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

¹İstanbul Medipol University Faculty of Medicine, Department of Dermatology, İstanbul, Turkey
²İstanbul Medipol University Faculty of Medicine, Department of Pathology, İstanbul, 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:

Kronik myelomonositer lösemide mikst tip matür plasmositoid dendritik hücreli tümörün spesifik kutanöz tutulumu

¹İstanbul Medipol University Faculty of Medicine, Department of Dermatology, İstanbul, Turkey
²İstanbul Medipol University Faculty of Medicine, Department of Pathology, İstanbul, Turkey

Altmış sekiz yaşında kemik iliği biyopsisi ile tanısı doğrulanmış kronik myelomonositer lösemili (KMML) erkek hasta saçlı deride, gövde ve gluteal bölgelerde 2 yıldır var olan pruritik, endure papüller nedeniyle başvurdu. Ağırlıklı olarak gövde, karın, bel ve bacak bölgelerinde yer alan purpurik, infiltre miliyer papüller ve peteşiler görüldü. Histopatolojik incelemede kıvrımlı, düzensiz şekilli kromatinli atipik monositik hücreler ve eşlik eden histiyositler görüldü. Hasta sonuç olarak KMML’nin matür plazmositoid dendritik hücreli tümörmikst tipinin spesifik deri tutulumu tanısı aldı. İmmünohistokimyasal testlerde CD4 (+), CD56 (+), CD123 (+) matür plazmositoid dendritik hücreler, CD1a (+) belirsiz dendritik hücreler, CD68 (+) monositik hücreler saptandı. Mikst tipmatür plazmositoid dendritik hücreli tümör KMML’nin en iyi prognozlu ve agresif olmayan lezyonların görüldüğü klinikopatolojik alt tipi olarak bilinmektedir. KMML’nin klinikopatolojik alt tiplerinin belirlenmesinin klinisyenlere kemoterapi protokollarında ve sağkalım tahmininde rehber olduğu sonucuna vardık.

Anahtar Kelimeler:

Pelin Üstüner, Ali Balevi, Mustafa Özdemir, Cuyan Demirkesen. Specific cutaneous involvement of a mixed-type mature plasmacytoid dendritic cell tumor in chronic myelomonocytic leukemia. . 2018; 52(2): 70-73

Corresponding Author: Pelin Üstüner, Türkiye