TMAs other than TTP
| Disorder . | Pathophysiology . | ASFA category for the role of TPE (see Table 4) . |
|---|---|---|
| TMA–Shiga toxin mediated | Direct endothelial damage with apoptosis due to effects of Shiga toxin | Presence of severe neurologic symptoms, III |
| Absence of severe neurologic symptoms, IV | ||
| TMA–complement mediated | Endothelial damage from unregulated complement activation resulting from the development of anti–factor H autoantibodies or mutations leading to abnormal complement regulatory proteins or abnormal complement factors | Complement factor gene mutations, III |
| Factor H autoantibodies, I | ||
| MCP mutations, III | ||
| TMA–hematopoietic stem cell transplantation associated | Endothelial damage due to infection, chemotherapy, radiation therapy, or graft-versus-host disease due to transplant. Of note, a significant percentage of affected individuals may have complement regulatory pathway mutations | III |
| TMA–drug associated | Mechanism varies depending on drug and includes direct endothelial damage as well as the development of ADAMTS13 autoantibodies | Depending on drug, I, III, or IV |
| TMA–malignancy associated | Activation of coagulation by tumor tissue factor expression. Possible complement regulatory pathway mutations | NC |
| TMA–Streptococcus pneumonia associated | Exposure of normally hidden endothelial antigens by bacterial neuramidase resulting in complement mediated endothelial damage | III |
| TMA–coagulation mediated | Mutations in DGKE, plasminogen, and thrombomodulin resulting in thrombosis and complement activation | III |
| HELLP syndrome | Mutations in alternate complement pathway regulatory elements | Postpartum, III |
| Antepartum, IV |
| Disorder . | Pathophysiology . | ASFA category for the role of TPE (see Table 4) . |
|---|---|---|
| TMA–Shiga toxin mediated | Direct endothelial damage with apoptosis due to effects of Shiga toxin | Presence of severe neurologic symptoms, III |
| Absence of severe neurologic symptoms, IV | ||
| TMA–complement mediated | Endothelial damage from unregulated complement activation resulting from the development of anti–factor H autoantibodies or mutations leading to abnormal complement regulatory proteins or abnormal complement factors | Complement factor gene mutations, III |
| Factor H autoantibodies, I | ||
| MCP mutations, III | ||
| TMA–hematopoietic stem cell transplantation associated | Endothelial damage due to infection, chemotherapy, radiation therapy, or graft-versus-host disease due to transplant. Of note, a significant percentage of affected individuals may have complement regulatory pathway mutations | III |
| TMA–drug associated | Mechanism varies depending on drug and includes direct endothelial damage as well as the development of ADAMTS13 autoantibodies | Depending on drug, I, III, or IV |
| TMA–malignancy associated | Activation of coagulation by tumor tissue factor expression. Possible complement regulatory pathway mutations | NC |
| TMA–Streptococcus pneumonia associated | Exposure of normally hidden endothelial antigens by bacterial neuramidase resulting in complement mediated endothelial damage | III |
| TMA–coagulation mediated | Mutations in DGKE, plasminogen, and thrombomodulin resulting in thrombosis and complement activation | III |
| HELLP syndrome | Mutations in alternate complement pathway regulatory elements | Postpartum, III |
| Antepartum, IV |
DGKE, diacylglycerol kinase-ε; HELLP, hemolysis, elevated liver enzyme levels, low platelet counts; NC, not categorized.