Oral and maxillofacial sequelae of sickle cell crises in a rare sickle cell disease genotype – HBS/β +-thalassemia

Introduction: Haemoglobin sickle-beta thalassemia (HbS/β-thalassemia) is a rare variant of sickle cell disease (SCD) occurring when both HbS and β-thalassemia genes are inherited. The severity of the condition depends on the amount of normal haemoglobin produced by the β-thalassemia gene. The oral and maxillofacial manifestations of sickle cell crises occurring in patients with this genotype have not been reported.

Methods: 208 SCD patients were surveyed via post, telephone or face-to-face interview on oral and maxillofacial sequelae of sickle-cell crises. Respondents identified as having HbS/β + -thalassemia were included in the study. HbS/β 0 -thalassemia patients, who produce no normal haemoglobin, were excluded.

Results: 12 patients met the inclusion criteria. 75% of patients reported sickle bone pain occurring at least once a month with the remainder experiencing pain less than once every 6 months. 50% described pain in the jaw and/or face during crises and 17% said that jaw pain had been confused with dental pain. One third of patients experienced sensory changes in the lip, chin or tongue. All patients who reported paraesthesia described it as temporary.

Conclusion: Clinicians should be aware of this rare group of patients as they may experience a high frequency of pain episodes. In order to avoid confusion with dental pain, it is suggested that patients who experience maxillofacial sequelae of sickle crises be referred to a Haematologist and Oral and Maxillofacial Surgeon for assessment and management. Future comparison with SCD genotypes will give better insight into the oral and maxillofacial manifestations of different haemoglobin variants of SCD.

Conflict of interest: None declared.

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Feb 5, 2018 | Posted by in Oral and Maxillofacial Surgery | Comments Off on Oral and maxillofacial sequelae of sickle cell crises in a rare sickle cell disease genotype – HBS/β +-thalassemia
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