Waldenstroms/Amyloid Flashcards
What is the main treatment approach for patients with AL Amyloidosis?
Those eligible for a transplant should be treated with Dara-CyBoRD followed by transplant for consolidation therapy if no VGPR not achieved. If achieved they don’t need a transplant.
What are some other regimens that you can be used for induction therapy for AL?
You can use Bortezomib with Cyclophosphamide and Dex or Len and Dex or you can do Bortezomib w/Dex alone
What are the treatment options for those who are not eligible for auto transplant for AL?
You can give Melphalan/Bortezomib/Dex or Mephalan/Dex
What are treatment options for those with AL refractory disease?
CyBoRD, Pom/Dex, Ixazomib plus (Cyclophosphamide)w/Dex or w/ Len/Dex, Dara-if not refractory, Len/Dex alone or w/Cyclophosphamide, Velcade +/- Dex
Is there a role of auto-SCT in the second line setting for AL amyloidosis?
Yes patients can still receive this in the second line setting. They don’t have to go forward with this in the first line setting if there is a good response.
When evaluating a patient with Waldenstroms what do you look for to guide whether or not you treat them?
If patients are completely asymptomatic from their disease you do not treat, it doesn’t matter what their lab values are!
What are the primary treatment options for Waldenstroms in the first line setting?
Bendamsutine/Rituximab, Velcade/Rituximab/Dex, Ibrutinib +/-Rituximab, Zanabrutinib
When using Rituximab in Waldenstroms you need to make sure patients don’t have what?
You need to make sure they don’t have hyperviscocity syndrome, because Ritux will make it worse.
If you have a patient with a IgM of 5,000-7,000 you have to keep in mind what?
They could have a IgM flare so you want to give cytotoxic chemo first or BTK inhibitor and then give Rituximab in the 2nd cycle
What is Bing Neel Syndrome? What do you use to treat?
Lymphoplasmacytic involvement of the cerebral cortex or meninges. Ibrutinib-crosses BBB
What is the benefit of using Zanabrutinib over Ibruitinib in Waldenstroms?
Zanabrutinib has less toxicity esp Afib/Aflutter, HTN, diarrhea. Neutropenia is higher, however less hospitalization w/Zanabrutinib bc of this.
What is the treatment of hyperviscocity?
Plasma exchange
What symptoms warrant treatment for Waldenstroms?
Bulky lymphadenopathy (greater than 5cm) or symptomatic lymph nodes, cytopenias, B-symptoms, concurrent AL Amyloidosis.
What are the tx options for second line disease for Waldenstroms?
If a patient had a durable response to a previous agent you can retreat them with that. Venetoclax is an option. Remember if they are asymptomatic you can just observe. You can refer to notes for the other options. Don’t think they will expect you to know.
Is there a role for transplant in relapsed Waldenstroms?
NCCN says you can consider auto or allogeneic transplant in young patients.
