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首页> 外文期刊>Journal of cutaneous pathology >Primary cutaneous marginal zone lymphoma with sequential development of nodal marginal zone lymphoma in a patient with selective immunoglobulin A deficiency
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Primary cutaneous marginal zone lymphoma with sequential development of nodal marginal zone lymphoma in a patient with selective immunoglobulin A deficiency

机译：选择性免疫球蛋白A缺乏症患者原发性皮肤边缘区淋巴瘤伴结节性边缘区淋巴瘤继发

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摘要

Multiple lymphoma subtypes occurring within one patient is rare in the context of B-cell lymphoma, and only few such cases have been reported in association with primary cutaneous marginal zone lymphoma (PCMZL). We herein describe the case of a 43-year-old patient who was diagnosed with PCMZL and subsequently developed a clonally unrelated nodal marginal zone lymphoma (MZL). At the time of diagnosis of PCMZL, multiple skin lesions were present. The atypical lymphoid infiltrate showed monotypic expression of immunoglobulin light chain lambda and heavy chain (IgM) on immunohistochemistry and an identical B-cell clone. No sign of systemic lymphoma was present in staging examinations. Complete remission was achieved utilizing rituximab. After a 3-year clinical course of repetitive cutaneous relapses and remissions, the patient additionally developed nodal lymphoma involvement by MZL which, however, harbored an immunophenotype and a genetic clone distinct from the cutaneous lymphoma counterpart. Therefore, the rare occurrence of two different types of MZL with sequential evolution was diagnosed. In this uncommon case, we hypothesize that selective immunoglobulin A deficiency may play a promoting role for the metachronous development of the two MZL that occurred in our patient.
机译：在B细胞淋巴瘤的情况下，在一名患者中发生多种淋巴瘤亚型的情况很罕见，并且仅报道了少数与原发性皮肤边缘区淋巴瘤（PCMZL）相关的病例。我们在本文中描述了一名43岁的患者，该患者被诊断患有PCMZL，随后发展为无支配性结节性边缘区淋巴瘤（MZL）。在诊断PCMZL时，存在多处皮肤病变。非典型淋巴样浸润在免疫组织化学和相同的B细胞克隆上显示免疫球蛋白轻链λ和重链（IgM）的单型表达。分期检查中未发现全身淋巴瘤的迹象。使用利妥昔单抗可实现完全缓解。经过3年的反复皮肤复发和缓解的临床过程后，患者还发展了MZL累及淋巴结的淋巴瘤，但该淋巴瘤具有与皮肤淋巴瘤对应物不同的免疫表型和基因克隆。因此，诊断出具有顺序进化的两种不同类型的MZL很少见。在这种罕见的病例中，我们假设选择性免疫球蛋白A缺乏可能对我们患者中发生的两个MZL的同时发展起促进作用。

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