Thrombotic Microangiopathy After Hematopoietic Stem Cell and Solid Organ Transplantation: A Review for Intensive Care Physicians

Author:

Nusrat Sanober1,Davis Hugh2,MacDougall Kira1ORCID,George James N.1,Nakamura Ryotaro3,Borogovac Azra4ORCID

Affiliation:

1. Department of Medicine, Division of Hematology-Oncology, University of Oklahoma Health Sciences Center, Oklahoma City, OK, USA

2. Division of Pulmonary and Critical Care Medicine, City of Hope, Duarte, CA, USA

3. Hematology and Hematopoietic Cell Transplantation, City of Hope, Duarte, CA, USA

4. Department of Hematology and Hematopoietic Cell Transplantation, Lennar Foundation Cancer Center, City of Hope, Irvine, CA, USA

Abstract

Intensive care physicians may assume the primary care of patients with transplant-associated thrombotic microangiopathy (TA-TMA), an uncommon but potentially critical complication of hematopoietic stem cell transplants (HSCTs) and solid organ transplants. TA-TMA can have a dramatic presentation with multiple organ dysfunction syndrome (MODS) associated with high morbidity and mortality. The typical presenting clinical features are hemolytic anemia, thrombocytopenia, refractory hypertension, proteinuria and worsening renal failure. Intestinal involvement, with abdominal pain, nausea and vomiting, gastrointestinal bleeding, and ascites are also common. Cardiopulmonary involvement may develop from various causes including pulmonary arteriolar hypertension, pleural and pericardial effusions, and diffuse alveolar hemorrhage. Due to other often concurrent complications after HSCT, early diagnosis and effective management of TA-TMA may be challenging. Close collaboration between ICU and transplant physicians, along with other relevant specialists, is needed to best manage these patients. There are currently no approved therapies for the treatment of TA-TMA. Plasma exchange and rituximab are not recommended unless circulating factor H (CFH) antibodies or thrombotic thrombocytopenic purpura (TTP; ADAMTS activity < 10%) are diagnosed or highly suspected. The role of the complement pathway activation in the pathophysiology of TA-TMA has led to the successful use of targeted complement inhibitors, such as eculizumab. However, the relatively larger studies using eculizumab have been mostly conducted in the pediatric population with limited data on the adult population. This review is focused on the role of intensive care physicians to emphasize the clinical approach to patients with suspected TA-TMA and to discuss diagnosis and treatment strategies.

Publisher

SAGE Publications

Subject

Critical Care and Intensive Care Medicine

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