Acta Dermato-Venereologica 99-12CompleteContent | Page 29

1176 SHORT COMMUNICATION Aggressive CD4 – CD8 – CD45RA + CCR10 – Primary Cutaneous Peripheral T-cell Lymphoma, Not Otherwise Specified: A Case Report Natsumi IKUMI 1 , Hideki FUJITA 2 *, Tadashi TERUI 2 , Hiromichi TAKAHASHI 1,3 *, Katsuhiro MIURA 1 , Yoshihiro HATTA 1 and Masami TAKEI 1 Division of Hematology and Rheumatology, 2 Division of Cutaneous Science, Department of Dermatology, and 3 Division of Laboratory Medicine, Department of Pathology and Microbiology, Nihon University School of Medicine, Tokyo, Japan. *E-mails: fujita.hideki@nihon-u. ac.jp and [email protected] 1 Accepted Sep 10, 2019; E-published Sep 10, 2019 Peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS), is a sub-type of lymphoma composed of all the PTCLs that cannot be characterized into any other established sub-types (1). Patients with primary cutaneous PTCL, NOS are commonly adults presenting with solitary, localized or, more frequently, generalized nodules or tu- mours with no site predilection. The prognosis of PTCL, NOS is generally poor, with a median 5-year survival rate of less than 20% (2). The expression pattern of cell surface antigens has been investigated in association with disease outcome in PTCL, NOS (3). In particular, chemokine receptor expression patterns are gaining much attention as prognostic markers. We report here a case of aggressive primary cutaneous PTCL, NOS with CD4 – /CD8 – , CD45RA + , CXCR3 + , and CCR4 – /10 – phenotype showing a very aggressive course. CASE REPORT A 73-year-old Japanese man presented with a 2-month history of generalized plaques and nodules on the skin. At initial visit, physical examination revealed multiple erythematous plaques and reddish nodules on his trunk (Fig. 1) and extremities. His lower legs were swollen, with haemorrhagic ulceration (Fig. S1 1 ). No superficial lymph nodes were enlarged. Laboratory investigations indicated a normal lactate dehydroge- nase level, low serum albumin level, and mild polymorphonuclear https://www.medicaljournals.se/acta/content/abstract/10.2340/00015555-3310 1 Fig. 1. Clinical photographs at initial visit. Indurated plaques, erythema and tumours on the trunk. leukocytosis with no peripheral atypical cells. Anti-human T-cell leukaemia virus type-1 antibody was negative. Skin biopsy specimen obtained from a plaque on the abdomen showed diffuse and dense infiltrate of small-sized atypical lym­ phoid cells in the dermis without epidermotropism (Fig. 2a, b). Immunohistochemical study revealed that the atypical lympho­ cytes were CD3 + CD4 – CD5 + CD8 – CD56 – CD45RA + CD45RO – TCRαβ + TCRγδ – EBER – granzyme B – (Fig. 2c–h and Fig. S2a–f 1 ). The expression profile of chemokine receptors was CXCR3 + CCR4 – CCR10 – (Fig. 2i–k). Approximately 80% of the tumour cells expressed Ki-67 (Fig. S2g 1 ). PCR and Southern blotting analyses revealed clonal T-cell re- ceptor Cβ rearrangement in the biopsy specimen. The karyotype of the biopsy sample was examined using G-banding method, and there were many unspecific complicated chromosomal aberrancies. Fig. 2. Histopathology and immunohistochemistry of the biopsy specimen from the erythematous plaque on the abdomen. (a) Dense lymphocytic infiltration in the whole dermis and subcutaneous fat (haematoxylin and eosin; H&E ×40). (b) Small atypical cells did not show epidermotropism (H&E ×400). Immunohistochemical staining for: (c) CD3, (d) CD4, (e) CD8, (f) CD56, (g) CD45RA, (h) TCRαβ, (i) CXCR3, (j) CCR4, and (k) CCR10 (×200). doi: 10.2340/00015555-3310 Acta Derm Venereol 2019; 99: 1176–1177 This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta Journal Compilation © 2019 Acta Dermato-Venereologica.