CD8-Positive Primary Cutaneous Anaplastic Large T-Cell Lymphoma (PCALCL): Case Report and Review of This Unusual Variant of PCALCL

Abstract

Primary cutaneous anaplastic large T-cell lymphoma (PCALCL) is a well-defined CD30-positive lymphoproliferative disorder with relatively good prognosis and response to treatment. We describe a case of PCALCL expressing CD8. The patient is a 57-year-old man that clinically presented with an ulcerated nodule in his left middle finger. Histopathologic sections showed an ulcerated epidermis with a diffuse lymphocytic infiltrate in the superficial dermis with focal epidermotropism. The large cohesive atypical cells were admixed with a reactive infiltrate composed of neutrophils, eosinophils, and small lymphocytes. Immunohistochemical studies showed the tumor cells to be strongly positive for CD8, CD30, and TIA-1, focally positive for CD3, and negative for CD4, CD20, CD56, Anaplastic Lymphoma Kinase (ALK-1), and HSV. Epstein-Barr virus by in situ hybridization was negative. The diagnosis of a CD8+ PCALCL was confirmed. There is limited precedent literature regarding CD8-positive PCALCL and this case falls within the clinical and histopathologic spectrum of CD30+ lymphoproliferative disorders. CD8/CD30 coexpression is rare in PCALCL and may have important clinical and prognostic implications. To the best of our knowledge there are only 4 previously reported cases describing similar findings.

Characterization of Primary Cutaneous CD8+/CD30+ Lymphoproliferative Disorders

“…The large cell portion comprised more than 50% of the infiltrate in both CD8 + LyP (9 of 15 cases) and PCALCL; however, it was more often found in cases of CD8 + PCALCL (11 of 11 cases, P = 0.0338). More cases of CD8 + PCALCL had 19 3 ALCL Cardoso et al 8 1 Lyp De Souza et al 9 11 LyP Fukunaga et al 20 1 ALCL Gelfand et al 21 1 ALCL Hellman et al 10 1 LyP Kadin et al 11 1 LyP Kikuchi et al 22 2 ALCL Magro et al 12 4 LyP Massone and Cerroni 23 6 ALCL McQuitty et al 14 26 8 ALCL, 18 LyP Plaza et al 13 20 8 ALCL, 12 LyP Plaza et al 25 1 ALCL Saggini et al 15 9 LyP Shimizu et al 26 1 ALCL Sim and Kim 16 1 LyP Wen et al 17 1 LyP Wu and Tsai 18 1 LyP Xu et al 27 There were no differences with regard to ulceration (P = 0.8728), spongiosis (P = 0.8578), parakeratosis (P = 0.5342), interface change (P = 0.4083), or pseudoepitheliomatous hyperplasia (P = 0.2143). Pseudoepitheliomatous hyperplasia was not a common feature in either CD8 + LyP or PCALCL.…”

“…[5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][25][26][27] Clinical characteristics are shown in Table 3. Patients with CD8 + LyP were significantly younger than patients with PCALCL (mean = 39.14 vs. 54.12 years, P = 0.0013).…”

Cutaneous CD30‐positive lymphoproliferative disorders with CD8 expression: a clinicopathologic study of 21 cases

“…Other reported cases suggest features clinically indistinguishable from conventional examples of LyP and cutaneous ALCL. 9,17 Whether one is dealing with borderline CD30+ LPD or overt ALCL, it is more typical for these cases to show a monomorphic infiltrate in regards to both the background inflammatory cell infiltrate as well as the nature of the neoplastic cell populace. In particular, several biopsies showed a dearth of eosinophils and neutrophils while granulomatous inflammation was not uncommon.…”

Comments on Cutaneous Lymphomas

“…37 By definition, more than 75% of the tumor cells have to express CD30. 39 Primary cutaneous ALCL lack the expression of anaplastic lymphoma kinase (ALK) and the t (2;5) translocation. CD8+ cases have been described.…”