TAFRO syndrome is an infrequent and severe systemic inflammatory condition marked by thrombocytopenia, anasarca, fever, reticulin fibrosis/renal dysfunction, and organomegaly. Although tocilizumab has shown clinical efficacy in inducing initial disease control, evidence on sustained long-term remission beyond three years following anti-interleukin-6 therapy remains limited. We present a 46-year-old female who was admitted with worsening edema, gingival bleeding, and severe thrombocytopenia, initially misdiagnosed as idiopathic thrombocytopenic purpura with no clinical response. A comprehensive diagnostic workup demonstrated multisite serosal effusions (pleural fluid accumulation, pericardial fluid accumulation, and abdominal free fluid), enlarged cervical lymph nodes, grade 1-2 reticulin myelofibrosis with megakaryocytic hyperplasia, Castleman disease-like features on nodal histopathological examination, and increased serum interleukin-6 levels (19.2 pg/mL). The patient fulfilled all major and multiple minor diagnostic criteria for TAFRO syndrome. Treatment consisted of combination therapy with cyclophosphamide, corticosteroids, plasmapheresis, and tocilizumab administered biweekly for a total of eight doses. Complete resolution of thrombocytopenia, anemia, and inflammatory markers was observed after five cycles of tocilizumab. Comprehensive clinical, laboratory, and radiological surveillance showed sustained complete remission for more than three years, without evidence of disease relapse. This report highlights the potential role of tocilizumab-based therapy in achieving prolonged clinical remission in selected patients with TAFRO syndrome, particularly when interpreted within the overall clinical and laboratory context.

Myelofibrosis (MF) is a Philadelphia chromosome-negative myeloproliferative neoplasm characterized by progressive bone marrow fibrosis, constitutional symptoms, cytopenias, and splenomegaly. Splenic enlargement, driven primarily by extramedullary hematopoiesis, represents a hallmark of MF and contributes substantially to symptom burden, portal hypertension, and worsening cytopenias through splenic sequestration. The identification of constitutive Janus kinase-signal transducer and activator of transcription (JAK-STAT) pathway activation as the central pathogenic mechanism in myeloproliferative neoplasms led to the development of JAK inhibitors, which have become the cornerstone of therapy for spleen volume reduction and symptom amelioration. Four JAK inhibitors-ruxolitinib, fedratinib, pacritinib, and momelotinib-are currently approved by regulatory agencies, each exhibiting distinct pharmacological profiles suited to different clinical phenotypes. Ruxolitinib remains the first-line standard for patients with adequate platelet counts, pacritinib is preferred in severe thrombocytopenia, and momelotinib is the agent of choice in anemia-dominant disease. Fedratinib serves as an effective second-line option following ruxolitinib failure. Emerging combination strategies, including pelabresib plus ruxolitinib and navitoclax plus ruxolitinib, have demonstrated superior spleen volume reduction in phase III trials and represent a promising therapeutic frontier. Conventional agents such as hydroxyurea and immunomodulatory drugs retain a role in select patients. Non-pharmacologic interventions-including splenectomy, splenic irradiation, partial splenic artery embolization, and radiofrequency ablation-remain valuable for patients refractory to or ineligible for medical therapy. This review provides a comprehensive and updated overview of the management of splenomegaly in MF, integrating all four approved JAK inhibitors, emerging combination therapies, peri-transplant considerations, and procedural approaches, and proposes a practical phenotype-driven treatment framework.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a systemic autoimmune disease, and central nervous system involvement, though uncommon, can be a severe manifestation. The co-occurrence of AAV and Evans syndrome (ES) is exceptionally rare and may reflect broader immune dysregulation. A 72-year-old female presented with progressive right-sided hemiplegia and mixed aphasia. Imaging revealed multiple acute cerebral infarcts. Laboratory findings showed anemia, thrombocytopenia, acute kidney injury, and elevated inflammatory markers. Serology confirmed myeloperoxidase (MPO)-ANCA positivity, supporting a diagnosis of AAV, most consistent with microscopic polyangiitis. Subsequent hematological workup supported concurrent autoimmune cytopenias consistent with probable ES. Despite corticosteroids and intravenous immunoglobulin, her condition deteriorated with left middle cerebral artery occlusion; during hospitalization, this deterioration coincided with a SARS-CoV-2 infection. Management was escalated to therapeutic plasma exchange and cyclophosphamide. This regimen stabilized renal and hematological parameters, although severe neurological deficits and thrombocytopenia persisted. This case highlights that stroke may be a catastrophic presenting feature of AAV and that concurrent autoimmune cytopenias may add substantial diagnostic and therapeutic complexity. Early ANCA testing in cryptogenic or progressive stroke with systemic involvement is vital, and management requires careful balance between immunosuppression and infection risk.

