Understanding thrombotic microangiopathies in children
Understanding the Disease
- 524 Downloads
Thrombotic microangiopathy (TMA) is an ultra-rare syndrome. The incidence in children is estimated to be ~ 3.0 cases/10 6 population per year (Fig. 1). Very importantly, TMA belongs to the Thrombocytopenia Associated-Multi Organ Failure (TAMOF) syndromes and, therefore, its diagnosis should be considered in critically-ill children. Thus, intensive care physicians and nurses should be familiar with this rare but specific cause of TAMOF. TMA is life-threatening, resulting from ischemic multi-organ failure and characterised by its diversity and high ICU mortality rate, ~ 20%, despite appropriate treatment [ 1, 2]. The common features for TMA are microangiopathic haemolytic anemia (haemolysis, elevated lactate dehydrogenase, reduced haptoglobin, and fragmentation of red blood cells) and thrombocytopenia. Organ injury, associated with disseminated thrombi in the microcirculation, further supports the diagnosis of TMA. Differential diagnosis includes idiopathic thrombocytopenic purpura (ITP),...
Compliance with ethical standards
Conflicts of interest
BSJ is recipient of the MaRIH research grant Alexion 2016; AV is a member of the French Advisory board on Caplacizumab (Ablynx); XLZ is a consultant for Ablynx and a member of the speakers’ bureau for Alexion.
- 2.George JN, Al-Nouri ZL (2012) Diagnostic and therapeutic challenges in the thrombotic thrombocytopenic purpura and hemolytic uremic syndromes. Am Soc Hematol Educ Progr 2012:604–609Google Scholar
© Springer-Verlag GmbH Germany, part of Springer Nature and ESICM 2018