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Does elevated hemoglobin F modulate the phenotype in Hb SD-Los Angeles?

Adekunle Adekile¹, Ali Mullah-Ali, Najwa Ali Akar

¹Department of Pediatrics, Kuwait University, Kuwait, Kuwait. adekile@hsc.edu.kw

Acta Haematologica

|January 30, 2010

Summary

Compound heterozygotes with Hemoglobin (Hb) SD-Los Angeles and elevated Hb F experience severe symptoms. Unlike Hb SS patients, elevated Hb F does not appear to improve the clinical outcome in Hb SD-Los Angeles.

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Area of Science:

Hematology
Genetics
Pediatrics

Background:

Hemoglobin (Hb) SD-Los Angeles compound heterozygotes typically present with severe clinical manifestations.
The impact of elevated fetal hemoglobin (Hb F) on the clinical phenotype of Hb SD-Los Angeles has not been previously documented.

Observation:

This study details 5 Kuwaiti children diagnosed with Hb SD-Los Angeles and Hb F levels exceeding 20%.
These children exhibited early-onset sickling-related symptoms (by age 2) and were monitored for 3-15 years.
All participants experienced severe clinical courses.

Findings:

The observed severe clinical course included splenic sequestration crises, acute chest syndrome, vaso-occlusive crises, osteomyelitis, and avascular necrosis of the femoral head.
This contrasts sharply with the typically milder presentation observed in Kuwaiti patients with Hb SS and elevated Hb F.
Elevated Hb F levels did not ameliorate the clinical phenotype in these Hb SD-Los Angeles patients.

Implications:

Fetal hemoglobin (Hb F) may not offer a protective effect against severe disease in Hemoglobin (Hb) SD-Los Angeles.
Further research is needed to elucidate the mechanisms underlying the lack of Hb F amelioration in Hb SD-Los Angeles.
Understanding these mechanisms could inform future therapeutic strategies for sickle cell disease variants.