Peri-treatment adverse events of primary mediastinal non-seminomatous germ cell tumors.

Primary mediastinal non-seminomatous germ cell tumors (PMNSGCT) are rare but life-threatening thoracic cancers. We report our experience from eight patients with peri-treatment adverse events. By analyzing changes in tumor extent, serum tumor markers, and pathologies between diagnosis and transfer, those events could be attributed to postbiopsy respiratory insufficiency, growing teratoma syndrome, secondary histiocytic malignancy, and PMNSGCT progression. Subjecting patients to respiratory therapy, conventional or high-dose chemotherapy, and surgery controlled the disease, with five of the eight patients surviving disease free. These outcomes indicate that integrated appropriate and timely approaches are important in tackling peri-treatment adverse events.

Evolution of Graves' Disease during Immune Reconstitution following Nonmyeloablative Haploidentical Peripheral Blood Stem Cell Transplantation in a Boy Carrying Germline SAMD9L and FLT3 Variants.

Graves' disease, characterized by hyperthyroidism resulting from loss of immune tolerance to thyroid autoantigens, may be attributable to both genetic and environmental factors. Allogeneic hematopoietic stem cell transplantation (HSCT) represents a means to induce immunotolerance via an artificial immune environment. We present a male patient with severe aplastic anemia arising from a germline SAMD9L missense mutation who successfully underwent HSCT from his HLA-haploidentical SAMD9L non-mutated father together with nonmyeloablative conditioning and post-transplant cyclophosphamide at 8 years of age. He did not suffer graft-versus-host disease, but Graves' disease evolved 10 months post-transplant when cyclosporine was discontinued for one month. Reconstitution of peripheral lymphocyte subsets was found to be transiently downregulated shortly after Graves' disease onset but recovered upon antithyroid treatment. Our investigation revealed the presence of genetic factors associated with Graves' disease, including HLA-B*46:01 and HLA-DRB1*09:01 haplotypes carried by the asymptomatic donor and germline FLT3 c.2500C>T mutation carried by both the patient and the donor. Given his current euthyroid state with normal hematopoiesis, the patient has returned to normal school life. This rare event of Graves' disease in a young boy arising from special HSCT circumstances indicates that both the genetic background and the HSCT environment can prompt the evolution of Graves' disease.

Therapeutic Drug Monitoring of Busulfan in Patients Undergoing Hematopoietic Cell Transplantation: A Pilot Single-Center Study in Taiwan.

(1) Background: Busulfan has been used as a conditioning regimen in allogeneic hematopoietic cell stem transplantation (HSCT). Owing to a large inter-individual variation in pharmacokinetics, therapeutic drug monitoring (TDM)-guided busulfan dosing is necessary to reduce graft failure and relapse rate. As there exists no TDM of busulfan administration for HCT in Taiwan, we conducted a pilot study to assess the TDM-dosing of busulfan in the Taiwanese population; (2) Methods: Seven patients with HCT from The Koo Foundation Sun Yat-Sen Cancer Center, Taipei, Taiwan, received conditioning regimens consisting of intravenous busulfan and other chemotherapies. After the initial busulfan dose, blood samples were collected for busulfan TDM at 5 min, 1 h, 2 h, and 3 h. Busulfan was extracted and detected by performing stable-isotope dilution LC-MS/MS. Plasma busulfan concentration was quantified and used for dose adjustment. Potential adverse effects of busulfan, such as mucositis and hepatic veno-occlusive disease (VOD), were also evaluated; (3) Results: The LC-MS/MS method was validated with an analyte recovery of 88-99%, within-run and between-run precision of <15%, and linearity ranging from 10 to 10,000 ng/mL. Using TDM-guided busulfan dosing, dose adjustment was necessary and performed in six out of seven patients (86%) with successful engraftments in all patients (100%). Mild mucositis was observed, and VOD was diagnosed in only one patient; (4) Conclusions: This single-center study in Taiwan demonstrated the importance of busulfan TDM in increasing the success rate of HCT transplantation. It is also necessary to further investigate the optimal busulfan target value in the Taiwanese population in the future.

[The analysis of HLA-A, B and DRB1 allelic polymorphism in Han race population in Lanzhou region of China].

