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

BACKGROUND Here, we report the novel presentation of a factor VII inhibitor in association with a new diagnosis of splenic marginal zone lymphoma in a previously healthy 38-year-old woman. There are only 4 reported cases of factor VII inhibitors, none of which are secondary to a splenic marginal zone lymphoma. CASE REPORT Our patient, a 38-year-old woman, presented reporting increased abdominal swelling and early satiety. She was found to have pancytopenia, an elevated international normalized ratio (INR), normal partial thromboplastin time (PTT), and massive splenomegaly. Further investigation revealed a morphology and immunophenotype most consistent with splenic marginal zone lymphoma. A mixing study was unable to bring the INR into normal range after 60 min, confirming a factor VII inhibition. Therefore, the final diagnosis was primary splenic marginal zone lymphoma and secondary factor VII inhibitors. Owing to the elevated INR, both chemotherapy and splenectomy were avoided and we began a 4-week course of weekly rituximab infusions. After a second course of 4 treatments, there was a resolution of both the coagulopathy and the splenomegaly. At this point, the splenectomy was safely performed. Maintenance rituximab continued for 2 years. Our patient has now been in remission 12 years. CONCLUSIONS We successfully treated a rare factor VII inhibitor and its underlying splenic marginal zone lymphoma with rituximab immunotherapy. A complete response was documented by splenectomy. The patient's 12-year remission of both the lymphoma and the inhibitor helps to support the causative relationship between the lymphoma and the factor VII inhibitor.