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Review
. 2020 Jul;39(7):2227-2234.
doi: 10.1007/s10067-020-05061-z. Epub 2020 Apr 10.

A tale of autoimmunity: thymoma, thymectomy, and systemic lupus erythematosus

Arash Mollaeian  1 , Christopher Haas  2   3
Affiliations
Review

A tale of autoimmunity: thymoma, thymectomy, and systemic lupus erythematosus

Arash Mollaeian et al. Clin Rheumatol. 2020 Jul.

Abstract

The thymus plays an integral role in immune system regulation, modulating the development, diversity, and selection of T lymphocytes, a critical feature for the prevention of T cell-mediated autoimmunity. Thymoma is a rare tumor of the thymus. Autoimmune diseases and paraneoplastic syndromes such as myasthenia gravis, pure red blood cell aplasia, and systemic lupus erythematosus, although relatively uncommon, have been described in association with thymomas. Rare cases of post-thymectomy autoimmune related diseases, including systemic lupus erythematosus and pure red cell aplasia, have been reported in the literature. Here, we present the case of a 65-year-old male who developed systemic lupus erythematosus 2 years after thymectomy in the setting of thymoma-associated pure red cell aplasia.

Keywords: Autoimmunity; SLE; Thymectomy; Thymoma; Thymus.

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Figures

Fig. 1
Fig. 1
a Chest X-ray before thymectomy and thymomectomy. b, c Chest CT scan: right-sided, anterior mediastinal well-circumscribed mass without evidence of gross infiltration or necrotic, cystic, or calcified components
Fig. 2
Fig. 2
Thymoma biopsy. a H&E stained slide at × 20; cellular nodular well-circumscribed tumor with fibrous bands (lower right) and a cellular nodule of tumor. b H&E stained slide at × 40; well-circumscribed nodule of tumor with fibrous band (upper left) and tumor nodule (lower right). c H&E stained slide at × 100; type AB thymomas have two microscopic components; there is a homogeneous population of neoplastic spindle cells without nuclear atypia (right mid to lower) and scattered foci which are rich in lymphocytes (seen predominantly in left lower and mid portion of image). d H&E stained slide at × 400; higher power image with neoplastic spindle cells admixed with lymphocyte rich foci
Fig. 3
Fig. 3
Bone marrow biopsy. a, b Hypocellular marrow (20–30%) with difficult to identify erythroid precursors and a myeloid-erythroid ratio of greater than 10:1. Vast majority of cells are in the myeloid series with many mature neutrophils. Scattered megakaryocytes are present
See this image and copyright information in PMC

Comment in

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