Introduction: Therapeutic plasma exchange (TPE) has emerged as a rescue therapy in thyroid storm. Multiple cycles are often required to achieve adequate reduction in circulating thyroid hormone levels. We report a case of thyroid storm with multiorgan failure successfully managed with only two cycles of TPE, followed by carbimazole. : A 43-year-old female with no significant past medical history presented with a 5-month history of altered bowel habits. She has a small goiter, which was not investigated before. She underwent elective esophagogastroduodenoscopy and colonoscopy at a private centre, both of which were unremarkable. Post-procedure, she developed unstable tachyarrhythmia and cardiac arrest. Return of spontaneous circulation was achieved after three cycles of cardiopulmonary resuscitation, and she was transferred to the intensive care unit. Biochemical evaluation revealed severe thyrotoxicosis (thyroid-stimulating hormone [TSH] <0.008 mIU/L, free thyroxine 4 [FT4] >100 pmol/L). BurchWartofsky Point Scale was 90. Her clinical course was complicated by multiorgan failure, including ischemic hepatitis with coagulopathy and oliguric acute kidney injury requiring continuous venovenous hemodialysis. Lugol’s iodine and corticosteroids were initiated as thionamides were contraindicated due to severe hepatic dysfunction. She developed refractory tachyarrhythmia despite electrical cardioversion. Following a multidisciplinary discussion, TPE was initiated due to the limited choice of antithyroid medication. After two TPE sessions, thyroid function improved markedly (TSH 0.01 mIU/L, FT4 26.2 pmol/L), accompanied by improvement in hepatic and renal function, allowing safe commencement of carbimazole. Thyroid function remained stable with FT4 of 19.3 pmol/L 1 week post TPE. Echocardiography demonstrated mildly reduced left ventricular ejection fraction (45–50%) with bi-atrial dilatation. Conclusion This case highlights the role of limited-cycle TPE as an effective rescue therapy and bridging strategy to definitive treatment in severe thyroid storm with multiorgan failure. Timely TPE initiation may improve biochemical control and clinical outcomes in critically ill patients.
Plasma Exchange ; Thyroid Crisis

Introduction: Severe thyrotoxicosis is particularly difficult to manage when antithyroid drugs are contraindicated. Therapeutic plasma exchange (TPE) is an adjunctive option in complicated hyperthyroidism, including thyroid storm and refractory thyrotoxicosis, through the rapid removal of circulating thyroid hormones, hormone-binding proteins, cytokines, and thyroid autoantibodies. However, its precise role and indications remain incompletely defined. We report two patients with Graves’ disease complicated by carbimazole-induced agranulocytosis in whom TPE was used as bridging therapy before total thyroidectomy. Cases: The first patient was a 32-year-old female who developed carbimazole-induced agranulocytosis 1 month after the diagnosis of hyperthyroidism. Biochemical control remained unsatisfactory despite second-line therapy with high-dose lithium, cholestyramine, propranolol, corticosteroids, and 5 days of Lugol’s iodine. Over 8 days, free thyroxine (fT4) increased by 13%, necessitating TPE for preoperative stabilization. Following three cycles over 4 days, fT4 decreased by 20%, from 52 to 41 pmol/L, enabling successful total thyroidectomy. The second patient was a 26-year-old female who presented with neutropenic sepsis and severe agranulocytosis 2 months after being diagnosed with Graves’ disease. She received second-line therapy, and neutrophil recovery occurred only after 7 days of granulocyte colony-stimulating factor. TPE, together with Lugol’s iodine, was then initiated as bridging therapy before surgery. After 5 days of Lugol’s iodine and three TPE cycles, fT4 decreased by 43%, from 58 to 33 pmol/L, permitting total thyroidectomy. Conclusion These cases highlight TPE as a useful bridging strategy in Graves’ thyrotoxicosis when antithyroid drugs are precluded by agranulocytosis, and conventional secondline therapy fails to achieve adequate biochemical control. TPE may facilitate timely stabilization and safe progression to definitive surgical treatment.
Plasma Exchange ; Agranulocytosis ; Graves Disease

