Cutaneous Lymphoid Hyperplasia and Cutaneous Marginal Zone Lymphoma

Abstract

Cutaneous marginal zone lymphoma (MZL) is a recently described low-grade B-cell lymphoma that usually follows an indolent course. This tumor shares many histologic and clinical features with cutaneous lymphoid hyperplasia (CLH), a benign reactive lymphoid proliferation. Sixteen biopsy specimens from 14 patients with CLH were studied, and compared with 16 cases of cutaneous MZL (9 primary cutaneous, 7 with secondary involvement of the skin) to determine whether there were features that would permit their distinction on routinely fixed, paraffin-embedded tissue sections. Both disorders showed a female preponderance (CLH: 9 F, 5 M; MZL: 11 F, 5 M). The median age was also similar (CLH: 54 years; cutaneous MZL: 55 years). CLH was most common on the arm (8) and the head and neck (7) but also involved the trunk (1); primary cutaneous MZL most often involved the limbs (3), trunk (3), and head and neck (3). Lymphoma did not develop in any of the 14 CLH patients (follow-up ranging from 9 to 246 months, mean 62 months). Six of 9 patients with primary cutaneous MZL and all 7 patients with secondary cutaneous MZL experienced relapses, most commonly isolated to skin or a subcutaneous site. On hematoxylin-eosin stained sections, a diffuse proliferation of marginal zone cells (p < 0.0001), zones of plasma cells (p = 0.01), the absence of epidermal change (p = 0.01), reactive germinal centers (p = 0.03), and a diffuse pattern of dermal or subcutaneous infiltration (p = 0.03) were more often seen in cutaneous MZL. A dense lymphocytic infiltrate, bottom-heavy or top-heavy growth pattern, eosinophils, and a grenz zone were seen equally often in both disorders. Dutcher bodies were observed only in cutaneous MZL. Immunoperoxidase stains on formalin-fixed paraffin-embedded tissue sections showed monotypic expression of immunoglobulin light chains by plasma cells in 11 of 16 MZL cases. By definition, no case with monotypic plasma cells was diagnosed as CLH. In CLH, T cells usually outnumbered B cells, and a B:T cell ratio > or = 3:1 was not observed in any case. By contrast, 40% of the MZL cases showed a B:T cell ratio > or = 3:1. No coexpression of CD20 and CD43 was seen in any case of either MZL or CLH. In summary, the clinical presentations of CLH and MZL are similar. In contrast to historical criteria for diagnosing cutaneous lymphoid infiltrates, the presence of reactive follicles favors a diagnosis of cutaneous B-cell lymphoma (CBCL). In addition, a bottom-heavy or top-heavy growth pattern is not a distinctive finding. Marginal zone cells and zones or sheets of plasma cells are strong morphologic indicators of marginal zone lymphoma. The diagnosis of CBCL can be supported in 40% of the cases by demonstrating a B:T cell ratio of > or = 3:1, and confirmed in 70% of the cases by demonstrating monotypic light chain expression of plasma cells on paraffin sections.

Pseudolymphoma of the liver associated with primary biliary cirrhosis: A case report and review of literature

“…It is usually localized and well demarcated from surrounding tissue [1] . Pseudolymphoma may occur at numerous sites including the stomach [7] , lung [8] , ocular adnexa [9] , hard palate and oral mucosa [10] , skin [11] , and breast [12] . To the best of our knowledge, 32 cases (including ours) of hepatic pseudolymphoma have been reported in the English and Japanese literature (Table 1).…”

“…All seven reported cases of breast pseudolymphoma were female [12] , but breast examination is limited almost exclusively to females, which could bias these findings. Reported male-female ratios of pseudolymphoma of the hard palate and oral mucosa [10] , lung [8] , and skin [11] were 1:2.8, 1:1.2 and 1:1.8, respectively, while ocular adnexal lymphoid hyperplasia affects men and women about equally [9] . Thus the extreme female asymmetry of hepatic pseudolymphoma is a unique characteristic.…”

A review of 55 cases of cutaneous lymphoid hyperplasia: reassessment of the histopathologic findings leading to reclassification of 4 lesions as cutaneous marginal zone lymphoma and 19 as pseudolymphomatous folliculitis

“…Although these conditions were formerly known as lymphadenosis benigna cutis, including synonyms such as lymphocytoma cutis, pseudolymphoma of Spiegler-Fendt or cutaneous lymphoplasia, and lymphocytic infiltration of Jessner, the term cutaneous lymphoid hyperplasia (CLH) is currently the recommended term [1,2]. In particular, there are various opinions about whether lymphocytic infiltration of Jessner is a distinct entity [3].…”

“…Recently, it has been suggested that lesions that are really cutaneous marginal zone lymphoma (CMZL) may have been erroneously diagnosed as CLH [1,2,5]. Both CMZL and cutaneous follicular lymphoma are uncommon in the Japanese population [6].…”

Primary cutaneous marginal zone B-cell lymphoma: a molecular and clinicopathological study of cases from Asia, Germany, and the United States

“…In contrast, this finding was almost absent in Germany and the United States cases. Tissue eosinophilia has been reported in 4 of 15 American cases, 23 although the degree of eosinophilia was not specified. In malignant lymphoma, tissue eosinophilia is often associated in Hodgkin's lymphoma and T-cell lymphoma.…”