Low-Grade Cutaneous B-cell Lymphoma in African American Patients
December 2018 | Volume 17 | Issue 12 | Editorials | 1334 | Copyright © December 2018
Shamir Geller MD, Melissa Pulitzer MD, Patricia L. Myskowski MD, Meenal Kheterpal MD
Memorial Sloan Kettering Cancer Center and Weill Cornell Medicine, New York, NY
Introduction: Cutaneous marginal zone lymphoma (CMZL) and cutaneous follicle center lymphoma (CFCL) are rare indolent cutaneous B-cell lymphomas (CBCL). Their incidence in African American (AA) patients is extremely low. While cutaneous T-cell lymphomas appear to be more aggressive in AA individuals, there is no data on the presentation and course of disease of CBCL in this group. In this study, we aimed to characterize CMZL/CFCL in AA patients.
Methods: A retrospective chart review identified 10 AA patients with CMZL/CFCL. We compared demographics, clinical features, and systemic disease incidence between AA and white patients.
Results: Of 288 patients with CMZL/CFCL, 10 patients were AA (3.5%), and 266 were white. AA patients trended toward diagnosis at a younger age compared to white individuals (median age of 41 vs 54 years; P=0.07). AAs presented with more regional and generalized cutaneous disease (T2-T3 in 70%), while most white patients presented with a solitary lesion (T1 in 55%). Head and neck involvement was more common in AA patients. Extracutaneous systemic disease at initial staging was not significantly different between the groups. One AA patient with primary CMZL developed extracutaneous MZL after16 years. No deaths were reported among AAs.
Discussion: CMZL/ CFCL in this series of AA patients had an earlier age of onset with preferential head and neck involvement and a higher T classification at presentation. Despite these features, systemic involvement was uncommon, and no deaths were recorded. This data supports an indolent course of CMZL and CFCL in the AA population; larger studies are needed to confirm these findings.
J Drugs Dermatol. 2018;17(12):1334-1337.
Cutaneous B-cell lymphomas (CBCLs) are indolent lymphomas presenting in the skin. Primary CBCL is classified according to the WHO/EORTC into three types1: primary cutaneous marginal zone lymphoma (PCMZL), primary cutaneous follicle center lymphoma (PCFCL), and primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT). While PCMZL and PCFCL are generally recognized as indolent diseases with 95% to 99% 5-year survival rate, PCDLBCL-LT has a more aggressive behavior with 5-year survival rates of 50%.2 The incidence of cutaneous T-cell lymphomas (CTCL), specifically of mycosis fungoides (MF), is increased significantly in African Americans (AAs) versus white individuals.3-6 AA patients with CTCL present with an earlier age of onset, and a higher stage at presentation.3 MF in AA patients is associated with disease progression and poorer survival.7-9Primary CBCL is much less common than MF.4 Unlike MF, its incidence in AA patients is significantly lower compared to white patients.3,4,10,11 Given its rarity, there is limited data on presentation and clinical behavior of CBCL in AAs.12 We attempted to characterize the disease presentation and course in this group.
We retrospectively searched the tumor registry and pathology reports database for patients with CMZL/CFCL who were diagnosed and followed at our institution between 1997-2016. We defined CMZL/CFCL as skin lesions with histology either consistent with primary CBCL or secondary cutaneous presentation of systemic B-cell lymphoma. Our inclusion criteria were: (1) CMZL/CFCL histology confirmed by pathologists at MSKCC; (2) skin as initial site of involvement by the lymphoma; (3) staging imaging study [positron emission tomography (PET) scan and/or chest, abdomen, and pelvic computed tomography (CT) scan] completed within 12 months of diagnosis. This study was approved by the institutional review board of Memorial Sloan Kettering Cancer Center.Demographic and clinical data were collected from the medical records including age, sex, race and ethnicity, disease distribution, and clinical T classification (according to the ISCL/EORTC TNM staging system13) initial staging results and follow-up data. Race and ethnicity were reported by the patient or their representative upon admission/registration in accordance with the US Census Bureau’s race and ethnicity categories.All demographic and clinical parameters were compared using