Epidemiology and outcomes of pediatric transplant‐associated thrombotic microangiopathy in Hong Kong

Chan, Wilson Y K; Tak, Alison Lap; Chan, Eugene Yu-hin; Kan, Amanda; Ng, Wai Fu; Lee, Pamela; Cheuk, Dkl; Chiang, Alan Kwok Shing; Leung, Wing; Chan, Godfrey Chi-Fung

doi:10.1111/petr.14366

Cited by 3 publications

(1 citation statement)

References 49 publications

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“…1 Although infections and graft-versus-host disease (GVHD) have historically been the major contributors to transplant-related nonrelapse mortality, 2,3 transplant-associated thrombotic microangiopathy (TA-TMA) has increasingly become recognized as an independent entity with incidence rates of 8.2% to 39%. [4][5][6][7] It is characterized by the development of post-transplant hemolysis with schistocytes, thrombocytopenia, and organ dysfunction. Kidneys are the most common organs to become involved; intestinal involvement is also recognized.…”

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confidence: 99%

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

Nusrat,

Davis,

MacDougall

et al. 2023

J Intensive Care Med

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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.

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