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Glycosylated Ferritin as a Marker of Hemophagocytic Syndrome: Comment on the Article by Mecchella et al

We read with great interest the Clinicopathologic Conference by Mecchella et al published recently in Arthritis Care & Research about a patient with amyopathic dermatomyositis, complicated by babesiosis associated with a possible hemophagocytic syndrome (1). However, we consider that the diagnosis of hemophagocytic syndrome is uncertain in the present case. Hemophagocytic syndrome, also named hemophagocytic lymphohistiocytosis (HLH), can be either primary, with a family inheritance or a genetic etiology, or secondary, as associated with malignancies, autoimmune diseases, or infections (2). Diagnostic criteria of secondary HLH have been established, requiring 5 of the 8 following criteria: fever .38.5 C (101.3 F); splenomegaly; cytopenia (affecting at least 2 lineages in the peripheral blood); hypertriglyceridemia; hemophagocytosis in bone marrow, spleen, lymph nodes, or liver; low or absent NK-cell activity, ferritin .500 ng/ml; and elevated sCD25 (2). Indeed, the patient under discussion had only 3 diagnostic criteria for this syndrome (i.e., splenomegaly, cytopenias, and ferritin .500 ng/ml), and all these diagnostic criteria are not specific for HLH. Mild splenomegaly, normocytic hemolytic anemia, thrombocytopenia, and slight decrease of white blood cell count are common findings in the course of babesiosis (3), and a high ferritin level may be detected in multiple conditions. In a French study, high ferritin level was associated with systemic infections, hematologic diseases, malignancies, alcoholism, dialyzed chronic renal failure, liver diseases, hemochromatosis, and systemic inflammatory diseases (4). There was no difference according to the etiology with regard to the ferritin level between 600 and 1,000 ng/ml, 1,000 and 1,500 ng/ml, and .1,500 ng/ml. Moreover, experts consider that ferritin .3,000 ng/ml should raise concern for HLH, and that .10,000 ng/ml is highly suspicious of HLH (2). Rheumatologists are aware that a high ferritin level is a common finding in patients with adult-onset Still’s disease, but also in patients with HLH. In both conditions, the percentage of glycosylated ferritin below 20% has been reported (5,6). Also, the combination of a high ferritin level and low percentage of glycosylated ferritin is considered as a good marker of macrophage activation. In this context, the role of interleukin-1b increasing ferritin synthesis and of liver cell damage with ferritin release by macrophages after erythrophagocytosis is advocated (6). Therefore, could the authors indicate what the glycosylated percentage of ferritin in their patient was since this result could support the diagnosis of HLH? Claude Bachmeyer, MD Christopher Rein, MD Hôpital Tenon, Assistance Publique Hôpitaux de Paris Paris, France