Pericardial effusion in pediatric SCT recipients with thrombotic microangiopathy

Lerner, David; Dandoy, Christopher E.; Hirsch, Russel; Laskin, Benjamin L.; Davies, Stella M.; Jodele, Sonata

doi:10.1038/bmt.2014.40

Cited by 35 publications

(32 citation statements)

References 10 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The reported incidence of PEF in retrospective cohorts has varied from 0.2% to 19% in patients after HSCT [7,8]. In our institution, we found a high incidence of PEFs in patients with transplant-associated thrombotic microangiopathy (TA-TMA) [9]. LV dysfunction has been reported in patients after high-dose cyclophosphamide and anthracyclines, and patients with LV dysfunction can initially be asymptomatic [10–12].…”

Section: Introductionmentioning

confidence: 92%

Abnormal Echocardiography 7 Days after Stem Cell Transplantation May Be an Early Indicator of Thrombotic Microangiopathy

Dandoy

Davies

Hirsch

et al. 2015

Biology of Blood and Marrow Transplantation

View full text Add to dashboard Cite

Cardiac complications after hematopoietic stem cell transplantation (HSCT) can lead to significant morbidity and mortality. Cardiac evaluation during the first 100 days after HSCT is usually performed only if clinically indicated, and no studies have examined whether routine screening is beneficial in this patient population at high risk for tissue injury. We conducted a single-center prospective clinical study to screen for cardiac complications in pediatric and young adult patients. One hundred consecutive HSCT patients underwent scheduled echocardiographic screening on day +7 after transplantation, independent of their clinical condition. At least 1 abnormality was identified in 30% of cases. Seventeen children had a pericardial effusion, 13 elevated right ventricular pressure, and 3 reduced left ventricular function. Survival was reduced in children with any echocardiographic abnormality at day 7 (67% versus 80% in those with and without, respectively, abnormality, P = .073). Moreover, raised right ventricular pressure at day +7 was significantly associated with transplant-associated thrombotic microangiopathy (TA-TMA; P = .004) and may indicate early vascular injury in the lungs. These data suggest that echocardiography 7 days after HSCT can detect early cardiac complications of HSCT and may identify early vascular injury associated with TA-TMA.

show abstract

Section: Introductionmentioning

confidence: 92%

Abnormal Echocardiography 7 Days after Stem Cell Transplantation May Be an Early Indicator of Thrombotic Microangiopathy

Dandoy

Davies

Hirsch

et al. 2015

Biology of Blood and Marrow Transplantation

View full text Add to dashboard Cite

show abstract

“…Pericardial effusion has been strongly associated with TA-TMA in HSCT patients [33,34]. Pericardial effusion can be suspected on chest X-ray or may be detected on echocardiographic screening even in asymptomatic patients.…”

Section: Refined Ta-tma Diagnostic Criteriamentioning

confidence: 99%

New approaches in the diagnosis, pathophysiology, and treatment of pediatric hematopoietic stem cell transplantation-associated thrombotic microangiopathy

Jodele

Dandoy

Myers

et al. 2016

Transfusion and Apheresis Science

101

View full text Add to dashboard Cite

Hematopoietic stem cell transplantation (HSCT)-associated thrombotic microangiopathy (TA-TMA) is an understudied complication of HSCT that significantly affects transplant-related morbidity and mortality. Over the past several decades, the cause of TA-TMA has remained unknown, limiting treatment options to non-specific therapies adapted from other diseases. Recent prospective studies dedicated to the study of TA-TMA have provided new insights into the pathogenesis of, and genetic susceptibility to TA-TMA, raising awareness of this important transplant complication and allowing for the identification of potentially novel therapeutic targets. Specifically, many patients with TA-TMA develop multi-organ tissue injury through endothelial damage mediated by the activation of the complement pathway, leading to rational therapeutic strategies including complement blockade. This new knowledge has the potential to favorably influence clinical practice and change the standard of care for how patients with TA-TMA are managed. In this review, we summarize novel approaches to the recognition and management of TA-TMA, using case examples to illustrate key clinical points that hopefully lead to improved short and long-term outcomes for these complex HSCT patients, who remain at significant risk for treatment-related morbidity and mortality.

show abstract

“…Multiorgan involvement typically manifests as pulmonary hypertension, polyserositis, gastrointestinal symptoms, central nervous system injury, and renal impairment. [7][8][9][10] The mechanisms causing endothelial injury in patients with TMA are only beginning to be studied. We recently reported that activation of the complement system may be involved in the pathogenesis of TMA and have described the potential therapeutic benefit of terminal complement blockade by eculizumab.…”

Section: Introductionmentioning

confidence: 99%

Diagnostic and risk criteria for HSCT-associated thrombotic microangiopathy: a study in children and young adults

Jodele

Davies

Lane

et al. 2014

Blood

342

443

View full text Add to dashboard Cite

• Proteinuria and elevated markers of complement activation at TMA diagnosis are associated with poor outcome.• Clinical interventions should be considered in HSCT patients with these high-risk features at the time TMA is diagnosed.Transplant-associated thrombotic microangiopathy (TMA) leads to generalized endothelial dysfunction that can progress to multiorgan injury, and severe cases are associated with poor outcomes after hematopoietic stem cell transplantation (HSCT). Identifying patients at highest risk for severe disease is challenging. We prospectively evaluated 100 consecutive HSCT recipients to determine the incidence of moderate and severe TMA and factors associated with poor overall outcomes. Thirty-nine subjects (39%) met previously published criteria for TMA. Subjects with TMA had a significantly higher nonrelapse mortality (43.6% vs 7.8%, P < .0001) at 1 year post-HSCT compared with those without TMA. Elevated lactate dehydrogenase, proteinuria on routine urinalysis, and hypertension were the earliest markers of TMA. Proteinuria (>30 mg/dL) and evidence of terminal complement activation (elevated sC5b-9) in the blood at the time of TMA diagnosis were associated with very poor survival (<20% at 1 year), whereas all TMA subjects without proteinuria and a normal sC5b-9 serum concentration survived (P < .01). Based on these prospective observations, we conclude that severe TMA occurred in 18% of HSCT recipients in our cohort and propose an algorithm to identify the highest-risk patients who might benefit from prompt clinical interventions. (Blood. 2014;124(4):645-653)

show abstract

Pericardial effusion in pediatric SCT recipients with thrombotic microangiopathy

Cited by 35 publications

References 10 publications

Abnormal Echocardiography 7 Days after Stem Cell Transplantation May Be an Early Indicator of Thrombotic Microangiopathy

Abnormal Echocardiography 7 Days after Stem Cell Transplantation May Be an Early Indicator of Thrombotic Microangiopathy

New approaches in the diagnosis, pathophysiology, and treatment of pediatric hematopoietic stem cell transplantation-associated thrombotic microangiopathy

Diagnostic and risk criteria for HSCT-associated thrombotic microangiopathy: a study in children and young adults

Contact Info

Product

Resources

About