The patient’s blood film shows target cells, sickle cell, Howell Jolly bodies. It is difficult to say what the underlying condition is other than a sickling syndrome with hyposplenism. Target cells are often found in all of these disorders, but especially haemoglobin C. It is possible this could be Hb SC (heterozygous for HbS and HbC), but often these patients do not become hyposplenic so Howell Jolly Bodies are perhaps less common. Another possibility is haemoglobin S/beta thalassaemia; again difficult to distinguish on film alone, although the anaemia will be less severe if associated with a ‘beta-plus’ chain rather than beta-zero as more haemoglobin A will be made. Microcytosis is seen in HbSC and HbS/beta thal, which the patient did not have (sorry that info wasn’t given!). However the only way to be sure is to perform further testing. In this case the SickleDex test was positive and below was the HPLC:
This shows the patient has almost all haemoglobin S (therefore sickle cell anaemia; Hb SS). The amount of haemoglobin F is slightly elevated which may confer some advantage with less crisis. The patient was not on hydroxycarbamide.
Important points in the management of these patients is:
- Prophylactic penicillin
- Vaccinations
- Folic acid
- Self help guide (keeping warm, self referral, analgesia etc.)
- Transcranial doppler in children
- Rh and Kell matched, sickle negative blood if needed
- Hydroxycarbamide if frequent complications
- Prenatal counselling/partner testing
TeamHaem 