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Class-switched Primary Cutaneous Marginal Zone Lymphomas Are Frequently IgG4-positive and Have Features Distinct From IgM-positive Cases

Publication ,  Journal Article
Carlsen, ED; Swerdlow, SH; Cook, JR; Gibson, SE
Published in: American Journal of Surgical Pathology
October 2019

Primary cutaneous marginal zone lymphoma (PCMZL) can be subdivided into 2 groups based on immunoglobulin (Ig) heavy chain usage: IgM-positive cases that constitute a less common and more T-helper type 1–driven process, and more common heavy chain class-switched cases that are predominantly T-helper type 2–driven. Although some report a significant IgG4-positive subset, others have found a much smaller proportion. To further evaluate the proportion of IgG4-positive PCMZL, to address whether IgG4-positive cases have any distinctive characteristics, and to assess whether additional features separating IgM-positive and class-switched cases could be identified, the clinicopathologic features of 26 PCMZL obtained from 19 patients were investigated. Twenty of 26 (77%) PCMZL were heavy chain class-switched (19 IgG-positive, 1 IgA-positive), including 9 that were IgG4-positive (35%). IgG4-positive and other class-switched PCMZL were morphologically similar. IgM-positive cases occurred in older individuals (median: 69 vs. 46.5 y; =0.0001), more often involved the subcutis (=0.002), demonstrated plasma cells diffusely scattered versus at the periphery of the lymphoid infiltrate (=0.005), uniformly showed follicular colonization (=0.0001), contained more numerous B cells (=0.0004), and were more likely to have a T-cell CD4:CD8 ratio of <3:1 (=0.03). None of the IgM-positive PCMZL harbored a L265P mutation. No significant differences in clinical outcome were documented. These results highlight the high frequency of IgG4-positive PCMZL, which are otherwise similar to other class-switched cases, provide additional evidence supporting the distinction between class-switched and IgM-positive cases, and emphasize the indolent nature of at least the class-switched PCMZL, which may warrant their categorization as a clonal chronic lymphoproliferative disorder.

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Published In

American Journal of Surgical Pathology

DOI

ISSN

0147-5185

Publication Date

October 2019

Volume

43

Issue

10

Start / End Page

1403 / 1412

Publisher

Ovid Technologies (Wolters Kluwer Health)

Related Subject Headings

  • Pathology
  • 1103 Clinical Sciences
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Carlsen, E. D., Swerdlow, S. H., Cook, J. R., & Gibson, S. E. (2019). Class-switched Primary Cutaneous Marginal Zone Lymphomas Are Frequently IgG4-positive and Have Features Distinct From IgM-positive Cases. American Journal of Surgical Pathology, 43(10), 1403–1412. https://doi.org/10.1097/pas.0000000000001363
Carlsen, Eric D., Steven H. Swerdlow, James R. Cook, and Sarah E. Gibson. “Class-switched Primary Cutaneous Marginal Zone Lymphomas Are Frequently IgG4-positive and Have Features Distinct From IgM-positive Cases.” American Journal of Surgical Pathology 43, no. 10 (October 2019): 1403–12. https://doi.org/10.1097/pas.0000000000001363.
Carlsen ED, Swerdlow SH, Cook JR, Gibson SE. Class-switched Primary Cutaneous Marginal Zone Lymphomas Are Frequently IgG4-positive and Have Features Distinct From IgM-positive Cases. American Journal of Surgical Pathology. 2019 Oct;43(10):1403–12.
Carlsen, Eric D., et al. “Class-switched Primary Cutaneous Marginal Zone Lymphomas Are Frequently IgG4-positive and Have Features Distinct From IgM-positive Cases.” American Journal of Surgical Pathology, vol. 43, no. 10, Ovid Technologies (Wolters Kluwer Health), Oct. 2019, pp. 1403–12. Crossref, doi:10.1097/pas.0000000000001363.
Carlsen ED, Swerdlow SH, Cook JR, Gibson SE. Class-switched Primary Cutaneous Marginal Zone Lymphomas Are Frequently IgG4-positive and Have Features Distinct From IgM-positive Cases. American Journal of Surgical Pathology. Ovid Technologies (Wolters Kluwer Health); 2019 Oct;43(10):1403–1412.

Published In

American Journal of Surgical Pathology

DOI

ISSN

0147-5185

Publication Date

October 2019

Volume

43

Issue

10

Start / End Page

1403 / 1412

Publisher

Ovid Technologies (Wolters Kluwer Health)

Related Subject Headings

  • Pathology
  • 1103 Clinical Sciences