Accuracy of the Criteria for Hemophagocytic Lymphohistiocytosis

Abstract

Introduction/Objective Regular assessment and analysis of diagnostic criteria for any type of human pathology is a prerequisite for ensuring the highest level of patient care. Hemophagocytic lymphohistiocytosis, also known as hemophagocytic syndrome, is a life-threatening condition. The syndrome can develop in critically ill patients with malignancies, severe infections, during chemotherapy, and may be associated with currently known genetic abnormalities, but this list is incomplete. We draw attention to the accuracy of diagnostic criteria, association with a variety of clinical conditions, pathophysiological mechanisms, and outcomes of the disease. Methods From the medical records in our hospital, we were able to extract several cases over a 10-year period. Based on hemophagocytosis features, our list included 13 patients representing 11 bone marrow aspirates, 4 lymph node biopsies, 1 liver biopsy, and 1 spleen sample; repeated examination of the slides confirmed the accuracy. Analyzing medical documentation, we evaluated the sequence and competence of the criteria used, the time required for diagnosis, management, and outcomes. Results We found that not all criteria were used for diagnosis, and the most sensitive and specific tests were bypassed. The preliminary diagnosis was made by a consultant (a rheumatologist or an oncologist-hematologist) on the 5th day of the hospital stay that delayed treatment. Of all the available treatment options, only a few were used. Conclusion The hemophagocytic syndrome is a very rare and fatal entity, it requires highly sensitive and specific diagnostic criteria for prompt diagnosis, targeted management, and thorough follow up. Every patient admitted to the hospital with a life-threatening condition should be suspected and tested for hemophagocytic syndrome on the first day. The criteria for hemophagocytic lymphohistiocytosis should be revised, with the most sensitive and specific ones are done in 100% of cases. Subsequently, each patient should be tested for the presence of genetic abnormalities that correlate with the syndrome.

Hepatic haemophagocytosis in haematology patients with hepatic dysfunction: prognostic impact and contribution of liver biopsy combined with the haemophagocytic syndrome diagnostic score (HScore)

“…Furthermore detecting HS in patients treated for haematological malignancies is challenging, especially when discriminating between infection‐induced HS that requires immunomodulation and infection‐induced aberrant hyperinflammation 24 . Current scoring systems, based on paediatric haemophagocytic lymphohistiocytosis (HLH) 2004 criteria 25 and the HS diagnostic score (HScore), 26 rely on non‐specific clinical and biological variables and have proven to be unsuitable for HS diagnosis 27 . However, they may be helpful tools in estimating individual risk for reactive HS.…”

“… 24 Current scoring systems, based on paediatric haemophagocytic lymphohistiocytosis (HLH) 2004 criteria 25 and the HS diagnostic score (HScore), 26 rely on non‐specific clinical and biological variables and have proven to be unsuitable for HS diagnosis. 27 However, they may be helpful tools in estimating individual risk for reactive HS.…”