Mycosis Fungoides Associated With Lesions in the Spectrum of Primary Cutaneous CD30+ Lymphoproliferative Disorders: The Same Process or 3 Coexisting Lymphomas?

Abstract

Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma, primary cutaneous CD30+ lymphoproliferative disorders (pc CD30+ LPD) being the second most prevalent. There is evidence that MF and pc CD30+ LPD may coexist and share T-cell clonality, suggesting a common origin. These findings were supported by a T-cell receptor clonality assessment by the polymerase chain reaction coupled with capillary electrophoresis, although results produced by this method may be ambiguous. We describe an otherwise healthy 46-year-old man who developed, over the course of 5 months, a tumor consisting of primary cutaneous anaplastic large cell lymphoma and, subsequently, several papules of lymphomatoid papulosis (LyP). Both lymphomas appeared on a single patch of MF, which had been present on the patient's right buttock for at least 2 years. T-cell receptor clonality of the 3 types of neoplastic lesions and apparently non-involved skin were assessed by a next-generation sequencing-based method. We found that MF, primary cutaneous anaplastic large cell lymphoma and LyP harbored the same top 2 clones. Non-involved skin harbored other T-cell clones. In this patient, these findings suggest that MF, LyP and pc CD30+ LPD were different clinicopathological manifestations arising from the neoplastic proliferation of the same T-cell clone.

Anaplastic Large Cell Transformation of Mycosis Fungoides: Case Report and Review of the Literature

“…subsequent development of pcCD30 + LPD with anaplastic-like or HRS-like morphology where both initial and transformed lesions shared the same clone [15][16][17][18][19][20][21][22][23] (Table 1). Additional genomic studies such as ALK and DUSP22 rearrangements, chromosome analysis, or NGS were performed in some cases, which supported the nature of transformation.…”

“…15,16,19 Histologically, the transformed lesions showed sheets of large cells with variable morphology including anaplastic, 19 atypical blastic, 16 histiocytic, 22 large pleomorphic, 23 or appeared as a medium-sized atypical lymphocytic infiltrate. 17 Anaplastic cells resembling Hodgkin cells, such as HRS-like, lacunar, or histiocytic-like cells, have been described in more than half of the cases. Immunophenotypically, CD30 was universally positive in all reported cases.…”

“…Transformed MF usually has a more accelerated progression and requires systemic therapy in selected patients, whereas most cases of pcALCL have indolent behavior and rarely require systemic treatment. Of the 9 reported cases in the clonally related group, 2 patients had a rapidly progressive clinical course and died due to the complications of lymphoma, 18,19 5 had stable disease courses, 16,17,[20][21][22] and 2 had no follow-up. 15,23 Because the number of reported cases was small and follow-up was only mentioned in some of the reports, these results may not draw a reliable conclusion on the clinical behavior of these patients.…”

“…However, transformed MF with pure anaplastic morphology and phenotype of ALCL is rare. A search of English literature with keywords of “ALCL,” “anaplastic cells,” “CD30-positive large T-cell lymphoma,” “HRS-like large cell T-cell lymphoma” identified 9 cases of MF with subsequent development of pcCD30 + LPD with anaplastic-like or HRS-like morphology where both initial and transformed lesions shared the same clone 15–23 (Table 1). Additional genomic studies such as ALK and DUSP22 rearrangements, chromosome analysis, or NGS were performed in some cases, which supported the nature of transformation.…”

Linfoma cutáneo primario anaplásico de células grandes CD30+ relacionado con micosis fungoide. Informe de un caso

“…Se diferencia del LCPACG porque son lesiones diseminadas que comprometen varios segmentos corporales y consisten en múltiples pápulas eritematosas, algunas veces hemorrágicas o necróticas, característicamente polimórficas, es decir, en diferentes estadios de desarrollo. Se autolimitan en tres a 12 semanas y a menudo dejan al resolverse una lesión cicatrizal superficial varioliforme 8 . De acuerdo con la clasificación actualizada en 2018 de la WHO-EORTC³ se distinguen seis tipos de PL: -Tipos A (80%) y C: se superponen al LCPACG, con células atípicas de gran tamaño, anaplásicas, de núcleo irregular, nucleolo prominente, y citoplasma eosinófilo y abundante.…”