OBJECTIVE: To investigate the HLA-A, B and DRB1 allele polymorphism of the Han race population in Lanzhou area. METHODS: Polymerase chain reaction-sequence specific primer was used to detect HLA-A, B and DRB1 alleles in 200 unrelated healthy Han individuals from Lanzhou region, Northwest China, and the results were compared with those of Han populations in North, South and Northwest China, and Hui, Uigur and Tibetan population in China. RESULTS: Fourteen of alleles were detected and identified for HLA-A; 32 for HLA-B; and 13 for HLA-DRB1. HLA- A*01, A*02,A*11,A*24, A*30, A*31, A*33; HLA- B*13, B*15, B*40, B*44, B*46, B*51, B*58, B*60; HLA- DRB1*04,. DRB1*07, DRB1*08, DRB1*09, DRB1*11, DRB1*12, DRB1*14 and DRB1*15 were the most common alleles. The frequencies of HLA-A, B and DRB1 genes of Lanzhou Han race were close to that of North China Hans and Hui population in Northwest China, and a little different to that of South China Hans. The HLA-DRB1 alleles were significantly different to those of Uigur and Tibetan race population of China. CONCLUSION: The allelic polymorphism of HLA-A,B and DRB1 loci of Han race population in Lanzhou area was between North and South Han race of China, close to Northwest China Hui, and markedly different to Northwest China Uigur and Tibetan race populations.

[Experimental study of double-root implantation with the aid of template in rabbits].

PURPOSE: To evaluate the feasibility of double-root implantation with the aid of template. METHODS: Implant templates were fabricated based on CT data from the tibia of 12 rabbits. 12 double-root implants were placed in the tibia of rabbits under the guidance of implant templates, and single-root implants were placed in contralateral tibia of the same rabbits. Rabbits were sacrificed at 8 and 12 weeks. Implant mobility was checked directly. Specimens were examined by X-ray to measure bone gray value using Image Pro-Plus6.0 software. Super-rigidity slices were prepared to test bone-implant contact ratio using Image Pro-Plus6.0 software. The data were analyzed with SPSS20.0 software package. RESULTS: The surgical procedures were completed successfully. There was no obvious loosening of implants. No significant difference was found between the experimental and control groups in bone gray value and bone-implant contact ratio at different time(P>0.05). CONCLUSIONS: The implanting method of double-roots implant with the aid of template is feasible, and osteointegration is ideal.

Mediastinal Germ Cell Tumor-associated Histiocytic Proliferations Treated With Thalidomide Plus Chemotherapy Followed by Alemtuzumab-containing Reduced Intensity Allogeneic Peripheral Blood Stem Cell Transplantation: A Case Report.

Mediastinal nonseminomatous germ cell tumor (MNSGCT)-associated histiocytic proliferations are rare and rapidly fatal disorders. Standard treatment modalities have yet to be established.We report a case of MNSGCT-associated hemophagocytic syndrome that evolved into malignant histiocytosis/disseminated histiocytic sarcoma (MH/HS), which was initially treated with intravenous immunoglobulin, corticosteroids, and cyclosporine. Then, thalidomide plus cyclophosphamide, adriamycin, oncovin, prednisolone chemotherapy followed by alemtuzumab-containing reduced-intensity allogeneic peripheral blood stem cell transplantation (PBSCT) was used as salvage therapy.The severe constitutional symptoms and pancytopenia resolved shortly after thalidomide with cyclophosphamide, adriamycin, oncovin, prednisolone. After PBSCT, the patient developed steroid-dependent skin graft-versus-host disease, but maintained a functional life for 1.5 years. Rapid resolution of chronic graft-versus-host disease preceded the fulminant recurrence of hemophagocytic syndrome and MH/HS.Thalidomide plus chemotherapy followed by alemtuzumab-containing reduced intensity allogeneic PBSCT is effective in allaying MNSGCT-associated histiocytic disorders, but does not prevent eventual relapse. However, further posttransplant immune modulation should be developed to completely eradicate the residual MH/HS cells.

Long-term cancer-free survival after infusion of autologous cord blood cells for rejection-unrelated cord blood graft after myeloablative conditioning for t-MDS: a case report.

