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 male, 52-year-old patient visited the Instituto Nacional de Cancerología (INC) in Bogotá, Colombia, because of the development of a mass in the back region. There had been progressive growth and multiple drainage attempts due to a suspected abscess at various referral sites without relief. He was given non-oncological surgical resection in another institution, and his pathology report was compatible with high-grade sarcoma with positive margins. Additionally, he referred to bilateral vision loss that had been evolving for one month. The initial clinical exam revealed a patient with normal vital signs who was disoriented with regard to space and time though awake and had poor communication with the examiner. He presented a hyperpigmented tumor measuring 10 x 8 cm in the back region, along with a scarred postsurgical lesion without inflammatory signs. The biopsy was reviewed by the INC Pathology Department, which indicated a high-grade sarcoma compatible with myxofibrosarcoma.\nHe was examined by the Ophthalmology Department, which considered bilateral retinal detachment probable. An abdominal computed tomography (CT) scan was performed, which showed a diffuse density alteration of the subcutaneous cellular tissue, without evidence of lesions, probably due to edema. A chest computed tomography (CT) scan with contrast showed an undefined heterogeneous mass dependent on the soft tissues in the posterior thorax wall, with infiltration signs in the muscular plane and the density alteration of the subcutaneous cellular tissue and skin (See Figure ). He was examined by clinical oncology, surgical oncology, and radiation oncology services, which indicated he was not a candidate for chemotherapy, surgical treatment, or radiotherapy because of the significant extension of the tumor into the upper and lower back, the inadequate response of these tumors to chemotherapy, and the patient’s poor Zubrod performance score.\nDuring inpatient care, the patient showed substantial clinical worsening characterized by the progressive deterioration of his consciousness state and persistent oral cavity bleeding. Laboratory tests were requested, which showed the prolongation of blood clotting time due to extended partial thromboplastin time (PTT), with the test value for prothrombin time (PT) being 11.9 seconds and that for PTT being 50.1 seconds (normal value until 28 seconds). The mixing study showed PTT fixing with a 50:50 normal plasma sample that lasted for the first and second hour, suggesting intrinsic pathway coagulation factor deficiency. Even though the mixing study was not suggestive of the presence of inhibitors, the medical history of the patient, the presence of an active neoplasm, and the recent beginning of bleeding created a clinical suspicion of acquired hemophilia. For these reasons, a new mixing study, VIII factor assay, and Bethesda assay to measure factor VIII inhibitors were requested. In addition, treatment with prednisone at 1 mg/kg/day and factor VIII at 1700 international unit (IU) every 12 hours was initiated. The results of these assays showed a basal PTT of 67 seconds, which with the 50:50 sample with normal plasma, was reduced to 38,2 seconds and maintained at around normal values two hours afterwards, with a value of 36,3 seconds. Factor VIII was measured at 4% and remained at this same value (4%) 10 minutes after the injection of intravenous factor VIII. The specific factor VIII inhibitor assay reported 1.6 Bethesda units. A diagnosis of acquired hemophilia was made based on the presence of hidden low-titer inhibitors in the mixing study.\nBased on the findings above, the persistence of oral cavity bleeding, and daily transfusion support in the form of two red blood cell units, IV treatment with an activated prothrombin complex like the factor eight (VIII) inhibitor bypassing activity (FEIBA) at a dose of 3500 units/12 h (50 U/kg every 12 hours) was initiated. During the patient’s evolution, hypofibrinogenemia development, the continuous elevation of D-dimer levels, and progressive thrombocytopenia suggested a diagnosis of disseminated intravascular coagulation (DIC). Because of the unresolved bleeding, it was decided to initiate a transfusion of cryoprecipitate and tranexamic acid oral rinse while augmenting the FEIBA dose. The patient developed a low response to the established treatment, along with more profound neurological impairment (central nervous system bleeding was ruled out) and the persistence of red blood cell, platelet, and cryoprecipitate transfusion requirements. Additionally, the patient had severe hyponatremia in the form of an electrolyte imbalance. Electrolyte correction was initiated, and because of the persistence of bleeding, oral cyclophosphamide was added to the immunosuppressive treatment, with the objective of eradicating the inhibitors. Seven days afterward, because of the lack of clinical response to management and the impossibility of providing specific antineoplastic treatment, with the agreement of the patient´s family, transfusion support was suspended, and palliative care was initiated. The patient died one week after.