Intravenous Leiomyomatosis: An Uncommon Cause of Pulmonary Embolism.

BACKGROUND Intravenous leiomyomatosis (IVL) is a rare benign smooth muscle tumor originating in the uterus or in the uterine vessels. It is characterized by continuous intraluminal growth that may extend through iliac veins and inferior vena cava (IVC) to right chambers of the heart and pulmonary vasculature, leading to life-threatening complications. This case report describes an uncommon cause of non-thrombotic pulmonary embolism in young woman caused by extensive IVL. CASE REPORT A 39-year-old woman was admitted after multiple syncopal episodes. She was initially found to have a bilateral pulmonary embolism and large right atrial mass believed to be a thrombus. After an unsuccessful attempt to remove the thrombus with AngioVac (AngioDynamics, Latham, NY), subsequent sternotomy revealed a large pedunculated mass extending to the infra-hepatic IVC. Further abdominal imaging showed multiple uterine masses, with the largest about 17 cm, infiltrating the parauterine vessels and extending through the right iliac vein and inferior vena cava up to the right atrium. Pathology examination of the atrial mass revealed benign leiomyoma consistent with further pathology findings after hysterectomy. The pulmonary embolism was believed to be caused by tumor tissue, and anticoagulation was abandoned. Pulmonary nodule raised a suspicion of benign pulmonary metastases, but, fortunately, remained stable during follow-up and the patient had a successful recovery. CONCLUSIONS Available information about IVL is scarce. This tumor, although benign and rare, should be included in the differential diagnosis of cardiac tumors and non-thrombotic pulmonary emboli in women with predisposing risk factors, as potential complications are life-threatening.

Utilization of Emicizumab in Acquired Factor VIII Deficiency.

BACKGROUND Acquired hemophilia A (AHA) is a rare autoimmune disease caused by immunoglobulins that bind and inactive factor VIII, thereby predisposing to life-threatening bleeding. Bleeding is typically stabilized by utilizing bypassing agents, such as recombinant factor VIIa (rVIIa). Select case reports have demonstrated the success of alternative prophylaxis for clearance of factor VIII inhibitors through the use of emicizumab, a current FDA approved medication for treatment of congenital hemophilia A. In this case report we present the efficacy of utilizing emicizumab as a prophylactic agent in a patient that was unable to tolerate first-line therapy for prophylaxis. CASE REPORT A 91-year-old male presented for ongoing hematuria for 5 weeks with prior workup unrevealing. He was given a day's course of recombinant factor VIIa to stabilize his bleeding and was started on cyclophosphamide and prednisone after a revealing hematological workup including activated partial thromboplastin time (aPTT) >100 seconds and factor VIII inhibitor level of 44 BU/mL. He continued to require VIIa infusions to control his bleeding and was started on emicizumab once stabilized. His bleeding remained controlled and his inhibitor decreased after 6 months of therapy with repeat factor VIII inhibitor level of 1.9 BU/mL. CONCLUSIONS The success of utilizing emicizumab for bleeding prophylaxis in AHA is demonstrated by this patient's resolution of bleeding. The high frequency of dosing and higher risk for thrombosis with factor VIIa, in conjunction with our patient's medical history and ease of administration, make emicizumab an ideal agent for bleeding prophylaxis while awaiting clearance of factor VIII inhibitors.

Spontaneous Bacterial Peritonitis in Cardiogenic Ascites.

The most common cause of ascites is liver cirrhosis. Additional causes such as heart failure, cancer, and pancreatitis among others can also precipitate this abnormality. Spontaneous bacterial peritonitis (SBP) is an infection of ascitic fluid that happens without any evidence of an intra-abdominal surgically-treatable cause. Ascites of cardiac origin can also be complicated by SBP. Here we present a case of a 62-year-old male with extensive cardiac history who presented to our service with ongoing dyspnea and orthopnea. He also had significant abdominal distention and pitting edema. The patient was found to have constrictive pericarditis and was admitted for pericardiectomy. Ascitic fluid was consistent with a transudative process. Lab and imaging did not show evidence of liver or kidney disease. Ascitic fluid was indicative of ascites of cardiac origin. Postoperatively patient developed intermittent fevers initially thought to be due to pericarditis but later found to be due to SBP complicating his recurrent ascites. Such a temporal association of SBP that complicates ascites after pericardiectomy has not been discussed frequently in literature.