A Rare Case of Factor VII Inhibitor in a Patient Presenting with Primary Splenic Marginal Zone Lymphoma

Naismith, Kendra; Allevato, Pat A.; Hamm, Caroline

doi:10.12659/ajcr.932704

Cited by 5 publications

(5 citation statements)

References 14 publications

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“…Acquired FVII deficiency was also associated with neoplastic disorders such as pleural liposarcoma, Wilms tumor, atrial myxoma and some hematological tumors, including multiple myeloma, acute myeloid leukemia and lymphoma. Nevertheless, these conditions were mostly linked to the presence of inhibitors [ 13 , 16 , 17 , 18 , 19 , 20 , 21 , 22 ].…”

Section: Discussionmentioning

confidence: 99%

Factor VII Deficiency in Systemic Mastocytosis with an Associated Myeloid Neoplasm

Rosati,

Camerlo,

Fornari

et al. 2024

Hematology Reports

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Factor VII (FVII) deficiency is a rare bleeding disorder that can be classified as congenital or acquired, and the majority of acquired cases are due to vitamin K deficiency or liver disease. Isolated acquired FVII deficiency is a rare occurrence and has been associated with inhibitors or auto-antibodies. Here, we describe a patient with polycythemia vera who developed systemic mastocytosis and FVII deficiency simultaneously. FVII deficiency was not caused by inhibitors and improved with antineoplastic treatment. Acquired FVII deficiency has been reported in cases of sepsis, possibly due to proteolytic degradation induced by the activation of monocytes or endothelial cells. Malignancies have been shown to cause a depletion in circulating FVII through the direct binding of cancer cells. This case report suggests a potential association between SM associated with a hematological neoplasm (SM-AHN) and acquired FVII deficiency. Further evaluations are recommended in patients with systemic mastocytosis to gain a better understanding of the relationship between pathological mast cells and clotting factor concentrations.

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Section: Discussionmentioning

confidence: 99%

Factor VII Deficiency in Systemic Mastocytosis with an Associated Myeloid Neoplasm

Rosati,

Camerlo,

Fornari

et al. 2024

Hematology Reports

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“…A decrease in FVII activity occurs in hereditary conditions as well as in the presence of an inhibitor (12). Although FVII inhibitors have been reported in certain cases (13)(14)(15), the incidence of this condition is extremely rare. In fact, an inhibitor of FVII was not identified in our patient.…”

Section: Discussionmentioning

confidence: 99%

Autoimmune Hepatitis Complicated by Undiagnosed Factor VII Deficiency: A Pitfall of Coagulopathy

Kishimoto,

Kakisaka,

Abe

et al. 2024

Intern. Med.

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Prothrombin time (PT) is a key parameter for assessing the severity of liver disease. We present the case of a 37-year-old woman with severe acute liver injury due to autoimmune hepatitis. Although prednisolone drastically improved her hepatocyte function, her PT did not recover to the reference range. A review of her medical records revealed that the patient had normal transaminase levels and prolonged PT 2 years previously. Further examinations of her coagulopathy revealed that she had low factor VII activity, suggesting a diagnosis of factor VII deficiency. Our experience suggests that altered coagulopathy should be considered in cases of liver injury with an extraordinary PT.

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“…Acquired factor inhibitors and the development of new lupus anticoagulant antibodies are rare but known phenomena in lymphoproliferative disorders [ 9 - 14 ]. This paraneoplastic syndrome is frequently reported as a presenting symptom in recurrent or rare lymphomas that are frequently aggressive in nature.…”

Section: Discussionmentioning

confidence: 99%

Treating Acquired Factor VIII Inhibitor and Tumor-Induced Hypoglycemia in a Case of Relapsed Diffuse Large B-Cell Lymphoma

Baker¹,

Norton²,

Hossain³

2023

J Hematol

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Diffuse large B-cell lymphoma (DLCBL) is a heterogenous disease, with many phenotypic subtypes and occasional paraneoplastic syndromes being present. Herein, we describe a case of a 63-year-old woman, with relapsed/refractory DLBCL (RR-DLBCL) with artifactual hypoglycemia on laboratory testing, likely related to the mechanical effects of a new factor VIII inhibitor. We demonstrate our workup, consideration, treatment, and her clinical course. This patient did not present with a bleeding phenotype despite her aberrant laboratory results, and therefore determining her risk of bleeding to weigh against further diagnostic procedures presented a difficult decision. We utilized rotational thromboelastometry (ROTEM) to assist with clinical decision making regarding her paraneoplastic factor VIII inhibitor and the patient’s bleeding risk. This led to a short course of dexamethasone. Her ROTEM improved, and an excisional biopsy was performed without any bleeding. To our knowledge, this is the only reported instance where this technology was utilized in this setting. We believe utilizing ROTEM to determine bleeding risk may be a beneficial tool for clinical practice in such additional rare cases.

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A Rare Case of Factor VII Inhibitor in a Patient Presenting with Primary Splenic Marginal Zone Lymphoma

Cited by 5 publications

References 14 publications

Factor VII Deficiency in Systemic Mastocytosis with an Associated Myeloid Neoplasm

Factor VII Deficiency in Systemic Mastocytosis with an Associated Myeloid Neoplasm

Autoimmune Hepatitis Complicated by Undiagnosed Factor VII Deficiency: A Pitfall of Coagulopathy

Treating Acquired Factor VIII Inhibitor and Tumor-Induced Hypoglycemia in a Case of Relapsed Diffuse Large B-Cell Lymphoma

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