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CD4+/CD56+ hematodermic neoplasm ("blastic natural killer cell lymphoma"): neoplastic cells express the immature dendritic cell marker BDCA-2 and produce interferon.

Abstract
CD4+/CD56+ hematodermic neoplasm ("blastic natural killer [NK]-cell lymphoma") is a rare highly aggressive neoplasm associated with cutaneous manifestations followed by dissemination to blood, bone marrow, and other tissues. Neoplastic cells exhibit a lineage-negative CD4+/CD56+/CD43+/HLA-DR+ immunophenotype, initially suggesting an NK-cell derivation. The recent discovery of CD123 antigen expression by tumor cells has provided evidence for a relationship to immature dendritic cells (DCs), a group of myeloid and lymphoid early-committed progenitors capable of differentiating into antigen-presenting DCs. Based on flow cytometric analysis, myeloid DCs represent the majority of human peripheral blood DCs and are positive for blood dendritic cell antigen (BDCA)-1 (or CD1c), CD13, CD11c(high), CD33, and CD123(low). Plasmacytoid DCs are BDCA-2+/ CD123(high)+/CD13-/CD33- and produce interferon (IFN)-alpha when triggered by antigens. IFN-alpha production may be detected in tissue sections using antibodies for myxovirus A (MxA) protein, a surrogate marker. This report describes the clinical, histologic, immunophenotypic, cytogenetic, and molecular genetic findings for 3 cases of CD4+/CD56+ hematodermic neoplasms. In all cases, neoplastic cells were reactive for CD123, BDCA-2, and MxA protein, providing strong evidence for an immature plasmacytoid DC derivation for this rare neoplasm.
AuthorsMonika E Pilichowska, Mark D Fleming, Jack L Pinkus, Geraldine S Pinkus
JournalAmerican journal of clinical pathology (Am J Clin Pathol) Vol. 128 Issue 3 Pg. 445-53 (Sep 2007) ISSN: 0002-9173 [Print] England
PMID17709319 (Publication Type: Case Reports, Journal Article)
Chemical References
  • CD4 Antigens
  • CD56 Antigen
  • CLEC4C protein, human
  • Lectins, C-Type
  • MX1 protein, human
  • Membrane Glycoproteins
  • Myxovirus Resistance Proteins
  • Receptors, Immunologic
  • GTP-Binding Proteins
Topics
  • Adolescent
  • Aged
  • CD4 Antigens (metabolism)
  • CD56 Antigen (metabolism)
  • Dendritic Cells (metabolism)
  • Female
  • GTP-Binding Proteins (analysis)
  • Humans
  • Immunophenotyping
  • Lectins, C-Type (metabolism)
  • Lymphoma, Non-Hodgkin (diagnosis, pathology)
  • Male
  • Membrane Glycoproteins (metabolism)
  • Middle Aged
  • Myxovirus Resistance Proteins
  • Receptors, Immunologic (metabolism)

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