An Acquired Factor X Inhibitor: The Importance of Understanding Coagulation

Chan, Isaac S.; Ogunsile, F. Joy

doi:10.1016/j.amjmed.2017.01.046

Cited by 6 publications

(3 citation statements)

References 5 publications

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“…23 The third and least common mechanism involves a pathologic functional antibody that was difficult to detect by citrate-based assays because it is a calcium-ion dependent anti-FX IgG antibody. 8,24,25 All of these cases required complicated treatments not only to manage severe bleeding, but also to control the primary disease (Table 2).…”

Section: Discussionmentioning

confidence: 99%

“…7 Previous studies reported factor X inhibitor to be associated with respiratory tract infection, leprosy, gastrointestinal lymphoma, burn, and inflammatory bowel disease. [8][9][10][11] In contrast, the most common cause of acquired factor X deficiency is light chain amyloidosis, which is a plasma cell dyscrasias that is characterized by deposition of amyloid fibrils from abnormal monoclonal immunoglobulins. 12 The proposed mechanism of factor X deficiency in systemic amyloidosis is rapid clearance of factor X by amyloid fibril absorption and probably splenic sequestration.…”

Section: Introductionmentioning

confidence: 99%

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Successful Treatment of Factor X Deficiency in a Patient with Lymphoplasmacytic Lymphoma with Bendamustine Plus Rituximab Regimen: A Case Report and Literature Review

Rungjirajittranon¹,

Chinthammitr²,

Hantaweepant³

2021

JBM

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Background: Acquired factor X deficiency is an uncommon condition, and affected individuals have severe and spontaneous bleeding. The associated conditions include malignancy, infection, burn, and inflammatory bowel disease. Many previous studies reported association between lymphoproliferative disease and factor X disappearance. Amyloid deposition causing factor X absorption was the most common mechanism. Here, we report a case of stage IV lymphoplasmacytic lymphoma (LPL) with factor X deficiency who was successfully treated with bendamustine plus rituximab (BR) regimen. Case Presentation: A 52-year-old Thai woman presented with heavy menorrhea, hoarseness, and widespread ecchymosis at her extremities. On physical examination, the patient had bilateral periorbital purpura and vocal cord hematoma. Coagulation testing showed prolonged prothrombin time (PT) and prolonged activated thromboplastin time (aPTT); however, after mixing with 1:1 normal pooled plasma, PT and aPTT were both corrected to normal levels. Factor assays demonstrated markedly decreased factor X levels, but no presence of factor X inhibitor. Bone marrow examination revealed numerous abnormal lymphoplasmacytoid lymphocytes with kappa light chain expression. Serum free light chain assay also showed kappa light chain restriction [kappa 716.16 mg/L, lambda 16.96 mg/L, ratio 42.23 (0.26-1.65)]. The patient was diagnosed as lymphoplasmacytic lymphoma with factor X deficiency. She received chemotherapy with 6 cycles of bendamustine plus rituximab (BR) regimen. The patient responded favorably to treatment, she remains in lymphoma remission at one year after diagnosis, and her factor X level was more than 20%. Conclusion:We performed a literature review to identify previous case reports about lymphoma-associated factor X deficiency or inhibitor to determine a possible explanation in our patient. It is important to emphasize that when patients present with acquired factor deficiency, including factor X, lymphoproliferative disease is commonly one of the underlying conditions. Furthermore, the recovery of coagulation factor deficiency is possible if successful remission of lymphoma can be achieved.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Successful Treatment of Factor X Deficiency in a Patient with Lymphoplasmacytic Lymphoma with Bendamustine Plus Rituximab Regimen: A Case Report and Literature Review

Rungjirajittranon¹,

Chinthammitr²,

Hantaweepant³

2021

JBM

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“…AFXD develops in approximately 10% of patients with systemic light-chain amyloidosis, and in such cases, it is probably caused by absorption of FX by amyloid fibrils (2)(3)(4). In contrast, AFXD without amyloidosis is extremely rare, and only a small number of cases have been described in the literature (5)(6)(7)(8)(9)(10)(11). In the majority of cases AFXD is presumed to be caused by autoimmunity towards FX, and a specific FX inhibitor has been identified in some cases (5,8).…”

Section: Introductionmentioning

confidence: 99%

Successful Treatment of Life-threatening Bleeding Caused by Acquired Factor X Deficiency Associated with Respiratory Infection

et al. 2020

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Acquired factor X deficiency (AFXD) is a very rare coagulation disorder. A 40-year-old man with no comorbidities suffering from a fever, malaise, and severe hemorrhagic symptoms, including massive hematuria, was emergently admitted. His platelet count was normal, but his prothrombin time and activated partial thromboplastin time were markedly prolonged, which was thought to be due to autoantibody against a coagulation factor in the common pathway. Despite severe massive hematuria resulting in transient renal failure, he was successfully treated with urgent immunosuppressive therapy. Computed tomography revealed bronchopneumonia, which improved with antibiotic administration. AFXD without evidence of amyloidosis was subsequently diagnosed.

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Autoimmune Clotting Dysfunction

Königs¹

2020

The Autoimmune Diseases

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An Acquired Factor X Inhibitor: The Importance of Understanding Coagulation

Cited by 6 publications

References 5 publications

Successful Treatment of Factor X Deficiency in a Patient with Lymphoplasmacytic Lymphoma with Bendamustine Plus Rituximab Regimen: A Case Report and Literature Review

Successful Treatment of Factor X Deficiency in a Patient with Lymphoplasmacytic Lymphoma with Bendamustine Plus Rituximab Regimen: A Case Report and Literature Review

Successful Treatment of Life-threatening Bleeding Caused by Acquired Factor X Deficiency Associated with Respiratory Infection

Autoimmune Clotting Dysfunction

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