Common clinical presentations of SCD
| Clinical presentation | Symptoms | Laboratory findings | Treatment |
|---|---|---|---|
| Painful crisis | Pain, localised swelling, fever | Low Hb, high reticulocyte count, high LDH, high bilirubin, high CRP | Hydration Analgesia Antibiotics |
| Acute chest syndrome | Chest pain, fever, hypoxia, cough; may progress from painful crisis elsewhere | Low Hb, high reticulocyte count, high LDH, pulmonary infiltrates on CXR | Respiratory support, antibiotics, red cell exchange transfusion |
| Bacterial sepsis | Pain, localised swelling, fever | Low Hb, high reticulocyte count, high LDH, high CRP, positive cultures | Hydration Analgesia Antibiotics |
| Sequestration crisis | Pain, severe pallor, hepatomegaly or splenomegaly | Low Hb, high reticulocyte count | Urgent red cell transfusion, pain relief |
| Aplastic crisis | Pallor | Low Hb, low reticulocyte count, parvovirus B19 +ve | Urgent red cell transfusion |
| Acute ischaemic stroke | Hemiplegia, altered consciousness, seizures | MRI brain with characteristic findings | Urgent red cell exchange transfusion aim to reduce HbS to <30% |
| Girdle syndrome | Abdominal pain and distension, reduced or absent bowel sounds, pallor, fever | AXR may show dilated bowel loops. Low Hb, high reticulocyte count, high CRP | Nil by mouth, NG tube on free drainage, broad spectrum antibiotics with anaerobic cover, red cell exchange transfusion, surgical review |
| Priapism | Painful, persistent erection | Hydration, pain relief, urgent urology review and intervention: red cell exchange transfusion |
AXR, abdominal x-ray; CRP, C-reactive protein; CXR, chest x-ray; Hb, haemoglobin; HbS, sickle haemoglobin; LDH, lactate dehydrogenase; MRI, magnetic resonance imaging; NG, naso-gastric; SCD, sickle cell disease.