Abstract Number: PB0449
Meeting: ISTH 2021 Congress
Theme: Hemophilia and Rare Bleeding Disorders » Acquired Hemorrhagic Coagulation Disorders
Background: Acquired hemophilia A (AHA) is a rare bleeding disorder. Multiple myeloma (MM) is a rare cause of AHA. Management of bleeding control and inhibitor eradication remains controversial.
Aims: To report the challenge of bleeding treatment and inhibitor eradication management in MM patients with AHA.
Methods: We report two case studies of AHA in MM patients.
Results: Case 1: A female (71years) with unremarkable medical history was admitted to hospital due to soft tissue hematomas. Laboratory tests revealed normocytic anemia, prolonged APTT failed to correct in mixing study. AHA diagnosis was made with FVIII=9%, inhibitor titer=7.8BU/ml. Patient received immunosuppressive treatment (IST) and aPCC for bleeding control. After 3 weeks we observed partial resolution of hematomas. During diagnostic workup serum electrophoresis revealed IgG monoclonal protein-41.5g/l, trephine biopsy showed 70% monoclonal plasmocytes, also multiple bone fractures were identified by computed tomography. We diagnosed MM R-ISS2 and administered VCD regimen as a combination of IST and anti-myeloma treatment. After 1 cycle, patient achieved very good partial response of MM and complete remission (CR) of AHA-Table1.
Case 2: A female (70years) was admitted to hospital due to large subcutaneous hematomas. She has a history of light kappa chain MM, diagnosed -2016, treated with autologous stem cell transplantation -2017. In 10/2019 she developed progression treated with lenalidomide and dexamethasone. After 3 cycles she achieved partial remission (PR), then therapy was withdrawn due to ocular surgery. On admission: anemia and persistent APTT prolongation on mixing tests, FVIII<1%, inhibitor titer=108BU/ml. MM relapse with serum kappa light chain 290mg/l was confirmed. Patient received lenalidomide in combination with IST and bleeding control with aPCC. Two months later she achieved CR of AHA with PR of MM-Table1.
| Case 1 | Case 2 | ||
| Gender | F | F | |
| Age [years] | 71 | 70 | |
| Myeloma status | De novo | Relapse | |
| M protein type | IgG lambda | Kappa light chain | |
| Symptoms | Muscle hematomas, subcutaneous hematomas | Subcutaneous hematomas | |
| On AHA diagnosis |
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| Treatment | Bleeding management | aPCC 100IU/kg bw in 2 separated doses | aPCC 200IU /kg bw in 3 separated doses |
| Inhibitor eradication treatment | Prednisone 1mg/kg bw, VCD (bortezomib 1.3mg/m2 1,4,8,11d, cyclophosphamide 500mg/w, dexamethasone 40mg 1,4,8,11d;cycle 21d). | Prednisone 1mg/kg bw, Cyclophosphamide 1.5mg/kg/d, Lenalidomide 25mg/d | |
| After treatment |
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Case description Abbreviations: F – female, M protein – monoclonal protein, AHA – acquired hemophilia A, MM – multiple myeloma, aPCC – activated Prothrombin Complex Concentrate, bw – body weight, CR – complete response, VGPR – very good partial response, PR – partial response
Conclusions: Acquired hemophilia A can occur both in the onset and relapse of multiple myeloma. Combination of IST and anti-myeloma therapy is efficient in inhibitor eradication.
To cite this abstract in AMA style:
Hoppe A, Rupa-Matysek J, Gil L. Acquired Hemophilia A as a Complication of Multiple Myeloma [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/acquired-hemophilia-a-as-a-complication-of-multiple-myeloma/. Accessed November 30, 2023.« Back to ISTH 2021 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/acquired-hemophilia-a-as-a-complication-of-multiple-myeloma/