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

Background: 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.