Busch F H
Give Sygehus, medicinsk afdeling.
Ugeskr Laeger. 1990 Sep 3;152(36):2565-6.
Familial benign chronic neutropenia is a rare anomaly which is transmitted as an autosomally dominant trait and is characterized by normal or somewhat low total leukocyte counts, consistent neutropenia, and, usually, relative monocytosis and lymphocytosis, sometimes with eosinophilia. Affected individuals have a normal life expectancy. Many are asymptomatic, but some have histories of tendency to develop furuncles and/or periodontal disease. A Danish family with familial benign chronic neutropenia is reported. Four family members were affected, of these one had repeated attacks of severe stomatitis, two had histories of tendencies to develop furuncles, and one was asymptomatic.
家族性良性慢性中性粒细胞减少症是一种罕见的异常情况,以常染色体显性性状遗传,其特征为白细胞总数正常或略低、持续性中性粒细胞减少,通常伴有相对单核细胞增多和淋巴细胞增多,有时伴有嗜酸性粒细胞增多。患病个体的预期寿命正常。许多人没有症状,但有些人有发生疖肿和/或牙周病的倾向病史。本文报道了一个患有家族性良性慢性中性粒细胞减少症的丹麦家族。四名家族成员患病,其中一人反复发生严重口腔炎,两人有发生疖肿的倾向病史,一人无症状。