Humans ; Nephrotic Syndrome ; Hematopoietic Stem Cell Transplantation ; Tissue Donors ; Transplantation Conditioning ; Graft vs Host Disease

OBJECTIVE: To investigate the clinical application of high-frequency color Doppler ultrasound (HFCDU) in detecting perforators in the deep adipose layers for harvesting super-thin anterolateral thigh flap (ALTF). METHODS: Between August 2019 and January 2023, 45 patients (46 sides) with skin and soft tissue defects in the foot and ankle were treated, including 29 males and 16 females, aged from 22 to 62 years, with an average of 46.7 years. The body mass index ranged from 19.6 to 36.2 kg/m ², with an average of 23.62 kg/m ². The causes of injury included traffic accident injury in 15 cases, heavy object crush injury in 20 cases, mechanical injury in 8 cases, heat crush injury in 1 case, and chronic infection in 1 case. There were 20 cases on the left side, 24 cases on the right side, and 1 case on both sides. After thorough debridement, the wound size ranged from 5 cm×4 cm to 17 cm×11 cm. All patients underwent free super-thin ALTF transplantation repair. HFCDU was used to detect the location of the perforators piercing the deep and superficial fascia, as well as the direction and branches of the perforators within the deep adipose layers before operation. According to the preoperative HFCDU findings, the dimensions of the super-thin ALTF ranged from 6 cm×4 cm to 18 cm×12 cm. The donor sites of the flaps were directly sutured. RESULTS: A total of 55 perforators were detected by HFCDU before operation, but 1 was not found during operation. During operation, a total of 56 perforators were found, and 2 perforators were not detected by HFCDU. The positive predictive value of HFCDU for identifying perforator vessels was 98.2%, and the sensitivity was 96.4%. Among the 54 perforators accurately located by HFCDU, the orientation of the perforators in the deep adipose layers was confirmed during operation. There were 21 perforators (38.9%) traveled laterally and inferiorly, 12 (22.2%) traveled medially and inferiorly, 14 (25.9%) traveled laterally and superiorly, 5 (9.3%) traveled medially and superiorly, and 2 (3.7%) ran almost vertically to the body surface. Among the 54 perforators accurately located by HFCDU, 35 were identified as type 1 perforators and 12 as type 2 perforators (HFCDU misidentified 7 type 2 perforators as type 1 perforators). The sensitivity of HFCDU in identifying type 1 perforators was 100%, with a positive predictive value of 83.3%. For type 2 perforators, the sensitivity was 63.2%, and the positive predictive value was 100%. The surgeries were successfully completed. The super-thin ALTF had a thickness ranging from 2 to 6 mm, with an average of 3.56 mm. All super-thin ALTF survived, however, 1 flap experienced a venous crisis at 1 day after operation, but it survived after emergency exploration and re-anastomosis of the veins; 1 flap developed venous crisis at 3 days after operation but survived after bleeding with several small incisions; 3 flaps had necrosis at the distal edge of the epidermis, which healed after undergoing dressing changes. All 45 patients were followed up 6-18 months (mean, 13.6 months). Three flaps required secondary defatting procedures, while the rest had the appropriate thickness, and the overall appearance was satisfactory. CONCLUSION Preoperative application of HFCDU to detect the perforator in the deep adipose layers can improve the success and safety of the procedure by facilitating the harvest of super-thin ALTF.
Male ; Female ; Humans ; Thigh/surgery* ; Plastic Surgery Procedures ; Prospective Studies ; Skin Transplantation ; Free Tissue Flaps ; Burns ; Soft Tissue Injuries/surgery* ; Ultrasonography, Doppler, Color ; Crush Injuries/surgery* ; Perforator Flap ; Treatment Outcome

