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Assessment of Serum Ferritin, Thyroid Profile and Calcium Homeostasis in Beta-Thalassemia Major Patients.

Background 𝛽-thalassemia major (𝛽-TM) is the most severe form of 𝛽-thalassemia. Serum ferritin is considered as a surrogate marker of iron o … the mean serum ferritin value was 2328.6 ng/ml (159.60-5958.80), and 15 (42.8%) patients had severe hyperferritinemia. Female 𝛽-TM patients had severe vitamin D deficiency, and the frequency of hypocalcemia was higher in males and 4 (11%) patients showed deranged values of phosphate. 𝛽-TM patients had a deranged thyroid profile, 10 (28.5%) of the patients had low t …

Ruxolitinib combined with azithromycin for scrub typhus-associated hemophagocytic lymphohistiocytosis in a child: a case report and narrative literature review.

BACKGROUND: Scrub typhus-associated hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening complication in children, with r … travel to Yunnan Province, China. Laboratory findings revealed pancytopenia (platelets 40× 109/L), hyperferritinemia (>2,000 ng/mL), hypofibrinogenemia (1 g/L), and elevated interferon-gamma (135.48 pg/mL). Bone marrow aspiration demonstrated hemophagocytosis. Metagenomic next-generation sequencing confirmed Orientia tsutsugamushi infection. The patient met six of …

Immune Effector Cell-Associated HLH-Like Syndrome (IEC-HS) in CAR-T and TCE-Treated Myeloma and B-Cell Malignancies: Insights from a Pharmacovigilance Study.

Chimeric antigen receptor T-cell therapy (CAR-T) and T-cell engager antibody (TCE) revolutionized relapsed refractory multiple myeloma (RRMM), non-Hodgkin lymphoma (NHL), and acute lymphoblastic leukemia (ALL) treatment. Immune effector cell-associated hemophagocytic lymphohistiocytosis-like syndrome (IEC-HS) is a potentially fatal rare complication characterized by cytopenia, coagulopathy, transaminasemia, and hyperferritinemia. We aim to analyze the IEC-HS reported cases, patterns, and outcomes in RRMM and NHL patients undergoing CAR-T and TCE therapy utilizing the US Food and Drug Administr…

Serum Ferritin Predicts Neither Organ Dysfunction Nor Mortality in Pediatric Sepsis Due to Tropical Infections.

Objective: To evaluate serial ferritin levels measured in the initial 72 h of admission as a biomarker for new and progressive multi organ d … ritin levels with mortality and NPMODS. Secondary outcomes included estimation of the prevalence of hyperferritinemia and comparison of risk prediction scores with serial ferritin measurement in predicting unfavorable outcomes. Measurements and Main Results: In the 202 children enrolled, diagnosis could be established in 133 (65.8%) children. Scrub typhus and dengu …

[X-linked inhibitor of apoptosis deficiency manifested as Crohn's disease: a case report and literature review].

Objective: To analyze the clinical characteristics of X-linked inhibitor of apoptosis (XIAP) deficient patients with clinical manifestation … minotransferase 175 U/L, aspartate transaminase 229 U/L) , hypertriglyceridemia (4.41 mmol/L) , and hyperferritinemia (>1 650.0 μg/L) . Magnetic resonance enterography revealed the intestinal wall thickening and increased enhancement in parts of illeum and colon. Capsule endoscopy revealed multiple ulcers in jejunum. Colonoscopy showed multiple ulcers in colon and …

Hemophagocytic lymphohistiocytosis: a review inspired by the COVID-19 pandemic.

Hemophagocytic syndrome (HPS) or hemophagocytic lymphohistiocytosis (HLH) is an acute and rapidly progressive systemic inflammatory disorder characterized by cytopenia, excessive cytokine production, and hyperferritinemia. Common clinical manifestations of HLH are acute unremitting fever, lymphadenopathy, hepatosplenomegaly, and multiorgan failure. Due to a massive cytokine release, this clinical condition is considered as a cytokine storm syndrome. HPS has primary and acquired (secondary, reactive) forms. Its primary form is mostly seen in childhood and caused by various mutations with geneti…

[Sjögren disease complicated by primary breast lymphoma: A case report].

