Amyloidosis and bleeding: pathophysiology, diagnosis, and therapy.
Amyloid diseases can be associated with potentially life-threatening hemorrhage. Pathogenetic factors contributing to the abnormal bleeding tendency in this setting are heterogeneous and depend on the type of amyloidosis and pattern of organ involvement. In patients with light-chain (AL) amyloidosis, acquired hemostatic abnormalities, including coagulation factor deficiencies, hyperfibrinolysis, and platelet dysfunction, can be regarded as the most important pathogenetic factors. In patients with other types of amyloidosis, acquired hemostatic defects are rare, and amyloid deposition has also been reported to be the main cause of abnormal bleeding manifestations. Amyloid angiopathy with increased fragility of blood vessels and impaired vasoconstriction may promote bleeding in this setting. Rupture of solid organs caused by amyloid deposition also was reported. Whereas therapeutic options in bleeding caused by local amyloid deposition are restricted to supportive measures and, in severe cases, surgery, acquired hemostatic defects may be treated according to the causative mechanism. In this review, we focus on bleeding risks in patients with amyloid diseases. Current concepts with regard to pathophysiology, diagnosis, and treatment are summarized and discussed.
Sucker
Christoph
C
Department of Hemostasis, Heinrich Heine University Medical Center, Duesseldorf, Germany. sucker@med.uni-duesseldorf.de
Hetzel
Gerd Ruediger
GR
Grabensee
Bernd
B
Stockschlaeder
Marcus
M
Scharf
Ruediger E
RE
D000682:Amyloid / Q000032:analysis / Q000378:metabolism; D000686:Amyloidosis / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology* / Q000628:therapy; D020533:Angiogenesis Inhibitors / Q000627:therapeutic use; D001777:Blood Coagulation / Q000502:physiology; D001791:Blood Platelet Disorders / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology; D001808:Blood Vessels / Q000737:chemistry / Q000378:metabolism; D003029:Coagulants / Q000627:therapeutic use; D020147:Coagulation Protein Disorders / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology; D004358:Drug Therapy; D005342:Fibrinolysis / Q000502:physiology; D006470:Hemorrhage / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology* / Q000517:prevention & control; D006801:Humans; D012307:Risk Factors; D033581:Stem Cell Transplantation
D000682:Amyloid; D020533:Angiogenesis Inhibitors; D003029:Coagulants