Transfus Apher Sci
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Multiple myeloma is the leading indication of autologous hematopoietic cell transplantation (AHCT) worldwide. Hematopoietic progenitor cell mobilization (HPCM) is the first step of a successful AHCT. A minimum of 2×106 CD34+ cells/kg are needed for successful engraftment. ⋯ Plerixafor is successfully integrated into both growth factor-only and cyclophosphamide and growth factor mobilization strategies with significantly reducing the mobilization failure rate in myeloma patients. The best strategy to mobilize progenitor cells with the highest yield and lowest toxicity and cost in patients with multiple myeloma has not yet been determined. This review aims to summarize the current status of art mobilization in myeloma comparing the pros and cons of different mobilization strategies.
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In adults with sickle cell disease (SCD), the effects of the red cell storage lesion are not well defined. The objectives of this study were to: (1) describe the distribution of storage ages provided to adults with SCD, and (2) evaluate clinical outcomes associated with storage age. ⋯ In a cohort of chronically transfused adults with SCD, we provide evidence that receipt of older units is associated with a higher rate of admission for infection. Prospective studies will need to validate these data and explore potential mechanisms by which these older units promote infection.
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Antiphospholipid antibody syndrome (APS) is an enigmatic heterogeneous disorder despite several revelations in its pathobiology. Renal transplantation in patients with APS has been notoriously difficult due to the high risk of development of thrombotic microangiopathy (TMA), which is often refractory to conventional treatment modalities such as aggressive anticoagulation and plasmapheresis. We describe a case of a 58-year-old male with secondary APS undergoing living unrelated renal transplantation for end-stage renal disease from lupus nephritis. ⋯ Our experience adds to the limited body of literature suggesting the role of complement inhibition in facilitating renal transplantation in patients with APS spectrum of disorders, thus adding a new tool to the therapeutic armamentarium for this difficult disease. The optimal treatment schedule and long term safety data for eculizumab in complement mediated TMA is still unclear. The search for an optimal biomarker to help guide treatment duration is an area of active research.
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In the absence of a specific laboratory test of envenomation, there is a need for an alternative mechanism for the early recognition of envenomation following hematotoxic snake-bite in tropical countries. Abnormalities of clotting are commonly associated with hematotoxic snake bite either due to systemic envenomation or due to the release of an inappropriate tourniquet applied as 'first-aid' often by the rural people before presentation to the hospital. Thromboelastography (TEG) has been used to monitor the coagulation abnormalities in various clinical scenarios. Here we narrate our experience where regular monitoring of hemostasis by TEG had helped us to successfully manage a case of hematotoxic snake-bite in a 45 year old male patient from rural India.
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Daily therapeutic plasma exchange (TPE) transformed the historically fatal prognosis of acquired, anti-ADAMTS13 antibody-mediated thrombotic thrombocytopenic purpura (TTP), leading to the current overall survival rates of >80%. However, relapses occur in up to 40% of patients and refractory disease with fatal outcomes still occurs. In this context, the introduction of rituximab has probably been the second major breakthrough in TTP management. ⋯ In this life-threatening disease, relapses can be efficiently prevented in patients with a severe acquired ADAMTS13 deficiency and otherwise in remission with the use of rituximab. In the coming years, the TTP therapeutic landscape should be enriched by original strategies stemming from clinical experience and new agents that are currently being evaluated in large, ideally international, clinical trials. Promising agents under evaluation include N-acetylcysteine, bortezomib, recombinant ADAMTS13 and caplacizumab, an inhibitor of the glycoprotein-Ib/IX-von Willebrand factor axis.