Table 2.

Patient demographics and baseline laboratory characteristics

PtAge, ySexDiagnosisPreexposure inhibitors, BUPostexposure inhibitors, BUTime to peak anti-pFVIII inhibitor, dBleed siteBleed severityHb decrease, g/dLReceived prior IST*Autoimmune disorder
Anti-hFVIIIAnti-pFVIIIAnti-hFVIIIAnti-pFVIII
1 67 F Relapse 140 1 474 15 66 R forearm with compartment syndrome, gluteal bleed Major  No  
2 54 M New 225 0 225 48 32 Pectoral bleed, GI bleed, upper extremity subcutaneous hematoma Major 3.8 Yes  
3 68 F New 54 8 512 72 30 Forearm with compartment syndrome, hematuria Major 4.9 No  
4 78 M Relapse 71 — 71 N/A N/A Flank hematoma (non-muscular) Major 2.7 Yes  
5 48 F Relapse 69 0 69 0 N/A L forearm, compartment syndrome Major  Yes  
6 68 F New 86 — 86 2 N/A Knee hemarthrosis, soft tissue arm hematoma, GI bleed Major 2.8 No  
7 78 M New 1 — 1 1 N/A Hematuria following urologic procedure CRNMB  No  
8 59 F Relapse 287 0.8 554 212 139 Soft tissue thigh bleed, GI bleed Major 4.1 Yes RA 
9 67 M New 2 533 2 2 533 2 N/A Scrotal/perineal hematoma Major  Yes  
10 64 M New 135 0.9 238 7 26 Forearm muscle with compartment syndrome, Flank soft tissue hematoma Major 2.8 No  
11 85 M New 17 571 186 17 571 186 N/A Surgical wound bleeding (open reduction and internal fixation) Major 3.6 No  
12 81 M Relapse 18 1 18 1 N/A Forearm muscle with compartment syndrome, Thigh soft tissue hematoma Major 3.6 Yes RA, psoriasis 
13 77 F Relapse 348 1 348 47 186 Neck hematoma, soft tissue CRNMB 0.8 Yes  
14 81 M New 6 0 24 0.9 24 Hematuria, epistaxis, thigh soft tissue hematoma Major  No  
15 85 F Relapse 2 0 25 10 27 Uterine bleeding CRNMB†  Yes  
16 15 F New 4 0 4 0 N/A Cutaneous bleeding Major 3.7 Yes EBA, autoimmune hepatitis 
17 69 M New 305 5 473 5 N/A Lower GI bleed Major  Yes  
18 54 M New 61 6 61 6 N/A Knee hemarthrosis Major 2 Yes Bullous Pemphigoid 
PtAge, ySexDiagnosisPreexposure inhibitors, BUPostexposure inhibitors, BUTime to peak anti-pFVIII inhibitor, dBleed siteBleed severityHb decrease, g/dLReceived prior IST*Autoimmune disorder
Anti-hFVIIIAnti-pFVIIIAnti-hFVIIIAnti-pFVIII
1 67 F Relapse 140 1 474 15 66 R forearm with compartment syndrome, gluteal bleed Major  No  
2 54 M New 225 0 225 48 32 Pectoral bleed, GI bleed, upper extremity subcutaneous hematoma Major 3.8 Yes  
3 68 F New 54 8 512 72 30 Forearm with compartment syndrome, hematuria Major 4.9 No  
4 78 M Relapse 71 — 71 N/A N/A Flank hematoma (non-muscular) Major 2.7 Yes  
5 48 F Relapse 69 0 69 0 N/A L forearm, compartment syndrome Major  Yes  
6 68 F New 86 — 86 2 N/A Knee hemarthrosis, soft tissue arm hematoma, GI bleed Major 2.8 No  
7 78 M New 1 — 1 1 N/A Hematuria following urologic procedure CRNMB  No  
8 59 F Relapse 287 0.8 554 212 139 Soft tissue thigh bleed, GI bleed Major 4.1 Yes RA 
9 67 M New 2 533 2 2 533 2 N/A Scrotal/perineal hematoma Major  Yes  
10 64 M New 135 0.9 238 7 26 Forearm muscle with compartment syndrome, Flank soft tissue hematoma Major 2.8 No  
11 85 M New 17 571 186 17 571 186 N/A Surgical wound bleeding (open reduction and internal fixation) Major 3.6 No  
12 81 M Relapse 18 1 18 1 N/A Forearm muscle with compartment syndrome, Thigh soft tissue hematoma Major 3.6 Yes RA, psoriasis 
13 77 F Relapse 348 1 348 47 186 Neck hematoma, soft tissue CRNMB 0.8 Yes  
14 81 M New 6 0 24 0.9 24 Hematuria, epistaxis, thigh soft tissue hematoma Major  No  
15 85 F Relapse 2 0 25 10 27 Uterine bleeding CRNMB†  Yes  
16 15 F New 4 0 4 0 N/A Cutaneous bleeding Major 3.7 Yes EBA, autoimmune hepatitis 
17 69 M New 305 5 473 5 N/A Lower GI bleed Major  Yes  
18 54 M New 61 6 61 6 N/A Knee hemarthrosis Major 2 Yes Bullous Pemphigoid 

Summary of patient demographics, bleed location, and other factors present at diagnosis. Diagnosis status refers to whether treatment at our center represented a new diagnosis of AHA or a relapse of a known case. Baseline inhibitor titer reflects the titer present at the time of bleed treatment. Bleed severity is evaluated based on ISTH bleeding criteria for clinically relevant nonmajor bleeding (CRNMB) and major bleeding in nonsurgical patients. Prior IST refers either to IST given for an underlying disorder, or those who were given corticosteroids or rituximab prior to receiving a dose of rpFVIII.

—, not available or not obtained prior to intervention; EBA, epidermolysis bullosa acquisita; F, female; M, male; N/A, not applicable; Pt, patient; RA, rheumatoid arthritis.

*

Prior IST refers to patients who were either on IST for an existing autoimmune disorder (with diagnosis noted in the adjacent column) or who received IST for AHA prior to receiving rpFVIII. Our practice for initial IST is to give corticosteroids and rituximab due to general tolerability and acceptability to patients for inhibitor eradication.

†

Patient was treated for uterine bleeding after having received rpFVIII for a surgical procedure.

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