新生儿Rh溶血病不同血源换血疗法的疗效观察

目的:探讨新生儿 Rh溶血病实行换血疗法时的血源选择和换血量的确定。方法 :选取在我院接受换血治疗的 6 3例 Rh溶血病患儿 ,其中用 Rh阳性血换血 32例 ,Rh阴性血换血 31例 ,分析其换血前后血清总胆红素(TB)和血红蛋白 (Hb)变化以判断疗效。 结果 :两种换血术均获得满意疗效 ,换血后胆红素值明显下降 ,所有患儿均痊愈出院。 结论 :在紧急情况下 ,若无法及时得到 Rh阴性血 ,可用 Rh阳性血换血 ,换血量至少应 >2 0 0 m l/ kg(为 Rh阴性血的 2倍 ) ,若应用得当 ,是一项安全有效的替代疗法     

Rh血型不合新生儿溶血病检测方法及应用

产前检测Ｒｈ，Ｄ因子及抗人球蛋白（ｃｏｏｍｂｓ）试验是必要的。测定Ｒｈ，Ｄ因子及抗Ｄ滴度使用木瓜酶方法。通过对１１２６１例孕妇常规检查Ｒｈ，Ｄ因子，发现Ｄ阴性７４例。Ｒｈ，Ｄ阴性妇女占６．５‰。２２例Ｒｈ，Ｄ阴性的孕妇所分娩的新生儿均为Ｒｈ，Ｄ阳性。其中２例孕妇血清抗Ｄ滴度为１∶３２，病情严重，宫内输血无效，胎死宫内。初产妇１３例，占５９％。活产２０例，存活率９０％。Ｒｈ因子及抗人球蛋白试验方法简便、易行，一般医院均可进行。对有流产史、输血史的孕妇检查Ｒｈ因子是十分必要的。在有条件的医院，对Ｒｈ，Ｄ阴性的产妇分娩Ｒｈ，Ｄ阳性的新生儿之后，产妇应预防性注射抗Ｄ免疫球蛋白     

Refractory hemolytic uremic syndrome improved by plasmapheresis using cryosupernatant fraction as a replacement fluid: A case report

A 52-year-old woman who had had 6 months of chemotherapy using mitomycin C and cisplatin for cervical cancer presented with hemolytic uremic syndrome. Conventional plasmapheresis using whole-plasma fraction was ineffective. However, plasmapheresis using the cryosupernatant fraction dramatically improved symptoms of hemolytic anemia and thrombocytopenia in this case. The activity of factor VIII in the cryosupernatant fraction of plasma as a replacement fluid decreased after removal of cryoprecipitate, indicating effective removal of von Willebrand factor. The pathogenesis of her hemolytic uremic syndrome may have been associated with von Willebrand factor multimers contained in the cryoprecipitate of plasma. Similar use of the cryosupernatant fraction of plasma could not be found in other reports of cases of hemolytic uremic syndrome. Plasmapheresis using the cryosupernatant fraction of plasma may improve refractory hemolytic uremic syndrome.     

The popularization and application of cold storage red blood cells or whole blood at −80 °C of the Rh(D) negative patients in surgical operation

Summary The efficiency of cold storage red blood cells (CSRBC) or whole blood at −80 °C used in 27 Rh(D) negative patients during surgical operation was reported. The Rh(D) negative patients received the transfusion of CSRBC or whole blood stored at −80 °C for 180 to 360 days. The changes in the indexes, such as blood TB, DB, K⁺, Na⁺, BUN, Cr, urine protein (URPO), UOB, Hb, HCT, serum total protein, relative to hemolytic reaction and blood volume before and after transfusion were observed. The results showed that after transfusion of CSRBC or whole blood 27 cases were negative for urine protein and UOB, and the levels of BUN and Cr were normal (P>0.05). Blood TB, DB, Hb, and HCT were increased, while pH, blood K⁺ and blood Na⁺ was normal with the difference being not significant before and after operation (P>0.05). Plasma protein was decreased, but there was no significant difference before and after operation (P>0.05). It was suggested that CSRBC or whole blood at −80 °C could be safely infused to the Rh(D) negative patients without side effects during the surgical operation. YU Zhongqing, male, born in 1957, Technician in Charge     

Percutaneous transluminal coronary angioplasty in a patient with paroxysmal nocturnal hemoglobinuria

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired chronic hemolytic anemia associated with an unusual susceptibility to hemolytic crisis, infection, and venous thrombosis which would be aggravated by a number of factors including surgery. We report a case of PNH undergoing percutaneous transluminal coronary angioplasty and discuss the corresponding perioperative management.     

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.     

Clonality of cold agglutinins in patients with hemolytic anemia: an analysis by high-resolution two-dimensional gel electrophoresis.

High-resolution two-dimensional gel electrophoresis (2-DGE) was used to analyse plasma samples and partially purified cold agglutinins (CA) obtained from two selected patients. Both presented an acute hemolytic anemia with CA of high thermal amplitude, normal immunoglobulin levels, no detectable paraproteinemia, and no clinical evidence of a malignant B-cell disorder. The electrophoretograms of their plasma showed evident alternations of the "normal" protein profile, which were directly related to hemolysis (absence of the spots of haptoglobin and in one case of those of hemopexin), but no monoclonal gammopathy. The electrophoretograms of their purified CA revealed two clearly different spot patterns respectively corresponding to a monoclonal IgM and to polyclonal IgM. These results show that the clonality of CA associated with hemolytic anemia can be easily determined by 2-DGE. This technique may be very useful to discriminate chronic cold agglutinin disease in the early phase from "parainfectious" CA.     

Purine nucleoside phosphorylase deficiency: a new case report and identification of two novel mutations (Gly156A1a and Val217Ile), only one of which (Gly156A1a) is deleterious

Purine nucleoside phosphorylase (PNP) deficiency results in an autosomal recessive immunodeficiency disease characterized by initial involvement of cellular immunity and neurological manifestations with subsequent abnormalities of humoral immunity. The initial presentation and clinical course has varied widely in the relatively few published cases. The molecular basis has been reported in only 10 patients, precluding evaluation of phenotype-genotype relationships. We now report clinical, immunologic, and molecular findings in a new case of relatively early onset that emphasizes hypotonia and developmental delay as early manifestations. The patient carried two novel missense mutations (Gly56A1a and Val217Ile) on the same allele in apparent homozygosity. Expression of each of the mutant enzymes in vitro demonstrated that the Gly156A1a mutation abolished enzyme activity while the Val217Ile mutation was without obvious effect and is therefore a normal variant. Such "normal" polymorphisms might be associated with a variable response to the immunosuppressive PNP inhibitors currently in clinical trials.