TITLE:
Hemoglobin K-Woolwich (Hb KW): Its Combination with Sickle Cell Trait
AUTHORS:
Ruchika Sharma, Melissa Rhodes, Elizabeth Varga, Samir B. Kahwash
KEYWORDS:
Hemoglobin K-Woolwich, Hemoglobin Electrophoresis, Fast Hemoglobin Variant, Sickle-Cell Anemia
JOURNAL NAME:
Open Journal of Pathology,
Vol.4 No.3,
July
21,
2014
ABSTRACT: Hemoglobin K-Woolwich (Hb KW)
is a rare hemoglobin variant with very few cases reported. It is most prevalent
in West African countries, particularly Nigeria, Ghana, and the Ivory Coast.
Some reports suggest Hb KW may be a clinically benign trait, whereas others
indicate it may behave similarly to a β+
thalassemia. The combination of hemoglobin S and hemoglobin KW (Hb S/KW) is a
rare double heterozygous disorder with little known clinical characteristics.
We report the hematologic and clinical data on three patients with Hb S/KW to
help describe the characteristics of this patient population. The first two
cases represent first cousins, ages 3 and 2 years. They are clinically
asymptomatic. They have normal hemoglobin and mean corpuscle volume (MCV)
levels without reticulocytosis. The third case is of a 14-year-old male who is
non-anemic with no microcytosis. He has been clinically well except for
abdominal pain upon dehydration. On hemoglobin electrophoresis, these patients
have Hb S levels slightly higher than typically observed with sickle cell trait
and a delay of hemoglobin F to adult levels. There exists a need for more
reports to better delineate the clinical course and management of these
patients.