Sickle haemoglobin and haemoglobin Stanleyville II: possible confusion with sickle cell-haemoglobin C disease.
OBJECTIVE
To bring to the attention of East African practitioners, the characteristics of Hb Stanleyville II, its interaction with HbS, and the resemblance of the double heterozygote to sickle cell-haemoglobin C (SC) disease.


METHODS
A prospective study of 100 patients with Sickle Cell (SS) disease in the steady state attending the sickle cell Clinic at Mulago Hospital, Kampala, Uganda.


METHODS
Out of 100 patients with SS disease, two were also heterozygous for an alpha chain variant identified as Hb Stanleyville II.


CONCLUSIONS
In association with HbS, Hb Stanleyville II produces a hybrid haemoglobin band which on alkaline haemoglobin electrophoresis, travels in the position of HbC. Such cases may cause confusion with sickle cell-haemoglobin C (SC) disease. The index cases in both families had associated alpha thalassaemia but from this small group, no conclusions may be drawn on the haematological or clinical significance of the interaction of Hb Stanleyville II with SS disease.
Serjeant
G R
GR
Sickle Cell Trust, Jamaica, West Indies.
Wild
B
B
Tebasulwa
S
S
Mason
K P
KP
Serjeant
B E
BE
Ndugwa
C M
CM
D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood* / Q000175:diagnosis* / Q000235:genetics; D001797:Blood Protein Electrophoresis; D003937:Diagnosis, Differential; D005260:Female; D006450:Hemoglobin SC Disease / Q000097:blood / Q000175:diagnosis / Q000235:genetics; D006451:Hemoglobin, Sickle / Q000032:analysis*; D006455:Hemoglobins, Abnormal / Q000032:analysis*; D006801:Humans; D008297:Male; D010375:Pedigree; D011446:Prospective Studies; D014454:Uganda
D006451:Hemoglobin, Sickle; D006455:Hemoglobins, Abnormal; C037389:hemoglobin Stanleyville II