Clinical significance of bone marrow hemophagocytosis in adult patients with malignancy and non-malignancy-induced hemophagocytic lymphohistiocytosis

Abstract

Bone marrow hemophagocytosis is a frequently observed but not mandatory finding for the diagnosis of hemophagocytic lymphohistiocytosis (HLH). However, the impact of bone marrow hemophagocytosis on the diagnosis of HLH is still not clear in adult patients. Thus, we retrospectively analyzed adult patients with bone marrow hemophagocytosis between 2000 and 2014 to determine its clinical significance. Among 264 patients with bone marrow hemophagocytosis, malignant disorders were the predominant underlying cause (n = 170, 64 %), especially T/NK-cell (n = 88) and B-cell (n = 45) lymphomas compared to infectious disease (48/264, 18 %). The data for HLH-2004 diagnostic criteria was available in 182 patients, and only 29 % (77/264) of patients with ≥ five positive criteria could be diagnosed with HLH. Among the criteria for the diagnosis of HLH, increased serum ferritin (89 %) was more common than hypofibrinogenemia, hypertriglyceridemia, and bicytopenia (<40 %). The median overall survival was worse in patients with malignancy (9.0 months, 95 % confidence interval [CI] 5.6-12.5) than in those with non-malignant disorders (71.8 months, 95 % CI 56.5-87.1, P < 0.001). In patients with malignancy, the overall survival of patients fulfilling the HLH-2004 criteria was significantly worse than patients who did not (P < 0.001). In conclusion, our results suggest that bone marrow hemophagocytosis might be an important finding in the diagnosis of HLH in adult patients. Considering the high incidence of malignancy as a predisposing disorder for HLH, immediate evaluation should be performed in adult patients with bone marrow hemophagocytosis.

A Rare Case of Hemophagocytic Lymphohistiocytosis Associated with Parvovirus B19 Infection

“…However, it is not pathognomonic for HLH. It can be seen in a few other diseases including viral infections, especially EBV, and hematologic malignancies such as non-Hodgkin’s-lymphoma [10]. Its presence is estimated to have a sensitivity of 80% to 83% and a specificity of only 60% to diagnose HLH [4].…”

Weathering a Cytokine Storm

“… 1 - 3 It has been shown through multiple studies that malignancy-associated HLH carries a far worse prognosis than HLH associated with infections. 1 - 3 , 6 , 7 …”

The Maze of Diagnosing Hemophagocytic Lymphohistiocytosis: Single-Center Experience of a Series of 6 Clinical Cases

“…In all 6 cases, the ferritin level was excessively elevated, which has also been observed in 89% of 264 patients diagnosed with bone marrow hemophagocytosis [9]. No differential diagnosis exists that goes along with comparably high ferritin levels without a history of polytransfusions.…”