A 25 year old lady came with anemia, jaundice and recurrent joint pains. All of the following are true except:
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.
HbA will be undetectable
She may have retinopathy
She can present with pulmonary bleeds
Hydroxyurea would help her
Sickle Cell Disease, G6PD deficiency and other Hemolytic Anemia