An unusual etiology of thrombotic microangiopathy in an adolescent male: Answers
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CLINICAL QUIZ
An unusual etiology of thrombotic microangiopathy in an adolescent male: Answers Charushree Prasad 1 & Deborah M. Levy 2,3
&
Diane Hebert 1,3 & Rose Chami 4,5 & Chia Wei Teoh 1,3
Received: 12 February 2020 / Accepted: 20 February 2020 # IPNA 2020
Keywords Thrombotic microangiopathy . Thrombotic thrombocytopenic purpura . Childhood-onset systemic lupus erythematosus . Adolescent
Answers to initial questions 1. The diagnosis of thrombotic microangiopathy (TMA) is reflected by the triad of thrombocytopenia, microangiopathic hemolytic anemia, and end-organ injury, particularly acute kidney injury [1]. The differential diagnosis is broad and can encompass a variety of conditions leading to primary or secondary TMA (Fig. 1) [1]: (a) Primary hereditary TMA (thrombotic thrombocytopenic purpura (TTP) secondary to ADAMTS13 mutation, atypical hemolytic-uremic syndrome (aHUS) due to a complement gene mutation, diacylglycerol kinase epsilon (DGKE) TMA, Cobalamin C deficiency); (b) Primary acquired TMA (TTP with ADAMTS13 autoantibody, aHUS with Factor H autoantibody); (c) Secondary TMA post solid-organ transplant, post hematopoietic stem cell transplant, drug-induced, This refers to the article that can be found at https://doi.org/10.1007/ s00467-020-04514-6. * Chia Wei Teoh [email protected] 1
Division of Nephrology, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada
2
Division of Rheumatology, The Hospital for Sick Children, Toronto, Ontario, Canada
3
Department of Paediatrics, Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada
4
Department of Paediatric Laboratory Medicine, The Hospital for Sick Children, Toronto, Ontario, Canada
5
Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada
pregnancy and HELLP (syndrome of hemolysis, elevated liver enzymes, and low platelets) associated, glomerular disease associated with ANCAassociated vasculitis/membranous nephropathy/IgA nephropathy/C3 glomerulopathy etc., hypertension associated, autoimmune disease associated, i.e., with systemic lupus erythematosus (SLE), antiphospholipid antibody syndrome, and malignancy-associated TMA); and (d) Infection-associated TMA (Shiga toxin-producing Escherichia coli-HUS (STEC-HUS), pneumococcal HUS, HIV associated, and other associated infections). 2. The diagnostic workup should be approached by systematically examining each potential etiology for TMA and performing appropriate investigations. Detailed clinical history and physical examination may direct the clinician towards a specific diagnosis. In clinical practice, rapid decision-making is critical, with a severely ill patient for whom treatment decisions may be required prior to the availability of results of the diagnostic workup. Supportive management including vigilant monitoring, careful fluid and electrolyte management, judicious blood and platelet transfusion, and renal replacement therapy when clinically indicated should be instituted in parallel with
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