Journal
HISTOPATHOLOGY
Volume 66, Issue 6, Pages 846-855Publisher
WILEY-BLACKWELL
DOI: 10.1111/his.12529
Keywords
6p25.3 rearrangement; biphasic pattern; DUSP22-IRF4 translocation; lymphomatoid papulosis (LyP); primary cutaneous anaplastic large cell lymphoma (C-ALCL)
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Funding
- Asociacion Espanola Contra el Cancer (AECC)
- MINECO [SAF2013-47416-R]
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Aims CD30-positive primary cutaneous lymphoproliferative disorders include several entities with differing clinical presentation but overlapping histological features, including lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma (C-ALCL). DUSP22-IRF4 locus translocation is present in 20-57% of C-ALCLs, and has also been described in a series of 11 lymphomatoid papulosis patients, where it was associated with a particular biphasic histological pattern, including pagetoid reticulosis-type epidermal infiltration. We aimed to study whether the presence of this translocation may define distinctive histological features in C-ALCL. Methods and results We collected three cases of C-ALCL with histological features similar to those described in the new variant of lymphomatoid papulosis with 6p25.3 rearrangement. We studied their histological features and immunophenotype, using a panel of antibodies against CD30, TCR-F1, TCR-, CD4, CD8, CD20, Ki-67 and ALK. FISH analyses were performed using an IRF4-DUSP22 break-apart probe for the study of the 6p25.3 rearrangement. FISH results were positive in the three cases, which all showed distinctive histological and immunohistochemical features: a diffuse dermal infiltrate of atypical medium-to-large cells, and marked epidermotrophism with small, atypical intra-epidermal lymphocytes. Conclusions Our findings suggest that the presence of 6p25.3 rearrangement might be related to this particular biphasic pattern.
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