Comparison of Hemophagocytic Lymphohistiocytosis Diagnostic Criteria in Malignancy-Associated Hemophagocytic Lymphohistiocytosis Patients

Abstract

Background Fulfilling diagnostic criteria of hemophagocytic lymphohistiocytosis (HLH) is challenging due to unavailable laboratory tests. Hence, we aimed to reveal malignancy-associated-HLH (M-HLH) patients in our center, which can not be reached in all tests. Material and Methods Nine patients with M-HLH were analyzed retrospectively. Results The median age was 59 years. The distribution of the underlying diseases was like diffuse large B cell lymphoma in 3 patients, acute myeloid leukemia in 2 patients, Hodgkin lymphoma in 2 patients, T cell non-Hodgkin lymphoma in 1 patient, and small cell lung cancer in 1 patient. According to HLH-2004 diagnostic criteria except for soluble CD25 and natural killer activity tests; one patient had 3/6, six patients had 5/6, two patients had 6/6 criteria while the median H-score was 258 at diagnosis. According to Tamamyan et al’s criteria; at the diagnosis, all patients had ≥7 (between 7-12) of 18 parameters. Patients fulfilled ≥5 parameters a median of 15 days (3-52 days) before the diagnosis and on that time six patients had 3/6 criteria of HLH-2004. 88.8% of the patients died. The median duration of survival was 8.5 days (1-18 days). Conclusions Unavailability of the tests in some countries and centers as in ours results in complications to fulfill 5 of 8 criteria and being delayed in diagnosis and treatment. We need to develop more specific and accessible criteria, and grading systems for M-HLH diagnose.

Keywords

• hemophagocytic lymphohistiocytosis
• malignancy
• diagnosis
• mortality

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