Jun 5, 2024 · Hyperferritinemia is a requisite for diagnosis of HLH in adults: Ferritin is generally >2,000 ug/L. Ferritin >6,000-10,000 ug/L are somewhat more specific, but less sensitive. Ferritin has prognostic value in HLH: higher values and failure to improve suggest worse outcomes.

Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threatening syndrome of excessive immune activation. It most frequently affects infants from birth to 18 months of age, but the disease is also observed in children and adults of all ages.

Jun 6, 2019 · Hyperferritinemia should always prompt inclusion of HLH in the differential diagnosis. 25 Ferritin values characteristic of HLH in adults are often >7000 to 10 000 µg/L and, rarely, may be >100 000 μg/L. 26 Ferritin levels >10 000 µg/L are >90% sensitive and specific for HLH in children, although other criteria need to be met to make the ...

Mar 5, 2015 · Highly elevated ferritin is not specific for hemophagocytic lymphohistiocytosis in adults. Marked hyperferritinemia in adults most often occurs in the setting of renal failure, hepatocellular injury, infection, or malignancy.

Hyperferritinemia (>10,000 ng/ml) is an important hallmark used as an indicator of infection triggered macrophage activation syndrome leading to hemophagocytic lympho histiocytosis (HLH). Measurement of serum ferritin can be used in diagnosis as ...

Haemophagocytic lymphohistiocytosis (HLH) may be inherited (AR; 5 subtypes, aka familial HLH) or acquired; may mimic severe sepsis, consider in apparent sepsis without source; likely under-diagnosed in ICU; HLH arising secondary to EBV or other viral infection is commonly termed X-linked lymphoproliferative disease (XLP) PATHOPHYSIOLOGY

1 day ago · In patients with systemic juvenile idiopathic arthritis, MAS-HLH is defined by the presence of a fever, a ferritin level exceeding 684 μg per liter, and any two of the following signs: a platelet ...

Ferritin levels above 10,000 microg/L appear to be specific and sensitive for HLH. In patients without a significant medical history and a new onset of febrile illness with highly elevated ferritin levels, the diagnosis of HLH should be evaluated.

Ferritin ≥500 ng/ml is a HLH diagnostic criterion. We evaluated the diagnostic potential of admission ferritin, in children with HLH. Pediatric patients of a referral teaching hospital from Feb 2010–Oct 2013 having been investigated for ferritin on admission were included.

Nov 29, 2023 · Peak ferritin in HLH is often higher than ferritin at presentation, and rapid increases can be seen. Serial ferritin measurements are, therefore, recommended to monitor trends or confirm diagnosis if diagnostic thresholds are not met at initial presentation.

Hyperferritinemia, defined as serum ferritin above 500 ng/mL, can be seen in hemophagocytic lymphohistiocytosis (HLH) and its secondary subcategory, macrophage activation syndrome (MAS), catastrophic antiphospholipid syndrome (CAPS), septic shock, adult-onset Still’s Disease (AOSD), and more recently multi-inflammatory syndrome related to ...

Ferritin in hemophagocytic lymphohistiocytosis (HLH): current concepts and controversies Clin Chim Acta . 2020 Nov:510:408-415. doi: 10.1016/j.cca.2020.07.053.

Hemophagocytic lymphohistiocytosis (HLH) covers a wide array of related life-threatening conditions featuring ineffective immunity characterized by an uncontrolled hyperinflammatory response. HLH is often triggered by infection.

Hemophagocytic lymphohistiocytosis (HLH) is a rare disease that usually occurs in infants and young children. It may also occur in adults. Children usually inherit the disease. In adults, many different conditions, including infections and cancer, can cause HLH.

Dec 3, 2015 · RESULTS: We identified 1,329 patients with a serum ferritin ≥500 mcg/L. Of these, HLH was diagnosed in 28 (2.1%) patients (malignancy-associated HLH in 11 patients, infection-associated HLH in 4 patients, autoimmune-associated HLH in 7 …

Feb 3, 2022 · Studies have shown that a significantly elevated serum ferritin is a common feature of all types of HLH, and the HLH-2004 guidelines have included “Ferritin ≥500 µg/L” as one of the eight diagnostic criteria for HLH.

Nov 1, 2020 · Hemophagocytic lymphohistiocytosis (HLH) is a state of hyperinflammatory state. Hyperferritinemia (≥500 ng/ml) has been incorporated as a criterion in diagnosis. ≥10,000 ng/ml ferritin is diagnostic of pediatric HLH. Adult HLH is heterogeneous disease with diverse etiologies and >2000 ng/ml ferritin may be more relevant.

Oct 30, 2024 · Hemophagocytic lymphohistiocytosis (HLH) ... hyperferritinemia (ferritin >500 ng/mL, although it is usually >3000 ng/mL) elevated soluble CD25 (IL-2 receptor alpha, two standard deviations above age-adjusted norms) low or absent natural killer cell activity (cytotoxicity assay)

Rare immune-mediated conditions such as hemophagocytic lymphohistiocytosis (HLH), where monocytes and macrophages seem to play a vital role through the production and release of ferritin, may cause extremely elevated ferritin levels .

Nov 16, 2012 · We examined if hyperferritinemia (all patients with ferritin level >10,000 mχγ/L between 2007 and 2012) correlated with diagnosis of HLH in 94 patients (73 adult; 21 pediatric) at our institution.

Jan 29, 2025 · The “optimized HLH inflammatory” (OHI) index developed for malignancy-associated HLH and based solely on ferritin (>1000 µg/L) and sIL-2R (>3900 U/mL) showed a mean Youden's index of 0.235, whereas it was 0.366 in a cohort of 348 patients aged ≥13 years with lymphomas only [1, 10] The clinical implications of this report are wide.

Dec 7, 2017 · Ferritin concentration is a surrogate marker for inflammation, and repeated measurements might be associated with disease severity and outcome. We explored the prognostic significance of serum ferritin in adult HLH.

Hemophagocytic lymphohistiocytosis (HLH) is a critical syndrome with a high mortality rate. In clinical practice, some patients with fever of unknown origin (FUO) can develop HLH, further complicating the diagnosis and treatment. ... (especially ferritin), and nonspecific skin lesions. 1, 5, 12 The epidemiology of HLH varies across studies, ...

Studies have shown that a significantly elevated serum ferritin is a common feature of all types of HLH, and the HLH-2004 guidelines have included “Ferritin ≥500 µg/L” as one of the eight diagnostic criteria for HLH.