An Approach to Diagnosis and Management of Thrombotic Microangiopathies - Flowchart
An Approach to Diagnosis and Management of Thrombotic Microangiopathies - Flowchart Thrombotic Thrombocytopenic Purpura Thrombotic Thrombocytopenic Purpura
«Flowchart»

Thrombocytopenia + MAHA

Thrombocytopenia + MAHA

Thrombocytopenia + MAHA

Thrombocytopenia + MAHA

Not TTP/aHUS


Specific management

Not TTP/aHUS


Specific management

Not TTP/aHUS

Not TTP/aHUS


Specific management


Specific management

End

End

End

* Indicates therapies under investigation.

* Indicates therapies under investigation.

* Indicates therapies under investigation.

*

aHUS:


Eculizumab
Rituximab (anti–CFH)
Transplantation

aHUS:


Eculizumab
Rituximab (anti–CFH)
Transplantation

aHUS:

aHUS:


Eculizumab
Rituximab (anti–CFH)
Transplantation


Eculizumab
Rituximab (anti–CFH)
Transplantation

History & laboratory tests


ADAMTS13 activity
Stools for shiga toxins, Urine pneumococcal Ag
Autoimmune serology (ANA, APLA)
DIC panel, Coombs test, Vitamin B12
Blood cultures & HIV studies as indicated
Imaging studies, BM, tissue biopsy as indicated

History & laboratory tests


ADAMTS13 activity
Stools for shiga toxins, Urine pneumococcal Ag
Autoimmune serology (ANA, APLA)
DIC panel, Coombs test, Vitamin B12
Blood cultures & HIV studies as indicated
Imaging studies, BM, tissue biopsy as indicated

History & laboratory tests

History & laboratory tests


ADAMTS13 activity
Stools for shiga toxins, Urine pneumococcal Ag
Autoimmune serology (ANA, APLA)
DIC panel, Coombs test, Vitamin B12
Blood cultures & HIV studies as indicated
Imaging studies, BM, tissue biopsy as indicated


ADAMTS13 activity ADAMTS13
Stools for shiga toxins, Urine pneumococcal Ag
Autoimmune serology (ANA, APLA)
DIC panel, Coombs test, Vitamin B12 12
Blood cultures & HIV studies as indicated
Imaging studies, BM, tissue biopsy as indicated

Co-morbidity


Non TTP/HUS diagnosis: STEC, T-activation, Vasculitis, Cancer, Stem-cell transplant, Renal transplant, Drug-induced TMA, DIC, Hypertension, HELLP, CAPS, Vascular devices, HIV, Cobalamin-C deficiency

Co-morbidity

Co-morbidity


Non TTP/HUS diagnosis: STEC, T-activation, Vasculitis, Cancer, Stem-cell transplant, Renal transplant, Drug-induced TMA, DIC, Hypertension, HELLP, CAPS, Vascular devices, HIV, Cobalamin-C deficiency


Non TTP/HUS diagnosis: STEC, T-activation, Vasculitis, Cancer, Stem-cell transplant, Renal transplant, Drug-induced TMA, DIC, Hypertension, HELLP, CAPS, Vascular devices, HIV, Cobalamin-C deficiency Non TTP/HUS diagnosis: Co-morbidity

Plasma exchange

Plasma exchange

Plasma exchange Plasma exchange

No severe ADAMTS13 deficiency/AKI


Complement factors


C3, C4, CFH, CFI, CFB


MCP expression
Anti-CFH
Genetic testing


Complement, DGK!!epsilon!!, Thrombomodulin

No severe ADAMTS13 deficiency/AKI

No severe ADAMTS13 deficiency/AKI ADAMTS13


Complement factors


C3, C4, CFH, CFI, CFB


MCP expression
Anti-CFH
Genetic testing


Complement, DGK!!epsilon!!, Thrombomodulin


Complement factors


C3, C4, CFH, CFI, CFB


C3, C4, CFH, CFI, CFB
MCP expression
Anti-CFH
Genetic testing


Complement, DGK!!epsilon!!, Thrombomodulin


Complement, DGK!!epsilon!!, Thrombomodulin No severe ADAMTS13 deficiency/AKI

Severe ADAMTS13 deficiency


Inhibitor assay
Anti-ADAMTS13 serology
Serial ADAMTS13
ADAMTS13 genetics

Severe ADAMTS13 deficiency

Severe ADAMTS13 deficiency ADAMTS13


Inhibitor assay
Anti-ADAMTS13 serology
Serial ADAMTS13
ADAMTS13 genetics


Inhibitor assay
Anti-ADAMTS13 serology ADAMTS13
Serial ADAMTS13 ADAMTS13
ADAMTS13 genetics ADAMTS13 Severe ADAMTS13 deficiency

Acquired TTP:


Plasma exchange
Steroid/Rituximab
Block GPIb-vWF*

Acquired TTP:

Acquired TTP:


Plasma exchange
Steroid/Rituximab
Block GPIb-vWF*


Plasma exchange
Steroid/Rituximab
Block GPIb-vWF* * * Acquired TTP

Hereditary TTP:


Plasma therapy
rADAMTS13*

Hereditary TTP:

Hereditary TTP:


Plasma therapy
rADAMTS13*


Plasma therapy
rADAMTS13* ADAMTS13 * * Hereditary TTP