Grand Rounds

Hematemesis in a child with beta thalassemia

Himali Meshram
Pediatric Gastroenterology, Happy Gut Child Superspeciality Clinic and Pediatric Gastroenterology unit, Nagpur, Maharashtra, India

Address for Correspondence: Himali Meshram, Ground floor, Amarjyoti palace, Wardha road, Lokmat square, Nagpur-440012, India. Email:

Keywords: hepatomegaly, thallesemia, hematemesis, portal cavernoma

Clinical Problem:
A 14 year old girl presented to us with hematemesis in April 2016. She was diagnosed to have heterozygous HBE thalassemia at the age of 2 years. Since then she was on regular blood transfusions. Her birth and development history is normal. On examination, weight was 32 kg (<3rd percentile), height was 147.5 cm (<3rd percentile). She had haemolytic faces. Abdominal examination revealed firm splenohepatomegaly. Other systems were normal. Complete blood count showed hemoglobin of 4.6 g/dl, total leukocyte count of 5,500 cells/mm3(polymorphs 55%, lymphocytes 40%), platelet count of 75,000 cells/mm3, total bilirubin- 0.9 mg/dl, direct bilirubin- 0.3 mg/dl, amino transferase -26 IU/L, alanine transferases- 28 IU/L, alkaline phosphatase- 56 IU/L, total protein- 5.2 gm/dl, albumin- 3 gm/dl, prothrombin time -12 seconds and INR- 1.3. HIV, Hepatitis C Elisa and HBsAg were negative. Ultrasound (USG) abdomen and doppler revealed portal cavernoma. Upper gastroesophageal endoscopy showed grade 3 varices which required endoscopic variceal ligation (EVL). She was also started on nonselective beta blocker (propranolol). As she had requirement of repeated blood transfusions and presence of portal cavernoma and hypersplenism; she underwent splenectomy with proximal lienorenal shunt in June 2016. She received low molecular heparin for 10 months post splenectomy. Pancytopenia improved hemoglobin of 10.3 g/dl, total leukocyte count of 10,000 cells/mm3 (polymorphs 44%, lymphocytes 49%, platelet count of 2, 86,000 cells/mm3). After 3 years of surgery, she again had hematemesis. Gastroduodenoscopy (Figure 1) showed grade 3 varices requiring EVL. USG abdomen and doppler showed portal cavernoma, with non-visualisation of lienorenal shunt. She is on regular follow up.

Figure1. Gastroduodenoscopy showing grade 3 varices requiring banding.
Hematemesis in a child with beta thalassemia

What is the cause of portal cavernoma in a thalassaemic child?

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