【精品推荐】慢性移植物抗宿主病

扁平苔藓，口干症，非感染性溃疡， 口腔干燥, 干燥性角结膜炎 角膜糜烂/非感染性结膜炎
食管狭窄，脂肪泻
Hale Waihona Puke Baidu
食欲减退，吸收不良，体重减轻， 腹泻，腹痛
无特异性
碱性磷酸酶升高, 转氨酶增高，胆 管炎，高胆红素血症
阴道狭窄，扁平苔藓
非感染性阴道炎，阴道萎缩
非特异性关节炎，肌炎，肌无力，浆 关节痛 膜炎，挛缩性关节固定
不同类型cGVHD的预后
Pérez-Simón J A et al. Haematologica 2012;97:1187-1195
Multivariate risk factor profiles acute GVHD and chronic GVHD
Flowers M, et al. Blood.2011;117(11):3214-3219)
Chronic GVHD: Pathophysiology and Novel Therapeutic Strategies
Ting Liu Department of Hematology
West China Hospital Sichuan University
2014. 4. Xiamen
内容
无特异性
血小板减少, 嗜酸性细胞增多，自 身免疫性血细胞减少
闭塞性细支气管炎
闭塞性细支气管炎伴机化性肺炎， 间质性肺炎
cGVHD: 多形性的皮肤病变
Epidermal cGVHD Lichen planus-like Papulosquamous Ichthyosiform Poikiloderma Keratosis pilaris-like Acral erythema
Chronic GVHD
Matched Sibling
N=3158
Matched Unrelated
N=941
18% 16%
31% 21%
42%
49%
Ringdén O, et al. Blood. 2009;113:3110-3118.
NIH 新的GVHD分类标准(2005)
Acute GVHD
• classic acute GVHD • late-onset acute GVHD
Chronic GHVD
• Classic chronic GVHD • Overlap syndrome
NIH分类标准最重要的变化是以临床表现和器官受累的程 度，而不是移植后时间来进行分类，这有利于临床医生作 出更符合病理生理学改变的诊断和治疗策略
Update of knowledges in cGVHD Progress in pathophysiology of cGVHD Treatment for cGVHD Novel therapeutic strategies of cGVHD
CIBMTR: GVHD 发病率
Acute GVHD Grade: II III-IV
Dermal cGVHD Lichen-sclerosus-like Dermal sclerosis
Subcutaneous cGVHD Subcutaneous sclerosis Fasciitis
cGVHD

cGVHD ：口腔黏膜溃疡
Treister N et al. Blood 2012;120:3407-3418
Filipovich AH, et al. Biol. Blood Marrow Transplant. 11(12), 945–956 (2005).
GVHD classification after the NIH consensus conference
Pavletic S Z , and Fowler D H Hematology 2012;2012:251-264
cGVHD发病的危险因素
Acute GVHD Older age of recipient and donor Female multiparous donor Mismatched and unrelated donors PBSC product Disease type: CML, Aplastic anemia ↓ High CD34 dose and/or T-cell dose Second transplants DLIs CMV?
Lee et al., Biol Blood Marrow Transplant 2003; 9:215-33.
受累器官系统 皮肤
黏膜 消化道 肝脏 泌尿生殖道 肌肉骨骼/浆膜 血液系统 肺
慢性GVHD的临床表现
肯定为慢性GVHD的临床表现
可能为慢性GVHD的临床表现
硬皮病，扁平苔藓，白癜风，疤痕性 湿疹样皮疹, 皮肤干燥, 斑丘疹, 脱 脱发，毛发角化过度，皮肤挛缩，甲 发, 色素沉着 床发育异常
影响cGVHD发病率的因素
Classification
Progressive poorest prognosis Quiescent de novo #1 risk factor: history of acute
GVHD
Changing risk factors
Older recipient age Donors(unrelated, haploidentic) Non-myeloablative conditioning Peripheral blood stem cell source Donor leukocyte infusions (DLI)
cGVHD危险度积分*
Mild – no significant impairment of function • Only 1-2 organs (except lungs) • Maximum organ score 1
Moderate – significant impairment but no major disability • Three or more organs with max score 1 • One organ with max score 2 • Lung score of 1