Elevation of Plasmin-α2-plasmin Inhibitor Complex Predicts the Diagnosis of Systemic AL Amyloidosis in Patients with Monoclonal Protein

Abstract

Objective The complication of systemic immunoglobulin light chain (AL) amyloidosis in patients with monoclonal immunoglobulin affects the prognosis, but amyloid deposition in tissues is sometimes difficult to detect due to bleeding tendencies and preferential distributions. However, fibrinolysis is known to be exacerbated in patients with systemic AL amyloidosis specifically. We therefore explored new biomarkers for predicting a diagnosis of systemic AL amyloidosis focusing on coagulation and fibrinolysis markers. Methods We reviewed the clinical features and treatment outcomes of patients with serum monoclonal protein, including primary systemic AL amyloidosis and multiple myeloma (MM), treated at our hospital between January 2008 and December 2014. Results Among several biomarkers, only the serum level of plasmin-α2-plasmin inhibitor complex (PIC) in patients with systemic AL amyloidosis (n=26) at the diagnosis was significantly higher than in patients with MM without AL amyloidosis (n=26) (mean±standard deviation, 3.69±2.82 μg/mL vs. 1.23±0.97 μg/mL, p<0.01). The cut-off for predicting a diagnosis of systemic AL amyloidosis in patients with serum monoclonal protein was 1.72 μg/mL with 84.6% sensitivity and 80.8% specificity. Hepatic involvement resulted in a significantly higher PIC level than no involvement in patients with systemic AL amyloidosis. The serum PIC level was also associated with the hematological response of systemic AL amyloidosis. Conclusion PIC is a useful biomarker for the diagnosis and management of patients with systemic AL amyloidosis.

Autoimmune Coagulation Factor X Deficiency as a Rare Acquired Hemorrhagic Disorder: A Literature Review

“…These parameters may be particularly important for excluding AL-Amyl from the differential diagnosis because patients with AL-Amyl demonstrate moderately elevated FDP and D-dimer values. 17 46…”

“…These parameters may be particularly important for excluding AL-Amyl from the differential diagnosis because patients with AL-Amyl demonstrate moderately elevated FDP and D-dimer values. 17,46 Fibrinolytic and coagulation parameters, i.e., such as plasmin-α 2 -plasmin inhibitor complex (PIC) and thrombin-antithrombin (TAT) complex, were reported to be important for recognizing a hyperfibrinolytic state and a hypocoagulation state, respectively, in bleeding patients with AL-Amyl. 17,46,47 However, PIC and TAT complexes were measured in only 1 of the 26 AiF10D cases, 8 although these parameters may be highly useful for differentiating AiF10D from AL-Amyl.…”

Clinical features and laboratory diagnostic issues of non‐immune, non‐amyloid related acquired factor X deficiency

“…These parameters may be particularly important for excluding AL-Amyloidosis from the differential diagnosis. 8,9 Generally, haemostatic treatments were similar between the NINA and AiF10D groups (Table 1). Fresh frozen plasma (FFP) and Vita-min K were administered to 61% and 43% of NINA, respectively.…”

Cardiac Amyloidosis: Internist and Cardiologist Insight

“…Они показаны не только при внутрисердечных тромбозах, ФП (независимо от количества баллов по шкале CHA2DS2-VASc), предсердных аритмиях, но и при синусовом ритме при эхокардиографических признаках механической дисфункции левого предсердия [5]. При ALамилоидозе нередко встречается дефицит Х фактора свертывания, геморрагии, и поэтому назначать антикоагулянты необходимо с осторожностью [5,59,60].…”