Performance of HScore in Reactive Hemophagocytic Lymphohistiocytosis
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ORIGINAL ARTICLE
Performance of HScore in Reactive Hemophagocytic Lymphohistiocytosis Neerav Khare1 • Sree Rekha Jinkala2
•
Srikanta Kanungo3
Received: 17 March 2020 / Accepted: 26 August 2020 Ó Indian Society of Hematology and Blood Transfusion 2020
Abstract Hemophagocytic lymphohistiocytosis (HLH) is a life threatening systemic hyperinflammatory disorder that leads to multiple organ damage. The most widely used diagnostic guidelines are the HLH 2004 guidelines proposed by the Histiocyte society. Recently, Fardet et al. has proposed a Scoring system, ‘‘HScore’’ for diagnosis of reactive HLH. To test the performance of ‘‘HScore’’ in diagnosis of reactive HLH and compare the credibility of the same with the existing gold standard 2004 guidelines in a cohort of Indian patients. This was a descriptive study of cases referred to the department of Pathology for bone marrow examination with suspicion or diagnosis of HLH from Jan 2015 to June 2017. The clinical records of these patients were analysed and diagnosed as positive and negative for HLH using the HLH 2004 guidelines and HScore, the scoring system developed by Fardet et al. Fifty cases fulfilled the inclusion criteria. The variables fever, hepato and /or splenomegaly, high triglyceride levels and marrow hemophagocytosis are highly significant to predict an individuals risk of HLH. We propose a cut-off of C 166 for the diagnosis of HLH by using HScore. If 4 of the 7 criteria are fulfilled by HLH 2004 guidelines, there is an 80% probability of having HLH by HScore. The HScore is & Sree Rekha Jinkala [email protected] 1
VI Semester MBBS, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry 605006, India
2
Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry 605006, India
3
Department of Preventive and Social Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry 605006, India
less restrictive in confirming a diagnosis of HLH compared to the HLH 2004 guidelines. HScore is a simple, and cost effective tool for diagnosis of reactive HLH. The higher the HScore, more is the probability of HLH. Keywords Hemophagocytic lymphohistiocytosis Hscore Hemophagocytosis
Introduction Hemophagocytic lymphohistiocytosis (HLH) or Hemophagocytic syndrome is a clinical syndrome characterised by persistent activation of mononuclear phagocytic system leading to sustained hyper inflammatory response which can be triggered by underlying immune stimulating disease or a host of genetic factors which predispose individuals towards abnormal activation of the immune system [1]. There are two categories of the disease: primary (familial) and secondary (acquired or reactive). Primary HLH has a genetic basis, i.e., is caused by genetic defects in cytotoxic function of T-lymphocytes and Natural killer (NK) cells. This type primarily occurs in children [2]. Mutations occur most often in the perforin gene or in genes important for the functio
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