OBJECTIVE: To report the blood group antigen and antibody specificity identification methods for a patient with high-frequency antibodies, and the process of finding and providing compatible blood for the patient. METHODS: A patient sent from the Blood Transfusion Department of Shanxi Provincial People's Hospital to Blood Transfusion Technology Research Laboratory of Taiyuan Blood Center in November 2022 was selected for the study. Classical serological methods were used to determine the patient's blood type, screen for unexpected antibodies, identify antibodies, and perform crossmatching. High-frequency antibody identification was carried out using red blood cells treated with various enzymes. Blood group genotyping was conducted using Matrix-Assisted Laser Desorption/Ionization Time-of-Flight Mass Spectrometry (MALDI-TOF) and Sanger sequencing. Multiple strategies were employed to address the patient's blood source problem. The study was approved by the Medical Ethics Committee of Taiyuan Blood Center [Ethics No. 2024 Ethics Review No.(2)]. RESULTS: The patient's blood type was B, RhD positive. Initial screening of the patient's serum with multiple screening cells and antibody identification cells in saline medium was negative, but positive in antiglobulin medium. The patient's serum showed varying reaction intensities with red blood cells treated with different enzymes. MALDI-TOF mass spectrometry and Sanger sequencing revealed a homozygous nonsense variant c.376C>T (p.Gln126Ter) in the ABCG2 gene, resulting in the Jr(a-) phenotype. During family donor selection, the patient's son was found to have a heterozygous variant c.376C>T (p.Gln126Ter), and another heterozygous variant c.421C>A (p.Gln141Lys), which predicted a Jr(a+w) phenotype. Crossmatch tests confirmed the compatibility of blood from the patient's son, which was used to address the urgent blood requirement. Later, rare blood from a Jr(a-) donor from the Guangzhou Blood Center was used for the patient's ongoing treatment, saving the patient's life. CONCLUSION Combining classic serological testing with blood group gene typing techniques successfully identified the rare Jr(a-) blood type and high-frequency anti-Jra antibodies. Enzyme-treated red blood cell identification methods confirmed the presence of anti-Jra antibodies. By searching within the family and seeking help from other blood centers, compatible blood was found. This approach may provide insights for resolving similar complex blood matching problems in the future.
Humans ; Blood Grouping and Crossmatching/methods* ; Blood Group Antigens/immunology* ; Blood Transfusion ; Male ; Isoantibodies/blood* ; Female ; Genotype

OBJECTIVE: To analyze the distribution frequency and characteristics of HLA-A and HLA-B loci in patients with immune-mediated Platelet transfusion refractoriness (iPTR) in order to provide data support for investigating HLA gene matching strategies for platelet transfusion and improving transfusion efficacy. METHODS: A total of 532 iPTR patients who applied for gene matched platelet at the Blood Center of Zhejiang Province between January 2020 and June 2024 were selected as the study subjects. Genomic DNA was extracted from peripheral blood samples from the patients, and the HLA-A and HLA-B loci were detected simultaneously using a PCR-sequence specific oligonucleotide probe method (PCR-SSO) and PCR-sequence based typing (PCR-SBT). Statistical methods were used to analyze the distributions of HLA-A and HLA-B antigens and genotypes on the platelet surface of the patients. An analysis of the differences was conducted to compare the results with the Common and Well-Documented (CWD) allele in the Chinese population from the China Marrow Donor Program (CMDP) database. This study was approved by the Medical Ethics Committee of the Blood Center of Zhejiang Province (Ethics No.: Provincial Blood Center Ethics Review 2023Yan No.004). RESULTS: Among the 532 iPTR patients, 19 HLA-A antigens (including 32 HLA-A alleles) and 37 HLA-B antigens (including 64 HLA-B alleles) were detected. The antigens with the highest frequencies were A2, A11, A24, and B46, B60, B58, with the combined distribution frequency of the top three antigens reaching 71.43% and 36.94%, respectively. The most prevalent alleles of the HLA-A and HLA-B loci were A*11:01, A*24:02, A*02:07 and B*46:01, B*40:01, B*58:01. The frequencies of common alleles A*01:01, A*02:07, A*11:02, A*30:01 and B*13:02, B*27:04, B*40:01, B*44:03, B*46:01 showed significant differences (P < 0.05) compared to the normal population in the CWD table (version 2.4) of CMDP. CONCLUSION The HLA-A and HLA-B genes of the iPTR patients showed great divergence, and the distribution frequencies of certain alleles have differed significantly from those of the normal population. This study has provided genetic data for exploring the molecular mechanism underlying iPTR, which is of significant clinical importance for searching HLA gene matched donors.
Humans ; HLA-B Antigens/genetics* ; Alleles ; Female ; HLA-A Antigens/genetics* ; Male ; Platelet Transfusion/adverse effects* ; Gene Frequency ; Middle Aged ; Adult ; Genotype ; Aged ; Adolescent ; China

