PRIMARY CUTANEOUS MARGINAL ZONE B-CELL LYMPHOMA – PITFALLS IN THE DIAGNOSIS LIMFOMUL CUTANAT PRIMAR CU CELULA B DE ZONĂ MARGINALĂ – CAPCANE ALE DIAGNOSTICULUI

Aurel Doru Chiriţă, Irina Margaritescu

Abstract


Primary cutaneous marginal zone B-cell lymphoma represents a low grade B-cell lymphoma that originates in the skin with no evidence of extracutaneous disease at the time of diagnosis. The diagnosis of this type of lymphoma can be very difficult especially when presenting

with unusual features. Clinically, the lesions may simulate inflammatory processes or benign conditions. Histologically, the reactive T-cell infiltrate can be so abundant that the true neoplastic B-cell infiltrate is overlooked. Hence, integration of all clinical, histological, immunohistochemical and molecular data is critical in establishing the correct diagnosis and initiation of appropriate treatment. We present the case of a primary cutaneous marginal zone B-cell lymphoma with multifocal lesions, spontaneous resolution and formation of anetodermic scars that was misinterpreted as lupus erythematosus tumidus, Jessner-Kanof infiltrate, or benign lymphoid hyperplasia for 7 years. We emphasize the difficulties encountered in the diagnosis of this particular type of cutaneous lymphoma.

Limfomul cutanat primar cu celula B de zonă marginală reprezintă un limfom cu celule B de grad jos care apare la nivelul pielii, fără să existe afectare extracutanată la momentul diagnosticului. Diagnosticul acestui tip de limfom poate fi foarte dificil, în special atunci când se prezintă cu anumite trăsături neobişnuite. Clinic, leziunile pot simula un proces inflamator sau o afecțiune benignă. Histologic, infiltratul reactiv cu celule T poate fi atât de abundent încât adevăratul infiltrat neoplazic cu celule B poate fi trecut cu vederea. De aceea, integrarea tuturor datelor clinice, histologice, imunohistochimice şi moleculare este esenţială pentru stabilirea unui diagnostic corect şi pentru iniţierea unui tratament adecvat. Prezentăm cazul unui limfom cutanat primar cu celula B de zona marginala cu leziuni multifocale, rezoluţie spontană şi formarea de cicatrici anetodermice şi care a fost greşit interpretat timp de

7 ani ca lupus eritematos tumidus, infiltrat Jessner-Kanof sau hiperplazie limfoidă benignă. Subliniem dificultăţile întâlnite în diagnosticul acestui tip de limfom cutanat.



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