Acquired Factor XIII inhibitor associated with mantle cell lymphoma

Nixon, Christian P.; Prsic, Elizabeth Horn; Guertin, Christine A.; Stevenson, Ryan; Sweeney, Joseph D.

doi:10.1111/trf.13947

Cited by 10 publications

(9 citation statements)

References 28 publications

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“…Many previously published studies reported acquired coagulation inhibitors accompanying lymphoma, mostly acquired hemophilia A or factor VIII inhibitors. [15][16][17][18][19][20] Similarly, acquired factor X deficiencies or inhibitors showed association with lymphoproliferative diseases via three mechanisms. 7,21 The most prevalent mechanism was amyloid fibril absorption.…”

Section: Discussionmentioning

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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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%

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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“…Paraneoplastic manifestations of lymphoma include hematologic abnormalities and neurological deficits [ 1 ]. As summarized in Table 1 , paraneoplastic coagulopathies have rarely been reported in the setting of systemic involvement by lymphoma [ 2 , 3 , 4 , 5 , 6 , 7 , 8 ]. We report the first case of a paraneoplastic coagulopathy in isolated primary central nervous system lymphoma (PCNSL).…”

Section: Tablementioning

confidence: 99%

Acquired factor VII inhibitor associated with primary central nervous system Lymphoma: A case report

Goyal

Salmasi

Leavitt

et al. 2022

eJHaem

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Paraneoplastic coagulopathies are uncommon in patients with lymphoma. We report the first case of an acquired coagulopathy in a patient with isolated primary central nervous system lymphoma (PCNSL) demonstrating large-cell histology. In our patient, a paraneoplastic factor VII inhibitor significantly delayed a diagnostic lumbar puncture despite fresh frozen plasma and inactivated prothrombin complex concentrate. While her coagulopathy was effectively overcome with recombinant activated factor VIIa and subsequently with lymphoma-directed therapy, her delayed diagnosis likely contributed to a poor outcome. Our case highlights the importance of rapidly identifying and correcting paraneoplastic coagulopathies when PCNSL is suspected.

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“…The erythroblast count is a percentage of leukocyte count In this case series, cancer treatment allowed FXIII recovery. Acquired FXIII deficiency has also been described in several lymphoid pathologies including monoclonal gammopathy of undetermined significance, 6 mantle cell lymphoma, 7 and lymphoblastic leukemia. 8 These deficiencies are related to the presence of an antibody directed against FXIII that accelerates FXIII clearance and reduces FXIII activity.…”

Section: Induction Treatment Was Started By Elam02 Protocol (Nct00149162) Remission Was Achieved With No Erythroblastsmentioning

confidence: 99%

Acquired factor XIII deficiency in a child with pure erythroid leukemia

Feugray

Buchbinder

Buchonnet

et al. 2021

Pediatric Blood & Cancer

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Acquired Factor XIII inhibitor associated with mantle cell lymphoma

Cited by 10 publications

References 28 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

Acquired factor VII inhibitor associated with primary central nervous system Lymphoma: A case report

Acquired factor XIII deficiency in a child with pure erythroid leukemia

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