Table 1. Summary of published case reports on thrombosis in acquired hemophilia A.
| Study | Age/sex | Prothrombotic risk factors | Site of 1. Bleeding 2. Thrombosis |
Relative timing | At presentation (of bleed) | Management of 1. Major Bleed 2. Inhibitor eradication 3. Thrombosis |
FVIII b (%) | 30-day survival | |||
|---|---|---|---|---|---|---|---|---|---|---|---|
| APTT a (s) | Hb (g/dL) | FVIII (%) | Inhibitor (BU) | ||||||||
| Siow et al (1982) 15 | 70/M | Idiopathic | 1. Right thigh hematoma, right knee hemarthrosis 2. Proximal right lower limb DVT |
DVT first, with B12 deficiency | 38 (13–38) |
5.1, then 10 | – | – | 1. FFP 2. Steroids: hydrocortisone, prednisolone (4 wk) and cyclophosphamide (5 wk) 3. Anticoagulation (stopped after bleeding) |
– | Yes |
| Hematoma 18 days later on anticoagulation | 120 (13–38) |
8.5 | – | Present | |||||||
| Poli et al (1997) 16 | 36/F | Pregnancy (postpartum) | 1. Ecchymoses 2. Distal right lower limb DVT c |
Distal right DVT first | 57 (on IV heparin) |
– | – | – | 1. Vitamin K 2. Prednisolone 1 g/kg/d (4 wk) 3. Heparin, then warfarin (stopped after bleeding) |
Normal | Yes |
| Ecchymoses on warfarin | 110 | – | 1.9 | 1.9 | |||||||
| Deitcher et al (2002) 13 | 60/F | Idiopathic | 1. Forearm hematoma 2. Right peroneal DVT |
Simultaneous | 54.3 (24–33) |
– | 5.3 | 57 | 1. FVIII concentrate and DDAVP; avoided aPCCs to reduce risk of propagating venous thrombosis 2. Not described 3. Anticoagulation for existing DVT stopped after bleeding manifested, IVC filter inserted; peroneal DVT not treated |
149 | Yes |
| 80/M | Lymphoma d | 1. Left flank and thigh hematoma 2. Proximal left lower limb DVT |
Simultaneous | 61.4 (22–33) |
– | 2 | 47 | 353 | Yes | ||
| 76/M | Postoperative | 1. Large ecchymosis over trunk 2. Proximal left lower limb DVT |
DVT first, bleeding while on warfarin | 54.8 (22–33) |
– | 2 | 5 | 88 | Yes | ||
| Spencer et al (2011) 17 | 37/F | Pregnancy (postpartum) |
1. Bruising on upper limbs and right calf hematoma 2. Proximal right lower limb DVT |
DVT first, with prolonged APTT (preheparin) | 3.01 (0.88–1.16) |
9.1 | – | – | 1. rFVIIa 2. Prednisolone 1 mg/kg/d (6 wk) and cyclophosphamide 100 mg/d (6 mo) 3. Heparin, then warfarin (stopped after hematoma) |
62 | Yes |
| Bleeding 9 days later on warfarin | 5.03 (0.88–1.16) |
8.1 | 2 | 35 | |||||||
| Paudel et al (2016) 18 | 21/M | Abdominal trauma (gunshot wounds) and surgery | 1. Gastrointestinal and mucosal bleeding, abdominal hematoma 2. Proximal right lower limb DVT, bilateral cephalic vein thrombi |
DVT at day 14 postoperation | – | – | – | – | 1. Vitamin K, FFP, rFVIIa 2. Methylprednisolone 120 mg/d, then Rituximab 375 mg/m 2 weekly (4 doses) 3. Heparin, then warfarin (stopped after bleeding) |
– | Yes |
| Major bleeding at day 24 on warfarin | >100 | – | <1 | 12 | |||||||
| Wool et al (2017) 20 | 73/M | Idiopathic | 1.Right thalamic hemorrhage with intraventricular extension 2. PE after rFVIIa |
Right thalamic hemorrhage first, PE on day 5 rFVIIa therapy |
76.9 (24–34) |
– | 4 | 27 | 1. FVIII concentrate (2 days), then rFVIIa (30 mcg/kg/dose) till plasma exchange (TPE) 2. Dexamethasone 8 mg BD; TPE day 5–6, then Rituximab 375 mg/m 2 weekly (2 doses) 3. IVC filter insertion |
275 | No e |
| Maral et al (2018) 19 | 46/F | Malignancy (adrenal) | 1. Severe ecchymosis on extremities 2. IVC thrombosis (pre-existing) |
Ecchymoses while on long-term warfarin for IVC thrombosis | 76.9 (24–34) |
– | 3 | 350 | 1. Not discussed 2. Prednisolone 1 mg/kg/d and cyclophosphamide 500 mg/wk 3.Warfarin (stopped after ecchymoses) |
Normal | Yes |
| Present case | 72/M | Idiopathic | 1. Right flank and right deltoid hematoma, right psoas hematoma, malena 2. Proximal left lower limb DVT |
Simultaneous | 79 (25–36) |
3.7 | <1 | 82 | 1. rFVIIa (90 mcg/kg/ dose) 2. Prednisolone 1 mg/kg/d (12 wk) and cyclophosphamide 100 mg/d (6 wk), Rituximab 375/m 2 (from day 26, 4 doses) 3. IVC filter, clexane |
217 | Yes |
Abbreviations: aPCCs, activated prothrombin complex concentrates; APTT, activated partial thromboplastin time; BD, bis in die meaning twice a day; DDAVP, 1-desamino-8-d-arginine vasopressin/desmopressin; DVT, deep vein thrombosis; FVIII, human factor VII; FFP, fresh frozen plasma; Hb, hemoglobin; IVC, inferior vena cava; PE, plasma exchange; rFVIIa, recombinant activated FVII; TPE, therapeutic plasma exchange.
a
Values in brackets represent control levels.
b
Levels after completion of treatment.
c
Interpreted as inappropriately diagnosed muscle hematoma.
d
Splenic marginal zone lymphoma.
e
Died on day 2s3 from arrhythmia.