2016
DOI: 10.1503/cmaj.160142
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Thrombotic microangiopathies: a general approach to diagnosis and management

Abstract: U nderstanding of the pathophysiology of thrombotic microangiopathies-a group of rare yet life-threatening hematologic conditions-has evolved in recent years along with better access to diagnostic testing. Identifying secondary causes requires a thorough diagnostic workup, but appropriate treatment of the underlying condition usually leads to resolution. We review evidence on the pathophysiology, diagnosis and management of this group of conditions for the generalist (Box 1). We summarize disease mechanisms an… Show more

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Cited by 124 publications
(160 citation statements)
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“…Among drugs associated with TMA, there is a strong association with gemcitabine and calcineurin inhibitors [ 4 , 5 ], frequently used as chemotherapy and immunosuppressants in solid-organ transplant recipients, respectively. In the case of malignancies, the majority of cases represent adenocarcinomas, with another large part being secondary to hematologic diseases.…”
Section: Discussionmentioning
confidence: 99%
See 1 more Smart Citation
“…Among drugs associated with TMA, there is a strong association with gemcitabine and calcineurin inhibitors [ 4 , 5 ], frequently used as chemotherapy and immunosuppressants in solid-organ transplant recipients, respectively. In the case of malignancies, the majority of cases represent adenocarcinomas, with another large part being secondary to hematologic diseases.…”
Section: Discussionmentioning
confidence: 99%
“…Regardless of the pathophysiology, it is important to recognize early signs of this disease, due to its high mortality rate in case of not administering appropriate treatment. Despite the implication of a qualitative or quantitative deficiency of the enzyme ADAMTS13 in the pathophysiology of the disease, the clinicopathological findings of hemolytic anemia and thrombocytopenia in the context of certain diseases or exposure to medications or infections should lead to suspicion of this entity [ 5 ].…”
Section: Discussionmentioning
confidence: 99%
“…The other changes (eg the inflammatory response leading to pulmonary fibrosis) are secondary to the vascular/capillary damage caused by alveolar capillary thrombosis/microangiopathy. Therapy directed at thrombotic microangiopathy (such as plasma exchange/plasma infusion) should be useful in treating paraquat toxicity (6,12,(14)(15)(16).…”
Section: Discussionmentioning
confidence: 99%
“…Localized microangiopathy to be defined histologically is, in this sense, similar to kidney-limited thrombotic microangiopathy. [ 21 , 22 ] It should be discriminated from systemic thrombotic microangiopathy characterized by microangiopathic hemolytic anemia and thrombotic thrombocytopenia [ 23 ] ; however, microangiopathic hemolytic anemia and thrombotic thrombocytopenia in the patients with extremely sever PAH were previously described in the literatures. [ 4 , 5 ]…”
Section: Discussionmentioning
confidence: 99%