Abstract：
BACKGROUND: Idiopathic multicentric Castleman disease (iMCD) is a rare polyclonal lymphoproliferative disease often associated with pulmonary involvement. Recently, transbronchial lung cryobiopsy (TBLC) has been reported to be useful for the diagnosis of diffuse interstitial lung disease. However, there have been no reports of pathological assessment of TBLC for iMCD.
METHODS: To clarify the efficacy of TBLC in the diagnosis of iMCD, we retrospectively reviewed four iMCD patients who had undergone both TBLC and surgical lung biopsy (SLB).
RESULTS: The median age was 44 years; 2 males and 2 females. Two or three TBLC specimens were taken from each patient. All patients had no complications other than minimal bleeding. The size of the TBLC specimens was approximately 5-6 × 3-4 mm, and the alveolar region, and centrilobular and perilobular areas were adequately sampled. As with SLB, the extent of lung lesions and inflammatory cell infiltration could be sufficiently evaluated by TBLC. The presence of lymphoid follicles could also be assessed by TBLC; however, the germinal centers with lymphoid follicles were difficult to evaluate. The TBLC specimens could also be evaluated for immunostaining, especially IgG4 immunostaining, to rule out IgG4-related lung disease. Pulmonary pathological grading showed a high concordance rate between major pathological findings of TBLC and SLB. The pathologist\'s confidence level of TBLC for the diagnosis of iMCD was high in all cases.
CONCLUSIONS: TBLC exhibits a high concordance rate with SLB in the pathological evaluation of iMCD, which may be useful for the diagnosis of iMCD.
METHODS: To clarify the efficacy of TBLC in the diagnosis of iMCD, we retrospectively reviewed four iMCD patients who had undergone both TBLC and surgical lung biopsy (SLB).
RESULTS: The median age was 44 years; 2 males and 2 females. Two or three TBLC specimens were taken from each patient. All patients had no complications other than minimal bleeding. The size of the TBLC specimens was approximately 5-6 × 3-4 mm, and the alveolar region, and centrilobular and perilobular areas were adequately sampled. As with SLB, the extent of lung lesions and inflammatory cell infiltration could be sufficiently evaluated by TBLC. The presence of lymphoid follicles could also be assessed by TBLC; however, the germinal centers with lymphoid follicles were difficult to evaluate. The TBLC specimens could also be evaluated for immunostaining, especially IgG4 immunostaining, to rule out IgG4-related lung disease. Pulmonary pathological grading showed a high concordance rate between major pathological findings of TBLC and SLB. The pathologist\'s confidence level of TBLC for the diagnosis of iMCD was high in all cases.
CONCLUSIONS: TBLC exhibits a high concordance rate with SLB in the pathological evaluation of iMCD, which may be useful for the diagnosis of iMCD.
摘要：
背景：特发性多中心Castleman病（iMCD）是一种罕见的多克隆淋巴增生性疾病，通常与肺部受累有关。最近,据报道，经支气管肺冷冻活检（TBLC）可用于诊断弥漫性间质性肺疾病。然而,没有关于TBLC对iMCD的病理学评估的报道.
方法：为了阐明TBLC在iMCD诊断中的功效，我们回顾性分析了4例同时接受TBLC和外科肺活检(SLB)的iMCD患者.
结果：中位年龄为44岁；2男2女。从每个患者采集两个或三个TBLC样本。除最小出血外，所有患者均无并发症。TBLC样品的尺寸约为5-6×3-4毫米，和肺泡区域，并对小叶正中和小叶周围区域进行了充分采样。与SLB一样，TBLC可以充分评估肺部病变和炎症细胞浸润的程度。淋巴滤泡的存在也可以通过TBLC来评估；然而，有淋巴滤泡的生发中心难以评估。还可以评估TBLC标本的免疫染色，尤其是IgG4免疫染色，排除IgG4相关的肺部疾病。肺病理分级显示TBLC的主要病理结果与SLB的一致性较高。在所有病例中，病理学家对TBLC诊断iMCD的信心水平都很高。
结论：在iMCD的病理评估中，TBLC与SLB的一致性较高，这可能有助于诊断iMCD。
方法：为了阐明TBLC在iMCD诊断中的功效，我们回顾性分析了4例同时接受TBLC和外科肺活检(SLB)的iMCD患者.
结果：中位年龄为44岁；2男2女。从每个患者采集两个或三个TBLC样本。除最小出血外，所有患者均无并发症。TBLC样品的尺寸约为5-6×3-4毫米，和肺泡区域，并对小叶正中和小叶周围区域进行了充分采样。与SLB一样，TBLC可以充分评估肺部病变和炎症细胞浸润的程度。淋巴滤泡的存在也可以通过TBLC来评估；然而，有淋巴滤泡的生发中心难以评估。还可以评估TBLC标本的免疫染色，尤其是IgG4免疫染色，排除IgG4相关的肺部疾病。肺病理分级显示TBLC的主要病理结果与SLB的一致性较高。在所有病例中，病理学家对TBLC诊断iMCD的信心水平都很高。
结论：在iMCD的病理评估中，TBLC与SLB的一致性较高，这可能有助于诊断iMCD。
