ABSTRACT
AIM: To evaluate clinical, laboratory, imaging findings, and outcomes of adult patients with bone marrow haemophagocytosis (BMH) who meet the diagnostic criteria for haemophagocytic lymphohistiocytosis (HLH) with those who do not meet the criteria. MATERIALS AND METHODS: A pathology database search was performed from 2009 to 2019 to identify adult patients with BMH. Electronic medical records of 41 patients were reviewed to distinguish those who fulfil the HLH-2004 diagnostic guidelines, which identified 22 patients (11 men; mean age, 53.5 years) who met the criteria (HLH+) and 19 patients (13 men; mean age, 54.7 years) who did not meet the criteria (HLH-). Multi-modality imaging was reviewed to record imaging features. Clinical, laboratory, imaging findings, and outcomes were compared between the two groups using Fisher's exact test and Wilcoxon test. RESULTS: Malignancy (non-Hodgkin's lymphoma) was the major trigger for both groups. 86% of HLH+ and 31% of HLH- patients presented with fever. Compared to the HLH- group, the HLH+ group exhibited higher serum ferritin, triglycerides, and lower fibrinogen levels (p<0.05). Alveolar opacities and hepatosplenomegaly were the most common imaging findings identified in both groups. Median overall survival of HLH+ and HLH- were 123.5 (interquartile range [IQR]: 40.7-681.7 days) and 189 days (IQR: 52-1680 days), respectively. Distribution of imaging features and overall survival did not differ between the groups. CONCLUSION: Malignancy is the major trigger for BMH in both HLH+ and HLH- groups. HLH+ and HLH- groups have similar imaging manifestations or clinical outcomes. Therefore, presence of BMH alone is correlated with high morbidity and mortality.
