AL AMYLOIDOSIS :
Approach & Management
A Tweetorial
Excerpts from my lecture at ISNCON 2021
ISNCON 2021 was a class apart academic feast & kudos to the organisers & ISN committe
#Isncon2021
@isn_india
@srigola751
@arvindcanchi
@ISNeducation
@isncon2021
Amyloidosis =
A Disorder of Protein Folding
AL Amyloidosis = Amyloidosis of Light chain origin
Questions we all wish to seek answers..!
There are Four major Types of Systemic Amyloidosis
AL Amyloidosis = Plasma Cell Clonal Proliferation = Amenable to Chemotherapy
Other Types of Amyloidosis are non neoplastic & Therefore no scope of chemotherapy
AL Amyloidosis is a Prototype example of MGRS.
Plasma Cell clone in Myeloma is Malignant while in AL amyloidosis is premalignant in the majority.
Why only few but not all develop AL Amyloidosis ?
Time to know the intricacies of The Biology of AL Amyloidosis..!
Three Biological Aberrations are necessary to manifest AL Amyloidosis..!
The First Prerequisite for AL Amyloidosis to manifest is the presence of Plasma Cell Clone.
A Clone which is Not as Malignant as in Myeloma.
The Second Prerequisite:
Light Chains with Fold Instability.
Such instability leads to deviation from Stable Alfa Helical structure ➡️ Beta Pleated Sheet configuration
?? Culprit : Mutations in Immunoglobulin Light Chain Variable region
The Third Prerequisite:
Defective Scavenging mechanisms in Tissues which tend to counter against abnormal Protein Aggregates
When to Suspect AL Amyloidosis ??
Suspect AL Amyloidosis in any Elderly Non Diabetic Nephrotic with Unexplained cardiomyopathy/ ELEVATED PRO BNP / intractable Paresthesias / Hepatomegaly with raised ALP / Autonomic Manifestations
Diagnostic Criteria =
Four Components
All need to be met
First Criterion:
The Classical Clinical Syndrome
Renal : 70 %
Cardiac : 60 %
First Criterion:
The Classical Clinical Syndrome
Macroglossia and Racoon Eyes = Highly Characteristic
Any organ other than Brain can be involved
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