This case report describes the diagnostic and therapeutic management of a 67-year-old female with a 40-year history of Sjögren disease (SjD) … laboratory findings including borderline positive IgM λ M-protein, elevated lactate dehydrogenase, hyperferritinemia, and increased β2-microglobulin levels raised suspicion for lymphoproliferative disorders, given the established association between SjD and lymphoma. Bone marrow aspiration showed no significant abnormalities, but PET/CT imaging detected hypermetab …

Metastatic Crohn's Disease and Acute Myeloid Leukemia: A Diagnostic Paradox in a Severe Hyperinflammatory State.

Crohn's disease (CD) is a chronic immune-mediated inflammatory disorder in which extraintestinal manifestations occur in up to 40% of patien … g a diagnosis of AML without maturation (FAB M1). Concurrently, overt hemophagocytosis with extreme hyperferritinemia confirmed concomitant sHLH (HScore: 227). This case highlights a critical and underrecognized risk: TNF-α may act as an endogenous immunological brake against clonal expansion. In our patient, TNF-alpha blockade may have contributed to the loss of i …

Adult-onset Still's disease: A disease at the crossroad of innate immunity and autoimmunity.

Adult-onset Still's disease (AOSD) is a rare disease affecting multiple systems and organs with unknown etiology, and the clinical symptoms are usually described as spiking fever, arthritis, evanescent salmon-pink eruptions, lymphadenopathy, splenomegaly, and other manifestations. The laboratory indicators are not specific, often presenting as increased leukocyte counts and neutrophil percentage, elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), hyperferritinemia, and increased inflammatory factors. ANA, ENA, and RF are negative. According to those unspecific clinical…

Transfusion-Related Iron Overload in Children With Acute Lymphoblastic Leukemia and Lymphoblastic Lymphoma: Addressing an Overlooked Treatment Complication.

INTRODUCTION: Transfusion-related iron overload (TRIO) is a common antineoplastic treatment complication in children undergoing aggressive c … 36.2 to 1924 ng/mL during the follow-up (FU). Upon cessation of intensive chemotherapy, significant hyperferritinemia was detected in 96% of the patients tested and during the FU in 60% of them. Risk factors for TRIO included age six and older, high-risk (HR) disease, and a substantial transfusion load (≥10 red blood cell transfusions (RBCTs)). Hepatic and/or cardi …

Эпштейн-Барр Вирус Серопозитивность, Иммун Дайз Регулирование и Смертность в педиатрии Сепсис.

ВАЖНОСТЬ: Серопозитивность вируса Эпштейн-Барр (ЭБВ) связана с хроническими расстройствами иммунной дисрегуляции, включая множественный склероз, системную волчанку эритематоза, состояние после COVID-19 и множественные раковые заболевания. Сепсис — это острая иммунная дисрегуляция, вызванная 1 из 5 смертей в мире. ЦЕЛЬ: Оценка причинно-следственных связей между серопозитивностью, регулированием иммунной дисперсии и смертностью среди детей, страдающих сепсисом.DESIGN, SETTING и УЧАСТНИКИ: В этом когортном исследовании было проанализировано 320 детей, страдающих сепсисом, в 9-центральном исследов

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IMPORTANCE: Epstein-Barr virus (EBV) seropositivity is associated with chronic immune dysregulation conditions, including multiple sclerosis … ssion. A total of 172 (53.8%) had causal associations with death directly and through the mediators hyperferritinemia and macrophage activation syndrome (MAS) and also had direct causal associations with increased C-reactive protein, ferritin, and interleukin 18 binding protein, which in turn had direct causal associations with decreased ADAMTS 13 activity and decr …

Defining Kawasaki disease and pediatric inflammatory multisystem syndrome-temporally associated to SARS-CoV-2 infection during SARS-CoV-2 epidemic in Italy: results from a national, multicenter survey.

