Acquired hemophilia A (AHA) is a rare but potentially life-threatening bleeding disorder. This single-center retrospective study aimed to assess clinical features, treatment strategies, and prognostic indicators in adult AHA patients. Eleven patients diagnosed between 2008 and 2024 were reviewed. Clinical data, laboratory findings, treatments, and outcomes were analyzed. Survival estimates and prognostic factors were evaluated using Kaplan-Meier and univariate analyses. Median age was 41 years; 54.5% of the patients were female. Pregnancy-associated AHA (36.4%) had excellent outcomes with steroid monotherapy and no relapse. Idiopathic and autoimmune cases required combination therapy and had higher relapse rates. The median follow-up duration was 27 months. All patients achieved remission (median response time: 62 days), though 36.4% relapsed. High inhibitor titer (>20 Bethesda unit) predicted delayed response (p=0.038); male sex and major bleeding were linked to shorter relapse-free survival. Baseline inhibitor burden and disease etiology influence AHA prognosis. Tailored therapy and multicenter validation are warranted to refine management strategies.

Pediatric traumas and critical illnesses are known to confer increased risk for hospital-acquired venous thromboembolism (HA-VTE) in children. The development of HA-VTE in children is associated with increased mortality and disabling co-morbidities. In this narrative review, we discuss the current literature on HA-VTE in children with severe trauma and those who are critically ill. The pediatric literature in this field continues to grow with randomized trials and guidelines actively being developed. We describe the available data related to the frequency of HA-VTE specific to these populations, as well as the pathophysiological concepts and considerations for its development and management. We outline an approach to HA-VTE in these two groups by delving into risk assessment and the identification of risk factors that accrue in these children, the need to assess for thromboprophylaxis and balance its risks and benefits, the clinical presentation and imaging modalities to confirm the diagnosis of HA-VTE, and management principles for developed HA-VTE. We use some of the currently available guidelines, including those of the Eastern Association for the Surgery of Trauma and Pediatric Trauma Society and the recently updated 2024 American Society of Hematology/International Society on Thrombosis and Haemostasis guidelines on the management of VTE in children, to aid in our discussion.

Hemophilia A (HA) is an X-linked hereditary bleeding disorder caused by variants in the coagulation factor VIII (F8) gene, with a current estimated prevalence of 17.1 per 100,000 male individuals. This study aimed to establish a gene variant spectrum in China using long-distance polymerase chain reaction (PCR) and next-generation sequencing. Long-distance PCR was used to detect intron inversions and next-generation sequencing gene panels were used to identify small sequence variants. Fifty-two different F8 variants were identified in 78 patients from unrelated families, including single-nucleotide alterations (missense, nonsense), frameshifts (small deletions/insertions), splicing-site changes, complex variations, and large rearrangements (inv22 or inv1). The nine variants reported here for the first time include two missense variants, two nonsense variants, four frameshifts, and one splicing alteration. The F8 gene mutation spectrum of patients with HA from Guangxi Province was established and genotype-phenotype correlations were explored. This study contributes data to the existing F8 mutation database and helps systematically identify the mutation spectrum of the gene for HA in southern China.

Transfusion therapy is critical for many patients with β-thalassemia or sickle cell disease (SCD). We aimed to review current practices and document chronic transfusion therapy for patients with hemoglobinopathies in the transfusion service centers of Türkiye. A survey with 16 structured questions was distributed electronically to adult and pediatric hematologists in Türkiye. Responses were received from 37 centers across 18 cities, representing 1449 patients diagnosed with β-thalassemia major, β-thalassemia intermedia, and SCD. Although 79% of centers reported performing extended red cell antigen typing prior to the first transfusion, adherence to national transfusion guidelines was inconsistent. Only 16% of centers routinely performed indirect antiglobulin testing before each transfusion despite guideline recommendations. Antibody identification capabilities varied, with 26% of centers lacking the capability onsite. Elution and adsorption testing were always performed at 13% of centers only, predominantly including university hospitals. Nearly half of the centers were always able to provide D, C, E, c, e, and Kell compatible red cell units, but one-fourth reported that they were unable to consistently provide compatible units due to limited supply. There was no access to red cell genotyping in the country. Our survey revealed disparities in transfusion practices and transfusion service laboratory infrastructure across Türkiye. There is a need for national policy initiatives to mandate adherence to national and international guidelines, expand immunohematology testing capabilities, and ensure the equitable distribution of phenotype-matched red cell units. These findings will contribute to discussions on establishing a centralized immunohematology reference laboratory and enabling red cell genotyping within the country to improve transfusion safety and health equity in hemoglobinopathy care.