Wunderlich Syndrome is a rare potentially life-threatening phenomenon that involves spontaneous non-traumatic retroperitoneal hemorrhage. At present, identifying the course of conservative management in these patients, especially in pregnancy, has not been clinically established. Presented here is a known case of Tuberous Sclerosis Complex with a Bilateral, 10cm Angiomyolipoma in a 31-year-old female, initially managed with active surveillance. At 27 weeks of pregnancy, she presented with a sudden onset of left flank pain with a hematocrit of 22%. Anemia was corrected with blood transfusions. A contrast-enhanced MRI of the abdomen showed a large subcapsular perirenal hematoma of the left kidney. Renal angioembolization of the bleeding segmental renal artery was done. The patient was conservatized until 37 weeks of pregnancy and underwent cesarean section delivery.

Four months after angioembolization, she had a recurrence of the left flank pain associated with gross hematuria and hypovolemic shock. The patient underwent emergency renal exploration of the left kidney via a transabdominal approach. Three liters of hemoperitoneum and a large expanding left retroperitoneal hematoma were noted intraoperatively. Early vascular control before nephrectomy of the left kidney was done. The postoperative course was unremarkable and the patient was discharged with improved condition.

This case displays a unique course in the management of a bleeding angiomyolipoma especially during pregnancy. Renal angioembolization can aid in achieving the age of viability in pregnancy. However, close monitoring for rebleeding should be kept in mind. A lower threshold for conservative management should be utilized when patients have a previous history of bleeding.

Human ; Female ; Adult: 25-44 Yrs Old ; Abdomen ; Anemia ; Angiomyolipoma ; Arteries ; Blood ; Blood Transfusion ; Cesarean Section ; Conservative Treatment ; Emergencies ; Female ; Flank Pain ; Hematocrit ; Hematoma ; Hematuria ; Hemoperitoneum ; Hemorrhage ; History ; Hypovolemia ; Insemination, Artificial, Heterologous ; Kidney ; Life ; Nephrectomy ; Pain ; Patients ; Pregnancy ; Recurrence ; Renal Artery ; Research Report ; Sclerosis ; Shock ; Syndrome ; Tuberous Sclerosis ; Watchful Waiting

Objective To investigate the therapeutic efficacy of HLA-genotype matched platelet transfusion using a platelet donor database for severe platelet transfusion refractoriness (PTR) caused by HLA antigen-antibody incompatibility. Methods Using real-time quantitative PCR (qPCR) to identify he patient's HLA class I genotype, followed by searching the platelet donor database for matching donors, and selecting highly compatible donors for transfusion. Platelets with higher compatibility levels were prioritized for transfusion recommendations. Results Among the 19 patients studied, 7 patients identified donors with B2U or higher compatibility, 6 patients identified donors with BX or higher compatibility, and 6 patients did not find a suitable donor. The transfusion efficacy was evaluated by calculating the corrected count increment (CCI) 24 hours post-transfusion, and all transfusions were effective. Conclusion The optimal strategy to prevent and treat patients with severe platelet transfusion refractoriness is to ensure patients receive platelet transfusions that are matched to their HLA genes, and this approach significantly enhances transfusion efficacy.
Humans ; Platelet Transfusion/adverse effects* ; HLA Antigens/immunology* ; Male ; Middle Aged ; Female ; Adult ; Blood Platelets/immunology* ; Aged ; Genotype

Objective Analyze the human leukocyte antigen(HLA) class-I antibody specificity and antigenic determinants in patients with ineffective platelet transfusion, to provide theoretical basis for the establishment and application of platelet donor bank in Shandong Province. Methods 69 patients with ineffective platelet transfusion, the patients specimens were subjected to specific detection of HLA class-I antibody, and the possible antigenic determinants were analyzed using HLA Matchmaker software, and the relative immunogenicity of the antigen was calculated. Results A total of 113 specific antibodies were detected in 69 patient specimens. Among which 33 were antibodies to the HLA-A loci, with the highest frequency of HLA-A*69:01 (54%), 54 were antibodies to the HLA-B loci, and the highest frequency of HLA-B*15:12 (64%); 25 antibodies against Cw loci with low platelet expression were detected, with HLA-C*17:01 having the highest frequency (38%). Using HLA Matchmaker software, a total of 221 HLA class I epitopes were detected, among which 163LG and 163L had the highest probability, reaching 59.4%. Among the HLA-A loci, the allele HLA-A*02:03 has the highest relative immunogenicity at 137.157, while the allele HLA-A*02:05 has the lowest relative immunogenicity at 0.1450. In the HLA-B locus, the relative immunogenicity of HLA-B*73:01 allele is the highest, reaching 229.885, while the relative immunogenicity of HLA-B*13:02 allele is the lowest, reaching 0.121. Conclusion This study obtained the distribution characteristics of HLA class-I antibodies in PTR patients in Shandong population, providing theoretical basis for precise platelet transfusion, improving transfusion efficiency, and establishing and applying platelet supply banks.
Humans ; Platelet Transfusion ; Male ; Female ; Middle Aged ; Epitopes/genetics* ; China ; Adult ; Histocompatibility Antigens Class I/genetics* ; Antibodies/blood* ; Aged ; Young Adult ; HLA-A Antigens/genetics* ; Adolescent ; HLA-B Antigens/genetics*

