慢性移植物抗宿主病PPT课件
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Chronic GVHD: Pathophysiology and Novel Therapeutic Strategies
Ting Liu Department of Hematology
West China Hospital Sichuan University
2014. 4. Xiamen
内容
Lee et al., Biol Blood Marrow Transplant 2003; 9:215▪-.33.
▪7
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
GVHD
Changing risk factors
Older recipient age
Donors(unrelated, haploidentic)
Non-myeloablative conditioning
Peripheral blood stem cell source
Donor leukocyte infusions (DLI)
▪14
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
无特异性 闭塞性细支气管炎
血小板减少, 嗜酸性细胞增多，自 身免疫性血细胞减少
闭塞性细支气管炎伴机化性肺炎， 间质性肺炎
▪.
▪8
cGVHD: 多形性的皮肤病变
Epidermal cGVHD Lichen planus-like Papulosquamous Ichthyosiform Poikiloderma Keratosis pilaris-like Acral erythema
▪.
▪12
不同类型cGVHD的预后
▪. ▪ Pérez-Simón J A et al. Haematologica 2012;97:1187-1195
▪13
Multivariate risk factor profiles acute GVHD and chronic GVHD
Flowers M, et al. Blood.2011;117(11):3214-32▪1.9)
• 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
▪.
▪5
2012;2012:251-264
源自文库
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 ↓
Dermal cGVHD Lichen-sclerosus-like Dermal sclerosis
Subcutaneous cGVHD Subcutaneous sclerosis Fasciitis
▪.
▪9
cGVHD
▪.
▪10
▪.
▪11
cGVHD ：口腔黏膜溃疡
Treister N et al. Blood 2012;120:3407-3418
High CD34 dose and/or T-cell dose
Second transplants
DLIs
CMV?
▪.
▪6
影响cGVHD发病率的因素
Classification
Progressive poorest prognosis Quiescent de novo #1 risk factor: history of acute
Matched Sibling
N=3158
Matched Unrelated
N=941
18% 16%
31% 21%
Chronic GVHD
42%
49%
Ringdén O, et al. Blood. 2009;113:3110-3118.
▪.
▪3
NIH 新的GVHD分类标准(2005)
Acute GVHD
Filipovich AH, et al. Biol. Blood Marrow Transplant. 11(▪1.2), 945–956 (2005).
▪4
GVHD classification after the NIH consensus conference
Pavletic S Z , and Fowler D H Hematology
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
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
Update of knowledges in cGVHD Progress in pathophysiology of cGVHD Treatment for cGVHD Novel therapeutic strategies of cGVHD
▪.
▪2
CIBMTR: GVHD 发病率
Acute GVHD Grade: II III-IV
Ting Liu Department of Hematology
West China Hospital Sichuan University
2014. 4. Xiamen
内容
Lee et al., Biol Blood Marrow Transplant 2003; 9:215▪-.33.
▪7
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
GVHD
Changing risk factors
Older recipient age
Donors(unrelated, haploidentic)
Non-myeloablative conditioning
Peripheral blood stem cell source
Donor leukocyte infusions (DLI)
▪14
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
无特异性 闭塞性细支气管炎
血小板减少, 嗜酸性细胞增多，自 身免疫性血细胞减少
闭塞性细支气管炎伴机化性肺炎， 间质性肺炎
▪.
▪8
cGVHD: 多形性的皮肤病变
Epidermal cGVHD Lichen planus-like Papulosquamous Ichthyosiform Poikiloderma Keratosis pilaris-like Acral erythema
▪.
▪12
不同类型cGVHD的预后
▪. ▪ Pérez-Simón J A et al. Haematologica 2012;97:1187-1195
▪13
Multivariate risk factor profiles acute GVHD and chronic GVHD
Flowers M, et al. Blood.2011;117(11):3214-32▪1.9)
• 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
▪.
▪5
2012;2012:251-264
源自文库
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 ↓
Dermal cGVHD Lichen-sclerosus-like Dermal sclerosis
Subcutaneous cGVHD Subcutaneous sclerosis Fasciitis
▪.
▪9
cGVHD
▪.
▪10
▪.
▪11
cGVHD ：口腔黏膜溃疡
Treister N et al. Blood 2012;120:3407-3418
High CD34 dose and/or T-cell dose
Second transplants
DLIs
CMV?
▪.
▪6
影响cGVHD发病率的因素
Classification
Progressive poorest prognosis Quiescent de novo #1 risk factor: history of acute
Matched Sibling
N=3158
Matched Unrelated
N=941
18% 16%
31% 21%
Chronic GVHD
42%
49%
Ringdén O, et al. Blood. 2009;113:3110-3118.
▪.
▪3
NIH 新的GVHD分类标准(2005)
Acute GVHD
Filipovich AH, et al. Biol. Blood Marrow Transplant. 11(▪1.2), 945–956 (2005).
▪4
GVHD classification after the NIH consensus conference
Pavletic S Z , and Fowler D H Hematology
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
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
Update of knowledges in cGVHD Progress in pathophysiology of cGVHD Treatment for cGVHD Novel therapeutic strategies of cGVHD
▪.
▪2
CIBMTR: GVHD 发病率
Acute GVHD Grade: II III-IV