A 25 year old lady came with anemia, jaundice and recurrent joint pains. All of the following are true except:: (A) HbA will be undetectable, (B) She may have retinopathy, (C) She can present with pulmonary bleeds, (D) Hydroxyurea would help her
Answer is C. Anemia with jaundice points to hemolytic anemia and presence of joint pain indicates sickling leading to vaso-occlusive crisis. This is likely a case of Sickle cell anemia. The major hemoglobin in sickle cell anemia is HbS and HbA will be undetectable. Proliferative sickle retinopathy (PSR) is the most severe ocular change in SCD. Patients can present with acute chest syndrome and not pulmonary syndrome. Acute chest syndrome is- chest pain, tachypnea, fever, cough, and aerial oxygen desaturation. Hydroxyurea: 1. Increases fetal hemoglobin and reducing sickling. 2. Beneficial effects on RBC hydration. 3. Reduces vascular wall adherence of sticky reticulocytes.