1.Osteogenic and adipogenic differentiation of bone marrow-derived mesenchymal stem cells in patients with aplastic anemia
Haiyan WANG ; Tianling DING ; Yi XIE ; Xiaoping XU ; Long YU ; Tong CHEN
Chinese Journal of Internal Medicine 2009;48(1):39-43
Objective To investigate the osteogenie and adipogenic difference of bone marrowdeftved mesenchymal stem cells(MSCs)between patients with aplastic anemia(AA)and healthy volunteers and to explore the role of MSCs adipo-differentiation in the pathogenetic mechanism of AA.Methods MSCs were isolated from bone marrow of patients with AA and healthy donors and expanded in vitro.MSCs derived from the AA patients and healthy volunteers were compared with respect to morphology,in vitro proliteration capacity,phenotype,differentiation ability and gene expression during differentiation.Results The MSCs clones in the AA patients were(19.30±4.77)/(5×105 MNCs)7 days after culture,being significantly lower than those in the healthy volunteers,which was(47.72±3.46)/(5×105 MSCs)(P<0.05).Compared with those the healthy donors,MSCs from the AA patients had similar proliferative capacity in the first 8 passages and then decreased in the following passages.MSCs from different sources had the same Dhenotype.MSCs from the AA patients could differentiate more easily into adipocytes but less easily and slower into osteoblasts than those from the healthy volunteers.Conclusion The increased adipogenic capacity and decreased osteogenic capacity of MSCs in AA patients may contribute to the development and progress of AA.
2.Analysis of prognostic factors of the first relapsed/refractory primary central nervous system lymphoma
Yan HUANG ; Bobin CHEN ; Pei LI ; Yan YUAN ; Yan MA ; Tianling DING ; Yixia WANG ; Xiaoping XU
Chinese Journal of Clinical Oncology 2018;45(19):985-993
Objective: To analyze the clinical characteristics of patients with relapsed/refractory primary central nervous system lym-phoma (PCNSL) and to explore the factors that influence the prognosis, in order to provide evidence for the clinical diagnosis and treat-ment. Methods: Sixty-four patients with relapsed/refractory PCNSL diagnosed from October 2006 to August 2015 were selected. The clinical features, treatment plans, and laboratory examination data were retrospectively analyzed. Cox regression was used for multi-variate analysis. Results: Univariate and multivariate analyses showed that progression-free survival of first time (PFS1)≤1 year and Kar-nofsky performance status (KPS) score<70 points were independent prognostic factors in patients with first relapsed/refractory PCNSL. The median PFS2 and overall survival of second time (OS2) were 19 and 21 months, respectively, in patients with PFS1≥1 year, where-as the median progression free survival of second time (mPFS2) and OS2 were 10 and 14 months, respectively, in patients with PFS1<1 year. The median PFS2 (mPFS2) in patients with first relapse/refractory KPS score≥70 points and those with KPS score<70 points were 40 and 10 months, respectively, and the median OS2 were 43 and 12 months, respectively. The median PFS for the methotrexate (MTX) and non-MTX groups was 18 and 10 months, respectively. Multivariate analysis showed that the salvage therapy was a relevant factor influencing the patient's PFS. However, univariate analysis showed that the median OS2 in the MTX and non-MTX groups was 23 and 12 months, respectively, with significant difference but without any correlation with prognosis. Conclusions: progression-free sur-vival (PFS)≤1 year and KPS score<70 were independent prognostic factors in patients with first relapsed/refractory PCNSL. Patients with relapsed/refractory PCNSL who continuously received high-dose MTX-based treatment may have improved long-term treatment outcomes.
3.Management of adult Langerhans cell histiocytosis in the hypothalamic-pituitary region—Experiences from Huashan Hospital
Qian WANG ; Quanya SUN ; Min HE ; Li PAN ; Yongfei WANG ; Haixia CHENG ; Yue WU ; Tianling DING ; Hongying YE ; Wei WU
Chinese Journal of Endocrinology and Metabolism 2024;40(5):386-392
Objective:To summarize experience of managing adult Langerhans cell histiocytosis(LCH) in hypothalamic-pituitary region(HPR) from Shanghai Huashan Hospital.Methods:Adult HPR-LCH patients diagnosed at oar endocrinology department from January 2013 to February 2022 were included. Clinical characteristics and treatment response were retrospectively analyzed.Results:A total of 27 adult HPR-LCH patients were included, with 14 cases involving the hypothalamus(H group) and 13 cases without(group NH). The common radiological findings included thickening of the pituitary stalk(25/27, 92.6%). At the time of diagnosis, 14 cases(51.9%) presented with panhypopituitarism, and 19 cases(70.4%) exhibited metabolic abnormalities. The group H had higher proportions of adrenal insufficiency, central hypothyroidism, panhypopituitarism, and diabetes compared to group NH(78.6% vs 23.1%; 78.6% vs 23.1%; 92.9% vs 30.8%, 35.7% vs 0%, respectively, all P<0.05). Hypothalamus syndrome was identified in 71.4%(10/14) of group H. The inital diagnosis rate was 79.2%(19/24), with 48.1% and 51.9% through biopsy of sellar and extrasellar lesions, respectively. Repeated biopsies confirmed the diagnosis in 25.9%(7/27) of cases. The peripheral lesions included bone, thyroid, lung, lymph node, thymus and liver. Out of 20 cases treated with chemotherapy, the objective response rate was 85% at 12 weeks. Four cases received local therapy, one case received traditional Chinese medicine treatment, one case abandoned treatment, and one case was lost to follow-up. The median follow-up time was 28(range 15 to 54) months. During this period, there were 3 deaths in group H and 1 death in group NH. Conclusion:Adult HPR-LCH patients presented with diabetes insipidus and high prevalences of hypopituitarism, hypothalamus syndrome and metabolic abnormalities. Typical imaging features were pituitary stalk thickening. A solitary mass in the HPR was usually very small, posing a great challenge for early diagnosis. Systemic evaluation would help to clarify the diagnosis. Patients with hypothalamus involvement had a higher mortality rate, suggesting the hypothalamus as a risk organ with poor prognosis.
4.Clinical Characteristics and Diagnostic Experience of Adult Thyroid Langerhans Cell Histiocytosis with Diabetes Insipidus
Qian WANG ; Quanya SUN ; Min HE ; Shuo ZHANG ; Boni XIANG ; Qiufan LI ; Yong WANG ; Xialing ZHANG ; Tianling DING ; Hongying YE
JOURNAL OF RARE DISEASES 2023;2(3):346-352