Résumé
A 56-year-old female was admitted to Department of Gastroenterology at Peking Union Medical College Hospital with diarrhea for seven months, and abnormal liver function for six months. She had a history of type 1 diabetes. The main clinical manifestations were recurrent fatty diarrhea and abnormal liver function, accompanied by abdominal and retroperitoneal lymphadenopathy, elevated CA19-9 and CEA. Progressive impairment of hepatic synthetic function and shrinkage of liver developed in a short period of time. The pathology of liver biopsy suggested that nodular regeneration of hepatocytes was followed by hyperplasia of thin bile ducts after submassive necrosis. Intestinal mucosa biopsies were performed twice. The pathology showed that the intestinal villi were completely blunt, accompanied with crypt hyperplasia. Goblet cells disappeared with reduced mucin. Paneth cells were barely seen without intraepithelial infiltration of lymphocytes. Rifaximin was not effective, while glucocorticoids improved clinical situation. The diagnosis of autoimmune enteropathy was finally confirmed by multidisciplinary team including departments of gastroenterology, pathology, endocrinology, hematology, infectious diseases, and rheumatology. With the administration of glucocorticoid and sirolimus, diarrhea relieved and liver function returned to normal.
Résumé
Objective To study the clinical profile of patients with central nervous system (CNS) involvement in relapsing polychondritis (RP).Methods Clinical data of five patients of RP with CNS lesions were collected,and compared with those from the literatures.T test and Fisher exact test were used for statistical analysis.Results Among 205 patients with RP in Peking Union Medical College Hospital,five cases (2.4％) had CNS damage.All 5 cases presented an active onset and occurred in the active phase of RP.All of them manifested as meningoencephalitis,complicating cranial neuropathies in 2 cases (2/5).Cerebrospinalfluid examination revealed non-specific meningeal inflammation,and magnetic resonance image (MRI) showed long T2 signals in brain lesions.Four patients (4/5) showed good response to high-dose glucocorticosteroid plus immunosuppressive agent combined therapy.The average age of our patients was younger than those in the literatures [(44± 14),(58± 11) years,respectively; t=2.547,P＜0.05],while other clinical features was not significantly different between the two groups.Conclusion CNS involvement is a rare condition in RP patients,and usually occurrs in the early course of active RP.Meningoencephalitis/ meningitis is the major clinical manifestations.MRI and cerebrospinal fluid examination may help to confirm the diagnosis.Treatment with corticosteroid and immunosuppressant can result in favorable response.
Résumé
Objective To analyze the clinical characteristics of AIDS-related non-Hodgkin lymphoma(ARL)and review relative literature for the diagnosis and treatment of ARL.Method The clinical data of ARL patients admitted to Peking Union Medical College Hospital from April 2009 to April 2011 were retrospectively analyzed.Results Five male ARL patients aged 32 to 65 years old were included in this retrospective study.Among them,two patients were found to be HIV-positive for the first time,three were on regular highly active anti-retroviral therapy(HAART)for 7-8 months before the emergence of lymphoma-related symptoms.CD4+ T cell count was(69-232)× 106/L at presentation.Two patients firstly presented with sore throat and throat ulcer,one with cervical nodules,one with pelvic mass,one with fever and edema in right thigh.Through pathological analysis,four patients had B cell-originated lymphoma,with one Burkitt lymphoma and three diffuse large B cell lymphomas; one patient had T-cell lymphoma.Four patients were treated with chemotherapy,with one complete remission,one relapse,one non-response,and one death.One patient had radiotherapy only and had progressed disease.Bone marrow suppression and gastrointestinal disturbance were the main adverse effects of chemotherapy.Conclusions Lymphoma should be considered in any HIV-infected patients presented with unexplainable adenopathy,recurrent sore throat or throat ulcer,or fever of unknown origin.Biopsy should be rigorously carried out.Appropriate chemotherapy,together with HAART,may improve the prognosis greatly.