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Journal of the American Academy of Dermatology
Volume 63, n° 2
pages 292-308 (août 2010)
Doi : 10.1016/j.jaad.2009.08.044
Dermatopathology

Cutaneous CD4+ CD56+ hematologic malignancies
 

Cynthia M. Magro, MD a, , Pierluigi Porcu, MD c, Jochen Schaefer, MD a, Jack W. Erter, MD c, Richard R. Furman, MD b, Paul K. Shitabata, MD d, A. Neil Crowson, MD e
a Department of Pathology, Weill Medical College of Cornell University, New York, New York 
b Division of Hematology and Oncology, Weill Medical College of Cornell University, New York, New York 
c Division of Hematology and Oncology, Ohio State University, Columbus, Ohio 
d Pathology Inc, Torrance, California 
e Regional Medical Laboratories, St John Medical Center, University of Oklahoma, Tulsa, Oklahoma 

Reprint requests: Cynthia M. Magro, MD, Department of Pathology, Weill Cornell Medical Center, 1300 York Ave, F-309A, New York, NY 10044.
Abstract
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.

The full text of this article is available in PDF format.

Key words : cutaneous, hematologic, malignancy

Abbreviations used : AML, EBV, IL, MDS, MF, MxA, NK, TCL1, TCR, UV



 Funding sources: None.
 Conflicts of interest: None declared.



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