Harrison Jonathan S, Parmar Harsh, Wang Xiangbing D
Department of Medicine and the Carole and Ray Neag Cancer Center University of Connecticut School of Medicine Farmington Connecticut.
Department of Medicine Rutgers - Robert Wood Johnson Medical School New Brunswick New Jersey.
Clin Case Rep. 2018 Mar 11;6(5):847-850. doi: 10.1002/ccr3.1454. eCollection 2018 May.
Autoimmune polyglandular syndrome type 1 (APS1) is a rare autosomal recessive disorder, and large granular lymphocytic leukemia (LGLL) may, even more rarely, complicate APS1. LGLL may be subtle in presentation, but it is imperative to recognize LGLL in APS1 promptly, as outcome may otherwise be fatal, as described herein.
1型自身免疫性多腺体综合征(APS1)是一种罕见的常染色体隐性疾病,而大颗粒淋巴细胞白血病(LGLL)合并APS1的情况甚至更为罕见。LGLL的临床表现可能不明显,但必须及时识别APS1患者中的LGLL,因为否则后果可能是致命的,如下文所述。