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Review
. 2023 Jan 15;62(2):243-249.
doi: 10.2169/internalmedicine.9743-22. Epub 2022 Jun 14.

Concurrent de novo Thymoma-associated Paraneoplastic Type 1 Autoimmune Hepatitis and Pure Red Cell Aplasia after Thymectomy: A Case Report and Literature Review

Rie Sakata  1   2 , Po-Sung Chu  1 , Miho Kawaida  3 , Katsura Emoto  3 , Masatoshi Sakurai  4 , Risa Nishida  5 , Keisuke Asakura  5 , Rei Morikawa  1 , Nobuhito Taniki  1 , Keisuke Kataoka  4 , Takanori Kanai  1 , Nobuhiro Nakamoto  1
Affiliations
Review

Concurrent de novo Thymoma-associated Paraneoplastic Type 1 Autoimmune Hepatitis and Pure Red Cell Aplasia after Thymectomy: A Case Report and Literature Review

Rie Sakata et al. Intern Med. .

Abstract

The precise manipulation of immune tolerance is the holy grail of immunotherapies for both autoimmunity and cancer immunity. Thymomas are well known to be associated with autoimmune diseases. The exact mechanism by which autoreactivity is induced after thymectomy remains to be elucidated. We herein present the case of a 50-year-old lady with concurrent de novo type 1 autoimmune hepatitis (AIH) and pure red cell aplasia (PRCA), 1 month after undergoing a successful total thymectomy for combined squamous cell carcinoma and thymoma (Masaoka stage II). Corticosteroids yielded short-term effects for both AIH and PRCA. Literature on thymoma-associated AIH, an extremely rare immune-related comorbidity, was also reviewed.

Keywords: autoimmunity; immune tolerance; immunosuppression; thymic epithelial tumors.

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Conflict of interest statement

The authors state that they have no Conflict of Interest (COI).

Figures

Figure 1.
Figure 1.
Histopathology of the surgically resected thymic tumor. (A, B) A thymoma component including TdT+ immature T cells and (C) a component of squamous cell carcinoma are observed. A and C: Hematoxylin and Eosin staining, B: Immunohistochemistry for TdT. A scale bar equals to 200 μm. TdT: terminal deoxynucleotidyl transferase
Figure 2.
Figure 2.
Histopathology of a bone marrow biopsy. (A, B) A hypocellular marrow with the cellularity of 5-10%. (C) Only a few glycophorin A-stained erythroblastic nucleated cells are observed. A, B: Hematoxylin and Eosin staining, C: Immunohistochemistry for glycophorin A. A scale bar equals to 200 μm.
Figure 3.
Figure 3.
Histopathology of a liver biopsy. (A, B) Liver tissue shows the characteristic of mixed lymphoplasmacytic infiltrate across the portal area and parenchyma. (C, D) Most of the infiltrated CD3+ T lymphocytes are CD8 positive. A, B: Hematoxylin and Eosin staining. C, D: Immunohistochemistry for CD3 and CD8, respectively. A scale bar equals to 200 μm.
Figure 4.
Figure 4.
Summary of the clinical course after thymectomy. Hb: hemoglobin, T-Bil: total bilirubin, ALT: alanine transaminase, Ret No.: reticulocyte number, RBC: red blood cell, BMBx: bone-marrow biopsy, LBx: liver biopsy, PSL: prednisolone
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References

    1. Mack CL, Adams D, Assis DN, et al. . Diagnosis and management of autoimmune hepatitis in adults and children: 2019 practice guidance and guidelines from the American Association for the Study of Liver Diseases. Hepatology 72: 671-722, 2020. - PubMed
    1. Czaja AJ. Examining pathogenic concepts of autoimmune hepatitis for cues to future investigations and interventions. World J Gastroenterol 25: 6579-6606, 2019. - PMC - PubMed
    1. Terziroli Beretta-Piccoli B, Mieli-Vergani G, Vergani D. Autoimmmune hepatitis. Cell Mol Immunol 19: 158-176, 2022. - PMC - PubMed
    1. European Association for the Study of the Liver. EASL Clinical Practice Guidelines: autoimmune hepatitis. J Hepatol 63: 971-1004, 2015. - PubMed
    1. Bluestone JA, Anderson M. Tolerance in the age of immunotherapy. N Engl J Med 383: 1156-1166, 2020. - PMC - PubMed