Uncommon biphasic CAR-T expansion induces hemophagocytic lymphohistiocytosis-like syndrome and fatal multiple infections following BCMA CAR-T cell therapy: a case report.

B-cell maturation antigen(BCMA)-directed chimeric antigen receptor (CAR)-T-cell therapy has significantly improved the treatment of relapsed or refractory multiple myeloma (MM). Nevertheless, the uncommon phenomenon of biphasic CAR-T cell expansion in vivo and its related severe toxicities have not been methodically described and studied. Herein, we report a case of patients with MM who experienced two CAR-T cell expansion peaks and subsequently developed multiple severe toxicities following BCMA CAR-T cell infusion.

Griscelli Syndrome Type 2: Comprehensive Analysis of 149 New and Previously Described Patients with RAB27A Deficiency.

Griscelli syndrome type 2 (GS2) is a rare, life-threatening immunodysregulatory disorder characterised by impaired cytotoxic activity leading to susceptibility to haemophagocytic lymphohistiocytosis (HLH) and hypopigmentation. We completed a literature review and analysis of clinical data of 149 patients with GS2 including 8 new patients.We identified three founder mutations which show diverse phenotypic profiles (RAB27A c.

Adult-Onset Hemophagocytic Lymphohistiocytosis Associated With Epstein-Barr Virus, Lyme Disease, and Anaplasmosis.

Adult-onset hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome characterized by dysregulated immune activation. Diagnosing HLH poses significant challenges due to its nonspecific clinical presentation, which often mimics infections, malignancies, and autoimmune diseases. Early recognition and prompt initiation of immunosuppressive therapy are crucial, as delayed treatment is associated with a high risk of mortality.

Association of albumin to urea nitrogen ratio with 30-day mortality in adult hemophagocytic lymphohistiocytosis: a retrospective cohort study.

Hemophagocytic lymphohistiocytosis (HLH) is a severe disease with a grim prognosis. This study aims to investigate the potential role of albumin to urea nitrogen ratio (AUR) as a predictor of 30-day mortality in adult HLH patients. This retrospective analysis involved patients admitted to the hospital with a first-time diagnosis of HLH between January 2015 and September 2021.

The Evolving Global Epidemiology of Human Melioidosis: A Narrative Review.

Endemic in over 45 countries globally, recent reports of locally acquired melioidosis in novel geographical areas, such as the Southern US, have highlighted the expanding geographical range of . Climate change and severe weather events have been linked to an increase in cases of melioidosis, which follows environmental exposure to the bacterium. Healthcare professionals should be aware of the possibility of the disease, with its diverse and often delayed presentations, even in areas not previously known to have risk.

[Aggressive natural killer cell leukemia with hemophagocytic lymphohistiocytosis: a case report].

A boy, aged 14 years, was admitted due to recurrent cough and expectoration for more than 1 month, with aggravation and fever for 2 days. After admission, he presented with tachypnea and a significant reduction in transcutaneous oxygen saturation, and emergency chest CT examination showed large patchy exudation and consolidation of both lungs. The boy was given tracheal intubation and invasive mechanical ventilation immediately, and his condition was improved after active symptomatic treatment.

Dengue-Related Hemophagocytic Lymphohistiocytosis in an Adult: A Case Report and Brief Update.

Dengue virus is an arbovirus transmitted through the bite of infected Aedes mosquitoes. Many unusual clinical features are being reported in dengue. Dengue complicated with hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially fatal condition.

Clinical spectrum of primary hemophagocytic lymphohistiocytosis: experience of reference centers in Central and Southeast Anatolia.

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease, with a high mortality if left untreated. In addition, the disease has unique diagnostic challenges. Therefore, despite the existing guidelines on management, current clinical practice data is informative on the course and outcome.

Two-Month Duration of Esophageal Button Battery Impaction in a 23-Month-Old Boy: A Case Report.

Ingestion of foreign bodies, particularly button batteries (BB), is a common pediatric emergency. Esophageal impaction of BB is associated with life-threatening complications. Damage can be manifested within hours from ingestion; therefore, immediate removal is recommended.

Hemophagocytic lymphohistiocytosis caused by multiple infections during primary chemotherapy for pediatric acute lymphoblastic leukemia: a case report.

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory disorder that occurs as a consequence of immune dysregulation. HLH can be primary (familial or non-familial) or secondary to infection, autoimmune disease or malignancy. Malignancy-associated HLH is often accompanied by hematologic and lymphoid neoplasms.

Hemophagocytic lymphohistiocytosis in pregnancy-a case report.

