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A 41-year-old diabetic woman developed endocarditis of the aortic valve caused by Paecilomyces javanicus six years after insertion of a porcine mitral valve heterograft. The patient died shortly after aortic valve replacement. Autopsy revealed vegetations of the aortic heterograft, valve ring abscess and ascending aortitis due to Paecilomyces. There was no involvement of the mitral valve heterograft. Lesions due to mycotic emboli were found in the kidneys, spleen, and brain. Cultures of the surgically removed aortic valve and of the kidney at autopsy produced rapid growth of P. javanicus. The gross and microscopic pathologic and cultural characteristics of this organism are described with a review of the literature. Previously reported cases of Paecilomyces endocarditis occurred only in prosthetic heart valves. This is the first known report of P. javanicus endocarditis of a native valve and its prosthetic heart valve heterograft.

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Patient: Female, 38-year-old Final Diagnosis: Coagulopathy • splenic marginal zone lymphoma Symptoms: Abdominal swelling • early satiety Medication: — Clinical Procedure: Splenectomy Specialty: Hematology Objective: Rare disease 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 immunopheno-type 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.

An intracytoplasmic microfilamentous lattice, ultrastructurally identical to Hirano body, was found in an inflammatory cell within a leptomeningeal vessel wall infected by fungus Paecilomyces javanicus. The structure was well preserved and not associated with phagosomes. This is the first report of Hirano body found in an inflammatory cell at the site of fungal infection. The present finding suggests that the formation of Hirano body is non-specific and not restricted to the cell of the neuro-muscular system.