Splenectomy for Primary Sjögren’s Syndrome Associated with Massive Splenomegaly: Two Case Reports

Fang Gui,* Li-Xuan Zhou,* Xue-Mei Liu, Yong-Long He, Qi-Bin Yang Department of Rheumatology and Immunology, Affiliated Hospital of North Sichuan Medical College, Nanchong, Sichuan, 637000, People’s Republic of China*These authors contributed equally to this workCo...

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Main Authors: Gui F, Zhou LX, Liu XM, He YL, Yang QB
Format: Article
Language:English
Published: Dove Medical Press 2025-08-01
Series:International Medical Case Reports Journal
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Online Access:https://www.dovepress.com/splenectomy-for-primary-sjgrens-syndrome-associated-with-massive-splen-peer-reviewed-fulltext-article-IMCRJ
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author Gui F
Zhou LX
Liu XM
He YL
Yang QB
author_facet Gui F
Zhou LX
Liu XM
He YL
Yang QB
author_sort Gui F
collection DOAJ
description Fang Gui,* Li-Xuan Zhou,* Xue-Mei Liu, Yong-Long He, Qi-Bin Yang Department of Rheumatology and Immunology, Affiliated Hospital of North Sichuan Medical College, Nanchong, Sichuan, 637000, People’s Republic of China*These authors contributed equally to this workCorrespondence: Qi-Bin Yang, Department of Rheumatology and Immunology, Affiliated Hospital of North Sichuan Medical College, 1 Maoyuannan Road, Nanchong, Sichuan, 637000, People’s Republic of China, Tel +86-817-259-8221, Email yangqb_001@163.comBackground: Primary Sjögren’s syndrome (pSS) is an autoimmune disease characterized by exocrine glands involving mainly the lacrimal and salivary glands. Splenomegaly from pSS is a rare clinical feature unless the pSS is complicated by lymphoma. Splenectomy could exclude malignant lymphoma, reduce the risk of spontaneous spleen rupture and remove hypersplenism to improve pancytopenia.Case Presentation: We reported the case of a 49-year-old female patient who was diagnosed with pSS according to dry mouth, dry eyes, splenomegaly, multiple positive autoantibodies, positive Schirmer’s test and biopsy of labial gland with decreased number of acini and focal lymphocytic infiltration. Due to hematological involvement and liver dysfunction repeatedly, the pSS patient was admitted to the hospital and treated with glucocorticoids, immunosuppressants and hepatoprotective drugs. However, the pancytopenia failed to improve. Considering pancytopenia of the pSS patient caused by massive splenomegaly, splenectomy was recommended to the patient. The hematological involvement was significantly improved after splenectomy. Besides, another 46-year-old woman with a similar clinical manifestation was diagnosed with pSS complicated by massive splenomegaly, however, the patient refused to undergo the splenectomy.Conclusion: Splenectomy may be an effective treatment of pSS complicated by massive splenomegaly resulting in hematological involvement.Keywords: Sjogren’s syndrome, splenomegaly, splenectomy, autoimmune diseases, case report
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spelling doaj-art-65f14486c2454a4985d0bcdbdfeb7cf52025-08-20T04:00:44ZengDove Medical PressInternational Medical Case Reports Journal1179-142X2025-08-01Volume 18Issue 1963968105440Splenectomy for Primary Sjögren’s Syndrome Associated with Massive Splenomegaly: Two Case ReportsGui F0Zhou LX1Liu XM2He YLYang QB3Rheumatology and ImmunolgyRheumatology and ImmunolgyRheumatology ImmunologyRheumatology and ImmunolgyFang Gui,* Li-Xuan Zhou,* Xue-Mei Liu, Yong-Long He, Qi-Bin Yang Department of Rheumatology and Immunology, Affiliated Hospital of North Sichuan Medical College, Nanchong, Sichuan, 637000, People’s Republic of China*These authors contributed equally to this workCorrespondence: Qi-Bin Yang, Department of Rheumatology and Immunology, Affiliated Hospital of North Sichuan Medical College, 1 Maoyuannan Road, Nanchong, Sichuan, 637000, People’s Republic of China, Tel +86-817-259-8221, Email yangqb_001@163.comBackground: Primary Sjögren’s syndrome (pSS) is an autoimmune disease characterized by exocrine glands involving mainly the lacrimal and salivary glands. Splenomegaly from pSS is a rare clinical feature unless the pSS is complicated by lymphoma. Splenectomy could exclude malignant lymphoma, reduce the risk of spontaneous spleen rupture and remove hypersplenism to improve pancytopenia.Case Presentation: We reported the case of a 49-year-old female patient who was diagnosed with pSS according to dry mouth, dry eyes, splenomegaly, multiple positive autoantibodies, positive Schirmer’s test and biopsy of labial gland with decreased number of acini and focal lymphocytic infiltration. Due to hematological involvement and liver dysfunction repeatedly, the pSS patient was admitted to the hospital and treated with glucocorticoids, immunosuppressants and hepatoprotective drugs. However, the pancytopenia failed to improve. Considering pancytopenia of the pSS patient caused by massive splenomegaly, splenectomy was recommended to the patient. The hematological involvement was significantly improved after splenectomy. Besides, another 46-year-old woman with a similar clinical manifestation was diagnosed with pSS complicated by massive splenomegaly, however, the patient refused to undergo the splenectomy.Conclusion: Splenectomy may be an effective treatment of pSS complicated by massive splenomegaly resulting in hematological involvement.Keywords: Sjogren’s syndrome, splenomegaly, splenectomy, autoimmune diseases, case reporthttps://www.dovepress.com/splenectomy-for-primary-sjgrens-syndrome-associated-with-massive-splen-peer-reviewed-fulltext-article-IMCRJSjögren's syndromeSplenomegalySplenectomyAutoimmune diseasesCase report
spellingShingle Gui F
Zhou LX
Liu XM
He YL
Yang QB
Splenectomy for Primary Sjögren’s Syndrome Associated with Massive Splenomegaly: Two Case Reports
International Medical Case Reports Journal
Sjögren's syndrome
Splenomegaly
Splenectomy
Autoimmune diseases
Case report
title Splenectomy for Primary Sjögren’s Syndrome Associated with Massive Splenomegaly: Two Case Reports
title_full Splenectomy for Primary Sjögren’s Syndrome Associated with Massive Splenomegaly: Two Case Reports
title_fullStr Splenectomy for Primary Sjögren’s Syndrome Associated with Massive Splenomegaly: Two Case Reports
title_full_unstemmed Splenectomy for Primary Sjögren’s Syndrome Associated with Massive Splenomegaly: Two Case Reports
title_short Splenectomy for Primary Sjögren’s Syndrome Associated with Massive Splenomegaly: Two Case Reports
title_sort splenectomy for primary sj amp ouml gren amp rsquo s syndrome associated with massive splenomegaly two case reports
topic Sjögren's syndrome
Splenomegaly
Splenectomy
Autoimmune diseases
Case report
url https://www.dovepress.com/splenectomy-for-primary-sjgrens-syndrome-associated-with-massive-splen-peer-reviewed-fulltext-article-IMCRJ
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