Authors: M. Pevná 1; J. Kissová 2; M. Doubek 1; Z. Adam 1; M. Klabusay 1 Authors‘ workplace: Interní hematoonkologická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MU Dr. Jiří Mayer, CSc. 1; Oddělení klinické hematologie FN Brno, pracoviště Bohunice, přednosta prof. MU Dr. Miroslav Penka, CSc. 2 Published in: Vnitř Lék 2010; 56(Supplementum 2): 183-187 Category: Langerhans cell histiocytosis and some other Hematology rare diseases
CD4+CD56+ malignancies are rare hematological tumours with poor prognosis affecting primarily the skin; despite good initial response to chemotherapy, they result in early relapse and rapid progression and dissemination of the disease into the bone marrow, peripheral blood and lymphatic nodes. Even though the origin of tumorous cells was initially being associated with NK cells because of the CD56 expression, recent studies suggest the disease is derived from precursor plasmocytoid dendritic cells. It is the co - expression of CD4 and CD56 and an absence of line - specific markers that defines this new entity within the last WHO - EORCT classification for cutaneous lymphomas. Patients with this immunophenotype have common clinical features and morphological findings. The specific genetic anomaly is not known. Immunohistochemical and flow cytometric analyses have an exclusive place in the diagnostics. We present two cases of CD4+CD56+ malignancies with different clinical course. In an 18 years old female, the disease presented as an acute leukemia without the typical cutaneous lesions, was chemoresistant and the patient died 12 months following diagnosis for relapse of the disease after allogeneic hematopoietic stem cell transplantation. In a 64 years old male, the disease manifested as cutaneous lymphoma only. Chemotherapy resulted in an 8 months lasting first complete remission. Treatment of the first relapse with disease dissemination resulted in a short-term 2nd complete remission. The second relapse followed and the patient died 2 years following diagnosis.
Key words: CD4+CD56+ haematodermic neoplasm – DC2 cells – DC2 malignancy
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