Disappearance of Acquired Hemophilia A after Complete Remission in a Multiple Myeloma Patient

Innao, Vanessa; Allegra, Alessandro; Morreale, Rosalba A.; Russo, S.; Musolino, Caterina

doi:10.4274/tjh.2016.0146

Cited by 7 publications

(9 citation statements)

References 11 publications

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“…Paraproteins can act as neutralizing antibodies against multiple coagulation factors. Furthermore, in certain cases, coagulation abnormalities resolve after the treatment of the underlying plasma cell disease ( 5 , 6 ). In the present case, both hemorrhagic and thrombotic complications were observed during treatment for the underlying MM.…”

Section: Discussionmentioning

confidence: 99%

“…In addition, a series of case reports demonstrated that acquired hemophilia A (AHA) and acquired von Willebrand syndrome (AVWS) can cause severe bleeding in MM patients ( 4 ). In certain cases, treatment for MM and suppression of paraprotein production ameliorated concomitant coagulopathy ( 5 , 6 ), suggesting that paraproteins play a central role in the abnormal coagulation of MM patients. We herein present a case of MM complicated by a coagulation abnormality that resolved after allo-hematopoietic stem cell transplantation (allo-HCT), which was performed to treat MM.…”

Section: Introductionmentioning

confidence: 99%

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Combined Coagulopathy Can Induce Both Hemorrhagic and Thrombotic Complications in Multiple Myeloma

et al. 2018

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Coagulation abnormalities are a rare but critical complication associated with plasma cell diseases. We herein present a case of multiple myeloma (MM) with complicated coagulopathy. Initially, the patient showed severe bleeding tendency due to concomitant acquired hemophilia A and acquired von Willebrand syndrome. Interestingly, the patient also exhibited hyperactivation of factor IX. During treatment for MM, the bleeding complications were ameliorated; however, the patient had central retinal vein occlusion. All of the coagulation abnormalities were completely resolved after the complete remission of MM. This case suggests that MM patients may have concomitant risks for both bleeding and thromboembolic complications.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Combined Coagulopathy Can Induce Both Hemorrhagic and Thrombotic Complications in Multiple Myeloma

et al. 2018

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“…The described subject achieved simultaneous remission of both disorders after chemotherapy and, experiencing a protein M increase even without myeloma symptoms, he developed an immediate AHA relapse [20]. In the literature, various types of M protein have been described in myeloma patients with AHA [5,[19][20][21][22][23][24][25][26][27][28][29][30][31][32][33][34][35]. The most common M protein type is IgG kappa (31%), though some patients have been diagnosed with IgA and light chain disease (LCD), both kappa and lambda (Table I).…”

Section: Pathophysiologymentioning

confidence: 99%

Clinical challenge of managing patients with multiple myeloma and acquired hemophilia A with risk of both thrombosis and bleeding: a narrative review

Hoppe

Rupa‐Matysek²,

Gil³

2022

Acta Haematol Pol.

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“…[ 14 ] F 64 AHA MC, HP 17 5 rFVIIa S Normal coagulation IgM kappa VTD NA NA 11 Innao et al . [ 15 ] M 67 PCN NA 28 NA FVIII No treatment Normal coagulation IgG kappa, kappa light chain VMP, ASCT CR Alive 12 Brás, et al [ 16 ] M 87 PCN MC, IM 1.4 18.4 APPC S, C, B Normal APTT, FVIII:C 36%, FVIII inhibitor 0.8 BU/ml IgG kappa MTP, VD PR NA 13 Napolitano et al . 2017 [ 17 ] F 59 AHA MC, HA, A 12 70 rFVIIa, APCC S, R Normal coagulation IgG lambda VMP CR Alive 14 Kawashima et al .…”

Section: Case Presentationmentioning

confidence: 99%

Acquired hemophilia A and plasma cell neoplasms: a case report and review of the literature

et al. 2020

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Background Acquired hemophilia A is a rare autoimmune disease with clinically often significant bleeding diathesis resulting from circulating autoantibodies inhibiting coagulation factor VIII. Half of acquired hemophilia A cases are associated with an underlying disorder, such as autoimmune diseases, cancer, or use of certain drugs, or occur during pregnancy and in the postpartum period. In the other half, no underlying cause is identified. An association of acquired hemophilia A with plasma cell neoplasm seems to be extremely rare. Case presentation We describe a case of a 77-year-old Swiss Caucasian man who was diagnosed with acquired hemophilia A and smoldering multiple myeloma as an underlying cause. Acquired hemophilia A was treated with prednisolone, cyclophosphamide, and immunoadsorption. Extensive workup revealed a plasma cell neoplasm as the only disorder associated with or underlying the acquired hemophilia A. For long-term control of acquired hemophilia A, we considered treatment of the plasma cell neoplasm necessary, and a VRD (bortezomib, lenalidomide, and dexamethasone) regimen was initiated. Due to multiple complications, VRD was reduced to VRD-lite after two cycles. After nine cycles of induction therapy and five cycles of consolidation therapy, the patient is in complete remission of his acquired hemophilia A and very good partial remission of the plasma cell neoplasm. We conducted a literature review to identify additional cases of this rare association and identified 15 other cases. Case descriptions, including the sequence of occurrence of acquired hemophilia A and plasma cell neoplasm , treatment, evolution, and outcome are presented. Discussion and conclusions Our case, together with 15 other cases described in the literature, underscore the possibility of plasma cell neoplasm as an underlying cause of acquired hemophilia A. Physicians should consider including protein electrophoresis, immunofixation, and analysis of free light chains in laboratory diagnostics when treating a patient with acquired hemophilia A. The occurrence of excessive and unexplained bleeding in patients diagnosed with plasma cell neoplasm should raise suspicion of secondary acquired hemophilia A and trigger the request for coagulation tests, particularly in patients treated with immunomodulatory drugs such as thalidomide or lenalidomide. Additionally, early intervention with immunoadsorption can be lifesaving in cases with high-titer factor VIII inhibitors, especially when surgical interventions are necessary.

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Disappearance of Acquired Hemophilia A after Complete Remission in a Multiple Myeloma Patient

Cited by 7 publications

References 11 publications

Combined Coagulopathy Can Induce Both Hemorrhagic and Thrombotic Complications in Multiple Myeloma

Combined Coagulopathy Can Induce Both Hemorrhagic and Thrombotic Complications in Multiple Myeloma

Clinical challenge of managing patients with multiple myeloma and acquired hemophilia A with risk of both thrombosis and bleeding: a narrative review

Acquired hemophilia A and plasma cell neoplasms: a case report and review of the literature

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