@article{Stavrou_Lazarus_2010, title={THROMBOTIC MICROANGIOPATHY IN HAEMATOPOIETIC CELL TRANSPLANTATION:AN UPDATE}, volume={2}, url={https://www.mjhid.org/mjhid/article/view/2010.033}, DOI={10.4084/mjhid.2010.033}, abstractNote={<span style="font-size: 10pt; line-height: 115%; font-family: ">Allogeneic hematopoietic cell transplantation (HCT) represents a vital procedure for patients with various hematologic conditions. Despite advances in the field, HCT carries significant morbidity and mortality. A rare but potentially devastating complication is transplantation-associated thrombotic microangiopathy (TA-TMA). In contrast to idiopathic TTP, whose etiology is attributed to deficient activity of ADAMTS13, (a member of the <span style="text-decoration: underline;">A</span> <span style="text-decoration: underline;">D</span>isintegrin <span style="text-decoration: underline;">A</span>nd <span style="text-decoration: underline;">M</span>etalloprotease with <span style="text-decoration: underline;">T</span>hrombo<span style="text-decoration: underline;">s</span>pondin 1 repeats family of metalloproteases), patients with TA-TMA have > 5% ADAMTS13 activity. Pathophysiologic mechanisms associated with TA-TMA, include loss of endothelial cell integrity induced by intensive conditioning regimens, immunosuppressive therapy, irradiation, infections and graft-versus-host (GVHD) disease. The reported incidence of TA-TMA ranges from 0.5% to 75%, reflecting the difficulty of accurate diagnosis in these patients. Two different groups have proposed consensus definitions for TA-TMA, yet they fail to distinguish the primary syndrome from secondary causes such as infections or medication exposure. Despite treatment, mortality rate in TA-TMA ranges between 60% to 90%. The treatment strategies for TA-TMA remain challenging. Calcineurin inhibitors should be discontinued and replaced with alternative immunosuppressive agents.<span style="mso-spacerun: yes;">  </span>Daclizumab, a humanized monoclonal anti-CD25 antibody, has shown promising results in the treatment of TA-TMA. Rituximab or the addition of defibrotide, have been reported to induce remission in this patient population. In general, plasma exchange is not recommended.<span style="mso-spacerun: yes;">    </span></span>}, number={3}, journal={Mediterranean Journal of Hematology and Infectious Diseases}, author={Stavrou, Evi and Lazarus, Hillard Michael}, year={2010}, month={Oct.}, pages={e2010033} }