Autologous cord blood transplantations are rarely applied in patients with hematologic as well as metastatic solid cancers since contamination of malignant clones is a concern. We report a case of therapy-related myelodysplasia after metastatic neuroblastoma who suffered from graft rejection and life-threatening infections after a myeloablative unrelated cord blood transplantation. The patient experiences long-term survival, free from leukemia/neuroblastoma after infusion of autologous cord blood cells. It suggests that autologous cord blood transplantation after high dose therapy might be a curative strategy for certain hematologic or metastatic solid cancers.

Idiopathic Pneumonia Syndrome and Thrombotic Microangiopathy Following Nonmyeloablative Haploidentical Peripheral Blood Stem Cell Transplantation and Posttransplant Cyclophosphamide: A Case Report.

Posttransplant high-dose cyclophosphamide (pT-HDCy) following T-cell-replete haploidentical bone marrow (BM) transplantation has been successfully utilized to control alloreactivity, mainly in ameliorating graft-versus-host disease (GVHD) and graft rejection. Recently, peripheral blood stem cells (PBSCs) have also been suggested to be a feasible and effective graft alternative to BM in the same setting. We report a case with refractory Hodgkin lymphoma treated with haploidentical PBSC transplantation with nonmyeloablative conditioning and pT-HDCy. Although engraftment with complete donor chimerism was achieved without classical GVHD, the patient suffered from idiopathic pneumonia syndrome followed by thrombotic microangiopathy. Although idiopathic pneumonia syndrome and thrombotic microangiopathy improved after treatment, the patient's lymphoma rapidly progressed nonetheless. This outcome may suggest that the alloreactivity against the classical GVHD targets is successfully eradicated by pT-HDCy, but alloreactivity against the lungs and endothelial cells is differentially preserved when utilizing granulocyte colony-stimulating factor-mobilized PBSCs as the graft source. The graft-versus-Hodgkin lymphoma effect was not observed in our patient.

[The design and biomechanical evaluation of implantation on the mandibular defect reconstructed with rib graft].

PURPOSE: Applying geometric simulation and finite element analysis to an unilateral mandibular defect reconstructed with rib graft to evaluate the biomechanical properties and guide the implant restoration for this kind of mandibular defect. METHODS: A geometric and finite element analysis mechanic model of mandibular defect reconstructed with rib graft based on CT scan and graphic processing technique was established. Computer-aided simulation of implantation and finite element analysis were carried out to meet the prosthetic requirement. The feasibility and biomechanical properties of this design were also evaluated. RESULTS: Implant restoration on mandibular defect reconstructed with rib graft was feasible. The implant could be inserted perpendicularly to the rib graft and the stress after implantation was concentrated on the mesial aspect of the left mandibular second premolar area. At the later stage, a prosthetic restoration should be applied to adjust the occlusal relationship. CONCLUSIONS: The accuracy and success rate of an implant restoration on a mandibular defect reconstructed with rib graft can be raised via preoperative design of the location and angulation of implants aided by CT scan, graphic processing technique, biomechanical analysis, as well as stress reduction by prosthetic fabrication and occlusal adjustment at the later stage.

[IgG4-associated parotitis:case report and review of the literature].

A case of immunoglobulin G4 (IgG4)-associated parotitis was reported and related literatures were reviewed,in order to improve the recognization of this systemic diseases and reduce the misdiagnosis and mistreatment in clinical practice for the stomatologists.

[Therapeutic effect of staged treatment for huge mandibular cystic lesions].

PURPOSE: To investigate the therapeutic effect of staged treatment for huge mandibular cystic lesions. METHODS: The study enrolled 18 cases of huge cystic lesions whose extent in X-ray film exceeded 5 cm from 2005 to 2009 in our hospital, 6 of them presented mal-aligned dentition. Decompression was first given under local anesthesia to make the entire extent gradually reduced to half of its primary extent, then the cysts were enucleated secondarily combined with simultaneous Bio-oss insertion under general anesthesia, followed by X-ray examination monthly to observe the density of bone, and normal orthodontic treatment was given to 6 cases with malocclusion. RESULTS: The lesions reduced to around half of the primary extent in 4 to 6 months after decompression,the density of bone substitute became almost similar to adjacent bone 6 to 12 months after secondary operation, no case had recurrence within 2 to 3 years. 6 cases with malocclusion were corrected after 2 to 3 years of orthodontic therapy. CONCLUSIONS: The staged treatment can retain the whole mandible and teeth at the same time of enucleation of the cyst, and achieve excellent aesthetic result combined with orthodontic therapy.

