Iron Deficiency in Haemophilia- Severity, Correlation and Clinical Profile- A Cross Sectional Study from South India

Abstract

Haemophilia is an inherited X-linked recessive disorder caused by deficiency of Factor VIII or Factor IX which is required for the generation of Thrombin in the intrinsic pathway of coagulation. India has the second highest burden of haemophilia in the world. Iron deficiency is expected in these vulnerable population due to either presence of occult blood loss in urine/stool or deposition of iron in the synovium during the repeated episodes of bleeding. This study was to estimate the proportion of iron deficiency and to determine the association of severity of Haemophilia with the stage of iron deficiency in individuals with Haemophilia and to determine the clinic aetiological and haematological profile of iron deficiency in haemophiliac patients. Methods: The patients confirmed with Factor VIII/IX deficiency attending the Haemophilia OPD of Government Medical College, Kottayam during a period of 1 year were recruited. They were classified as mild (6-40%), moderate (1- 5%) and severe(<1%) based on their factor levels. Data collection was done using a case report form which included clinical characteristics of patients along with targeted blood investigations. The analysis was done using SPSS software. The data were summarized using descriptive and correlational statistics. Results: The proportion of iron deficiency in individuals with Haemophilia was found to be 27.1%. Of those with iron deficient erythropoiesis 66.7% had severe Haemophilia A and 9.5%had Haemophilia B. Of those with iron deficiency anemia 57.1% had severe Haemophilia A and 42.9% had severe Haemophilia B. Of the patients with low ferritin were 75% found to have iron deficient erythropoiesis while 25% were found to have iron deficiency anemia. The most common symptom associated with iron deficiency was muscular pain, joint pain, palpitations and shortness of breath .The most common sign associated with iron deficiency anemia was papillary atrophy of the tongue.

Conclusion: The proportion of iron deficiency in individuals with Haemophilia was found to be 27.1%. The more the severity of Haemophilia, the higher was the stage of iron deficiency of the patient.