Primary cutaneous B-cell lymphomas: clinical and molecular analysis of the Russian Group for the Study of Cutaneous Lymphomas

Introduction. Primary cutaneous B-cell lymphomas (PCBCL) are a rare group of lymphoproliferative diseases with diverse clinical manifestations, differential diagnostic algorithms, treatment options, and prognoses.

Aim: to characterize patients with PCBCL according to clinical, pathomorphological and cytogenetic parameters.

Materials and methods. Data from 57 patients with PCBCL, who had been observed and/or treated between January 2011 and December 2023, were retrospectively analyzed and included in the study conducted by the Russian Group for the Study of Cutaneous Lymphomas.

Results. The most common subtype was primary cutaneous diffuse large B-cell lymphoma (PC-DLBCL) (40 %), while primary cutaneous marginal zone lymphoma (PC-MZL) and primary cutaneous follicle center lymphoma (PC-FCL) accounted for 32 % and 28 %, respectively. The long-term overall survival rate of PCBCL was 90 % in the entire group and indicates a favorable prognosis for PCBCL. At the time of diagnosis verifi cation, 42 out of 55 patients had indications for therapy (33 % in indolent cases, 100 % in PCDLBCL). Of these, 89 % had a complete response after the fi rst line (PCFCL — 93 %, PCMZL — 94 %, PCDLBCL — 78 %). For patients with PCDLBCL, effi cacy was demonstrated when using systemic chemotherapy according to the mNHL BFM-90 protocol: 5-year disease-free survival (DFS) and event-free survival (EFS) were 83 % and 76 %, respectively. For PCFCL and PCMZL, effi cacy was demonstrated with surgical resection and radiation therapy (5-year DFS and EFS were 71 % and 67 %, respectively). The analysis of the identifi cation of factors of the unfavorable course of the disease revealed an increase in the risk of recurrence/ progression by 40 % with lower limb damage in all PCBCL variants.

Conclusion. For PCFCL and PCMZL, radiation therapy demonstrated the highest therapeutic effi cacy. In patients with PCDLBCL, various chemotherapy regimens are indicated at the stage of diagnosis verifi cation, with high-dose chemotherapy being the most effective. Localization of the pathological process in the lower limbs is associated with an increased risk of relapse across all PCBCL subtypes.

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