Secondary Hemophagocytic Syndrome

Introduction: Hemophagocytic lymphohistiocytosis (HLH) corresponds to a wide array of potentially fatal hyper-inflammatory diseases involving pathologic immune activation and engulfment of hematopoietic cells by activated macrophages. These disorders have common clinical and laboratorial features, such as severe cytopenias, fever, hepatosplenomegaly and hyperferritinemia, leading to a dismal prognosis when treatment is delayed. Secondary hemophagocytic syndromes may develop as a result of strong immunological activation of the mononuclear phagocyte system by underlying conditions, such as infection, autoimmune diseases, malignancies and metabolic disorders. Mortality rates are high, even with proper treatments, and can reach up to 50%, usually within the first two months of the diagnosis. Diagnosis of this condition is difficult and requires a high degree of suspicion, since the diagnostic criteria are non-specific. Up to 30% of patients with confirmed hemophagocytic syndrome do not show this morphologic aspect in bone marrow examination. We report the data of our institution, regarding the clinical aspects, treatment and outcome of patients with confirmed hemophagocytosis in bone marrow aspiration analysis.