To the editor, X-linked immunodeficiency with magnesium defect, Epstein-Barr virus (EBV) infection, and neoplasia (XMEN) is a rare primary immunodeficiency caused by mutations in the magnesium transporter 1 (MAGT1) gene [1,2].Due to its rarity and variable clinical course, each case offers valuable insight into this complex condition.A 29-year-old male presented with fever, jaundice, and fatigue.He had a childhood history of idiopathic thrombocytopenic purpura (ITP) and surgery for patent ductus arteriosus.Laboratory tests showed severe anemia (Hb: 4.6 g/dL), mild thrombocytopenia, indirect hyperbilirubinemia, elevated lactate dehydrogenase, C-reactive protein (CRP), and undetectable haptoglobin.The direct Coombs test was negative, and schistocytes were noted on peripheral smear.Thrombotic microangiopathy (TMA) was suspected, but paroxysmal nocturnal hemoglobinuria (PNH) and ADAMTS13 tests were normal.He received plasmapheresis and corticosteroids without benefit.Eculizumab was initiated for suspected atypical hemolytic uremic syndrome (aHUS), leading to hematologic improvement.Bone marrow biopsy showed erythroid hyperplasia with dyserythropoiesis.Computed tomography (CT) examination revealed pathologic axillary lymphadenopathy.Biopsy confirmed EBV-positive nodular lymphocyte predominant Hodgkin lymphoma (Stage 3A), treated with six cycles of R-CHOP, achieving remission.About 2.5 years after the initial presentation, he developed Coombs-positive autoimmune hemolytic anemia (AIHA) with thrombocytopenia.Steroids were ineffective; eltrombopag and rituximab were given.Cytomegalovirus (CMV) reactivation was treated with ganciclovir.Rituximab resolved cytopenias.Approximately 2 years later, severe hemolysis recurred after cefprozil.Rituximab was resumed but paused due to CMV reactivation, which was managed with valacyclovir.About 7 months later, hemolytic anemia relapsed.Azathioprine was started.Thrombocytopenia recurred with infections but responded to intravenous immunoglobulin (IVIG).Due to persistent EBV DNA positivity, as determined by PCR, and thrombocytopenia, eltrombopag was reinitiated.The summary of the treatment course is presented in Figure 1 .Recurrent cytopenias and frequent infections prompted an evaluation for underlying immunodeficiency.Serum immunoglobulin levels and subclasses were decreased (IgG1: 3.4 g/L; IgG2: 1.46 g/L).Family history revealed that the patient's brother had developed gait disturbance, ataxia, and dysarthria following a COVID-19 infection at the age of 26.Genetic analysis of the brother identified a hemizygous X-linked MAGT1 mutation and a heterozygous autosomal dominant COL4A1 mutation.The asymptomatic mother was heterozygous for the same MAGT1 variant.Genetic testing of the patient confirmed a hemizygous MAGT1 mutation (Figure 1).Flow cytometry revealed a CD4/CD8 ratio of 1.1.Serum magnesium concentrations in our case were consistently within normal limits.Hemoglobin and platelet counts returned to normal without further eltrombopag therapy.No evidence of lymphoma recurrence was observed.The patient was initiated on IVIG therapy every 21 days and placed under regular follow-up.Although the diagnostic criteria for XMEN disease have not yet been fully standardized, they can be summarized based on reported cases in the literature and current clinical guidelines.The diagnosis of XMEN disease is based on the identification of a loss-of-function mutation in the MAGT1 gene, which is located on the X chromosome.Hemizygous mutations are typically seen in males, while heterozygous female carriers are usually asymptomatic.Immunological findings often include CD4+ T-cell lymphopenia, impaired T-cell activation, decreased intracellular magnesium levels, and reduced surface expression of the NKG2D receptor on CD8 T cells and NK cells [1].Clinically, affected individuals may present with persistent or chronic EBV viremia, EBV-related lymphoproliferative disorders , recurrent infections and autoimmune cytopenias [3].Additional features may include dysgammaglobulinemia, lymphadenopathy, and splenomegaly.A decreased CD4/CD8 ratio and

