Mihai Emanuel HIMCINSCHI, Delia Codruta POPA, Sorina-Nicoleta BADELITA, Mihaela UTA, Daniel CORIU, Andrei ANGHEL
Abstract
Amyloidosis is a systemic disorder, characterised by the deposition of misfolded proteins in the extracellular matrix. Primary amyloidosis (AL) is a subtype in which immunoglobulin light chains, undergo the same misfolding and deposition process and are produced by an excessive production of plasma cells, similar to multiple myeloma. As AL can affect multiple organs, it is rather common for AL patients to present with haemostatic associated abnormalities.
In this article, we focused on providing a better insight into hemostatic events in the studied population. Patients who presented with bleeding or thrombosis underwent both global hemostasis tests (PT, aPTT, fibrinogen, D-Dimers) and specialised hemostasis tests (thrombelastography, thrombin generation time, platelet aggregation). Even if the global tests did not provide conclusive results, the specialised ones managed to unmask, under what is widely considered to be a normocoagulant profile, an accentuated production of thrombin, evaluated by increased values of the endogenous thrombin potential produced, of the maximum amplitude of it’s generation and a hypercoagulant profile revealed by thrombelastography. This hypercoagulant profile tends to be associated with renal failure in patients concerned.
At the opposite pole, the evaluation of bleeding events, in the context of liver failure, was highlighted by representative modifications of specialised hemostasis tests. Additionally, the evaluation of the impact that light chains have on type IV collagen was attempted through platelet aggregometry, but with results that were difficult to interpret. Complementary to the aggregation function, platelet adhesion was studied by performing an ex-vivo experiment, that tries to better integrate some of the intricate interactions between type IV collagen and serum from a AL patient.
As a general conclusion, thrombotic events were better put into perspective and more comprehensive medical care can be adjusted for these patients accordingly. On the other hand when studying bleeding phenomena, the methods used may give a theoretical perspective, but ultimately the results need to be backed up with more accurate research methods and a strong correlation must be observed between them.
Keywords: AL, bleeding, thrombosis, haemostasis
https://doi.org/10.59854/dhrrh.2024.2.4.159
