Hematology
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Myeloablative allogeneic hematopoietic stem cell transplantation (allo-HSCT) with a matched sibling donor (MSD) in first complete remission (CR1) is an effective consolidation for adult acute lymphoblastic leukemia (ALL), and matched unrelated donor (MUD) is an alternative stem cell source. ⋯ Myeloablative MUD allo-HSCT resulted in comparable survivals with MRD transplant. RIC allo-HSCT produced promising long-term DFS with a low TRM in adult ALL.
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Review Meta Analysis
Preoperative transfusion in patients with sickle cell disease to prevent perioperative complications: a systematic review and meta-analysis.
Sickle cell disease (SCD) is associated with perioperative vascular (SCD-related) and non-vascular complications. To minimize perioperative complications during elective surgery, either exchange blood transfusion or simple blood transfusion can be used. We systematically reviewed the literature and meta-analyzed randomized and observational trials comparing exchange transfusion to simple transfusion, as well as studies comparing preoperative transfusion to no transfusion to assess the relative risk (RR) and benefit of each strategy in sickle cell patients undergoing surgery. ⋯ Based on the current literature, neither preoperative exchange transfusion nor simple transfusion reduces perioperative complications in patients with SCD who are undergoing surgery; however, available studies were underpowered to detect a treatment effect.
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Review Case Reports
Exertional rhabdomyolysis and renal failure in patients with sickle cell trait: is it time to change our approach?
Based upon numerous reported cases and despite widespread beliefs to the contrary, sickle cell trait (SCT) may be deemed a quantifiable risk factor in certain subsets of patients. As a result of common misconceptions regarding SCT, most individuals with the condition are generally not informed regarding the possible consequences of certain activities such as venturing to high altitudes or participating in overly exertional physical activities. Acute exertional rhabdomyolysis is a potentially serious clinical illness and is caused by skeletal muscle injury resulting in the release of myoglobin and other cellular contents, including creatine kinase, into the circulatory system. ⋯ Several case reports have been published since the early 1970s describing significant morbidity and mortality of acute exertional rhabdomyolysis in patients with SCT. We present the case of a 27-year-old male with a past medical history significant only for SCT who presented after a 1.5 mile run with severe exertional rhabdomyolysis and subsequent acute renal failure requiring hemodialysis (HD). In presenting this case, we hope to raise awareness of a possible underlying cause to many cases of exertional rhabdomyolysis and encourage physicians to counsel their patients with SCT in order to avoid the significant morbidity and mortality that may be associated with the condition.
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Thrombotic microangiopathies (TMA) are microvascular occlusive disorders characterized by hemolytic anemia caused by fragmentation of erythrocytes and thrombocytopenia due to increased platelet aggregation and thrombus formation, eventually leading to disturbed microcirculation with reduced organ perfusion. Although several disease states may manifest as TMA, the two most relevant conditions associated with TMA are thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS), characterized by prominent brain or renal lesions, respectively. However, occasionally the clinical distinction between these two conditions can be difficult. In this review, we focus on the epidemiologic and diagnostic criteria as well as on the most recent insights into the pathophysiology and treatment of these two conditions.