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Venesection in haemoglobin Yakima, a high oxygen affinity haemoglobin
R J Grace1, P A Gover, D F Treacher
1Department of Haematology, St. Thomas' Hospital, London, UK.
Clinical and Laboratory Haematology
|January 1, 1992
Summary
High oxygen affinity hemoglobin causes polycythemia, leading to hyperviscosity symptoms. Reducing packed red cell volume (PCV) through venesection relieved these symptoms in infants with Hemoglobin Yakima.
Area of Science:
- Hematology
- Genetics
- Cardiovascular Physiology
Background:
- High oxygen affinity hemoglobins can lead to polycythemia, a condition of elevated red blood cells.
- Polycythemia necessitates cardiovascular adaptation to ensure adequate tissue oxygenation.
- This adaptation, however, can result in hyperviscosity and vaso-occlusive complications.
Observation:
- A family (kindred) presented with a high oxygen affinity hemoglobin variant, Hemoglobin Yakima.
- Two infants within this family exhibited intra-uterine growth retardation.
- These infants also suffered from symptoms indicative of hyperviscosity.
Findings:
- Hemoglobin Yakima was identified as the causative factor for high oxygen affinity hemoglobin in this kindred.
- Infants with Hemoglobin Yakima experienced hyperviscosity symptoms, including intra-uterine growth retardation.
- Reduction of packed red cell volume (PCV) via venesection led to the resolution of hyperviscosity symptoms.
Implications:
- High oxygen affinity hemoglobin disorders like Hemoglobin Yakima can have significant clinical manifestations, particularly in neonates.
- Management strategies for symptomatic polycythemia secondary to high affinity hemoglobin involve PCV reduction.
- Understanding these genetic and physiological links is crucial for diagnosing and managing related cardiovascular and hematological conditions.