Pemphigus Vulgaris Associated with Autoimmune Hemolytic Anemia and Elevated TNFα

A 76-year-old female was admitted with many bullae and erythema on her trunk and extremities. A biopsy specimen showed significant intercellular edema in the lower epidermis and eosinophilic infiltration into the dermis and the epidermis. Immunofluorescent staining revealed the deposition of IgG in the intercellular area of her prickle cells. From these histologic findings and the typical clinical features, we diagnosed her as having pemphigus vulgaris. Examination of her blood revealed that she also suffered from autoimmune hemolytic anemia. Despite intensive treatment with prednisolone, she finally died. This case is of interest because of its rarity and the TNFα detected significantly in the blister fluid of this patient.     

单用达那唑治疗慢性再生障碍性贫血19例临床观察

单用达那唑（ＤＮＺ）治疗１９例慢性再生障碍性贫血（ＣＡＡ）病人。结果表明，基本治愈１例，缓解５例，明显进步７例，有效者共１３例，总有效率为６８．４％，初治者５／７例有效，有效率为７１．４％；复治者８／１２例有效，有效率为６６．７％。治疗后与治疗前比较，血红蛋白、白细胞、血小板和网织红细胞均明显升高（Ｐ＜０．００１）；骨髓红系、粒系及巨核细胞明显增高（Ｐ＜０．０５）；Ｔ辅助细胞（ＣＤ４）明显增加、Ｔ抑制细胞（ＣＤ８）明显降低，ＣＤ４／ＣＤ８比值明显升高（Ｐ＜０．０５）。并结合文献对ＤＮＺ的作用原理进行讨论。     

乙二胺四乙酸铁钠强化酱油改善贫血效果观察

本研究比较了NaFeEDTA强化酱油、硫酸亚铁 (FeSO4)强化酱油对IDA改善效果。将 30 0名IDA学生分为对照组、NaFeEDTA强化酱油组Fe 5mg (人·日 )、FeSO4强化酱油组Fe5mg (人·日 ) ,比较新型铁强化剂 (NaFeEDTA)与传统铁剂 (FeSO4)对缺铁性贫血的改善作用。结果表明 ,对照组各项检验指标干预前后没有显著差异。其它各试验组呈现出较为一致的变化 ,表现为血红蛋白、血清铁、血清铁蛋白含量的显著性增加和原卟啉、总铁结合力、转铁蛋白的显著性下降。试验结果提示 ,乙二胺四乙酸铁钠与传统铁剂 (硫酸盐铁 )强化酱油均有改善学生贫血的作用 ,并且NaFeEDTA组的贫血改善率和Hb恢复水平优于FeSO4组。     

慢性再生障碍性贫血患者外周血T淋巴细胞亚群改变与中医分型的关系

用McAb和ABC法测定了38例慢性再生障碍性贫血(CAA)患者外周血T淋巴细胞亚群(简称T亚群)。与健康人相比,CAA的Ts明显增高(P<0.001),Th/T5明显降低(P<0.001)。并发现外周血Hb水平、病程、疗效及中医证型与T亚群相关。按气血两虚肾阳虚、肾阴虚、肾阴阳两虚的顺序,患者Th逐渐降低,Ts逐渐升高,Th/Ts明显降低。后两组比前两组Th/Ts下降更显著(P<0.01),提示阳损及阴时机体免疫机能发生更大的改变,从T亚群水平揭示了中医证型特别是肾阳虚、肾阴虚的内涵。     

小儿营养性贫血原因调查

对326名2岁以下婴幼儿进行了调查分析,认为小儿营养性贫血以6～24个月为高发年龄,母乳喂养儿的患病率明显低于人工喂养儿,提示母乳喂养可降低贫血的患病率,有利于婴儿的生长发育,但12个月以后母乳喂养的贫血患病率有明显上升的趋势,因此认为要在大力提倡母乳喂养的同时应强调无论母乳喂养或人工喂养都应及早添加辅食,防止小儿营养性贫血的发生。     

肾组织移植对慢性肾功能衰竭大鼠促红细胞生成素的调节作用

目的研究鼠婴肾组织移植于肾包膜下对促红细胞生成素的调节作用,为临床应用治疗肾性贫血提供理论依据。方法以Wistar雄性大鼠建立慢性肾功能衰竭动物模型为受体,将鼠婴肾组织块多点植入受体肾包膜下。治疗期间用EPO酶联免疫(ELISA)试剂盒测定血清EPO水平;观察肾组织病理改变,用免疫组化方法检测EPO在移植物中的表达。结果①60d时D组血清促红细胞生成素(1.768±0.140)mu/mL高于B组(1.160±0.324)mu/mL(P<0.01),与C组(2.329±0.125)mu/mL也有差异(P<0.05)。②移植后60d,移植物的体积由1mm3增至3-4mm3大小,表面血管网丰富,光镜下见肾小球、肾小管结构正常;③移植物EPO免疫组化,发现移植物EPO着色颗粒主要分布于肾皮质的肾小管,明显多于病例对照组大鼠肾组织(P<0.05)。结论通过对移植物的形态观察和功能测定,证明肾组织移植是一种有效的治疗手段,有可能为慢性肾性贫血提供一种新的途径。     

Paroxysmal nocturnal haemoglobinuria: Nature's gene therapy?

The development of paroxysmal nocturnal haemoglobinuria (PNH) requires two coincident factors: somatic mutation of the PIG-A gene in one or more haemopoietic stem cells and an abnormal, hypoplastic bone marrow environment. When both of these conditions are met, the fledgling PNH clone may flourish. This review will discuss the pathophysiology of this disease, which has recently been elucidated in some detail.     

Chronic anemia as a complication of parvovirus B19 infection in a pediatric kidney transplant patient

. This is a report of unexplained anemia that persisted for 4 months in an adolescent renal transplant patient receiving immunosuppression that included prednisone, tacrolimus, and mycophenolate mofetil. This patient required monthly blood transfusions for fatigue, palpitations, and hematocrit levels between 15% and 17%. In addition, his posttransplant course was notable for the development of insulin-dependent diabetes mellitus. While receiving low-dose prednisone, he was switched from tacrolimus to cyclosporin and tapered off insulin injections over the next 2 months. At 4.5 months post-transplantation, further diagnostic evaluation was suggestive of parvovirus B19 infection as the cause for our patient’s chronic anemia. After testing negative for serum-specific parvovirus B19 IgM and IgG antibodies, parvovirus B19 infection was detected in blood by the polymerase chain reaction. Treatment with intravenous immunoglobulin (1 g/kg per day × 2 days) resulted in normalization of both his reticulocyte count and hematocrit within 6 weeks. At 4 months after receiving the immunoglobulin infusion, he has maintained a normal hematocrit level and stable renal function without requiring further blood transfusions. Received August 23, 1996; received in revised form and accepted November 20, 1996