Autoimmune Acquired Factor XIII Deficiency: A Case Report

Figures & data

Figure 1 Changes in thrombin generation depending on FXIII antigen. (A) Before starting steroids. Baseline FXIII of 5%. (B) The patient received steroids for 1 week. Baseline FXIII of 34%. (C) The patient received steroids for 2 weeks. Baseline FXIII of 45%. Reference within normal parameters is shown in green and patient´s sample in blue.

Figure 2 Changes in thromboelastography. (A) The patient received corticosteroids for 1 week. (B) The patient received steroids for 2 weeks.

Abbreviations: CT, clotting time; CFT, clot formation time; MCF, maximum clot firmness.

Table 1 Changes in Hemostatic Parameters Throughout Time

	FXIII Antigen (%)	VWF Antigen (%)	VWF Activity (%)	Fibrinogen (mg/dl)	Bethesda Units (Inhibitor Titer, BU)	APTT Ratio	INR
At diagnosis	9	116	127	240	12	1.25	1.2
Corticosteroids discontinuation	35	145	130	290	1.4	1.15	1.3
Admission for mediastinal hematoma	20	180	160	270	3	1.28	1.2
Last blood sample	55	190	200	310	1	1.12	1.1

Notes: References values: FXIII>60%, VWF antigen:45–150%, VWF activity:45–150%, Fibrinogen:200–450 mg/dl, Bethesda Units<0.6 BU, APTT R: 0.8–1.25, INR: 0.8–1.30.

Abbreviations: VWF, Von Willebrand factor; APTT, activated partial thromboplastin time; INR, international normalized ratio.