CORRELATION OF HAEMOLYTIC FACTORS AND HYDROXYUREA TREATMENT IN SICKLE CELL ANAEMIA WITH PULMONARY HYPERTENSION
Journal Title: Journal of Evolution of Medical and Dental Sciences - Year 2017, Vol 6, Issue 60
Abstract
BACKGROUND Retrospective and prospective studies1-4 have shown that 20% - 40% of adult sickle cell patients have evidence of pulmonary hypertension on 2D Echocardiography evaluation. It is also an independent risk factor for death in sickle cell anaemia. Various studies4-6 reported 40% - 50% two-year mortality rate in sickle cell anaemia with pulmonary hypertension, which is quite high. Haemolysis is a proposed mechanism in development of PH in SS patients, while hydroxyurea has interesting relation with nitric oxide biology. The objectives of this study are- 1) Evaluation of haemoglobin level, WBC count, platelet count, reticulocyte count and their relationship with SS patients; 2) Evaluation of serum bilirubin, serum LDH in relation with PH in SS patients; and 3) To establish relation of HU treatment with PH in SS patients. MATERIALS AND METHODS It is a hospital-based cross-sectional study; 88 cases of Sickle Cell Anaemia (SS) diagnosed as SS patterned on Hb electrophoresis were evaluated for various haemolytic factors, rheological factors and treatment with hydroxyurea to establish their relationship with pulmonary hypertension in sickle cell anaemia patient (SS pattern on Hb electrophoresis) and grouped in 2 groups as SS with Pulmonary Hypertension (PH) and SS without PH by 2D Echocardiography evaluation with Tricuspid Regurgitant Jet Velocity (TRV) of > 2.5 m/s and < 2.5 m/s respectively. RESULTS There were 66 male and 22 female cases; 32 (36.36%) cases had PH and 56 (63.64%) cases were without PH. There was no significant age and gender relation with PH in SS. Mean number of hospital admissions in the past were 3.63 ± 1.29 in SS with PH and 2.76 ± 1.08 in SS without PH having highly significant difference (p value = 0.001). Mean duration of illness from diagnosis of SS was not significant. Mean number of blood transfusions received in the past in SS with PH were 2.21 ± 1.82 and 1.03 ± 1.11 in SS without PH with significant difference. There was no relation in number of vaso-occlusive crises in past with PH. Mean Haemoglobin was 7.63 ± 0.69 in SS with PH and 8.25 ± 1.15 gm/dL in SS without PH group. This difference was statistically significant. Reticulocytosis also had positive relation with PH. Biochemical parameters such as high serum Lactate Dehydrogenase (LDH) had statistically significant relation with PH. Patients of SS without PH receiving Hydroxyurea were 26.78%, while only 6.25% of SS with PH patients were receiving Hydroxyurea. This difference was statistically significant. Zinc and Folic acid did not find significant difference in relation with PH. CONCLUSION Serum LDH is a good laboratory marker of haemolysis as well as PH in SS patients along with low haemoglobin and reticulocytosis. It may be used as a predictor of PH in sickle cell anaemia patients. There is high risk of development of PH in those sickle cell anaemia patients who were not receiving hydroxyurea suggesting its use in SS patients for primary prevention of pulmonary hypertension in sickle cell anaemia.
Authors and Affiliations
Bharatsing D. Rathod, Sunil D Bhaisare, Keyur Kishor Dave
Keywords
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- EP ID EP226804
- DOI 10.14260/Jemds/2017/951
- Views 66
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