Congenital hemangioma with thrombocytopenia is rare and is often mistaken for Kasabach-Merritt phenomenon seen in kaposiform hemangioendothelioma. We present three neonatal cases with severe but transient thrombocytopenia that resolved without intervention. Accurate diagnosis, cardiac evaluation, and cautious management are essential, as most patients recover spontaneously, avoiding unnecessary interventions.

Betrixaban is an oral factor Xa inhibitor that is indicated for venous thromboembolism (VTE) prophylaxis in hospitalized patients. It has minimal renal excretion, but it is not well studied in patients with severe renal dysfunction or patients with end-stage renal disease (ESRD). In this evaluation, we discuss betrixaban use in a patient with ESRD on intermittent hemodialysis (HD) and a history of heparin-induced thrombocytopenia (HIT) and contraindication to heparin therapy due to a positive platelet factor 4 IgG antibody. The patient was started on betrixaban for VTE prophylaxis, dosed as 80 mg once followed by 40 mg daily for a total of 15 days of therapy before they were discharged. The patient was continued on their home dialysis schedule of three times weekly and received a total of seven sessions of HD while admitted. Several anti-Xa levels collected after the second dose of betrixaban ranged between 0.12 and 0.32 IU/mL, which were within the predicted therapeutic range for betrixaban based on previous pre-clinical studies. A trough level drawn on day 7 of 0.15 IU/mL indicated the absence of accumulation. Betrixaban was well tolerated with no reported bleeding or VTE events. This case report demonstrates the successful use of betrixaban without accumulation for a patient with ESRD on HD. Betrixaban may be a potential choice when standard options are unsuitable for VTE prophylaxis or in patients with contraindications to other treatments.

The TALAPRO-2 trial (NCT03395197) showed that the addition of talazoparib, a potent PARP inhibitor, to enzalutamide significantly improved radiographic progression-free survival in patients with mCRPC. Due to adverse events (AEs), approximately 62% of patients experienced dose interruption and 53% of patients experienced dose reduction of talazoparib in TALAPRO-2 trial. Hematologic AEs such as anemia, thrombocytopenia, and neutropenia were the most common AEs leading to dose interruptions or reductions. We investigated the relationship between talazoparib exposure as well as baseline patients/disease characteristics and Grade ≥ 3 anemia, thrombocytopenia, and neutropenia using graphical examination and Cox proportional hazard models. The results indicated that higher talazoparib exposure, Cavg, t, was associated with a higher risk of Grade ≥ 3 anemia, thrombocytopenia, and neutropenia. Additionally, among the other baseline factors, we observed that higher risk of all tested safety endpoints was associated with lower baseline hemoglobin. In addition, higher risk of anemia was associated with lower baseline body weight and higher baseline lactate dehydrogenase. Higher risk of neutropenia was associated with lower baseline absolute neutrophil count and lower baseline body weight. These findings support the proposed dose modification algorithm as an effective approach for management of AEs.

Severe Fever with Thrombocytopenia Syndrome (SFTS) is an acute infectious disease caused by the novel bunyavirus with high mortality rates. Current lack of systematic clinical classification standards and effective risk prediction tools necessitates early identification of severe patients to improve prognosis. We retrospectively analyzed clinical data from 437 SFTS patients admitted to The First Affiliated Hospital of USTC and Hefei Second People's Hospital from January 2022 to December 2024. Patients were classified into mild (177 cases, 40.5%), severe (202 cases, 46.2%), and critical (58 cases, 13.3%) categories based on disease severity. Univariate and multivariate logistic regression analyses were performed to identify independent risk factors for disease progression (severe+critical vs. mild). A risk prediction nomogram was constructed using R software with internal validation. Multivariate analysis revealed seven independent risk factors for disease progression: IL-6 > 50 pg/mL (OR = 7.82, 95%CI: 4.23-14.47, P < 0.001), altered consciousness (OR = 6.85, 95%CI: 3.94-11.91, P < 0.001), hemorrhagic manifestations (OR = 4.21, 95%CI: 1.85-9.58, P < 0.001), high-sensitivity CRP > 10 mg/L (OR = 3.45, 95%CI: 2.18-5.47, P < 0.001), pulmonary infection (OR = 2.94, 95%CI: 2.01-4.30, P < 0.001), AST > 200 U/L (OR = 2.13, 95%CI: 1.47-3.08, P < 0.001), and age ≥ 65 years (OR = 1.68, 95%CI: 1.15-2.46, P = 0.007). The nomogram model demonstrated good discriminative ability with a C-index of 0.806 (95%CI: 0.804-0.878) and ROC-AUC of 0.806. Calibration curves showed high concordance between predicted and actual probabilities (Hosmer-Lemeshow test P = 0.512), with sensitivity of 78.5% and specificity of 81.9%. This nomogram prediction model incorporating seven independent risk factors can effectively assist clinical identification of high-risk SFTS patients and has significant clinical utility for early intervention and personalized treatment strategies.