Hemophagocytic syndrome in an elderly with chronic lymphocytic leukemia

An 84-year-old man was hospitalized because of a febrile neutropenia (neutrophils 400/mm). His medical history included chronic lymphocytic leukemia (CLL) known since 2005. After 4 years of indolent clinical course (Binet stage A), lymphadenopathies associated with a high rate of lymphocytes (119 10/mm) appeared, leading to chemotherapy. Chlorambucil was not effective. Therefore, he was administered fludarabine that allowed remission but had to be stopped because of anemia. Six months later, the patient presented with relapsing fever without any clinical nor any chest radiographic abnormalities. Upon admission, he had fever (40 8C), splenomegaly, inguinal and axillary lymphadenopathies, jaundice, and dyspnea. The laboratory values were as follows: hemoglobin 8,3 g/dL, reticulocytes 17,000 10, platelets 28,000/mm, leukocytes 200/mm, lactate dehydrogenase 584 U/L (N: 230–460), hypertriglyceridemia 2,40 g/L, ferritin 39088 IU/L (N: 30–300), alkaline phosphatase (295 IU/L) and increase of aminotransferases (62 ALAT, 99 ASAT IU/L). A thoracoabdomino-pelvic computed tomography scan revealed left axillary, hilar, mediastinal, mesenteric and retroperitoneal lymph nodes with a thoracic alveolar process. Intravenous immunoglobulin with subcutaneous granulocyte colony-stimulating factor was initiated. In addition, the patient received a broad-spectrum empiric antibiotic therapy with intravenous piperacillin/tazobactam (4.0/0.5 g thrice daily), oral ciprofloxacin (500 mg twice daily) and anti-fungal medication with voriconazole (400 mg/day). A bone marrow biopsy led to the diagnosis of hemophacocytic syndrome (HPS) (Fig. 1). Infection screen included Epstein-Barr Elderly