ABSTRACT

We retrospectively reviewed 15 patients diagnosed and treated in our clinic between 2010 and 2019. Statistical analysis was performed with kruskal-wallis test.

The median age of diagnosis of 15 patients diagnosed and treated in our clinic was 18 months (1 month-17 years). The female to male ratio was 6/9. Fever was seen in all patients, hepatosplenomegaly was 80%. The mean ferritin value was 33.927 ± 51.461μ / l (964-201.074). In our patient group, consanguineous marriage rate was 53% (n = 8) and it was high and the factor was EBV in all patients who developed secondary to infection. 33% (n = 5) of the cases were primary and 67% (n = 10) were secondary. 40% (n = 4) of secondary cases had malignancy in etiology, 50% (n = 5) infection and 10% (n = 1) metabolic disease.

HLH is important for early diagnosis in cases with persistent high fever, hepatosplenomegaly and high ferritin conditions in our country. In cases presenting with HLH, malignancy should not be forgotten in etiology besides primary causes.

Hemophagocytic lymphohistiocytosis (HLH) is a rare inflammatory syndrome resulting from uncontrolled proliferation in the monocyte / macrophage system. It is divided into two classes as familial (primary) and secondary. Since the rate of consanguineous marriage in our country is high with 29.2%, the frequency of HLH is also increasing. Secondary HLH can be seen at any age, but its true incidence is unknown. It can accompany malignancies, infections and inflammatory processes. Major clinical findings are persistent high fever, cytopenia, splenomegaly and / or hepatomegaly. The first finding is usually fever. The goal of treatment is to stop abnormal inflammation and treat the underlying cause.