Case Presentation
A 42-year-old male Ethiopian patient presented to Yekatit 12 Hospital Medical College with a two-day history of worsening of shortness of breath, which followed multiple episodes of massive bilateral epistaxis. He also noted light-headedness, dizziness, tinnitus, easy fatigability, intermittent vomiting of ingested matter, generalized body swelling which started from the legs, palpitation, orthopnoea and paroxysmal nocturnal dyspnoea.
Further inquiry revealed history of bilateral nasal bleeding which initially started 6 years back. Over time, the epistaxis became more frequent and severe, sometimes bleeding up to 1 liter per episode. He also had passage of black tarry stools for the last 7 months which was first noticed after hospitalization. Before his current admission, he was hospitalized 3 times in the last 2 years, with diagnosis of high output heart failure secondary to severe anemia. On his last admission, he received 5 units of packed red blood cells and was also given oral iron, folic acid and proton pump inhibitors. His mother had epistaxis through bilateral nostrils which started in her early adulthood. She died of unknown cause at the age of 60.
Physical examination was significant for tachycardia (pulse rate of 106 beats per minute), pale conjunctivae and tiny erythematous lesions over the dorsum of his tongue which blanch on pressure (Figure 1), bibasilar lung rales, raised JVP, ejection systolic murmur at the apex, and pitting leg edema.
Laboratory investigations revealed hemoglobin of 4g/dl (MCV 78fL), iron studies revealed low serum iron level of 20ug/dl (reference range: 33-193ug/dl) and low ferritin level of 16.8ng/ml (reference range: 30-150ng/ml); liver enzymes and renal function tests which were with in normal limits and normal coagulation profile. Peripheral blood smear showed microcytic and hypochromic anemia. Chest x-ray revealed grade I pulmonary edema and borderline cardiomegaly but echocardiography showed signs of high output heart failure with ejection fraction of 70%. Upper GI endoscopy showed multiple telangiectatic spots in the stomach and duodenum oozing heme while colonoscopy showed pale mucosa with normal vascular architecture (Figure 2). Abdominal ultrasound with doppler study showed minimal bilateral pleural effusion and hepatic AV malformations (Figure 3).