CD30+ Lymphoproliferative Disorders: An Expanding and Versatile Group of Lymphomas

Angelica Selim, MD

Distinction of intralymphatic from blood vascular location may impart important prognostic and therapeutic information.

CD30+ T-cell lymphoproliferative disorders (TLPDs) include anaplastic large cell lymphoma (ALCL) and lymphomatoid papulosis (LyP). Because their morphologies overlap, some cases need follow-up to properly subclassify the tumors. Recently, CD30+ TLPDs with intravascular involvement have been described. These authors retrospectively studied 18 cases of cutaneous intravascular large cell lymphoproliferations.

They included nine CD30+ TLPDs (5 ALK− ALCLs), one ALK+ ALCL, six B-cell lymphomas, and two benign microscopic intravascular T-cell proliferations. Twelve were lymphoproliferative. Median patient age was 70, and lesion location varied. The ALK− ALCLs presented as plaques, and the ALK+ ALCL as peau-d'orange.

Of the nine patients with ALK− CD30+ TLPD, three had cutaneous relapses at last follow-up, three were disease free, one had unknown status, one had systemic disease, and one had an unrelated death. The ALK+ ALCL patient had multiple systemic relapses after chemotherapy. Most patients with large B-cell lymphomas had systemic disease or related death.

D2-40 staining highlighted lymphatic spaces filled with CD30+ TLPDs, which was not seen in the large B-cell lymphomas. Rearrangement of the DUSP22-IRF4 locus was noted in the 5 CD30+ TLPDs. The characteristic histologic presentation in all cases was of large cells in vascular spaces. In addition, one of five intravascular large B-cell lymphomas showed an extravascular component; while the ALK+ ALCL and four of the CD30+ TLPDs had a small T-cell perivascular infiltrate.

Citation(s):

Samols MA et al. Intralymphatic cutaneous anaplastic large cell lymphoma/lymphomatoid papulosis: Expanding the spectrum of CD30-positive lymphoproliferative disorders. Am J Surg Pathol 2014 Sep; 38:1203.