2016
DOI: 10.1093/ajcp/aqw076
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Performances of the H-Score for Diagnosis of Hemophagocytic Lymphohistiocytosis in Adult and Pediatric Patients

Abstract: The adapted HLH-2004 guidelines seem less powerful and H-score seems to be more appropriate for children, which may be due to less significantly marked biological features. For adults, H-score performances are better when determined at presentation. The cutoff value of the H-score should be adapted depending on the target population to obtain optimal specificity.

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Cited by 173 publications
(150 citation statements)
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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%
“…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%
“…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%
“…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%