慢性移植物抗宿主病1例

报告一例慢性移植物抗宿主病。患者,男,26岁,骨髓移植后全身皮肤硬化、瘙痒伴色素沉着1年余。皮肤科检查：躯干、四肢皮肤弥漫性硬化、色素沉着,其上覆叶片状鳞屑,肢端部分皮疹破溃,少许渗液结痂。皮损组织病理检查：表皮萎缩,基底细胞液化变性及明显色素失禁,真皮全层胶原增生、增厚、均质化,血管周围稀疏淋巴细胞浸润,病理符合硬皮病样皮肤改变。     

慢性移植物抗宿主病1例

慢性移植物抗宿主病是同种异体器官移植后发生的一种严重的慢性并发症。现将笔者诊治的1例报告如下。     

慢性移植物抗宿主病1例

1临床资料 患者,男,50岁,因"确诊慢粒16月,异基因外周造血干细胞移植术后1年,全身红色皮疹伴口唇糜烂1月",于2003年2月19日入院.患者1年前体检时发现外周血白细胞156×109/L,脾大,查骨髓像:慢性粒细胞白血病,经羟基脲2g/天治疗,血象控制正常.     

慢性移植物抗宿主病1例

1临床资料患者,女,26岁。1年半前因患"急性粒细胞性白血病"而接受其姐为供体的"同种异体骨髓造血干细胞移植术"。术后一直连续口服"环孢素A"和"泼尼松"抗排异治疗,未发生急性排斥反应。半年前于腰、腹、四肢、腋下及双腘窝出现红褐色丘疹,近2     

慢性移植物抗宿主病一例

患者男,48岁。因乏力6年,喘憋4 年,四肢皮肤变硬、肌肉萎缩3年于2004 年4月来我院就诊。6年前患者在当地医院诊断为急性粒细胞白血病,部分分化型(AML-M2),并行同种异体基因骨髓移植(供者为其胞弟)。移植后1个月及3 个月分别行两次化疗。移植后4个月,出     

硬皮病样表现伴关节挛缩的慢性移植物抗宿主病一例

患儿,男,5岁。双手足质硬、红斑、关节挛缩2年。患儿自幼反复罹患肺脓肿并伴有发热,4年前行基因检测,诊断为JAK3基因相关重型联合免疫缺陷症,遂行异基因造血干细胞移植。2年前先于足部出现脱皮,后双手足背、腕、肘、膝及髋关节处出现红斑、脱皮、硬化,伴手足关节挛缩。皮肤组织病理示移植物抗宿主反应并病毒感染。     

肝移植术后急性移植物抗宿主病1例

急性移植物抗宿主病(acute graft-versus-host disease,aGVHD)是供体的同种反应性T细胞针对受体组织的破坏性免疫反应,常见于骨髓移植术后,发生于肝移植术后者少见,发生率为1%～2%,但死亡率高于85%[1].     

慢性移植物抗宿主病一例

患者女,３８岁。因双上肢硬化、萎缩,伴乏力、肌肉胀痛５个月而来诊,我科门诊以“慢性移植物抗宿主病？硬皮病？皮肌炎？”诊断收入院。２年前患者因乏力、消瘦伴发热住我院血液科,行骨穿诊断为慢性粒细胞白血病,尔后在北京行骨髓移植术,口服环孢素A２００mg／d,１年后逐渐减量至２５mg／d。半年前出现感冒伴高热,经抗炎治疗后好转。半个月后发现右上臂内侧出现数个豆粒大红斑、斑丘疹,腹部出现甲大水肿性红斑,曾服用抗组胺药无效。皮疹逐渐增多、融合,扩大为硬肿性红斑,３个月前皮疹逐渐蔓延至双上肢,出现挛缩、胀痛,腹部红…     

硬皮病样慢性移植物抗宿主病一例

患者,男,42岁。全身红斑、硬化、脱发9年。皮肤科查体：胸部、四肢、鼻周、口周等部位地图样斑块、色素脱失斑、萎缩、瘢痕,头顶部见脱发区,表面见色素脱失。腹部皮损组织病理示：表皮角化过度,基底细胞空泡变性伴色素失禁;真皮附属器减少,真皮浅层胶原纤维粗大、致密及玻璃样变。诊断：硬皮病样慢性移植物抗宿主病。     

硬皮病样慢性移植物抗宿主病3例

报告3例硬皮病样慢性移植物抗宿主病。患者均为男性，平均年龄48岁。因原发性血液系统疾病在化疗缓解后接受异体骨髓移植，移植后平均12．7个月出现仝身弥漫性硬化或泛发性硬斑，硬化严格以手腕、踝部和颈部为界，未累及肢体远端和面部，无雷诺现象和毛细血管扩张。其中2例伴有不同程度的肺间质病变。3例患者抗核抗体（ANA）、类风湿因子(RF）、抗Scl-70抗体和抗着丝点抗体均为阴性，组织病理检查显示真皮网状层胶原纤维束增粗、密集和肿胀。3例患者均接受中等剂量的糖皮质激素治疗，其中2例治疗1个月后皮肤明显软化，另1例软化不明显。     

Cutaneous graft-versus-host disease: rationales and treatment options

The treatment of cutaneous graft-versus-host disease (GVHD) is one of the most challenging clinical scenarios in a dermatological practice. Given the significant risk of morbidity and mortality in this patient group, it is important for a dermatologist to understand the pathophysiology of GVHD, as well as their role within a multidisciplinary practice where many immunosuppressants are prescribed. A significant proportion of the patients with GVHD will require a combination treatment regimen in order to stem the progression of their disease. In this review, the stages, type of GVHD and treatment options are reviewed for the dermatologist.     

