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| Article | Citation | Presentation | Findings | Outcome |
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| 41 | Michiels et al. [4] | 37 y/o G1P1 presented 4 months postpartum with bruises and hemarthrosis. | FVIII-0.02 U/mL
FVIII inhibitor: 7.9 BU | Treated with prednisone taper of 1 mg/kg with remission within 2 months while on 20 mg/d without recurrence at 5-year f/u. |
| 22 y/o G3P3 presented 7 months postpartum with menorrhagia and bleeding after tooth extraction. | FVIII: 0.01–0.02 U/mL
FVIII inhibitor: 12 BU | Treatment with cyclophosphamide 100 mg/day (0.7 mg/kg/d) without inhibitor disappearance; however, spontaneous remission developed 10 months later after discontinuing medication. Subsequent pregnancy without bleeding diathesis. |
| 31 y/o G2P2 presented 2 months postpartum with muscle and soft tissue bleeding, menorrhagia, ecchymoses, and hemarthrosis. | FVIII: <0.01 U/mL
FVIII inhibitor: 24 BU | Treated with 4000 U human factor VIII, prednisone 1 mg/kg/d for 6 weeks, and cyclophosphamide 2 mg/kg/d concomitantly from the 3rd to 6th week of prednisone treatment, with a 5-day course of high-dose gamma-globulin at 0.5 g/kg/day. Recurrence of the inhibitor with removal of agents resulting in IVIG being initiated. Remission at 28 months. |
| 24 y/o G2P1A1 presented after delivery of stillborn with PPH and subsequent hemarthrosis. | FVIII: <0.01
FVIII inhibitor: 295–625 BU | Treated with 1 mg/kg prednisone for 3 weeks without remission. Five years later presented with FVIII 600 BU and thus 10K U of cryoprecipitate. At 24 years after treatment, the patient still had not achieved remission. |
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| 38 | Coller et al. [5] | 22 y/o primigravid woman presented with severe vaginal bleeding postpartum day 6. | PT: 12.6/13.0 sec
PTT: 74/45 sec
FVIII: 8%
FIX: 90%
FVIII inhibitor: 15 BU
von Willebrand factor: 90% | Treated with 60 mg prednisone daily, became free of inhibitor within months, and had a successful second pregnancy without any subsequent bleeding diathesis. |
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| 6 | Chaari et al. [9] | 19 y/o F presented PPD one with large vaginal hematoma. | aPTT: 87/30 sec
FVIII: 7%
FVIII inhibitor: 64 BU | Treatment began with initial surgical intervention for evacuation of hematoma but was complicated by bleeding into the abdominal cavity and genitalia. The patient was given recombinant FVIIa and activated prothrombin complex concentrates. Due to symptom progression, the patient started on prednisone alone for 3 days followed by cotherapy with cyclophosphamide without symptom improvement. The patient passed away PPD 2/2 hemorrhagic shock. |
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| 5 | Seethala et al. [7] | 36 y/o woman presented with PPH s/p NSVD secondary to acquired factor VIII deficiency. | PTT: 71.7/45 sec
PT: 15.9 sec
INR: 1.3
FVIII: <1%
FVIII inhibitor: >54.3 BU | Received factor VII and desmopressin.
Treated with methylprednisolone, cytoxan, and plasmapheresis with appropriate decline in PTT but was discontinued because of sepsis prior to discharge. The patient was readmitted for bacteremia and succumbed to severe sepsis. |
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| 4 | Kotani et al. [8] | 31 y/o G1P1p developed postpartum hemorrhage after delivery.
A female neonate developed subcutaneous hemorrhage on dorsum of hand on day 1of life s/p routine blood draw secondary to transplacental transfer of the inhibitor. | PT: 10.6 sec
PTT: 76.9 sec
FVIII: <1%
FVIII inhibitor: 458 BU/mL
PT: 14.3 sec
PTT: 80.3 sec
FVIII: <1%
FVIII inhibitor: 199 BU/mL | Treatment for 31 y/o F consisted of 19 U PRBCS, 51 U FFP, steroid pulse therapy, and 3 vials of factor VII during hospital course and discharged on 30 mg/day of prednisolone. 10 months postpartum PTT WNL without any further complications.
