Zaidi Y, Sivakumaran M, Graham C, Hutchinson R M
Department of Haematology, Leicester Royal Infirmary.
J Clin Pathol. 1996 Sep;49(9):774-5. doi: 10.1136/jcp.49.9.774.
Sickle cell beta + thalassaemia is regarded as the mildest of the sickle cell haemoglobinopathy syndromes with a benign natural course. In contrast to sickle cell disease, severe life threatening complications are not usually associated with this genotype. A case of a 30 year old West Indian man who, previously asymptomatic for 10 years, sustained a fatal pulmonary bone marrow embolism, is reported. This case report illustrates that serious, even fatal, complications may occur in patients with this 'benign' condition and bone marrow embolism should be included in the differential diagnosis of acute crisis in these patients.
镰状细胞β+地中海贫血被认为是镰状细胞血红蛋白病综合征中最轻微的一种,其自然病程较为良性。与镰状细胞病不同,这种基因型通常不会引发严重的、危及生命的并发症。本文报告了一例30岁的西印度男子,他此前10年一直无症状,但却遭遇了致命的肺骨髓栓塞。该病例报告表明,患有这种“良性”疾病的患者可能会出现严重甚至致命的并发症,骨髓栓塞应纳入这些患者急性危象的鉴别诊断中。