PURPOSE: Intertrochanteric fractures undergoing proximal femoral nail antirotation (PFNA) surgery are associated with significant hidden blood loss. This study aimed to explore whether intramedullary administration of tranexamic acid (TXA) can reduce bleeding in PFNA surgery for intertrochanteric fractures in elderly individuals. METHODS: A randomized controlled trial was conducted from January 2019 to December 2022. Patients aged over 60 years with intertrochanteric fractures who underwent intramedullary fixation surgery with PFNA were eligible for inclusion and grouped according to random numbers. A total of 249 patients were initially enrolled, of which 83 were randomly allocated to the TXA group and 82 were allocated to the saline group. The TXA group received intramedullary perfusion of TXA after the bone marrow was reamed. The primary outcomes were total peri-operative blood loss and post-operative transfusion rate. The occurrence of adverse events was also recorded. Continuous data was analyzed by unpaired t-test or Mann-Whitney U test, and categorical data was analyzed by Pearson Chi-square test. RESULTS: The total peri-operative blood loss (mL) in the TXA group was significantly lower than that in the saline group (577.23 ± 358.02 vs. 716.89 ± 420.30, p = 0.031). The post-operative transfusion rate was 30.67% in the TXA group and 47.95% in the saline group (p = 0.031). The extent of post-operative deep venous thrombosis and the 3-month mortality rate were similar between the 2 groups. CONCLUSION We observed that intramedullary administration of TXA in PFNA surgery for intertrochanteric fractures in elderly individuals resulted in less peri-operative blood loss and decreased transfusion rate, without any adverse effects, and is, thus, recommended.
Humans ; Tranexamic Acid/administration & dosage* ; Hip Fractures/surgery* ; Male ; Aged ; Female ; Fracture Fixation, Intramedullary/adverse effects* ; Blood Loss, Surgical/prevention & control* ; Antifibrinolytic Agents/administration & dosage* ; Aged, 80 and over ; Bone Nails ; Middle Aged ; Blood Transfusion/statistics & numerical data*

To guide clinical blood transfusion practices for pediatric patients, the National Health Commission has issued the health standard "Guideline for pediatric transfusion" (WS/T 795-2022). Blood transfusion is one of the most commonly used supportive treatments for children with hematological diseases. This guideline provides guidance and recommendations for blood transfusions in children with aplastic anemia, thalassemia, autoimmune hemolytic anemia, glucose-6-phosphate dehydrogenase deficiency, acute leukemia, myelodysplastic syndromes, immune thrombocytopenic purpura, and thrombotic thrombocytopenic purpura. This article presents the evidence and interpretation of the blood transfusion provisions for children with hematological diseases in the "Guideline for pediatric transfusion", aiming to assist in the understanding and implementing the blood transfusion section of this guideline.
Humans ; Child ; Hematologic Diseases/therapy* ; Blood Transfusion/standards* ; Practice Guidelines as Topic

To guide clinical blood transfusion practices for pediatric patients, the National Health Commission has issued the health standard "Guideline for pediatric transfusion" (WS/T 795-2022). Blood transfusion for children undergoing hematopoietic stem cell transplantation is highly complex and challenging. This guideline provides recommendations on transfusion thresholds and the selection of blood components for these children. This article presents the evidence and interpretation of the transfusion provisions for children undergoing hematopoietic stem cell transplantation, with the aim of enhancing the understanding and implementation of the "Guideline for pediatric transfusion".
Humans ; Hematopoietic Stem Cell Transplantation ; Child ; Blood Transfusion/standards* ; Practice Guidelines as Topic