Catastrophic thrombotic syndromes are characterized by rapid onset of multiple thromboembolic occlusions affecting diverse vascular beds. Patients may have multiple events on presentation, or develop them rapidly over days to weeks. Several disorders can present with this extreme clinical phenotype, including catastrophic antiphospholipid syndrome (APS), atypical presentations of thrombotic thr...

Acute myeloid leukemia is a heterogeneous disease that accounts for approximately 20% of acute leukemias in children and adolescents. Despite the lack of targeted therapy for most subtypes and a dearth of new agents, survival rates have reached approximately 60% for children treated on clinical trials in developed countries. Most of the advances have been accomplished by better risk classificat...

Primary plasma cell leukemia (pPCL) is a rare and aggressive plasma cell proliferative disorder with a very poor prognosis and with distinct biologic, clinical, and laboratory features. Compared with multiple myeloma, pPCL presents more often with extramedullary involvement, anemia, thrombocytopenia, hypercalcemia, elevated serum 2-microglobulin and lactate dehydrogenase levels, as well as impa...

Clostridium difficile infection has been increasing since 2000 in children and in adults. Frequent antibiotics use, comorbidity, and the development of hypervirulent strains have increased the risk of infection. Despite the high carriage rates of C. difficile, infants rarely develop clinical infection. Discontinuing antibiotics and supportive management usually leads to resolution of disease. A...

PURPOSE OF REVIEW The aim of this article is to review new data on the use of antiretroviral therapy in primary HIV infection. RECENT FINDINGS The concept of 'hit HIV hard and early' supporting the use of antiretroviral therapy to treat the early stages of HIV infection has been revisited. Recent demonstration of massive, rapid and largely irreversible HIV-mediated destruction of memory CD4+ ...

Clinical practice must be based on the results of clinical trials, not on theories derived from epidemiology observations. The major trials of blood pressure reduction are described, and some of their shortcomings are discussed. Whenever drug treatment is necessary there will inevitably be a balance between benefits of treatment and unwanted effects of medication. The MRC trial of treatment in ...

This review presents a cohesive approach to treating patients with Gaucher disease. The spectrum of the clinical presentation of the disease is broad, yet heretofore there was only one diseasespecific treatment. In the past 2 years, a global shortage of this product has resulted in reassessment of the “one enzyme–one disease–one therapy” mantra. It has also showcased the multiple levels that en...