- The Role of Dermal Regeneration Template in Anterior Abdominal Wall Defect after Burst Abdomen: A Case Report in Acute Graft Versus Host Disease of the Gastrointestinal Tract in Aplastic Anemia. [Case Reports]J Indian Assoc Pediatr Surg. 2022 Nov-Dec; 27(6):760-763.JI
- Acute graft-versus-host disease of the gastrointestinal tract (GI-aGVHD) is a rare condition, often requiring multiple laparotomies, ultimately leading to a burst abdomen. We report the successful use of a dermal regeneration template (DRT), combined with negative pressure wound therapy (NPWT) and skin grafts, to reconstruct the abdominal skin in an 11-year-old boy. The patient was a case of apla…
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- Allogeneic Hematopoietic Stem Cell Transplantation in Severe Aplastic Anemia: A Single Centre Experience in Malaysia. [Journal Article]Blood Cell Ther. 2022 May 25; 5(2):45-53.BC
- CONCLUSIONS: Our study found that patients who received early allogeneic transplantation for SAA had better outcomes. Pre-engraftment failure was the major cause of transplant-related mortality within 100 d. Further studies are required to identify the factors responsible for delaying transplantation to improve treatment outcomes.
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- Transplant for non-malignant disorders: an International Society for Cell & Gene Therapy Stem Cell Engineering Committee report on the role of alternative donors, stem cell sources and graft engineering. [Journal Article]Cytotherapy. 2023 Jan 27 [Online ahead of print]C
- Hematopoietic stem cell transplantation (HSCT) is curative for many non-malignant disorders. As HSCT and supportive care technologies improve, this life-saving treatment may be offered to more and more patients. With the development of new preparative regimens, expanded alternative donor availability, and graft manipulation techniques, there are many options when choosing the best regimen for pat…
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- [Avatrombopag combined with standard immunosuppressive therapy in the treatment of severe aplastic anemia with hepatic impairment in six patients]. [Journal Article]Zhonghua Xue Ye Xue Za Zhi. 2022 Nov 14; 43(11):952-955.ZX
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- [Hematologic responses to avatrombopag switch in TPO-RA refractory aplastic anemia]. [Journal Article]Zhonghua Xue Ye Xue Za Zhi. 2022 Nov 14; 43(11):921-927.ZX
- Objective: Short-term efficacy and safety of afatrombopag conversion therapy in patients with aplastic anemia (AA) who were previously ineffectively treated with intense immunosuppressive therapy (IST) combined with TPO receptor Agonist (TPO-RA) or who were unable to tolerate the side effects of TPO-RA. Methods: Analysis of patients with severe aplastic anemia (SAA) treated in Institute of Hemato…
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- [Clinical characteristics and prediction model of early death in severe/very severe aplastic anemia with immunosuppressive therapy]. [Journal Article]Zhonghua Xue Ye Xue Za Zhi. 2022 Nov 14; 43(11):916-920.ZX
- Objective: Early death (ED) characteristics and predictive factors analysis in patients with severe/very severe aplastic anemia (SAA/VSAA) treated with intensive immunosuppression therapy and establish an ED prediction model. Methods: The clinical data of 232 patients with SAA/VSAA treated with Antithymocyte immunoglobulin (ATG) at the Peking Union Medical College Hospital from August 2003 to Aug…
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- [Guidelines for the diagnosis and management of aplastic anemia in China (2022)]. [Journal Article]Zhonghua Xue Ye Xue Za Zhi. 2022 Nov 14; 43(11):881-888.ZX
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- [The characterization analysis of pathogenic T cells in immune-mediated aplastic anemia mouse model]. [Journal Article]Zhonghua Xue Ye Xue Za Zhi. 2022 Jul 14; 43(7):587-593.ZX
- Objective: This study aims, in addition to characterizing pathogenic T cells trafficking to bone marrow (BM) and other organs, to establish immune-mediated AA C.B10 mouse model by DsRed mouse (B6 background) lymph nodes (LN) cells infusion after a total body irradiation (TBI) . Methods: The C.B10 mice received a 5 Gy TBI and then were infused with DsRed mouse (B6 background) LN cells at 5×10(6)/m…
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- [Kinetics of immune activated T cells in aplastic anemia mouse model]. [Journal Article]Zhonghua Xue Ye Xue Za Zhi. 2022 Jul 14; 43(7):581-586.ZX
- Objective: To explore the dynamic changes of donor derived T cells at different time points in the aplastic anemia mouse model. Methods: The aplastic anemia mouse model was induced and then the proportion of infiltrated donor derived T cells in spleen and bone marrow, expression of activation molecular markers, cell cycle and functional subsets were measured by flow cytometry at different time po…
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- Utility of Whole Exome Sequencing in the Early Diagnosis of Atypical Diamond-Blackfan Anemia. [Journal Article]J Pediatr Hematol Oncol. 2023 Jan 09 [Online ahead of print]JP
- Diamond-Blackfan anemia (DBA) is a rare congenital bone marrow failure syndrome, with a hallmark of erythroblastopenia and congenital anomalies. DBA demonstrates genetic heterogeneity and variable phenotypic expression. We present 2 cases of atypical DBA harboring de novo mutations in the RPS-19 gene with c.49 G>C and c.357-1G>T allelic variants. The 2 cases presented confounding critical illness…
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- Efficacy of eltrombopag with immunosuppressive therapy for children with acquired aplastic anemia. [Journal Article]
- CONCLUSIONS: Adding EPAG to standard IST as the first-line treatment for children with acquired SAA improved the rapidity of hematological response and the CR rate but did not improve the OR or EFS rates.
