Raghunathan Vikram M, Butera James N, Treaba Diana O
Department of Medicine, Brown University Alpert Medical School, Providence, RI, USA.
Division of Hematology and Oncology, Rhode Island Hospital, Providence, RI, USA.
Case Rep Hematol. 2017;2017:8174207. doi: 10.1155/2017/8174207. Epub 2017 Sep 28.
Hemoglobin (Hb) Sherwood Forest is a rare high-affinity hemoglobin first described in 1977, arising from an Arg to Thr substitution at codon 104 of the beta chain. This hemoglobin variant has been identified in few individuals and has been associated with a compensatory erythrocytosis in the homozygous state. Prior scarce case reports have noted that heterozygotes for this variant are phenotypically normal. Here we present a patient who was evaluated in our hematology clinic for chronic erythrocytosis and was found to be heterozygous for Hb Sherwood Forest. No other primary or secondary cause of his polycythemia was identified. This is the first described case of heterozygous Hemoglobin Sherwood Forest causing erythrocytosis.
血红蛋白（Hb）舍伍德森林是一种罕见的高亲和力血红蛋白，于1977年首次被描述，由β链第104密码子处的精氨酸被苏氨酸取代引起。这种血红蛋白变体仅在少数个体中被鉴定出来，并且与纯合状态下的代偿性红细胞增多症有关。先前稀少的病例报告指出，这种变体的杂合子在表型上是正常的。在此，我们报告一名在我们血液学诊所接受慢性红细胞增多症评估的患者，发现其为血红蛋白舍伍德森林的杂合子。未发现其红细胞增多症的其他原发性或继发性病因。这是首例关于杂合性血红蛋白舍伍德森林导致红细胞增多症的病例报道。
