Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2022 Jun 7;3(3):1000-1002.
doi: 10.1002/jha2.482. eCollection 2022 Aug.

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

Vanshika Goyal  1 , Giselle Salmasi  2 , Andrew D Leavitt  2   3 , James L Rubenstein  2 , Rahul Banerjee  2   4
Affiliations
Case Reports

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

Vanshika Goyal et al. EJHaem. .

Abstract

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.

Keywords: CNS lymphoma; case report; coagulation; factor VII; lymphoma.

PubMed Disclaimer

Conflict of interest statement

VG: No interests to disclose. GS: No interests to disclose. ADL: BioMarin, advising; Bioproducts Laboratory; Catalyst Biosciences, advising; Dova Pharmaceuticals, advising; Johnson & Johnson, shareholder; Merck, consulting. JLR: BMS, research support; Genentech, research support. RB: Curio Science, honoraria; Eradigm Consulting, consulting; i3 Health, advising; Guidepoint Global, consulting; Sanofi Pasteur, consulting; SparkCures, consulting.

Figures

FIGURE 1
FIGURE 1
Time course of patient's coagulopathy and treatment. Hospital days represent days since arrival to our facility. The reference interval for prothrombin time at our institution is shaded in grey. FFP, fresh frozen plasma; MTX, methotrexate; rFVIIa, recombinant activated factor VII; RTX, rituximab
See this image and copyright information in PMC

References

    1. Hagler KT, Lynch JW Jr. Paraneoplastic manifestations of lymphoma. Clin Lymphoma. 2004;5(1):29–36. - PubMed
    1. Gesierich W, Munker R, Geiersberger U, Pohlman H, Brack N, Hartenstein R. Spontaneous bleeding in a patient with malignant lymphoma: A case of acquired hemophilia. Onkologie. 2000;23(6):584–8. - PubMed
    1. Lee ES, Hibsman BK, Liebman HA. Acquired bleeding disorder in a patient with malignant lymphoma. Cancer. 2001;91(4):646–1. - PubMed
    1. Aljohani NI, Matthews JH. Acquired factor V inhibitor in a patient with mantle cell lymphoma presenting with hematuria followed by thrombosis: A case report. Int Med Case Rep J. 2014;7:27–30. - PMC - PubMed
    1. Meenhuis A, van Vliet R, Hudig F, Ypma PF, Schipperus MR, Hollestelle MJ. Successful treatment of a noninhibitory antibody‐mediated acquired factor X deficiency in a patient with marginal‐zone lymphoma. Clin Case Rep. 2015;3(7):587–93. - PMC - PubMed

Publication types