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

Authors : Hakkı Onur KIRKIZLAR, Tuğcan ALP KIRKIZLAR, Ufuk DEMİRCİ, Sedanur KARAMAN GULSARAN, Volkan BAS, Elif UMİT, Ahmet Muzaffer DEMİR

Pages : 180-189

Doi:10.46310/tjim.1121064

View : 39 | Download : 12

Publication Date : 2022-10-29

Article Type : Research Paper

Abstract :Background Fulfilling diagnostic criteria of hemophagocytic lymphohistiocytosis insert ignore into journalissuearticles values(HLH); is challenging due to unavailable laboratory tests. Hence, we aimed to reveal malignancy-associated-HLH insert ignore into journalissuearticles values(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 insert ignore into journalissuearticles values(between 7-12); of 18 parameters. Patients fulfilled ≥5 parameters a median of 15 days insert ignore into journalissuearticles values(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 insert ignore into journalissuearticles values(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