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- Successful treatment of pure red cell aplasia using therapeutic plasma exchange after ABO-incompatible hematopoietic stem cell transplant. [Case Reports]J Clin Apher. 2023 Jan 26 [Online ahead of print]JC
- Hematopoietic stem cell transplants (HSCTs) are widely used in the treatment of hematologic malignancies and bone marrow failure syndromes. ABO compatibility is typically of secondary importance, and up to 50% of HSCT are performed in ABO-incompatible pairings. In the literature, pure red cell aplasia (PRCA) occurs in 1% to 50% of all major/bidirectional ABO-incompatible stem cell transplants, bu…
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- Rise of the planet of rare anemias: An update on emerging treatment strategies. [Review]Front Med (Lausanne). 2022; 9:1097426.FM
- Therapeutic options for rare congenital (hemoglobinopathies, membrane and enzyme defects, congenital dyserythropoietic anemia) and acquired anemias [warm autoimmune hemolytic anemia (wAIHA), cold agglutinin disease CAD, paroxysmal nocturnal hemoglobinuria (PNH), and aplastic anemia (AA)] are rapidly expanding. The use of luspatercept, mitapivat and etavopivat in beta-thalassemia and pyruvate kina…
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- A CRISPR-Cas9 screen identifies EXO1 as a formaldehyde resistance gene. [Journal Article]
- Fanconi Anemia (FA) is a rare, genome instability-associated disease characterized by a deficiency in repairing DNA crosslinks, which are known to perturb several cellular processes, including DNA transcription, replication, and repair. Formaldehyde, a by-product of metabolism, is thought to drive FA by generating DNA interstrand crosslinks (ICLs) and DNA-protein crosslinks (DPCs). However, the i…
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- T-cell Depleted Peripheral Blood versus Unmanipulated Bone Marrow in Matched Sibling Transplantation for Aplastic Anemia. [Journal Article]Transplant Cell Ther. 2023 Jan 19 [Online ahead of print]TC
- CONCLUSIONS: Our study shows that TCD PB can be considered as a safe source for MSD-HSCT in SAA, with potential advantages in engraftment and GVHD that could challenge the standard with BM. These findings provide further insights for future research in a prospective controlled clinical trial.
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- Late graft failure with donor-derived GPI-deficient cells in a mixed chimera following allogeneic bone marrow transplantation for severe aplastic anemia. [Letter]Bone Marrow Transplant. 2023 Jan 21 [Online ahead of print]BM
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- Mass cytometry analysis identifies T cell immune signature of aplastic anemia and predicts the response to cyclosporine. [Journal Article]
- Aplastic anemia (AA) is an auto-activated T cell-mediated bone marrow failure. Cyclosporine is often used to treat non-severe AA, which demonstrates a more heterogeneous condition than severe AA. The response rate to cyclosporine is only around 50% in non-severe AA. To better predict response to cyclosporine and pinpoint who is the appropriate candidate for cyclosporine, we performed phenotypic a…
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- Aplastic Anemia in Triple X Syndrome. [Case Reports]
- Triple X syndrome is the most common sex chromosome aneuploidies (SCA) in females. Still, it is underdiagnosed because patients are usually without clear dysmorphism, and the syndrome is not associated with any significant congenital anomalies. We are reporting a case of a 5-year-old girl who presented with aplastic anemia, confirmed by a bone marrow aspiration and biopsy. Her complete workup sho…
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- [Chinese expert consensus on the diagnosis and treatment of Fanconi anemia (version 2022)]. [Journal Article]Zhonghua Yi Xue Za Zhi. 2023 Jan 31; 103(4):235-241.ZY
- Fanconi anemia (FA) is an autosomal recessive or X-linked hereditary bone marrow failure disease, in which mutations or deletions of FA-related genes lead to abnormalities in DNA repairment after damage and DNA cross-linking repair. The most common mutation genes include FANCA, FANCC, FANCG, FANCE and FANCF. FA is a disorder with high phenotypic and genotypic heterogeneity and mainly manifests as…
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- Antihuman T lymphocyte porcine immunoglobulin combined with cyclosporine as first-line immunosuppressive therapy for severe aplastic anemia in China: a large single-center, 10-year retrospective study. [Journal Article]
- CONCLUSIONS: p-ATG combined with CsA for the treatment of AA is effective and safe, and p-ATG can be used as an alternative ATG preparation for the standard IST regimen in areas in which h-ATG is not available.
