Center, Durham, NC 27710 (e-mail:

© 2010 Lippincott Williams & Wilkins, Inc.