Biochemistry and Molecular Genetics Department, Core Laboratory, Biomedical Diagnostic Centre, Hospital Clínic, Barcelona, Spain.
Biochem Med (Zagreb). 2023 Jun 15;33(2):020801. doi: 10.11613/BM.2023.020801. Epub 2023 Apr 15.
Kimura disease (KD) is an unusual inflammatory disease of unknown etiology. Despite being described many years ago, KD might cause diagnostic difficulty or be confused with other conditions. Here, we present a 33-year-old Filipino woman who was referred to our hospital for evaluation of persistent eosinophilia and intense pruritus. Blood analysis and peripheral blood smear review showed high eosinophil counts (3.8 x10/L, 40%) that did not show morphological abnormalities. Besides, high serum IgE concentration was detected (33,528 kU/L). Serological tests were positive for and treatment with albendazol was initiated. Nevertheless, increased eosinophil counts were still present after several months, alongside with high serum IgE concentrations and intense pruritus. During her follow-up, an inguinal adenopathy was detected. The biopsy revealed lymphoid hyperplasia with reactive germinal centers and massive eosinophil infiltration. Proteinaceous deposits of eosinophilic material were also observed. All these findings, together with peripheral blood eosinophilia and high IgE concentrations, confirmed the diagnosis of KD. The diagnosis of KD should be considered in the differential diagnosis of long-standing unexplained eosinophilia in association with high IgE concentrations, pruritus and lymphadenopathies.
木村病(KD)是一种病因不明的罕见炎性疾病。尽管多年前就有描述,但 KD 仍可能导致诊断困难或与其他疾病混淆。在这里,我们介绍一位 33 岁的菲律宾女性,她因持续性嗜酸性粒细胞增多和剧烈瘙痒而被转至我院进行评估。血液分析和外周血涂片检查显示嗜酸性粒细胞计数高(3.8x10/L,40%),但无形态异常。此外,还检测到高血清 IgE 浓度(33,528 kU/L)。血清学检查 阳性,开始用阿苯达唑治疗。然而,几个月后,嗜酸性粒细胞计数仍持续升高,同时伴有高血清 IgE 浓度和剧烈瘙痒。在随访过程中,发现腹股沟淋巴结肿大。活检显示淋巴组织增生,伴有反应性生发中心和大量嗜酸性粒细胞浸润。还观察到嗜酸性粒细胞物质的蛋白样沉积物。所有这些发现,加上外周血嗜酸性粒细胞增多和高 IgE 浓度,均证实了 KD 的诊断。对于长期原因不明的嗜酸性粒细胞增多症伴有高 IgE 浓度、瘙痒和淋巴结病,应考虑 KD 的鉴别诊断。