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Letters to the Editor

Indian Pediatrics 2005; 42:610-611

Hereditary Spherocytosis in a Family from Tamil Nadu


Hemolytic anemias are generally more prevalent in north India than south, and among the tribal population throughout the country. Of the hemolytic anemias, thalassemia, sickle cell anemia and G6PD deficiency are more prevalent than hereditary spherocytosis (HS)(1-3). Literature search did not reveal any report on this disorder from Tamil Nadu. We are describing a family with this disorder from Virudhachalam in Tamil Nadu.

Three siblings, two males aged twelve and nine years, and one female aged eleven years who were brought to our hospital for the complaints of breathlessness on exertion, puffiness of face and abdominal pain were observed to have mild jaundice, anemia and splenomegaly. The past history was unremarkable except for recurrent episodes of jaundice in all of them. Since the jaundice showed fluctuation in severity and several members of their family also had jaundice and were doing well they did not show further interest in the symptom. The family tree is shown in the Fig. I

Fig. 1. Pedigree chart.

From the history and clinical findings a diagnosis of familial hemolytic anemia was made. Investigations were suggestive of hereditary spherocytosis. Ultrasound examination of the abdomen did not show any evidence of cholelithiasis. Screening of other family members could not be done because of practical difficulties. Based on the laboratory findings in these children and from the history, it was concluded that other family members with jaundice also probably had HS.

About 80% of patients with HS show an autosomal dominant pattern of inheritance(4) as seen in the present family.

In the dominantly inherited form, the deficiency is mild and hence the anemia is mild, while in the non dominant forms the deficiency is greater and the anemia severe. The mild nature of the disorder in our children could be because of the dominant pattern of inheritance in them.

Splenectomy results in significant improvement in the clinical condition and prevents complications such as cholelithiasis. However, the advantages of splenectomy have to be weighed against the complications of surgery. In mild cases splenectomy may not be indicated. Since our children had only a mild disorder, splenectomy was not advised. They were put on folic acid suppplementation and are doing well on follow up.

M.R. Das,
S. Ananthakrishnan

Department of Pediatrics,
Mahatma Gandhi Medical College and Research Institute,
Pillayarkuppam, Pondicherry,
India.
E-mail: [email protected]

References

1. Dash S, Dash RJ. Spectrum of hemolytic anemias in Punjab, North India. Trop Geogr Med 1980; 32: 312 -316.

2. Kamble M, Chaturvedi P. Epidemiology of sickle cell disease in a rural hospital of Central India. Indian Pediatr 2000; 37: 391-396.

3. Mehta J, Harjai K, Vasani J, Banghar P, Sanklecha M, Singhal S, et al. Hereditory spherocytosis: experience of 145 cases. Indian J Med Sci 1992; 46: 103-110.

4. Lane PA, Nusa R, Ambruso DR. Hematologic disorders. In: Hayward RR, Levin MJ, Sandheimer JM. Current Pediatric Diagnosis and Treatment.15th edn. New York: McGraw- Hill; 2001. p 846-848.

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