Sjögren’s syndrome is a chronic autoimmune disease characterized by lymphocytic infiltration of the exocrine glands, specifically in the salivary glands, lacrimal glands and musculoskeletal system,resulting in xerostomia, xerophthalmia and arthralgia. Other systems or organs, such as respiratory, digestive, hematology system, and kidney, are also involves in this syndrome, leading to interstitial pneumonia, hepatic damage, thrombocytopenia, and renal dysfunction.4 Immunologic thrombocytopenic purpura is a common acquired autoimmune disease. Both antibody-mediated platelet destruction and impaired platelet production are involved in its pathogenesis. About 5% of patients with Sjögren’s syndrome eventually develop lymphomas, while patients with Sjögren’s syndrome having a 44-fold increased risk of developing non-Hodgkin’s lymphoma. MALT lymphoma arising in the thymus is extremely rare, only 37 cases have been reported before.1,5 Chronic B-cell stimulation by these antibodies may contribute to the development of MALT lymphoma. Immunologic disorders are strongly associated with thymic MALT lymphoma tumorigenesis and that thymic MALT lymphoma did not cause these disorders.6 The symptoms and antibody abnormalities could remain unimproved after thymectomy. Irregular antibodies screening is a routine preoperative test, because these harmful antibodies, for instance, anti-D, can result in severe hemolytic disease. In a retrospective review of 28 303 (21 327 Chinese) antenatal attendances from 1997 to 2001༌it is found that the prevalence of clinically significant antibodies amongst Chinese pregnant women is 0.27% and there is only one case which has anti-c + anti-E irregular antibodies among the 58 Chinese women.3 Tiwari et al.7 found that the incidence of irregular antibody varies across different clinical disciplines. Cardiology and cardio-thoracic surgery, hepatology, oncology are the most common clinical disciplines. According to our knowledge, however, the association between irregular antibody and thymic MALT lymphoma is unclear.
As for the surgical treatment of the patient, there is many critical points to achieve a well performance. Firstly, perioperative examination and preparation for clinical diagnosis and operative conditions, such as transfusion preparation. Then, for complicated disease, especially in old people with many comorbidities, MDT should be taken into consideration by enough specialized doctor, such as surgeons, physicians, radiologists and anesthesiologists. Finally, postoperative management should focus on surgical complications, for instance, bleeding after operation due to wound errhysis and stress to decrease the platelets counts. To monitor postoperative condition, patient was warned to routine assessment in the outpatient clinic.
Here we report one case, to our knowledge, that is the first case of thymic MALT lymphoma associated with 2 rare parathymic syndromes: Sjögren's syndrome, immunologic thrombocytopenic purpura and accompanying a rare blood group.