Case PresentationAN INFANT, age 2½ months, was referred to this hospital from the Dhahran Health Center with a history of vomiting fresh blood and clots six weeks prior to admission and recurrent melena. She had required three admissions to Dhahran Health Center and at one time was in shock with a hemoglobin of 4.5 gm/dl. There was no history of jaundice. The patient was investigated for bleeding tendency and for anatomical causes of bleeding. She had thrombocytopenia. Studies for DIC were negative. Esophagoscopy and gastroscopy revealed small bleeding points in the stomach. She was referred to the King Faisal Specialist Hospital for further investigation.Parents were not consanguinous; she had seven healthy siblings. There was no family history of bleeding tendency. The patient was the product of a full-term pregnancy and normal vaginal delivery, with no prenatal or neonatal complications. Her development was adequate for her age. She was fed Similac formula and had had no immunizations.On admission she had a height of 60 cm, weight 4.8 kg, temperature 37.7°C, respiratory rate 32/min, pulse 130/min. Height and weight were normal for age. Mucous membranes were pale, but there was no telangiectasis. ENT was negative, lungs clear, and heart regular and rhythmic with no murmurs. On abdominal examination, there was no hepatosplenomegaly, no masses, or bruit; and bowel sounds were normal. Genitalia were normal; examination of extremities showed no abnormalities. Neurologic examination was physiologic.Laboratory data: White blood cell count was 14,900/mm 3 , segmented neutrophils 20, band neutrophils 6, lymphocytes 66, monocytes 2, eosinophils 3, metamyelocytes 2, myelocytes 1, nucleated RBCs 2, hemoglobin 7.6 gm/dl, hematocrit 21.8, mean corpuscular volume (MCV) 78.4 µm 3 , mean corpuscular hemoglobin (MCH) 27.6 µg/dl, mean corpuscular hemoglobin concentration (MCHC) 35.2 gm/dl, reticulocyte 7.2%. Smear revealed polychromasia, hypochromia, and microcytosis. Platelet count was 76,000/mm 3 . Fibrinogen was 250 mg/dl. Prothrombin time was normal. Urinalysis revealed 2 to 3 RBCs per high power field; BUN was 29 mg/dl, creatinine 0.5 mg/dl, sodium 135 mEq/1, potassium 5.5 mEq/1, chloride 105 mEq/1, carbon dioxide 13 mmol/1, liver enzymes were normal, total bilirubin 0.2 mg/dl, total protein 5.2 gm/dl, albumin 3.8 mg/dl, alkaline phosphatase 303 IU/1, lactic dehydrogenase (LDH) 389 IU/1. Several stool specimens were examined and revealed no ova, parasites, fat globules, or fibers. Stools were positive for occult blood. Findings from chest x-ray and upper GI series were negative.
Course in hospital:The infant continued to have recurrent melena requiring multiple blood transfusions. Fourteen units of packed cells and multiple platelets and fresh plasma transfusions were given. DIC studies were negative. There was a decreased platelet count. After studies for a bleeding tendency, the child was investigated for anatomical cause of the bleeding. An upper GI series was negative. Upper GI endoscopy revealed multiple hemorrhagic a...