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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.

Publication ,  Journal Article
Papalas, JA; Van Mater, D; Wang, E
Published in: Am J Dermatopathol
December 2010

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.

Duke Scholars

Published In

Am J Dermatopathol

DOI

EISSN

1533-0311

Publication Date

December 2010

Volume

32

Issue

8

Start / End Page

821 / 827

Location

United States

Related Subject Headings

  • Young Adult
  • Skin Neoplasms
  • Skin
  • Neutrophils
  • Middle Aged
  • Male
  • Lymphoma, Primary Cutaneous Anaplastic Large Cell
  • Immunophenotyping
  • Immunohistochemistry
  • Immunocompromised Host
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Papalas, J. A., Van Mater, D., & Wang, E. (2010). Pyogenic variant of primary cutaneous anaplastic large-cell lymphoma: a lymphoproliferative disorder with a predilection for the immunocompromized and the young. Am J Dermatopathol, 32(8), 821–827. https://doi.org/10.1097/DAD.0b013e3181d81dc3
Papalas, John A., David Van Mater, and Endi Wang. “Pyogenic variant of primary cutaneous anaplastic large-cell lymphoma: a lymphoproliferative disorder with a predilection for the immunocompromized and the young.Am J Dermatopathol 32, no. 8 (December 2010): 821–27. https://doi.org/10.1097/DAD.0b013e3181d81dc3.
Papalas, John A., et al. “Pyogenic variant of primary cutaneous anaplastic large-cell lymphoma: a lymphoproliferative disorder with a predilection for the immunocompromized and the young.Am J Dermatopathol, vol. 32, no. 8, Dec. 2010, pp. 821–27. Pubmed, doi:10.1097/DAD.0b013e3181d81dc3.

Published In

Am J Dermatopathol

DOI

EISSN

1533-0311

Publication Date

December 2010

Volume

32

Issue

8

Start / End Page

821 / 827

Location

United States

Related Subject Headings

  • Young Adult
  • Skin Neoplasms
  • Skin
  • Neutrophils
  • Middle Aged
  • Male
  • Lymphoma, Primary Cutaneous Anaplastic Large Cell
  • Immunophenotyping
  • Immunohistochemistry
  • Immunocompromised Host