A 78-year-old man was admitted with a chief complaint of black stool. Ten days prior to his recent hospitalization, the patient was admitted to a nearby hospital with the same complaint. Esophagogastroduodenoscopy (EGD) was performed, and hemostatic procedures were done three times because of exudative hemorrhage and a Dieulafoy’s lesion in the lesser curvature of the antrum and upper body of the stomach (). Melena was observed during digital rectal examination at the time of admission, and fresh red-colored blood was noted in gastric lavage via a nasogastric tube. The patient has been taking medications for hypertension, heart failure, and asthma, excluding anticoagulants or antiplatelet medications. He was a non-smoker and non-alcoholic.\nAt the time of visit, no symptoms other than the main complaint was noted during a systems review. Upon physical examination, he had an acute ill-looking appearance, and the conjunctiva and skin were slightly pale. There was no scleral jaundice and liver or spleen enlargement. His blood pressure was 110/70 mm Hg, pulse rate was 98 times/min, respiratory rate was 20 times/min, and body temperature was 38.6℃. Chest X-ray revealed no abnormalities, and the electrocardiogram showed sinus tachycardia.\nLaboratory tests were conducted. Peripheral blood tests evaluated the leukocyte (19,570/mm3) and platelet count (205,000/mm3) and hemoglobin level (5.8 g/dL). Biochemical tests included measurement of aspartate aminotransferase (17 mg/dL), alanine aminotransferase (46 mg/dL), total protein (4.1 g/dL), albumin (2.2 g/dL), total bilirubin (1.0 mg/dL), serum sodium (135 mEq), and serum potassium (3.7 mEq/L) levels. Blood urea nitrogen (41 mg/dL), creatine (1.24 mg/dL), C-reactive protein (11.89 mg/dL) levels were all elevated. The prothrombin time (PT) was 15.4 seconds, and the activated partial thromboplastin time (aPTT) was 49.8 seconds, both of which were prolonged.\nSince disseminated intravascular coagulation (DIC) due to repeated bleeding was suspected, the patient underwent an EGD based on the findings of melena and hemoglobin decline. Vascular formation was observed in the lesser curvature of the mid-body of the stomach, and argon plasma hemostasis was done (). On day 3 of admission, the hemoglobin level decreased, and follow-up EGD was performed. Argon plasma hemostasis revealed oozing bleeding in the upper body of the stomach (). The patient complained of back pain, and a bruise was observed on the right flank (). Computed tomography revealed a hematoma approximately 7 cm in size in the right psoas muscle (). On day 4 of admission, 650 cc of melena was noted. Hence, EGD was performed. Results showed the presence of a gastric ulcer directly below the esophagogastric junction. An epinephrine solution was injected around the ulcer. On day 5, the patient complained of 200 cc of melena, and the hemoglobin level declined. Surgery was performed on the seventh day to achieve surgical hemostasis. At the time of surgery, there was no source of bleeding except on the area just below the esophageal junction. On the day following surgery, exudative hemorrhage from the surgical wound and 350 cc of black stool were observed. Hemoglobin level was reduced to 6.0 g/dL, PT was prolonged to 14.9 seconds, and aPTT was prolonged to 55.4 seconds. PT was corrected in the plasma mixing test; however, the aPTT was not corrected. In the additional test, antibody against blood coagulation FVIII (2.52 BU) was observed. Based on this test, the patient was diagnosed with AHA and bleeding. Then, he was transferred to the Hematology Department, and recombinant activated FVIII was administered. Consequently, the bleeding seemed to stop. Nonetheless, he refused recombinant activated FVIII because of the cost burden, and he died of shock on the 16th day of admission despite continued supportive care.
A 74-year-old Caucasian female with myasthenia gravis admitted with melena and a hemoglobin of 5.8 g/dl. Her history was significant for small bowel perforation requiring bowel resection and anastomosis two years before the current presentation followed by multiple admissions for anemia treated with blood transfusions and iron supplementations. She did not have any personal or family history of malignancies or bleeding disorders. She was diagnosed with myasthenia gravis a year before the current presentation and was started on pyridostigmine and high dose steroids initially. The steroids were tapered down to prednisone 5 mg daily, six months before the current presentation. She reported non-compliance with her medications for myasthenia gravis. One month before the current presentation, she had an episode of life-threatening acute gastrointestinal bleeding; however, endoscopy did not reveal a clear bleeding source, and the cause was attributed to erosive esophagitis. During the current admission for melena, both endoscopy and colonoscopy failed to show active bleeding. Tagged red blood cell scan, CT angiogram (CTA), capsule endoscopy showed oozing from the bowel anastomotic area. Surgical exploration was deferred given her multiple comorbidities. Later embolization of the bleeding vessel at the site of anastomosis was done by interventional radiology. One week after the procedure, she started to have melena and CTA demonstrated active extravasation again from the anastomotic area. Coagulation studies were performed in the setting of repeated bleeding and showed isolated activated partial thromboplastin time (APTT) prolongation which was not corrected by mixing study. Her FVIII activity was markedly reduced to <1% and factor IX activity was normal. Prothrombin time, fibrinogen, von Willebrand factor, lupus anticoagulant, malignancy workup, and hepatitis panel were normal. Bethesda titer showed elevated inhibitor levels at 91 Units. Steroids and rituximab were administered, and she was discharged with a plan of weekly rituximab for four weeks. She was readmitted two days after the discharge with spontaneous chest wall hematoma. CTA showed a large left pectoral hematoma measuring 14 x 13 x 5 cm with active extravasation (Figure ). Factor Eight Inhibitor Bypassing Agent (FEIBA), recombinant factor VIIa, and steroids were given to control active bleeding. Clinically patient had stability of bleeding. However, during her subsequent hospital course, she acutely became unresponsive and resuscitative efforts proved to be unsuccessful, and the patient passed away. Intracranial hemorrhage was considered as a possible cause for her sudden death; however, an autopsy was not performed according to her family’s wishes.