Neonate required no treatment and factor VIII, and PTT returned to baseline at postnatal month 4. |
| 35 | Chaari et al. [6] | 31 y/o woman presented with several areas of ecchymoses over lower extremities several days prior to delivery. | FVIII: 18%
FVIII inhibitor: 1, 4 BU | Treated with prednisone and rituximab. |
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| 8 | Porteous et al. [10] | 32 y/o primigravid woman developed postpartum hemorrhage hours after delivery. | PTT: 78.2 sec
PT: 10.9 sec
FVIII: <1%
FVIII inhibitor: 15 BU | Treated with 1 mg/kg/day (60 mg) prednisolone, 5 doses of recombinant FVIIa 360 IU, 2 doses of DDAVP 0.3 µg/kg, 20 U PRBCs, tranexamic acid 1 g daily over 20 days with continued bleeding. Bleeding was controlled with bilateral internal pudendal artery embolization. Maintenance therapy was achieved with prednisolone 60 mg and tranexamic acid 500 mg tds. |
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| 23 | Sebastian et al. [11] | 25 y/o F with a history of secondary APS in the setting of SLE and acquired factor VII inhibitor at 10 weeks of gestation developed retroperitoneal bleeding. Prior to current conception, the patient was symptomatic having suffered from several areas of ecchymoses on extremities, episcleritis, and urticaria and having been diagnosed with a PE 6 months prior. | PTT: 94/37 sec
FVIII: 1.33%
FVIII inhibitor: 614.4 BU
C3: 0.57
C4: <0.08
Anti-ANA: 1 : 320
SS-A and Ro52: present
Anti-dsDNA: 18.35/100 IU/mL | Treatment consisted of methylprednisolone IV, recombinant factor VII, prednisone 1 mg/kg, and cyclosporine 250 mg/day. The patient consented to terminate pregnancy. Continued maintenance therapy with cyclosporine 200 mg/day, chloroquine 250 mg/day, and methylprednisolone. |
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| 7 | Rodrigues et al. [12] | 34 y/o primigravid F presented PPD forty with complaints of spontaneous hematomas on extremities and intramuscular bleeding on the back, forearms, ankle, and thighs. | APTT: 62/<38 sec
FVIII: 3.5%
FVIII inhibitor: 10 BU
FIX: 124%
Fibrinogen: 430/450 mg/DL | Treated with prednisone 1 mg/kg/d plus tranexamic acid. The patient had remission of the disease with normalization of FVIII without the presence of the inhibitor in ∼2 years. |
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| 29 | Lee et al. [13] | 18 y/o F primigravid presented 9 months postpartum with painful swelling of knees and ankles, multiple bruises on hands and feet, and menorrhagia. | PT: 10.2/(10–14 sec)
INR: 0.95/1.5 sec
APTT: 84.3/40 sec
FVIII: 1.4%
Anti-FVIII: 28 BU | Treated with activated prothrombin complex concentrate and FEIBA anti-inhibitor coagulant complex at a dose of 50–100 units/kg q12 h. Corticosteroids were considered but not indicated due to remission of symptoms. |
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| 39 | Azam et al. [14] | 26-year-old multiparous woman presented PPD 2 with hemoperitoneum following lower segment C-section. | PTT: 90 sec (0–32 sec)
Normal PT/INR
FVIII level: 1%
Positive inhibitor
Normal lupus anticoagulant
Normal vWF
Normal antiphospholipid IgM and IgG | Treated with prednisone 60 mg/day initially with taper and initiation azathioprine. |
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| 2 | Qian et al. [15] | 35-year-old PPD 48 with chest pain and was found to have pleural hemorrhage. | PT: 15.2 sec
PTT: 68.40 sec
FVIII inhibitor: positive
Inhibitor: 7.4% | Treated with aPCC, human factor VIII concentrates, corticosteroids, and plasma. At 6 months, no recurrence. |
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