TMA

Question 1

Management of complement mediated TMA during pregnancy and postpartum

Answer 1

*Essentially the same as for patients outside of pregnancy.
Eculizumab or ravulizumab. CM-TMA is not a reason to alter obstetric management. It is not a contraindication to pregnancy.
MFM consult

Question 1

Approved drugs for complement mediated TMA

Answer 1

eculizumab or ravulizumab

Question 2

Atypical HUS workup

Answer 2

Autoantibody to complement factor H (but not a great test per UTD. Discuss with send-out lab)
- Renal biopsy to confirm TMA (Rule out other cause of AKI)
- C3, C4, CH50 (Helpful if positive, but low sensitivity so can’t rule out, still send IMO since positive is helpful and suggestive)
- Nephrology consult (confirm no other causes of AKI apparent)

Question 3

Findings on peripheral smear with TMA

Answer 3

• schistocytes
• thrombocytopenia
• polychromasia (immature RBCS different color basically)

Question 4

other clinical feature relatively common in atypical HUS

Answer 4

new/severe HTN (40-50%)

Question 5

PLASMIC score components

Answer 5

• plt count <30k
• creatinine < 2.26
• hemolysis index +
• MCV <90
• INR <1.5
• no active cancer
• no solid organ or HSC transplant

Question 6

Ethnicity at higher risk of TTP

Answer 6

African American

Question 7

Components of immune TTP management

Answer 7

Remove the antibody
1) PLEX
Suppress the immune system from producing more antibody
2) Steroids
3) rituxan (now recommended for all first diagnosis given highly effective in reducing relapse)
4) Caplacizumab

Question 8

Primary SE of caplacizumab

Answer 8

Mild mucocutaneous bleeding (causes VWD type phenotype)

Question 9

Benefit of caplacizumabs

Answer 9

improved plt recovery so less PLEX and shorter hospital stay
**reduces exacerbations

Question 10

Refractory TTP management

Answer 10

• Caplacizumab if not started yet
• intensify immunosuppression (rituxan, cyclosporine, vincristine, cytoxan, velcade, dara)
• splenectomy
• Recombinant ADAMTS13 (once on market)

Question 11

Long term sequelae of TTP

Answer 11

neuropsychiatric:
- stroke
- cognitive impairment
- depression, PTSD
Other morbidities:
- HTN
- lupus
- obesity

Question 12

Genes most commonly implicated in atypical HUS

Answer 12

• CFH
• MCP

Question 13

Bleeding and clotting risk in acute vs. chronic DIC

Answer 13

• Acute DIC bleeding more common, clotting more common in chronic DIC

Question 14

Common culprits for drug induced TMA

Answer 14

• gemcitabine
• mitomycin
• docetaxel
• oxali
• bleomycin
• doxorubicin
• bevacizumab
• sunitinib
• everolimus
• imatinib
• checkpoint inhibitors
• carfilzomib

Question 15

Catastrophic APS management

Answer 15

1) Methylprednisone IV 1g daily for 3 days, then plan to transition to prednisone 1 mg/kg with gradual taper
IF persistent thrombosis, plan to add retuximab
2) Consult transfusion medicine for PLEX
3) Therapeutic heparin gtt
Plan to eventually transition to warfarin (goal INR 2-3)
4) Optimize management of underlying trigger

Question 16

Condition that can masquerade as TTP

Answer 16

B12 deficiency

Question 17

Management of patient with TMA who’s not improving on exchange

Answer 17

Start complement inhibitor for complement-mediated TMA