BACKGROUND: There is mounting evidence on the existence of a Pediatric Inflammatory Multisystem Syndrome-temporally associated to SARS-CoV-2 infection (PIMS-TS), sharing similarities with Kawasaki Disease (KD). The main outcome of the study were to better characterize the clinical features and the treatment response of PIMS-TS and to explore its relationship with KD determining whether KD and PIMS are two distinct entities. METHODS: The Rheumatology Study Group of the Italian Pediatric Society launched a survey to enroll patients diagnosed with KD (Kawasaki Disease Group - KDG) or KD-like (Ka

Precision nutrition to reset virus-induced human metabolic reprogramming and dysregulation (HMRD) in long-COVID.

SARS-CoV-2, the etiological agent of COVID-19, is devoid of any metabolic capacity; therefore, it is critical for the viral pathogen to hija … 9 condition could manifest into 3 clinical phases: (i) hypoxia and hypoxemia (Warburg effect), (ii) hyperferritinemia ('cytokine storm'), and (iii) thrombocytosis (coagulopathy). The mean incubation period for COVID-19 onset was estimated to be 5.1 days, and most cases develop symptoms after 14 days. The mean viral clearance times were 24, 30, and 39 days for acute …

Elevated ferritin as a companion biomarker of myeloid-driven inflammation in RNP/Sm co-positive, treatment-resistant SLE endotype.

BACKGROUND: The profound molecular heterogeneity of SLE remains a fundamental barrier to therapeutic progress. METHODS: We integrated clini … ure defines a high-risk, refractory, myeloid-driven lupus endotype characterised by activity linked hyperferritinemia that likely requires therapies directed at the underlying interferon and myeloid-centred pathways. FUNDING: BEAT-Lupus: Arthritis UK and GSK. ACCESS and CALIBRATE: National Institute of Allergy and Infectious Diseases of the NIH. CONTEXT AND SIGNI …

[Diagnostic significance of Th1/Th2 cytokine pattern in childhood hemophagocytic lymphohistiocytosis].

OBJECTIVE: To illustrate the diagnostic value of Th1/Th2 cytokine pattern in childhood hemophagocytic lymphohistiocytosis (HLH) and its diag … longed high-grade fever (50/50), hepatomegaly (44/50), splenomegaly (38/50), hemocytopenia (47/50), hyperferritinemia (49/50), coagulopathy (44/50), hemophagocytosis in bone marrow (42/50), liver dysfunction (42/50) and hypertriglyceridemia (42/50). The IFN-γ, TNF, IL-10, IL-6, IL-4 and IL-2 levels for healthy children were (4.6 ± 1.8) ng/L, (4.0 ± 1.2) ng/L, (6.5 …

Precision genomic profiling in Gaucher disease: insights from atypical presentations.

BackgroundGaucher disease (GD) is characterized by significant phenotypic heterogeneity, even among patients with identical GBA1 genotypes, suggesting the role of genetic and/or epigenetic modifiers. The enzymatic defect and pathological accumulation of glucosylceramide (GlcCer) lead to chronic metabolic inflammation, providing ample opportunities for interaction with other biological pathways to influence disease expression. Herein, we developed a model of precision medicine in this prototype single-gene disorder.MethodsThis study leveraged a well-characterized, longitudinally followed cohort

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… Hyperferritinemia and iron overload Hyperferritinemia with normal iron saturation is a well-recognized feature of GD, often occurring as part of chronic inflammation rather than true iron overload ( Motta et al., 2024 ). … Despite a good response to therapy, he had persistent hyperferritinemia and marked splenic iron overload on MRI. WES revealed homozygosity for the c.44-24G>C and p.Val221 = variants in the SLC40A1 gene. …

Mechanistic Insights in Hemophagocytic Lymphohistiocytosis: Subsequent Acute Hepatic Failure in a Multiple Myeloma Patient Following Therapy with Ixazomib-Lenalidomide-Dexamethasone.