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- A Case with Iron Deficiency Anemia Developed Aplastic Crisis. [Journal Article]Kobe J Med Sci. 2022 Jul 06; 68(1):E1-E4.KJ
- A toddler with an unbalanced diet and gastrointestinal bleeding by juvenile polyp developed an aplastic crisis due to the human parvovirus B19 (HPVB19). Although he exhibited microcytic anemia without iron deficiency in the acute phase of HPVB19 infection, he presented with iron deficiency anemia (IDA) in the chronic phase. IDA results in erythroblast hyperplasia and shortened red blood cell life…
- Prolonged use of eltrombopag in patients with severe aplastic anemia in the real world. [Journal Article]Clin Exp Med. 2023 Jan 16 [Online ahead of print]CE
- Eltrombopag (EPAG) can improve the efficacy of immunosuppressive therapy (IST) consisting of antithymocyte immunoglobulin (ATG) and cyclosporin in severe aplastic anemia (SAA) patients. This study explored whether patients with SAA could benefit from continuous usage of EPAG beyond 6 months.Seventy-four treatment-naive Chinese patients with SAA were administrated with rabbit ATG-based IST plus EP…
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- Determination of chloramphenicol in food using nanomaterial-based electrochemical and optical sensors-A review. [Journal Article]Food Chem. 2023 Jan 09; 410:135434.FC
- Chloramphenicol (CAP) is a widely used antibiotic for the treatment of sick animals owing to its potent action and low cost. However, the accumulation of CAP in the human body can cause irreversible aplastic anemia and hematopoietic toxicity. Accordingly, development of various analytical techniques for the rapid detection of CAP in animal products and the related processed foods is necessary. Am…
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- Fanconi anemia-associated signature in cancer. [Journal Article]Nat Genet. 2023 Jan; 55(1):1.NGen
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- Genome-wide siRNA screens identify RBBP9 function as a potential target in Fanconi anaemia-deficient head-and-neck squamous cell carcinoma. [Journal Article]
- Fanconi anaemia (FA) is a rare chromosomal-instability syndrome caused by mutations of any of the 22 known FA DNA-repair genes. FA individuals have an increased risk of head-and-neck squamous-cell-carcinomas (HNSCC), often fatal. Systemic intolerance to standard cisplatin-based protocols due to somatic-cell hypersensitivity underscores the urgent need to develop novel therapies. Here, we performe…
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- Current status of producing autologous hematopoietic stem cells. [Review]Curr Res Transl Med. 2023 Jan 05; 71(1):103377.CR
- Hematopoietic stem cells (HSCs) transplantation is an established therapy for many diseases of the hematopoietic system, for example aplastic anemia, acute myeloid leukemia and acute lymphoblastic leukemia. With the development of the HSCs research, HSCs provide an attractive method for treating hereditary blood disorders and immunotherapy of cancer by introducing gene modification. Compared with…
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- Extraction, structure and bioactivities of polysaccharides from Rehmannia glutinosa: A review. [Review]J Ethnopharmacol. 2023 Apr 06; 305:116132.JE
- CONCLUSIONS: Based on its effects of promoting hematopoiesis, antitumor and enhancing immunity, RGPs have been clinically applied in the treatment of chronic aplastic anemia and esophageal cancer, but other effects of RGPs have not been reflected in the clinical practice. In the future, more in-depth research can be conducted on the molecular structure analysis, toxicity, side effects and clinical pharmacological effects of RGPs to further explore the pharmacological effects of RGPs and to lay the foundation for safe clinical application and expansion of application scope.
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