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. 2021 May 25;11(6):467.
doi: 10.3390/jpm11060467.

Thrombotic Microangiopathy: Multi-Institutional Review of Pediatric Patients Who Underwent HSCT

Archana Ramgopal  1 , Shiva Sridar  2 , Jignesh Dalal  2 , Ramasubramanian Kalpatthi  1
Affiliations

Thrombotic Microangiopathy: Multi-Institutional Review of Pediatric Patients Who Underwent HSCT

Archana Ramgopal et al. J Pers Med. .

Abstract

Thrombotic microangiopathy (TMA) is a rare but serious complication of hematopoietic stem cell transplantation (HSCT). The purpose of our study is to estimate the incidence, prevalence, and analyze the risk factors and outcome of TMA in children receiving HSCT. Patients under the age of 21 who underwent HSCT at one of the 42 Pediatric Health Information System (PHIS) hospitals from 2000-2012 were analyzed, including demographics, hospitalizations, TMA, and other HSCT-related complications. From 2000 to 2012, a total of 12,369 unique pediatric patients who received HSCT were identified. Among these, 93 (0.8%) children were identified to have the diagnosis of TMA. TMA was significantly associated with allogeneic HSCT, peripheral blood stem cell trasnplants (PBSCT), cytomegalovirus (CMV), human herpes virus 6 (HHV6), fungal infection, graft-versus-host disease (GVHD), and veno-occlusive disease (VOD) (p = 0.01). Multivariate logistic regression analysis of mortality showed only HHV6 was an independent risk factor associated with increased mortality in patients with TMA (hazard ratio: 2.86 [1.01, 8.39], p = 0.05). The prevalence of TMA in our study is 0.8% with a mortality in our pediatric TMA cohort of 30%, which is in contrast to the higher mortality reported in previously published, small-case series. HHV6 emerged as not only a risk factor for TMA but also as associated with increased mortality in these patients.

Keywords: graft-versus-host disease; hematopoietic stem cell transplant; pediatric; thrombotic microangiopathy; transplant-associated thrombotic microangiopathy.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Thrombotic microangiopathy (TMA) trend in pediatric HSCT (hematopoietic stem cell transplantation) recipients, the circles represent average percentages per year with the lines representing the range of percentages.
Figure 2
Figure 2
Overall mortality in HSCT patients with human herpes virus 6 (HHV-6).
Figure 3
Figure 3
Flow chart of patient study group and the population with HHV6 who developed TMA.
Figure 4
Figure 4
Overall mortality in HSCT patients with TMA.
See this image and copyright information in PMC

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