Specific cutaneous involvement of a mixed-type mature plasmacytoid dendritic cell tumor in chronic myelomonocytic leukemia

Specific cutaneous involvement of a mixed-type mature plasmacytoid dendritic cell tumor in chronic myelomonocytic leukemia [Turkderm-Turk Arch Dermatol Venereol]

Turkderm-Turk Arch Dermatol Venereol. 2018; 52(2): 70-73 | DOI: 10.4274/turkderm.25675

Specific cutaneous involvement of a mixed-type mature plasmacytoid dendritic cell tumor in chronic myelomonocytic leukemia

Pelin �st�ner¹, Ali Balevi¹, Mustafa �zdemir¹, Cuyan Demirkesen²
¹�stanbul Medipol University Faculty of Medicine, Department of Dermatology, �stanbul, Turkey
²�stanbul Medipol University Faculty of Medicine, Department of Pathology, �stanbul, Turkey

A 68-year-old man with chronic myelomonocytic leukemia (CMML), proven by bone marrow biopsy, presented to our clinic with pruritic indurated papules on his scalp, trunk and gluteal regions that have been present for two years. Purpuric, infiltrated miliary papules and petechiae were predominantly observed on his trunk, abdomen, back and legs. Histopathological examination revealed atypical monocytic cells with convoluted, irregularly shaped chromatin accompanied by histiocytes. He was finally diagnosed with CMML with specific cutaneous involvement of mixed-type mature plasmacytoid dendritic cell tumor. Immunohistochemical tests showed positive CD4, CD56, and CD123 mature plasmacytoid dendritic cells, positive CD1a indeterminate dendritic cells, and positive CD68 monocytic cells. Mixed-type mature plasmacytoid dendritic cell tumor is known as a clinicopathological subtype of CMML that has the best prognosis, involving non-aggressive lesions. We conclude that identification of clinicopathologic subtypes in CMML will guide clinicians on chemotherapy protocols and estimation of survival.

Keywords: Chronic, dendritic cells, leukemia, myelomonocytic

Kronik myelomonositer l�semide mikst tip mat�r plasmositoid dendritik h�creli t�m�r�n spesifik kutan�z tutulumu

Altm�� sekiz ya��nda kemik ili�i biyopsisi ile tan�s� do�rulanm�� kronik myelomonositer l�semili (KMML) erkek hasta sa�l� deride, g�vde ve gluteal b�lgelerde 2 y�ld�r var olan pruritik, endure pap�ller nedeniyle ba�vurdu. A��rl�kl� olarak g�vde, kar�n, bel ve bacak b�lgelerinde yer alan purpurik, infiltre miliyer pap�ller ve pete�iler g�r�ld�. Histopatolojik incelemede k�vr�ml�, d�zensiz �ekilli kromatinli atipik monositik h�creler ve e�lik eden histiyositler g�r�ld�. Hasta sonu� olarak KMML�nin mat�r plazmositoid dendritik h�creli t�m�rmikst tipinin spesifik deri tutulumu tan�s� ald�. �mm�nohistokimyasal testlerde CD4 (+), CD56 (+), CD123 (+) mat�r plazmositoid dendritik h�creler, CD1a (+) belirsiz dendritik h�creler, CD68 (+) monositik h�creler saptand�. Mikst tipmat�r plazmositoid dendritik h�creli t�m�r KMML�nin en iyi prognozlu ve agresif olmayan lezyonlar�n g�r�ld�� klinikopatolojik alt tipi olarak bilinmektedir. KMML�nin klinikopatolojik alt tiplerinin belirlenmesinin klinisyenlere kemoterapi protokollar�nda ve sa�kal�m tahmininde rehber oldu�u sonucuna vard�k.

Anahtar Kelimeler: Kronik, dendritik h�creler, l�semi, myelomonositik

Pelin �st�ner, Ali Balevi, Mustafa �zdemir, Cuyan Demirkesen. Specific cutaneous involvement of a mixed-type mature plasmacytoid dendritic cell tumor in chronic myelomonocytic leukemia. Turkderm-Turk Arch Dermatol Venereol. 2018; 52(2): 70-73

Corresponding Author: Pelin �st�ner, T�rkiye

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