OBJECTIVE: To explore the feasibility and effectiveness of a foldable pedicled latissimus dorsi myocutaneous flap to repair soft tissue defects in the shoulder and back. METHODS: Between August 2018 and January 2023, the foldable pedicled latissimus dorsi myocutaneous flaps were used to repair soft tissue defects in the shoulder and back of 8 patients. There were 5 males and 3 females with the age ranged from 21 to 56 years (mean, 35.4 years). Wounds were located in the shoulder in 2 cases and in the shoulder and back in 6 cases. The causes of injury were chronic infection of skin and bone exposure in 2 cases, secondary wound after extensive resection of skin and soft tissue tumor in 4 cases, and wound formation caused by traffic accident in 2 cases. Skin defect areas ranged from 14 cm×13 cm to 20 cm×16 cm. The disease duration ranged from 12 days to 1 year (median, 6.6 months). A pedicled latissimus dorsi myocutaneous flap was designed and harvested. The flap was divided into A/B flap and then were folded to repair the wound, with the donor area of the flap being pulled and sutured in one stage. RESULTS: All 7 flaps survived, with primary wound healing. One patient suffered from distal flap necrosis and delayed healing was achieved after dressing change. The incisions of all donor sites healed by first intention. All patients were followed up 6 months to 4 years (mean, 24.7 months). The skin flap has a good appearance with no swelling in the pedicle. At last follow-up, 6 patients had no significant difference in bilateral shoulder joint motion, and 2 patients had a slight decrease in abduction range of motion compared with the healthy side. The patients' daily life were not affected, and linear scar was left in the donor site. CONCLUSION The foldable pedicled latissimus dorsi myocutaneous flap is an ideal method to repair the soft tissue defect of shoulder and back with simple operation, less damage to the donor site, and quick recovery after operation.
Male ; Female ; Humans ; Young Adult ; Adult ; Middle Aged ; Plastic Surgery Procedures ; Myocutaneous Flap/surgery* ; Shoulder/surgery* ; Skin Transplantation ; Superficial Back Muscles/transplantation* ; Soft Tissue Injuries/surgery* ; Wound Healing ; Treatment Outcome ; Perforator Flap

OBJECTIVE: To summarize the progress of the roles and mechanisms of various types of stem cell-based treatments and their combination therapies in both animal studies and clinical trials of lymphedema. METHODS: The literature on stem cell-based treatments for lymphedema in recent years at home and abroad was extensively reviewed, and the animal studies and clinical trials on different types of stem cells for lymphedema were summarized. RESULTS: Various types of stem cells have shown certain effects in animal studies and clinical trials on the treatment of lymphedema, mainly through local differentiation into lymphoid endothelial cells and paracrine cytokines with different functions. Current research focuses on two cell types, adipose derived stem cells and bone marrow mesenchymal stem cells, both of which have their own advantages and disadvantages, mainly reflected in the therapeutic effect of stem cells, the difficulty of obtaining stem cells and the content in vivo. In addition, stem cells can also play a synergistic role in combination with other treatments, such as conservative treatment, surgical intervention, cytokines, biological scaffolds, and so on. However, it is still limited to the basic research stage, and only a small number of studies have completed clinical trials. CONCLUSION Stem cells have great transformation potential in the treatment of lymphedema, but there is no unified standard in the selection of cell types, the amount of transplanted cells, and the timing of transplantation.
Animals ; Endothelial Cells ; Lymphedema/therapy* ; Stem Cell Transplantation ; Cytokines

Objective: To summarize the characteristics of patients with newly diagnosed multiple myeloma (NDMM) admitted at Ruijin Hospital affiliated to Shanghai Jiaotong University School of Medicine. We compared the clinical characteristics and prognoses among patients with non-extramedullary disease (EMD), bone-related extramedullary (EM-B) disease, and extraosseous extramedullary (EM-E) disease and further explored the effects of autologous hematopoietic stem cell transplantation (ASCT) for EMD. Methods: From January 2015 to January 2022, data of 114 patients (22%) with EMD out of 515 patients with NDMM were retrospectively analyzed; 91 (18%) and 23 (4%) patients comprised the EM-B and EM-E groups, respectively. The clinical characteristics of patients in all groups were compared with the Chi-square test. Progression-free survival (PFS) and overall survival (OS) of patients were analyzed by the Kaplan-Meier method. Independent prognostic factors were determined using multivariate Cox proportional hazard model. Results: There were no significant differences in age, gender, ISS stage, light chain, creatinine clearance, cytogenetic risk, 17p deletion, ASCT, and induction regimens among the three groups. Overall, 13% of EM-E patients had IgD-type M protein, which was significantly higher than that in EM-B patients (P=0.021). The median PFS of patients in the non-EMD, EM-B, and EM-E groups was 27.4, 23.1, and 14.0 months; the median OS was not reached, 76.8 months, and 25.6 months, respectively. The PFS (vs non-EMD, P=0.004; vs EM-B, P=0.036) and OS (vs non-EMD, P<0.001; vs EM-B, P=0.002) were significantly worse in patients with EM-E, while those were not significantly different between patients with EM-B and those with non-EMD. In the multivariate analysis, EM-E was an independent prognostic factor for OS in patients with NDMM (HR=8.779, P<0.001) and negatively impacted PFS (HR=1.874, P=0.050). In those who did not undergo ASCT, patients with EM-B had significantly worse OS than those with non-EMD (median 76.8 months vs. not reached, P=0.029). However, no significant difference was observed in the PFS and OS of patients with EM-B and those with non-EMD who underwent ASCT. Conclusions: Compared to patients with either non-EMD or EM-B, those with EM-E had the worst prognosis. EM-E was an independent risk factor for OS in patients with NDMM. ASCT can overcome the poor prognosis of EM-B.
Humans ; Multiple Myeloma/therapy* ; Retrospective Studies ; China ; Hematopoietic Stem Cell Transplantation ; Prognosis ; Transplantation, Autologous

