AL amyloidosis facts

Question 1

% of lambda in AL amyloidosis

Answer 1

80%

Question 2

presence of t(11:14) on AL amyloidosis

Answer 2

40-60%
Associated with poor response and poor OS

Question 3

Standard of care 1st line Tx in AL amyloidosis

Answer 3

ANDROMEDA
DVCd for 6 cycles
Followed by Dara main up to 24 months
vs VCd X6
80% VGPR
CR 50% vs 14%

Question 4

Timing of ASCT in AL amyloidosis

Answer 4

CR2
usually deferred until relapse and not in CR1due to high TRM

Question 5

AL amyloidosis goals of Tx

Answer 5

VGPR after 2 cycles

Question 6

Tx of MRD positivity in AL amyloidosis

Answer 6

In fit pts in CR but without organ response- MRD can guide the need for further Tx

Question 7

IgM-related AL amyloidosis

Answer 7

6-10% of AL amyloidosis
most are LPL and not PC
Tx is based on the clone

Question 8

Normal GLS

Answer 8

> 18%

Question 9

Phyisical exam signs in AL amyloidosis

Answer 9

Periorbital hematoma
Enlarged tongue
Shoulder pad
Hepatomegaly
Ansarca

Question 10

VGPR in AL amyloidosis

Answer 10

dFLC < 4 mg%

Question 11

Cardiac response in AL amyloidosis

Answer 11

NT-BNP decrease of > 30% (at least 300)

Question 12

Renal response in AL amyloidosis

Answer 12

> 50% decrease in proteinuria

Question 13

ASCT in AL amyloidosis

Answer 13

High TRM
Select pts carefully
No OS advantage in RCT and meta anylasis