[An animal experiment of immediate construction of mandibular defect].

PURPOSE: To observe the effect of immediate construction of mandibular defect with or without teeth missing by bone composite and absorbable Bio-gide membrane. METHODS: 40 New Zealand rabbits were randomly divided into 3 groups. For the mandibular defect, group A was filled with nothing, group B was filled with bone composite and Bio-gide membrane, group C was filled with bone composite and Bio-gide membrane as well as autogenous tooth implantation. The rabbits were sacrificed 4,8,12 weeks after surgery for evaluation of gross morphology, X-ray and histopathology. RESULTS: After 12 weeks, the defect in group A was still there but narrower, the defect in group B was filled with new bone, the density of which was same to the bone X-ray, and lamellar bone were under microscope, the implanted teeth in group C had been fixed tightly in 17 animals, no transparent areas in 13 animals from X-ray, but absorbable areas in 13 animals under microscope were noted. CONCLUSIONS: Immediate construction of mandibular defect with bone composite and Bio-guide membrane is feasible and effective,autogeneous tooth implantation on the construction area is also stable.

[Extraarticular diffuse pigmented villonodular synovitis of the temporomandibular joint: report of one case and review of the literature].

Diffuse pigmented villonodular synovitis (PVNS) in the temporomandibular joint (TMJ) is distinctly rare. This article reported a case of extra-articular type of PVNS of the TMJ, and reviewed the previously reported cases in the literature. It is concluded that PVNS can be diagnosed by characteristic MRI finding, wide local excision including a total synovectomy and postoperative radiotherapy should be adopted.

[ABO typing error caused by anti-IgM activated complements].

In order to determine the appraisal of ABO blood group accurately and ensure the safety of blood transfusion, an investigation was made on the influence of complement activated by antibody in appraising ABO blood group by using hemoagglutination test and direct antiglobulin test. Two samples of ABO blood group were collected from two donors. The results showed that the obverse and reverse patterns did not accord each other in two samples of ABO blood group, and their blood samples were both positive for anti-C(3) and were both negative for anti-IgG. It is concluded that the inconsistency of the obverse and reverse patterns in two samples of ABO blood group were proved to be caused by the complements activated by the antibody against IgM. Blood group O was finally determined for the two samples, and the influences of IgM antibody and complement on ABO blood group were excluded when the test proceeded. This method will be useful to determine ABO blood group accurately.

[Clinical features of primary Sjögren's syndrome in childhood].

OBJECTIVE: To assess the clinical features of primary Sjgren's syndrome (pSS) in childhood and adult patients to understand the differences between them. METHOD: Data of 21 childhood and 400 adult patients with definite primary Sjgren's syndrome were analyzed retrospectively. RESULTS: In adult patients, initial clinical symptoms were various, dry mouth and dry eyes, arthritis and arthralgia, parotid swelling, renal tubular abnormalities (RTA) were more common. Compared with adult patients, RTA, parotid swelling and rash were more common in childhood, pulmonary abnormalities and neurologic system involvement were less common. The frequency of renal tubular abnormalities (52.4%) and rash (47.6%) in childhood pSS were higher than those of adults, but the frequencies of dry eyes (61.0%) and pulmonary interstitial fibrosis (25.2%) in adults pSS were higher than those in childhood (P < 0.01); serum rheumatoid factor (RF) and gamma-globulin were higher in all childhood patients (P < 0.01). Anti-nuclear antibody (ANA), anti-SSA and anti-SSB antibodies, and IgG were significantly higher in childhood cases than in adult patients (P < 0.05 or 0.01). CONCLUSION: The major clinical characteristics of childhood pSS cases included: (1) RTA, parotid swelling and rash which appeared earlier, but dry mouth and dry eyes appeared later and were mild. (2) The frequency of pulmonary abnormalities, nervous system involvement and Raynaud's phenomenon were less commonly seen. (3) Positivity of RF, anti-SSA and anti-SSB antibodies, and serum IgG were higher.