Acquired Factor VIII Inhibitor in a Non-Haemophilic Patient: Successful Treatment with Plasma Exchange Associated with Factor VIII Concentrate and Immunosuppressors

Ml, Paracchini; Gm, Rocchini; Renoldi, P; Gf, Fiorini; Gosso, P; Onida, L; Geniram, A; Marini, U; Gibelli, A

doi:10.1159/000215065

Cited by 6 publications

(4 citation statements)

References 7 publications

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“…In 18 cases immune suppressive therapy with prednisone alone (n = 4) or combined with cyclophosphamide (n = 13) or azathioprine (n = 1) was administered. In 10 cases (2,6,8,12,13,16,17,19,24,25 ) this treatment was successful within a relatively short time. In 9 of them complete remission was achieved within two months.…”

Section: Treatmentmentioning

confidence: 97%

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Solid Tumors and Factor VIII Antibodies

Hauser¹,

Lechner²

1999

Thromb Haemost

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Summary Purpose: To analyze the association between solid tumors and the occurrence of factor VIII antibodies and the response to treatment. Patients and Methods: Published case reports describing the association of a solid tumor and a factor VIII antibody retrieved from 20 data bases. 40 cases were reported and 27 cases were analyzable. Results: Factor VIII antibodies occurred in a close temporal relationship with the detection of the tumor in most cases. No association with a specific type of tumor could be identified. Immune suppressive treatment with prednisone ± cyclophosphamide was successful in the majority of cases. Conclusion: It is likely that there is a causal association between some solid tumors and factor VIII antibodies, but it is an extremely rare complication of cancer. The immunoglobuline nature of the inhibitor and the good response to immune suppressive treatment suggests that it is an autoimmune phenomenon. The pathogenesis is unknown.

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Section: Treatmentmentioning

confidence: 97%

“…In all the latter cases signs of cancer were still present at the time of diagnosis of the inhibitor. In 11 cases (6,8,10,13,14,16,17,20,23,25,27) the inhibitor developed between 1 week and 6 months after surgery or an invasive diagnostic procedure (Fig. 1).…”

Section: Temporal Relationship Between the Diagnosis Of Cancer And Ofmentioning

confidence: 99%

Solid Tumors and Factor VIII Antibodies

Hauser¹,

Lechner²

1999

Thromb Haemost

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“…Plasma anti‐FVIII:C auto‐antibody removal aims to achieve normal haemostasis during this lag time interval. Plasmapheresis has been shown to be effective in lowering the anti‐FVIII:C titre, thus restoring the efficacy of FVIII concentrates (Pintado et al , 1975; Erskine et al , 1981; Slocombe et al , 1981; Francesconi et al , 1982; Sultan et al , 1983; Paracchini et al , 1984; Hambley et al , 1985). However, the lack of specificity of the clearing activity leads to a simultaneous decrease in other coagulation factors, resulting in a bleeding or thrombotic risk.…”

Section: Discussionmentioning

confidence: 99%

Protein A sepharose immunoadsorption: immunological and haemostatic effects in two cases of acquired haemophilia

Guillet¹,

Kriaa²,

Huysse³

et al. 2001

Br J Haematol

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Summary. Acquired haemophilia is a life-threatening disorder caused by circulating auto-antibodies that inhibit factor VIII coagulant activity (FBIII:C). Immunoadsorption on protein A sepharose (IA-PA) was performed in two bleeding patients with acquired haemophilia: we observed a dramatic and quick decrease in the anti-FVIII:C inhibitor titre leading to a normal, albeit transient, haemostatic status. In one case, IA-PA was the only procedure which succeeded in stopping massive haemorrhage. In the second case, IA-PA reinforced the haemostatic effect of recombinant activated factor VII by increasing the endogenous plasma factor VIII level. The efficacy of IA-PA was sustained with immunosuppressive treatment introduced, respectively, 10 and 15 d before the IA-PA procedures. Our experience with IA-PA suggests that this extracorporeal anti-FVIII:C removal procedure is a valuable therapeutic tool for acquired haemophilia and can alleviate life-threatening haemorrhages.

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“…In our literature search, we identified 13 cases of cancer patients who developed a FVIII inhibitor following cancer surgery [10][11][12][13][14][15][16][17][18][19][20][21][22] (Table 1). The EACH2 registry includes three patients with acquired haemophilia and a solid cancer as underlying disease, which has been treated by surgery, and 56 cancer patients in whom FVIII inhibitors were diagnosed because of postoperative bleeding [7].…”

Section: Cases Of Postoperative Paraneoplastic Fviii Inhibitors Repormentioning

confidence: 99%

Postoperative paraneoplastic factor VIII auto‐antibodies in patients with solid tumours

et al. 2011

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Paraneoplastic FVIII antibodies may occur concurrent with the diagnosis or at various times after diagnosis and treatment of cancer. Between 2002 and 2009, we observed two patients with acquired haemophilia A due to an FVIII auto-antibody, which appeared 4 and 5 months after uncomplicated cancer surgery. We aimed to evaluate if such an association of cancer surgery and FVIII antibody formation has been observed previously. We retrieved all published case reports of cancer-associated FVIII auto-antibodies from PubMed for the period 1950-2010. The search in the literature revealed 13 patients in whom a FVIII inhibitor developed after uncomplicated surgery for cancer and a bleeding-free time interval of up to 6 months; 11/15 patients had abdominal cancers (five colon cancer, four pancreatic cancer, gastric cancer and choledochus carcinoma one each). The median time period between surgery and antibody detection was 3 months (1 week-6 months). In most cases, the antibody titre was low (median: 14 BU mL⁻¹, range: 1.7-64 BU mL⁻¹). Immunosuppressive treatment was successful in most of the cases - nine of the treated patients reached a sustained CR of the antibody after a median time of 3 months. Postoperative paraneoplastic FVIII inhibitors may be regarded as a special, not yet recognized subgroup of acquired FVIII antibodies. They share some characteristics with postpartum FVIII inhibitors with regard to the latency period between the triggering event and the appearance of the antibody, and between the usually low antibody titres and their good response to immunosuppressive treatment.

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Acquired Factor VIII Inhibitor in a Non-Haemophilic Patient: Successful Treatment with Plasma Exchange Associated with Factor VIII Concentrate and Immunosuppressors

Cited by 6 publications

References 7 publications

Solid Tumors and Factor VIII Antibodies

Solid Tumors and Factor VIII Antibodies

Protein A sepharose immunoadsorption: immunological and haemostatic effects in two cases of acquired haemophilia

Postoperative paraneoplastic factor VIII auto‐antibodies in patients with solid tumours

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