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KMID : 0363220180560010042
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Korean Journal of Dermatology
2018 Volume.56 No. 1 p.42 ~ p.48
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Primary Cutaneous Marginal Zone Lymphoma: A Clinical and Pathologic Study of Korean Patients
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Kang Hyun-Ji
Kim Hak-Tae
Choi Jae-Won
Lee You-Jin
Lee Ye-Jin
Lee Mi-Hye
Lee Woo-Jin
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Abstract
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Background: Primary cutaneous marginal zone lymphoma (PCMZL) reportedly accounts for 5∼15% of all cutaneous lymphomas. The incidence and clinical characteristics of PCMZL vary according to geography, and there have been few reports describing patients from Asian countries.
Objective: The purpose of this study was to assess the clinical and pathologic features of Korean patients with PCMZL and compare them with features in Western patients.
Methods: We retrospectively analyzed 19 cases of PCMZL over 17 years. Patient medical records were reviewed to gather clinical, histologic, and follow-up data.
Results: The mean patient age was 40 (range: 15∼56) years and there was a slight female predominance. More than half of the patients presented with eyelid swelling. In addition to ocular involvement, erythematous papules or nodules on the head and neck occurred in five of nine patients in contrast with previous reports that PCMZL presented predominantly on the trunk and extremities. Immunoglobulin (Ig) M immunophenotyping showed different rates of positivity between ocular and cutaneous lesions (71.4 vs. 33.3%). Treatment strategies included excision, radiotherapy, and chemotherapy, all of which showed excellent long-term prognosis.
Conclusion: Korean patients differed from Western patients in that PCMZL commonly occurred on the head and neck. We identified two types of PCMZL according to Ig expression, and IgM immunohistochemical analysis seemed to help differentiate adnexal marginal zone lymphoma from PCMZL.
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KEYWORD
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Marginal zone B-cell lymphoma, Skin
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