We conducted a study to assess the incidence and outcome of hemophagocytic histiocytosis (HH) in thrombocytopenic patients with sepsis syndrome or septic shock and to define the possible associations between HH, disseminated intravascular coagulation (DIC), and platelet-associated IgG (PAIgG) in promoting thrombocytopenia. Twenty immunocompetent thrombocytopenic patients were included. Bone marrow aspirates were obtained from each patient to identify hemophagocytic histiocytes. Coagulation parameters, PAIgG levels, and bacterial and viral infections were studied. Twelve patients with HH were identified. The presence of DIC and of PAIgG were often associated with this disease. No herpesvirus infection was demonstrated. Eight of the 12 patients with HH and four of the eight patients without HH died (P = NS). The results of this study suggest that HH could be involved in the development of thrombocytopenia in immunocompetent patients with sepsis syndrome or septic shock. HH does not seem to be associated with increased mortality.

We report the case of a patient with homozygous sickle cell disease (SCD) and chronic myeloid leukaemia (CML) whose vaso-occlusive crises were resolved with imatinib therapy.The patient, born in 1983, underwent a splenectomy at the age of 7 years for acute splenic sequestration. Hospitalization for vaso-occlusive crisis (VOC) occurred about once a year until 2007. The patient was never treated with hydroxycarbamide. In 2008, the patient was hospitalized five times for bone VOC with high fever (40°C) but no clinical signs of infection and negative microbiologic cultures were found; the white blood cell (WBC) count was elevated to 43AE2 ·

No abstract

Summary.We report four patients with Waldenstro Èm's macroglobulinaemia with an unusual neurologic complication, neurolymphomatosis, characterized by meningeal and root nerve in®ltration by lymphoplasmacytic cells. Patients presented with rapidly progressive leg proximal weakness. Examination of cerebrospinal¯uid disclosed lymphoplasmacytic cells. Magnetic resonance imaging of the lumbar spine was suggestive of a tissular in®ltration of leptomeninges and nerve roots. Chemotherapy and irradiation of involved tissues led to a remarkable and sustained neurological improvement.

No abstract