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Volume 63, Issue 2 , Pages 292-308, August 2010

Cutaneous CD4⁺ CD56⁺ hematologic malignancies

Cynthia M. Magro, MD
- Department of Pathology, Weill Medical College of Cornell University, New York, New York
- Reprint requests: Cynthia M. Magro, MD, Department of Pathology, Weill Cornell Medical Center, 1300 York Ave, F-309A, New York, NY 10044.
Pierluigi Porcu, MD
- Division of Hematology and Oncology, Ohio State University, Columbus, Ohio
Jochen Schaefer, MD
- Department of Pathology, Weill Medical College of Cornell University, New York, New York
Jack W. Erter, MD
- Division of Hematology and Oncology, Ohio State University, Columbus, Ohio
Richard R. Furman, MD
- Division of Hematology and Oncology, Weill Medical College of Cornell University, New York, New York
Paul K. Shitabata, MD
- Pathology Inc, Torrance, California
A. Neil Crowson, MD
- Regional Medical Laboratories, St John Medical Center, University of Oklahoma, Tulsa, Oklahoma

published online 11 June 2010.

Background

Hematologic malignancies expressing CD4 and CD56 are most commonly associated with the recently described CD4⁺ CD56⁺ hematodermic neoplasm.

Methods

Thirteen cases of CD4⁺ CD56⁺ hematologic malignancies were prospectively encountered in the routine and referral practices of the authors.

Results

Patients 1 and 2 were elderly men exhibiting an acute onset of skin, bone-marrow, and peripheral blood involvement, both dying of their disease within less than 12 months. CD3⁺ phenotype and a clonal T-cell receptor β rearrangement indicated categorization as a CD4⁺ natural killer T-cell lymphoma. Patient 3 developed a CD56⁺ anaplastic large cell lymphoma and is without disease after excision and radiation. Indolent CD4⁺ CD56⁺ poikilodermatous mycosis fungoides defined case 4. There were 7 patients with CD123⁺ CD4⁺ CD56⁺ hematodermic neoplasm, 4 dying within 18 months of presentation with peripheral blood/marrow involvement in 6 of the 7 cases. Two patients with granulocytic sarcoma dying within 100 days of presentation defined the last two cases.

Limitations

There were relatively small numbers in each of the categories and the follow-up was limited in those cases where death was not reported.

Conclusion

Cutaneous malignancies composed of CD4⁺ CD56⁺ hematopoietic cells define a varied group and oftentimes have an aggressive clinical course although not in every case.

Key words: cutaneous, hematologic, malignancy

Abbreviations used: AML, acute myelogenous leukemia, EBV, Epstein-Barr virus, IL, interleukin, MDS, myelodysplastic syndrome, MF, mycosis fungoides, MxA, myxovirus protein, NK, natural killer, TCL1, T-cell leukemia 1 oncogene, TCR, T-cell receptor, UV, ultraviolet