Dear Editor, Megakaryocytes (MKs) comprise 0.01% of all nucleated cells in the bone marrow (BM) [1].Recent studies show that MKs play an important part in platelet production, inflammation, and immune function.As an"omnipotent"cell type, MKs participate in regulating coagulation, inflammation, and immunity [2].MKs in peripheral blood smears (PBSs) may indicate hematological neoplasms, such as myelodysplasia, granulocytic leukemia, or other myeloproliferative disorders [3].MKs are more rarely observed in PBSs of patients with non-hematological diseases, including Sheehan's syndrome, lumbar-disc herniation, and hypertension [4].A total of 411 newborns were admitted to Qingdao Women and Children's Hospital Affiliated to Qingdao University from January 2024 to May 2024, were enrolled in this study.They are 0 to 30 days old.Here, we report on 80 cases of MKs discovered at the PBS's feathered edges of 411 children (Fig. 1).Three to five PBSs of each infant were made to detect MKs which were confirmed with blinded assessments by two experienced experts.The median age of the newborns was 4 days (range: 0 to 30 days), and 44 were boys.Thirty-four patients were diagnosed with hyperbilirubinemia, of whom all 34 had negative neonatal hemolysis test results and 20 were premature.Seventeen children were admitted to hospital because of infections, including pneumonia, enteritis, intrauterine infections and perinatal u

Classical Hodgkin lymphoma (CHL) is a common lymphoid neoplasm with a wide range of differential diagnoses. Although it has a specific immunophenotype, aberrant expression of antigens can cause problems at its diagnosis. In this study we evaluated the usefulness of GATA3 in the differential diagnosis of CHL. One hundred cases of CHL and a control group of 106 lymphoma cases, which included anaplastic large-cell lymphoma both positive and negative for anaplastic lymphoma kinase (ALK), Epstein-Barr virus (EBV)-positive large B-cell lymphoma, T-cell/histiocyte-rich B-cell lymphoma, primary mediastinal large B-cell lymphoma, nodular lymphocyte-predominant Hodgkin lymphoma, and mediastinal gray-zone lymphoma, were included in the study. GATA3 immunohistochemistry was applied to all cases and nuclear expression was accepted as positive. Expression status of GATA3 was compared between the CHL group and the control group, as well as among each lymphoma subtype. In addition, whether the biopsy type affected diagnostic performance was assessed. For CHL, the relationship with EBV status and GATA3 expression was evaluated. GATA3 expression was significantly higher in CHL cases compared to the control group (p<0.001). When compared among individual subgroups, GATA3 was found to be useful in the differential diagnosis of all except for ALK-negative anaplastic large-cell lymphoma (p=0.678) and mediastinal gray-zone lymphoma (p=0.327). GATA3 expression was significantly higher in EBV-negative CHL (p=0.02). In core-needle biopsies, the diagnostic performance was limited (p=0.178). GATA3 is a useful marker for differentiating CHL from B-cell non-Hodgkin lymphomas but its efficiency is limited in ALK-negative anaplastic large-cell lymphoma and mediastinal gray-zone lymphoma. Due to heterogeneous reactions, its diagnostic value is limited in core-needle biopsies.

Follicular lymphoma (FL) is the most common subtype of indolent non-Hodgkin lymphoma in Western countries. While FL is generally incurable, standard initial therapies are associated with high response rates and durable remissions for most patients. In addition, novel targeted agents and immunotherapies are changing the treatment algorithm for patients with relapsed or refractory disease. This review discusses the initial staging, prognosis, and treatment options for newly diagnosed and relapsed/refractory FL. Initial treatment options for FL include active surveillance, radiotherapy, rituximab monotherapy, and chemoimmunotherapy. Staging with positron emission tomography/computed tomography and bone marrow biopsy is crucial for identifying early-stage patients. Most patients with FL will receive chemoimmunotherapy as the initial treatment with options including rituximab or obinutuzumab plus cyclophosphamide, vincristine, and prednisone; cyclophosphamide, doxorubicin, vincristine, and prednisone; bendamustine; or lenalidomide. No significant differences in overall survival have been observed in randomized studies comparing these regimens. Maintenance therapy with rituximab or obinutuzumab in responders to initial chemoimmunotherapy improves progression-free survival. For relapsed/refractory FL, treatment options include chemoimmunotherapy, lenalidomide-based regimens, tazemetostat, chimeric antigen receptor (CAR)-T cell therapy (axicabtagene ciloleucel and tisagenlecleucel), and CD3/CD20 bispecific antibodies (BsAbs). Given the encouraging outcomes obtained with CAR-T cell therapy and BsAbs, multiple trials are testing these highly active agents in earlier lines of therapy and among high-risk patients with early relapse after frontline chemoimmunotherapy. Additional studies and follow-up are needed to understand how these novel agents may further change treatment algorithms for FL.