Splenic sequestration syndrome at mountain altitudes in sickle/hemoglobin C disease

Sickle Cell Disease

“…Splenic infarction occurs in sickle cell trait, 80 and splenic sequestration in patients with sickle hemoglobin C disease. 81 Acute vaso-occlusive pain also seems to be more common in patients living at high altitude.…”

Environmental determinants of severity in sickle cell disease

“…The most frequently reported complication is acute splenic infarction in people with sickle cell trait, occurring both in aircraft and mountainous areas, particularly Denver, Colorado (altitude 1600 m). 68 Splenic sequestration has been reported in adults and children with HbSC disease on ascent to high altitude, including a paper describing this complication in four children with HbSC disease who visited mountains at altitudes greater than 2700 m. 69 The third complication associated with altitude is the development of acute vaso-occlusive pain in patients with sickle cell anemia. This has been described both in Colorado 70 and Saudi Arabia; 71 in the latter country the authors found that the rate of acute vaso-occlusive complications in patients with SCD living in the highlands was about double that of lowlanders, associated with a 5% increase in hemoglobin levels.…”

Role of phlebotomy in the management of hemoglobin SC disease: Case report and review of the literature

“…Our patient experienced splenic infarction while driving at a high altitude. Four cases of splenic infarction in Hb SC patients following travel to altitudes of 9,000-12,000 feet in the Rocky Mountains have been documented; a fifth patient with Hb SC disease experienced splenic infarction 10 days after arriving at an altitude 5,280 feet above sea level [10]. Following splenectomy, our patient began to experience an increased frequency of painful events as well as new-onset dizziness and generalized malaise.…”