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He received multiple transfusions and trial of steroids with no benefit. He developed shortness of breath on exertion despite maintaining haemoglobin more than 10 g/dl. His Echocardiogram showed normal LV function. His pulmonary function tests revealed restrictive lung disease. He had high BNP (Brain natriuretic peptide) likely due to pulmonary hypertension with ongoing haemolysis, however pulmonary pressures were not measured. He had options of Eculizumab and Bone marrow transplant. In view of cost he chose to go with Bone marrow transplant. Androgen therapy and Immuno suppressants can be tried [3] in early part of diagnosis which is not offered in view of failure to respond to steroids and pulmonary hypertension.

Role of Allogenic Bone Marrow Transplant in Patients with Paroxysmal Nocturnal Haemoglobinuria