Cutaneous CD4+ CD56+ hematologic malignancies

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.