Immunohistochemistry of cutaneous graft-versus-host disease after allogeneic bone marrow transplantation

Graft-versus-host disease (GVHD) is an immunologically mediated disease occurring most frequently after allogeneic bone marrow transplantation. The aim of this study was to evaluate the contribution of immunohistochemistry in the diagnosis of cutaneous GVHD. Patients transplanted for either leukemia or beta-thalassemia were included in the study. Skin lesions of acute and chronic GVHD were examined both by direct immunofluorescence to detect immunoglobulin deposits and by an avidin-biotin-peroxidase complex technique to evaluate the inflammatory cell infiltrate. Epidermal and dermal fluorescent bodies (IgG and IgM) were frequently found in both acute and chronic GVHD. Most of the infiltrating cells were CD3+ T lymphocytes, with CD8+ cells representing the major cell population invading the epidermis both in acute GVHD and in chronic lichenoid GVHD. A small proportion of the dermal cells were CD14+ macrophages; no B cells were detected. HLA-DR, but not HLA-DQ antigens, were variably expressed by keratinocytes in all cases of acute GVHD and in chronic lichenoid GVHD. KL-1, a monoclonal antikeratin antibody specific for the 56.5 KD acidic polypeptide usually present in suprabasal keratinocytes, stained all epidermal layers, including the basal layer. Langerhans cells were dramatically reduced in number in the epidermis of both acute and chronic lichenoid GVHD. It is concluded that immunohistologic analysis may be supportive in the diagnosis of acute and early chronic lichenoid cutaneous GVHD.     

Very late appearance of acute graft-versus-host disease after tapering immunosuppression.

Graft-versus-host disease (GVHD) is a major complication of stem cell transplantation. Here we report a 40-year-old woman who developed an acute GVHD 30 months after transplantation. Late and very late appearance of acute GVHD has only been described in rare cases.     

Phototherapy for the treatment of cutaneous graft versus host disease

Graft versus host disease (GvHD) occurs in half the patients who receive allogenic haematopoietic stem cell transplantation and is a major contributor for the morbidity and mortality in these patients. Immunosuppressant therapy cannot suppress all the manifestations of GvHD and new ways of treating the condition are needed. The focus of this review is the treatment of cutaneous GvHD through phototherapy. Of the six acute and ten chronic cutaneous GvHD case series examined the overall trend was that phototherapy was able to reduce the presence of cutaneous lesions of GvHD and, as a consequence, steroid use could be reduced. This provides a positive outlook for phototherapy as a treatment for cutaneous GvHD but there is a need for future studies to include larger numbers of patients in order to obtain more data.     

移植物抗宿主病皮肤表现 (附 3例报告 )

报道3例外周血干细胞移植后致移植物抗宿主病（GVHD），皮疹具本病临床和组织学特点，皮损的严重性与供体有相关性，典型的皮疹及组织浓助于GVHD诊断。     

Acute graft-versus-host disease following hematopoietic stem-cell transplantation

Hematopoietic stem-cell transplantation has become the standard of care for numerous malignant and nonmalignant conditions. As the number of stem-cell transplants performed worldwide rises, it is imperative that dermatologists taking care of these patients are able to understand the methods of transplantation, as well as to recognize and treat the cutaneous complications that commonly follow transplant, particularly acute graft-versus-host disease.     

Many faces of graft-versus-host disease

Allogeneic haematopoietic stem cell transplantation is increasingly used in the treatment of malignant and non-malignant disorders. Despite ongoing advances in the field, morbidity and mortality related to graft- versus -host disease remains a major barrier to its application. Graft- versus -host disease is a difficult-to-diagnose disease. Dermatologists are involved due to its diverse cutaneous expression. In order to appropriately diagnose, classify and treat this complex disease, knowledge of its expanding cutaneous expression is required. This review provides a synopsis of the clinical manifestations of acute, lichenoid and sclerodermatous phases of graft- versus -host disease with a look at the current evidence surrounding its differential diagnosis.     

Chronic cutaneous graft-versus-host disease simulating hypertrophic lupus erythematosus--a case report of a new morphologic variant of graft-versus-host disease

The spectrum of clinical and histopathologic features associated with chronic graft-versus-host disease (GVHD) is broad, with recognized variants simulating scleroderma, lichen sclerosus, eosinophilic fasciitis, and de novo diffuse melanoderma. We report a case of a patient with multiple myeloma who presented approximately 1 year after his allogeneic hematopoietic stem cell transplantation with lesions of chronic lichenoid GVHD that harbored features of hypertrophic lupus erythematosus (LE) and that was initially mistaken for a superficial well-differentiated squamous cell carcinoma (SCC). However, in 4 years of follow-up, the patient failed to develop any evidence of cutaneous or systemic LE, actinic damage, or SCC, and the lesions cleared with topical and systemic treatments appropriate for chronic GVHD. For proper interpretation of the histologic findings of GVHD, it is important for the dermatopathologist to be aware of unusual manifestations. Knowledge of the occurrence of hypertrophic LE and familiarity with its histologic features is also important to avoid an erroneous diagnosis of SCC in immunosuppressed patients.