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition that is a rare occurrence in pregnancy and can be elusive in its diagnosis thereby delaying treatment. We report the case of a 30-year-old female patient at 36 weeks of pregnancy who presented with a persistent fever that did not respond to antibiotics. After we investigated her thoroughly, considering the persistent fever, we performed a bone marrow biopsy as part of the workup for prolonged pyrexia.

Autoinflammatory patients with Golgi-trapped CDC42 exhibit intracellular trafficking defects leading to STING hyperactivation and ER stress.

Most autoinflammatory diseases are caused by mutations in innate immunity genes. Previously, four variants in the RHO GTPase CDC42 were discovered in patients affected by syndromes generally characterized by neonatal-onset of cytopenia and auto-inflammation, including hemophagocytic lymphohistiocytosis and rash in the most severe form (NOCARH syndrome). However, the mechanisms responsible for these phenotypes remain largely elusive.

The Microbiome Modifies Manifestations of Hemophagocytic Lymphohistiocytosis in Perforin-Deficient Mice.

Primary hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome caused by inborn errors of cytotoxicity. Patients with biallelic PRF1 null mutations (encoding perforin) usually develop excessive immune cell activation, hypercytokinemia, and life-threatening immunopathology in the first 6 months of life, often without an apparent infectious trigger. In contrast, perforin-deficient (PKO) mice only develop HLH after systemic infection with lymphocytic choriomeningitis virus (LCMV).

Characteristics and therapeutic outcomes of subcutaneous panniculitis-like T-cell lymphoma with and without germline HAVCR2 mutations in Thai children and adolescents.

Background: Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare subtype of non-Hodgkin lymphoma associated with hemophagocytic lymphohistiocytosis (HLH)/HLH-like systemic illnesses and germline HAVCR2 mutations. Although previous studies suggested successful treatment of SPTCL with immunosuppressive therapy (IST) without chemotherapy, IST data in pediatric SPTCL remain scarce. To explore characteristics and therapeutic outcomes, comparing IST-based and chemotherapy-based regimens in pediatric SPTCL, in this retrospective cohort study, patients with SPTCL diagnosed at age ≤20 years during 2007-2023 were enrolled from 6 hematology/oncology centers in Thailand.

The role of blood cell morphology in understanding and diagnosing severe fever with thrombocytopenia syndrome (SFTS): Insights from a case report.

Rationale: Severe fever with thrombocytopenia syndrome (SFTS) is an emerging tick-borne zoonotic disease characterized by a high fatality rate of 12% to 15%. Despite hematologic abnormalities being frequently reported in SFTS patients, detailed characterization of peripheral blood cells, especially in severe cases, is scarce. This case report aims to fill this gap by describing the unique morphological features of blood cells in a patient with a severe outcome.

Epstein-Barr Virus-Driven T-Cell Lymphoma with Haemophagocytic Lymphohistiocytosis: A Life-Threatening Disorder Extending Beyond Childhood.

Unlabelled: An 18-year-old previously healthy Filipino male presented with abdominal pain, vomiting, dyspnoea and fever. Initial investigations revealed severe hepatosplenomegaly, pancytopaenia, elevated liver enzymes, coagulopathy and extremely high ferritin levels. Bone marrow biopsy confirmed an abnormal CD8+ T-cell population with haemophagocytosis.

Health-Related Quality of Life Among Discharged Patients With Hemophagocytic Lymphohistiocytosis: A Follow-Up Study.

Purpose: Evaluating the post-discharge health-related quality of life (HRQoL) in hemophagocytic lymphohistiocytosis (HLH) and exploring its influencing factors.

Patients And Methods: The study was conducted at a regional pediatric medical center and involved pediatric patients diagnosed with HLH between July 2017 and July 2022. Healthy children of the same age and sex were included as the control group.

Timeline and outcomes of viral and fungal infections after chimeric antigen receptor T-cell therapy: a large database analysis.

Objectives: This large database analysis aims to describe the incidence, timeline, and risk factors for viral and fungal infections after chimeric antigen receptor (CAR) T-cell therapy.

Methods: We queried a global research network database, TriNetX, for patients who received CAR T-cell therapy, who were identified and followed for the development of viral and fungal infections. Baseline demographic, oncologic history, laboratory data and medication histories were collected.

Nationwide Analysis of Variables Associated With Sarcoid Inpatient Mortality.

Background: Sarcoidosis is a multisystem autoimmune disease that can result in significant morbidity and mortality. This study aims to identify factors associated with in-hospital death for sarcoid patients on a national level.

Methods: We performed a medical records review study of all adult sarcoid hospitalizations from 2016 to 2020 National Inpatient Sample database.