Humans ; Hematopoietic Stem Cell Transplantation ; Myelodysplastic Syndromes/therapy* ; Nuclear Proteins/genetics* ; Transplantation Conditioning

Humans ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma ; Hematopoietic Stem Cell Transplantation ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/therapy* ; T-Lymphocytes ; Immunotherapy, Adoptive ; Antigens, CD19

Humans ; Shwachman-Diamond Syndrome ; Myelodysplastic Syndromes/therapy* ; Transplantation, Homologous ; Hematopoietic Stem Cell Transplantation ; Transplantation Conditioning

Adoptive therapeutic immune cells, such as chimeric antigen receptor (CAR)-T cells and natural killer cells, have established a new generation of precision medicine based on which dramatic breakthroughs have been achieved in intractable lymphoma treatments. Currently, well-explored approaches focus on autologous cells due to their low immunogenicity, but they are highly restricted by the high costs, time consumption of processing, and the insufficiency of primary cells in some patients. Induced pluripotent stem cells (iPSCs) are cell sources that can theoretically produce indefinite well-differentiated immune cells. Based on the above facts, it may be reasonable to combine the iPSC technology and the CAR design to produce a series of highly controllable and economical "live" drugs. Manufacturing hypoimmunogenic iPSCs by inactivation or over-expression at the genetic level and then arming the derived cells with CAR have emerged as a form of "off-the-shelf" strategy to eliminate tumor cells efficiently and safely in a broader range of patients. This review describes the reasonability, feasibility, superiority, and drawbacks of such approaches, summarizes the current practices and relevant research progress, and provides insights into the possible new paths for personalized cell-based therapies.
Humans ; Receptors, Chimeric Antigen/genetics* ; Induced Pluripotent Stem Cells ; Killer Cells, Natural ; Cell- and Tissue-Based Therapy ; T-Lymphocytes ; Immunotherapy, Adoptive ; Neoplasms/genetics*

Objective: To investigate the genetic and genomic profiling of juvenile myelomonocytic leukemia (JMML) and factors affecting its survival rate. Methods: Clinical characteristics, cytogenetics, molecular biology results and survival status of children with 27 JMML cases admitted to the Hematology Department of Children's Hospital, Capital Institute of Pediatrics from December 2012 to December 2021 were analyzed retrospectively, and the outcomes of the children were followed up. Kaplan-Meier method was used for survival analysis. Univariate analysis was used for analyzing factors affecting the overall survival (OS) rates of patients who received hematopoietic stem cell transplantation (HSCT). Log-Rank test was used for comparison of survival curves. Results: Among 27 JMML cases, there were 11 males and 16 females. The age of disease onset was 28 (11,52) months. There are 20 cases of normal karyotype, 4 cases of monosomy 7, 1 case of trisomy 8,1 case of 11q23 rearrangement and 1 case of complex karyotype. A total of 39 somatic mutations were detected.Those involved in RAS signal pathway were the highest (64%(25/39)), among which PTPN11 mutation was the most frequent (44% (11/25)). A total of 17 cases (63%) received HSCT, 8 cases (30%) did not receive HSCT, and 2 cases (7%) lost follow-up. For children receiving transplantation, the follow-up time after transplantation was 47 (11,57) months. The 1-year OS rate of high-risk transplantation group (17 cases) and high-risk non transplantation group (6 cases) was (88±8)% and (50±20)% respectively, with a statistically significant difference (χ²=5.01, P=0.025). The 5-year OS rate of the high-risk transplantation group was (75±11)%. The survival time of those who relapsed or progressed to acute myeloid leukemia after transplantation was significantly shorter than that of those who did not relapse (χ²=6.80, P=0.009). The OS rate of patients with or without PTPN11 mutation was (81±12) % and (67±19)% respectively (χ²=0.85, P=0.356). Conclusions: The main pathogenesis involved in JMML is gene mutation related to RAS signaling pathway, and the most common driver gene of mutation is PTPN11. Allogeneic HSCT can significantly improve the survival rate of high-risk JMML patients. The recurrence or progression after transplantation was related to poor prognosis.
Male ; Female ; Child ; Humans ; Child, Preschool ; Leukemia, Myelomonocytic, Juvenile/therapy* ; Retrospective Studies ; Survival Analysis ; Mutation ; Hematopoietic Stem Cell Transplantation