Table 2.

Drugs associated with thrombotic microangiopathy

DrugPossible pathogenesis and treatment
Ticlodopine Specific to ticlodopine and not other thienopyridine derivatives Association with ADAMTS13 antibodies and respond to PEX 
Estrogen-containing drugs, eg, COCP Precipitation of congenital TTP Association with immune TTP cases 
Quinine Antibodies against platelets, leukocytes, erythrocytes, and endothelial cells, leading to damage to endothelial cells
Question role of PEX 
Gemcitabine Dose-dependent endothelial damage, affecting primarily the glomerular arterioles and capillaries Response recorded to eculizumab 
Mitomycin C Delayed onset, dose-dependent toxicity, and cumulative
Microthrombi affect glomerular capillaries and arterioles
Cumulative dose results in irreversible renal damage and no response to PEX 
VEGF inhibitors, eg, bevacizumab and aflibercept TMA features limited to the glomerular structures differentiate anti-VEGF-induced thrombotic microangiopathy from other drug causes Supportive care and drug discontinuation 
Proteosome inhibitors, eg, carfilzomib and bortezomib Occurs at a median of 3 weeks following medication initiation Favorable response to stopping therapy 
Thyrosine kinase inhibitors, eg, carfilzomib and bortezomib Small molecule inhibition of the VEGF receptor
Supportive care and drug discontinuation 
Interferon-β Direct dose-dependent effect causing endothelial hyperplasia, luminal occlusion, and microaneurysm formation Very delayed (years after initiation) Variable recovery 
Calcineurin inhibitors, eg, ciclosporin and tacrolimus Primarily affect glomerular arterioles Reducing the dose/stopping the drug can improve/reverse the TMA 
Platinum-based drugs, eg, oxaliplatin Anemia and thrombocytopenia could result from oxaliplatin-dependent antibodies against erythrocytes and platelets 
Emicizumab (in conjunction with bypassing agents) Unknown, question excess thrombin resulting in endothelial damage Stop drug, symptomatic symptom control
PEX used Drug has been reintroduced on resolution 
DrugPossible pathogenesis and treatment
Ticlodopine Specific to ticlodopine and not other thienopyridine derivatives Association with ADAMTS13 antibodies and respond to PEX 
Estrogen-containing drugs, eg, COCP Precipitation of congenital TTP Association with immune TTP cases 
Quinine Antibodies against platelets, leukocytes, erythrocytes, and endothelial cells, leading to damage to endothelial cells
Question role of PEX 
Gemcitabine Dose-dependent endothelial damage, affecting primarily the glomerular arterioles and capillaries Response recorded to eculizumab 
Mitomycin C Delayed onset, dose-dependent toxicity, and cumulative
Microthrombi affect glomerular capillaries and arterioles
Cumulative dose results in irreversible renal damage and no response to PEX 
VEGF inhibitors, eg, bevacizumab and aflibercept TMA features limited to the glomerular structures differentiate anti-VEGF-induced thrombotic microangiopathy from other drug causes Supportive care and drug discontinuation 
Proteosome inhibitors, eg, carfilzomib and bortezomib Occurs at a median of 3 weeks following medication initiation Favorable response to stopping therapy 
Thyrosine kinase inhibitors, eg, carfilzomib and bortezomib Small molecule inhibition of the VEGF receptor
Supportive care and drug discontinuation 
Interferon-β Direct dose-dependent effect causing endothelial hyperplasia, luminal occlusion, and microaneurysm formation Very delayed (years after initiation) Variable recovery 
Calcineurin inhibitors, eg, ciclosporin and tacrolimus Primarily affect glomerular arterioles Reducing the dose/stopping the drug can improve/reverse the TMA 
Platinum-based drugs, eg, oxaliplatin Anemia and thrombocytopenia could result from oxaliplatin-dependent antibodies against erythrocytes and platelets 
Emicizumab (in conjunction with bypassing agents) Unknown, question excess thrombin resulting in endothelial damage Stop drug, symptomatic symptom control
PEX used Drug has been reintroduced on resolution 

Included are some of the more common drugs documented in association with causing TMA. Most are associated with acute kidney injury and usually present with severe hypertension.

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