Extreme Hyperferritinemia

Sackett, Katie; Cunderlik, Maros; Sahni, Nishant; Killeen, Anthony A.; Olson, Andrew

doi:10.1093/ajcp/aqw053

Cited by 59 publications

(22 citation statements)

References 13 publications

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“… 14) The magnitude of elevation in serum ferritin has been associated with a worse prognosis. 15) Our study showed an extremely elevated ferritin level in the HLH-KD group ( Fig. 2 ).…”

Section: Discussionsupporting

confidence: 52%

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Differentiation between incomplete Kawasaki disease and secondary hemophagocytic lymphohistiocytosis following Kawasaki disease using N-terminal pro-brain natriuretic peptide

et al. 2018

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PurposeHemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome with many causes, including Kawasaki disease (KD). The purpose of this study was to identify the laboratory tests needed to easily differentiate KD with HLH from incomplete KD alone.MethodsWe performed a retrospective study on patients diagnosed with incomplete KD and incomplete KD with HLH (HLH-KD) between January 2012 and March 2015. We compared 8 secondary HLH patients who were first diagnosed with incomplete KD with all 247 incomplete KD diagnosed patients during the study period. The complete blood count, erythrocyte sedimentation rate, platelet count, and serum total protein, albumin, triglyceride, C-reactive protein, N-terminal pro-brain natriuretic peptide (NT-proBNP), and ferritin levels were compared. Clinical characteristics and echocardiography findings were also compared between the 2 groups.ResultsThe total duration of fever was longer in the HLH-KD group than in the KD group. White blood cell and platelet counts were higher in the KD group. Alanine aminotransferase, ferritin, and coronary artery diameter were increased in the HLH-KD group compared with those in the KD group. The median of NT-proBNP was significantly higher in the HLH-KD group than in the KD group at 889.0 (interquartile range [IQR], 384.5–1792.0) pg/mL vs. 233.0 (IQR, 107.0–544.0) pg/mL.ConclusionThe NT-proBNP level may be helpful in distinguishing incomplete KD from KD with HLH. The NT-proBNP level should be determined in KD patients with prolonged fever, in addition to the white blood cell count, platelet count, and ferritin level, to evaluate secondary HLH.

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“… 14) The magnitude of elevation in serum ferritin has been associated with a worse prognosis. 15) Our study showed an extremely elevated ferritin level in the HLH-KD group ( Fig. 2 ).…”

Section: Discussionsupporting

confidence: 52%

“…Hypercytokinemia is believed to drive the overactivation of immune cells, resulting in tissue damage together with these cells. 4 14 15) …”

Section: Introductionmentioning

confidence: 99%

Differentiation between incomplete Kawasaki disease and secondary hemophagocytic lymphohistiocytosis following Kawasaki disease using N-terminal pro-brain natriuretic peptide

et al. 2018

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“…No cutoff value has been defined as critical; however, high levels of ferritin (> 50,000 μg/L) are associated with a poor prognosis. 13 Hypofibrinogenemia is the main factor for blood cells velocity that also stated as bad prognostic factor for HLH. Riviere et al found higher LDH levels in patients with hemophagocytic syndrome (positive cases) than in negative and undetermined patients.…”

Section: Discussionmentioning

confidence: 99%

Hemophagocytic Lymphohistiocytosis in Adults: Low Incidence of Primary Neoplasm as a Trigger in a Case Series From Turkey

Arslan

2018

Mediterr J Hematol Infect Dis

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Hemophagocytic Lymphohistiocytosis (HLH) is an indicator of an exaggerated immune response and eventually adverse outcomes. This study aimed to investigate the clinical and laboratory features and outcomes of patients with HLH. The medical records of 26 HLH adult patients (≥ 16 years of age) were retrospectively analyzed. Gender, age, the duration of fever, time to diagnosis, etiology and laboratory data were extracted from the records. The mean age was 38 ± 18 years, and 15 (58%) patients were female. A total of nine cases had infectious diseases; four cases had rheumatologic diseases, three cases had hematological malignancies while nine cases could not have a definitive diagnosis. The median time to detection of HLH was 20 days (IQR: 8–30 d). Of the 25 patients, 11 (44%) died. The erythrocyte sedimentation rates of the surviving and non-surviving patients were 39 ± 22 mm/h and 15 ± 13 mm/h, respectively. When a long-lasting fever is complicated by bicytopenia or pancytopenia (especially), clinicians should promptly consider the possibility of HLH syndrome to improve patients’ prognosis.

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“…On the contrary, recent reports from adult intensive care units (ICUs) have suggested a lower ferritin cut off value of 3000 to 4000 μg/L with >80% sensitivity and specificity in HLH diagnosis [17]. While hyperferritinemia is not specific to HLH, the same in the clinical context of fever, worsening cytopenia (s), and splenomegaly is highly valuable in the ICUs where sepsis is the major overlapping clinical condition [18]. Recent studies have shown that a high serum soluble interleukin 2 receptor to ferritin ratio is an important biomarker in distinguishing lymphoma associated HLH compared to benign disease associated HLH (8.56 vs. 0.66, respectively, P = 0.0004) [19].…”

Section: Diagnostic Criteriamentioning

confidence: 99%

Hepatic Involvement in Hemophagocytic Lymphohistiocytosis

Padhi¹,

Sarangi²,

Patra³

et al. 2020

Hepatitis a and Other Associated Hepatobiliary Diseases

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Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome which results in uncontrolled systemic proliferation of benign macrophages in all reticuloendothelial organs producing worsening peripheral blood cytopenia(s); hypercytokinemia leading to hepatic injury producing hyperferritinemia, hypertriglyceridemia, hypofibrinogenemia; and if not diagnosed and treated early may lead to disseminated intravascular coagulation (DIC), multiorgan dysfunction, and death in nearly all individuals. It is postulated that hepatic injury/dysfunction starts early in the course of the disease which may mimic nonspecific hepatitis like prodrome to fulminant hepatic failure; possibly requiring liver transplant. While HLH as an entity is being increasingly recognized nowadays across wide specialties (both pediatric and adults); hepatic involvement in this setting has been poorly characterized. This chapter is aimed to highlight on the diagnosis and classification of HLH with a special emphasis on the pathophysiology of hepatic dysfunction, histomorphology of liver; and the current concept and controversies on the role of liver transplantation in this clinical setting.

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Extreme Hyperferritinemia

Abstract: Although this supports the relationship between extreme hyperferritinemia and HLH, it maintains that the positive predictive value of hyperferritinemia for HLH is quite low, and one should consider more common explanations before suspecting HLH.

Cited by 59 publications

References 13 publications

Differentiation between incomplete Kawasaki disease and secondary hemophagocytic lymphohistiocytosis following Kawasaki disease using N-terminal pro-brain natriuretic peptide

Differentiation between incomplete Kawasaki disease and secondary hemophagocytic lymphohistiocytosis following Kawasaki disease using N-terminal pro-brain natriuretic peptide

Hemophagocytic Lymphohistiocytosis in Adults: Low Incidence of Primary Neoplasm as a Trigger in a Case Series From Turkey

Hepatic Involvement in Hemophagocytic Lymphohistiocytosis

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