Acquired hemophagocytic syndrome in a patient with synovial sarcoma: A case report

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by severe hyperinflammation due to an overwhelming ineffective immune response to different triggers. Most important symptoms are fever, hepatosplenomegaly and cytopenias. Biochemical signs include elevated ferritin, hypertriglyceridemia and low fibrinogen. Hemophagocytosis in the bone marrow is a hallmark of this syndrome. Based on the pathogenetic mechanism, it can be classified into primary (inherited) or secondary (acquired) HLH. We report, to our knowledge, the first case of acquired hemophagocytic syndrome that arose in a 20-year-old man affected by synovial sarcoma as a complication during chemotherapy. The importance of this case report resides mainly in the difficulty of identifying a rare syndrome, as the hemophagocytic lymphohistiocytosis is, which can gather and justify several unspecific clinical and biochemical signs and symptoms. Hardly, prolonged fever, hepatosplenomegaly and a lasted cytopenia arising in a patients with solid tumor (a synovial sarcoma in our case) during chemotherapy, not directly attributable and not fully justifiable by infections, cytotoxic treatment or by the cancer itself, lead clinicians assuming the hemophagocytosis as a possible etiology. Therefore, describing a peculiar case, which promptly responded to corticosteroid therapy, can help to bring about an early diagnosis and set quickly an adequate treatment so as to change the patient's prognosis.