Performances of the H-Score for Diagnosis of Hemophagocytic Lymphohistiocytosis in Adult and Pediatric Patients

Mahadeb, Bhavna; Ferster, Alina; Meuleman, Nathalie; Rozen, Laurence; Demulder, Anne; Corazza, Francis

doi:10.1093/ajcp/aqw076

Cited by 162 publications

(150 citation statements)

References 13 publications

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“…6 Therefore, it could be useful considering new diagnose criteria, also in addition to those proposed by Delphi study and H score. 7,8 Only a few hemophagocytosis signs were detectable in patient’s bone marrow, in spite of the wide and aggressive clinical manifestation. Accordingly, we agree that the bone marrow aspect should not be considered as a gold standard, because the typical hemophagocytosis signs predominantly appear in the more advanced disease stages, and they are absent in about 40% of cases.…”

Section: Discussionmentioning

confidence: 96%

Hemophagocytic Lymphohistiocytosis, an Unclear Nosologic Entity: Case Report of an Adult Man with Rising of Amylase and Lipase and Spinal Cord Infiltration

et al. 2017

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Here we present the case of a 57-years old patient affected by hemophagocytic lymphohistiocytosis (HLH), a rare disease characterized by an uncontrolled immune activation, resulting in clinical and biochemical manifestations of extreme inflammation. In a previous hospitalization, the patient showed fever, hepato-splenomegaly, pancytopenia, hyperferrtitinemia, lymphadenopathy and cholestasis. No diagnosis was done, however, he totally recovered after splenectomy. Eight months later, he relapsed, showing also hypofibrinogenemia, hypertriglyceridemia, hemophagocytic signs in bone marrow, cholestatic jaundice, high LDH and high PT-INR. Interestingly, he presented increased levels of amylase and lipase in absence of radiologic signs of pancreatitis. He was treated with Dexamethasone and Cyclosporine according to HLH-2004 guidelines. The clinical and biochemical manifestations disappeared in a few weeks, but he was newly hospitalized for lower limbs hypotonia caused by a hemophagocytic lesion of the cauda equina and lumbar cord. The death occurred in a few days, despite the immunosuppressive treatment.

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Section: Discussionmentioning

confidence: 96%

Hemophagocytic Lymphohistiocytosis, an Unclear Nosologic Entity: Case Report of an Adult Man with Rising of Amylase and Lipase and Spinal Cord Infiltration

et al. 2017

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“…In spite of the above data however, an expert working group on adult HLH from the Histiocyte Society recently published guidelines reaffirming the role of the HLH‐94 criteria (including the ferritin cutoff of 500 ng/mL) as the standard diagnostic tool for adult HLH . Some newer diagnostic tools for adult HLH have been emerging in recent years, most notably the H‐score . However, this scoring system uses ferritin cutoffs of 2000 ng/mL and 6000 ng/mL (with more points conferred at the higher level), which based on the results of our own and the above‐cited studies are still woefully non‐specific.…”

Section: Discussionmentioning

confidence: 99%

Reevaluating the role of ferritin in the diagnosis of adult secondary hemophagocytic lymphohistiocytosis

Naymagon

Tremblay

Mascarenhas

2020

European J of Haematology

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Background The standard diagnostic criteria for hemophagocytic lymphohistiocytosis (HLH) include hyperferritinemia to >500 ng/mL. This ferritin threshold is based on pediatric data, and evidence for its application among adults with secondary HLH is lacking. Objective and Methods We conducted a retrospective study assessing the relationship between extreme hyperferritinemia and adult secondary HLH at our institution. All adult inpatients seen over a 10‐year period, with serum ferritin >5000 ng/mL, were included. Results Among 1055 patients with serum ferritin >5000 ng/mL, there were 69 cases of HLH (HLH prevalence of 6.5%). Mean ferritin among HLH patients was 70 398 ng/mL (SD 122 908), median 40 019 ng/mL (IQR 16 051‐68 326). The prevalence of HLH only reached 50% as serum ferritin approached 90 000 ng/mL. A variety of conditions were contributory to hyperferritinemia, most commonly bacterial sepsis (33%), hematologic malignancy (29%), renal failure (24%), and liver injury (18%). The optimal cutoff ferritin for diagnosis of HLH was 16 000 ng/mL (sensitivity 79.4%, specificity 79.2%, PPV 20.9%, and NPV 98.2%). Conclusions The threshold ferritin levels used in diagnostic criteria for adult secondary HLH are too low to be clinically relevant, and efforts should be undertaken to revise them upward. Similar reappraisals should be taken of the other criteria used to diagnose adult HLH.

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“…The HLH-2004 diagnostic criteria require that 5 of 8 of the following criteria are met: (1) fever, (2) splenomegaly, (3) cytopenias, (4) hypertriglyceridemia and/or hypofibrinogenemia, (5) hemophagocytosis in the bone marrow, spleen, or lymph nodes, (6) low or absent NK cell activity, (7) hyperferritinemia, and (8) high levels of soluble IL-2R. Though the HLH-2004 criteria were developed in the pediatric population, their use has been extended to the adult patient population in clinical practice empirically [17]. Tamamyan et al [16] proposed additional criteria for adult patients with malignancy-associated HLH in whom a partial laboratory evaluation may preclude the diagnosis of HLH.…”

Section: Methodsmentioning

confidence: 99%

Limited Role of Random Skin Biopsy in the Diagnosis of Intravascular Lymphoma in Adult Patients with Hemophagocytic Lymphohistiocytosis

Cho

Sheu²,

Kuo³

et al. 2017

Acta Haematol

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Background/Aims: This study examined the role of random normal skin biopsy in the diagnosis of intravascular lymphoma (IVL) in adult Western patients with clinically diagnosed hemophagocytic lymphohistiocytosis (HLH). Methods: In a retrospective chart review study, we analyzed a total of 59 skin biopsies that were performed to diagnose IVL in 21 adult patients with HLH seen at Stanford Hospital between 2004 and 2016. Results: Out of the 59 skin biopsies, 42 were taken from clinically normal-appearing skin and 17 from clinically abnormal-appearing skin. None of the 59 biopsies revealed a diagnosis of primary or metastatic malignancy, regardless of the malignancy history, clinical presentation, and biopsy and histopathologic characteristics. A review of 8 positive IVL cases at Stanford Hospital including 1 case associated with HLH showed 1 positive diagnosis by a targeted skin biopsy and other positive diagnoses by bone marrow (n = 4), lung (n = 2), brain (n = 2), muscle (n = 1), and nerve (n = 1). Conclusion: Random skin biopsies have a limited role in diagnosing IVL in adult patients with HLH, in the setting of a single academic institution in the USA. A review of the literature emphasizes the role of a full body skin exam with a selective skin biopsy in these patients.

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Performances of the H-Score for Diagnosis of Hemophagocytic Lymphohistiocytosis in Adult and Pediatric Patients

Cited by 162 publications

References 13 publications

Hemophagocytic Lymphohistiocytosis, an Unclear Nosologic Entity: Case Report of an Adult Man with Rising of Amylase and Lipase and Spinal Cord Infiltration

Hemophagocytic Lymphohistiocytosis, an Unclear Nosologic Entity: Case Report of an Adult Man with Rising of Amylase and Lipase and Spinal Cord Infiltration

Reevaluating the role of ferritin in the diagnosis of adult secondary hemophagocytic lymphohistiocytosis

Limited Role of Random Skin Biopsy in the Diagnosis of Intravascular Lymphoma in Adult Patients with Hemophagocytic Lymphohistiocytosis

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