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: gshimali@yahoo.com


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?


Previous Grand Rounds View All

G
Glycogen Storage Disease - Type 3
Suhani Jain, Ira Shah
A 9-month-old female child was referred for asymptomatic hepatomegaly detected by the local physician. Her birth history was normal and she had achieved milestones as per her age. The child had no fam....
T
Tuberculous Atlanto-axial dislocation – How to manage?
Suhani Jain, Ira Shah
An 8-year-old girl presented with pain and inability to extend her neck for three months. She had a history of occasional low-grade fever during this period, but she was afebrile at presentation. Ther....
A
Aberrant vasculature in the Liver
Suhani Jain, Ira Shah
A 2-month-old female infant presented for a secondary assessment following the identification of abnormal vessels during antenatal ultrasound. Subsequent imaging via CT abdomen at 1 month of age revea....
I
INH Monoresistance tuberculosis with inhA gene resistance - What should be the anti-tuberculosis regimen?
Vaidehi Mehta, Ira Shah
A 4-year-old boy presented with gradually increasing left axillary swelling for 7 months. There is no fever, cough, loss of appetite or weight loss or contact with a patient having tuberculosis (TB) c....
I
Is it Congenital toxoplasmosis?
Suhani Jain, Ira Shah
A 1 month old male child delivered by lower section cesarean section (LSCS) at 8 ½ months of gestation in view of meconium stained liquor with a birth weight of 2.09 kg was referred in view of a posit....
Disclaimer: The information given by www.pediatriconcall.com is provided by medical and paramedical & Health providers voluntarily for display & is meant only for informational purpose. The site does not guarantee the accuracy or authenticity of the information. Use of any information is solely at the user's own risk. The appearance of advertisement or product information in the various section in the website does not constitute an endorsement or approval by Pediatric Oncall of the quality or value of the said product or of claims made by its manufacturer.
0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0