Hemophagocytic lymphohistiocytosis (HLH) is a rare, elusive, and life-threatening condition that is characterized by the pathologic and uncontrolled secondary activation of the cytotoxic T-cells, natural killer cells (NK-cells), and macrophages of the innate immune system. This condition can develop in sporadic or familial contexts associated with hematological malignancies, as a paraneoplastic syndrome, or linked to an infection related to immune system deficiency. This leads to the systemic inflammation responsible for the overall clinical manifestations. Diagnosis should be thorough, and tr

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… Hyperferritinemia is an indicator of macrophage activation and reflects a high generation of TNF-α [ 47 ]. … The enhancement of ferroportin-mediated iron efflux by growth differentiation factor 15 (GDF15) appears to be involved in the modulation of iron homeostasis and the development of hyperferritinemia in HLH [ 48 ]. …

Bone Marrow Aplasia after CAR-T-Cell Therapy for Relapsed/Refractory Burkitt's Lymphoma.

Chimeric antigen receptor T-cells (CAR-T) are now a standard approach for treating relapsed/refractory B-cell lymphomas. Immune effector cell-associated hemophagocytic lymphohistiocytosis-like syndrome (IEC-HS) is a newly described entity that can manifest following CAR-T. Bone marrow (BM) aplasia is an uncommon manifestation of IEC-HS reported after CAR-T-cell therapy and is defined as the reduction or absence of hematopoietic progenitor cells resulting in severe pancytopenia. We describe the case of a 44-year-old female with relapsed/refractory Burkitt lymphoma (BL) who received treatment wi

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… Clinical features include hyperferritinemia, cytopenias, and coagulopathy and may provide a pathologic basis for aplasia following CAR-T. Here, we report a novel case of aplasia in a patient with R/R BL after receiving CAR-T. … The only laboratory requirement for the diagnosis of IEC-HS is hyperferritinemia, which does not have strict parameters, instead using “rapidly increasing” to allow for physician discretion. …

Diagnostic challenges of systemic juvenile idiopathic arthritis with aberrant M-protein expression: a case report.

BACKGROUND: Systemic juvenile idiopathic arthritis (sJIA), a severe autoinflammatory subtype of JIA, presents diagnostic challenges due to non-specific clinical features and the absence of definitive biomarkers. Current ILAR classification criteria emphasize exclusion of mimicking conditions, yet misdiagnosis rates persist (20%-35%), particularly in early disease stages. CASE PRESENTATION: A 15-year-old female presented with persistent high-grade fever, anemia (Hb: 84→64→73→91 g/L), thrombocytosis (PLT 592→1,044 × 10⁹/L), and iron overload. Initial evaluations excluded infections (CMV-positive

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… Notably, serial ferritin monitoring is strongly recommended during diagnostic workup, as hyperferritinemia serves as both a diagnostic biomarker and severity indicator in sJIA. … In sJIA diagnostics, prioritization of established disease markers (e.g., IL-6 elevation, hyperferritinemia) over transient M-protein detection provides more reliable clinical correlation. Acknowledgements Not applicable. …

Clinical and Laboratory Features of Hemophagocytic Lymphohistiocytosis in People With Disseminated Histoplasmosis.

BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome involving pathologic excitation of the immune system. Disseminated histoplasmosis (DH) is a known trigger of HLH. However, the prevalence of HLH in DH is unknown. Limited data exist on risk factors and outcomes. The goals of this study are to determine the prevalence of HLH among participants with DH at a single center, identify risk factors for HLH in this population, and describe the treatment and outcomes of people with DH and HLH. METHODS: We retrospectively identified cases of DH at our institution from 2

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… However, hyperferritinemia alone has been shown to have poor specificity for HLH. In a 2015 review, only 19% of 113 adults with serum ferritin over 50 000 ng/mL had HLH [ 25 ]. … Marked hyperferritinemia does not predict for HLH in the adult population. Blood 2015; 125:1548–52. 26. Moore CJ, Ormseth M, Fuchs H. Causes and significance of markedly elevated serum ferritin levels in an academic medical center. J Clin Rheumatol 2013; 19:324–8. 27. …