BALANCED GLOBIN SYNTHESIS BY Hb K WOOLWICH HETEROZYGOTES

Zago, Marco Antônio; Costa, Fernando Ferreira; Greene, Lance; Bottura, C

doi:10.1111/j.1365-2141.1986.tb07590.x

Cited by 2 publications

(1 citation statement)

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“…Six years later, Zago et al described the globin synthesis and hematologic characteristics of a Brazilian black family with six Hb KW heterozygotes [9]. The proband was a 2-year-old boy who presented with microcytic and hypochromic anemia and ancylostomiasis (Hookworm).…”

Section: Discussionmentioning

confidence: 99%

Hemoglobin K-Woolwich (Hb KW): Its Combination with Sickle Cell Trait

Sharma¹,

Rhodes²,

Varga³

et al. 2014

OJPathology

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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.

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Section: Discussionmentioning

confidence: 99%