Diagnostic criteria to distinguish hypocellular acute myeloid leukemia from hypocellular myelodysplastic syndromes and aplastic anemia: recommendations for a standardized approach

Members of the French-American-British Cooperative Leukemia Working Group met to review cases of aplastic anemia, hypocellular myelodysplastic syndrome and hypocellular acute myeloid leukemia. Criteria were proposed and modified following three workshops. Additional input was obtained from another hematopathologist with a special interest in bone marrow histology and immunohistochemistry. Guidelines were recommended based on the workshop results as well as additional studies including selective immunohistochemistry, flow cytometry and cytogenetics.     

康力龙联合复方皂矾丸治疗慢性再生障碍性贫血的疗效观察

目的:探讨慢性再生障碍性贫血(CAA)治疗的方法及疗效。方法:采用随机对照研究办法比较复方皂矾丸及环孢素A(CsA)联合康力龙治疗CAA的疗效。结果:复方皂矾丸或CsA联合康力龙治疗CAA的总有效率分别为80.0%和87.5%,两组比较,差异无显著性(P>0.05)。结论:复方皂矾丸联合康力龙治疗CAA以其疗效好、不良反应少、治疗费用低等优点,易于基层医院推广使用。     

神经酰胺对再生障碍性贫血患者T淋巴细胞增殖与凋亡作用的影响

目的：研究神经酰胺对再生障碍性贫血（AA）外周血T淋巴细胞增殖与凋亡的影响。方法：分离纯化30例AA患者、6例正常人外周血T淋巴细胞，MTT法检测不同质量浓度神经酰胺（C2—ceramide）对T淋巴细胞增殖活性的影响；流式细胞仪检测C2—ceramide对AA外周血T淋巴细胞凋亡产生的影响。结果：C2-ceramide对AA外周血T淋巴细胞的生长具有质量浓度依赖性抑制作用，而对正常淋巴细胞的抑制作用不明显；流式细胞仪检测C2-ceramide处理后的AA外周血T淋巴细胞凋亡比例明显高于AAT淋巴细胞的自然凋亡率，DNA电泳可见凋亡特有的DNA片段。结论：神经酰胺可通过抑制AA外周血T淋巴细胞增殖及诱导凋亡的作用，对AA免疫紊乱进行调节。     

健脾补肾活血方及其拆方对免疫介导再生障碍性贫血小鼠骨髓造血细胞凋亡的影响

目的:采用细胞培养方法,从细胞凋亡角度探讨健脾补肾活血方对再生障碍性贫血(aplastic anemia,AA)模型小鼠的疗效及其促进骨髓造血的作用机制。方法:复制AA小鼠模型并制备AA小鼠血清,以健脾补肾活血方及其拆方灌胃大鼠制备含中药血清,共同培养正常小鼠骨髓造血细胞,24 h后采用流式细胞仪和透射电镜观察细胞的凋亡情况。结果:AA小鼠血清能诱导正常小鼠骨髓造血细胞发生凋亡,经健脾补肾活血方及其拆方的药物血清作用后,各组小鼠骨髓造血细胞的凋亡率均下降,其中,补肾方的疗效优于健脾方和活血方,健脾补肾活血方(全方)的效果最好。电镜观察造血细胞超微结构的结果亦表明补肾方的作用较好,但以全方作用最佳。结论:健脾补肾活血方及其拆方均可不同程度地降低骨髓细胞凋亡率,可促进骨髓恢复造血功能。     

参附注射液配合西药治疗慢性再生障碍性贫血对红细胞免疫功能的影响

目的观察参附注射液对慢性再生障碍性贫血(CAA)患者的临床疗效及对红细胞免疫功能的影响。方法将50例CAA患者随机分为参附组(参附注射液配合西药治疗)30例及对照组(单纯西药治疗)20例,观察2组患者治疗效果及红细胞免疫功能的变化。结果治疗前CAA患者C3bRR均显著性降低(P<0.01),治疗后均较治疗前显著升高(P<0.01)。治疗后2组C3bRR水平比较有显著性差异(P<0.05)。结论参附注射液可能通过调节免疫功能发挥作用,从而提高CAA的疗效。     

中医分型治疗慢性再生障碍性贫血临床研究

目的:探讨再造生血颗粒治疗慢性再生障碍性贫血(CAA)肾阴虚型和肾阳虚型患者的临床疗效和作用机理.方法:选取慢性再障患者40例,其中肾阴虚型22例,肾阳虚型18例.均口服再造生血颗粒0.5 a.检测治疗前后骨髓CD34 细胞bcl-2、bax蛋白的表达.结果:治疗后肾阳虚型基本治愈9例,缓解6例,明显进步2例,无效1例,有效率为90.9%.肾阳虚型分别为5例、5例、8例、4例,有效率为81.8%.两组比较,P＜0.05.肾阳虚型患者的疗效明显优于肾阴虚型者.结论:肾阳虚型治疗前bcl-2值明显高于肾阴虚型,bax则相反.     

Trilineage Response to rhG-CSF with Subsequent Clonal Hematopoiesis in a Patient with Severe Bone Marrow Aplasia

We treated a patient with severe aplastic anemia with long-term administration of recombinant human granulocyte-colony stimulating factor (rhG-CSF). When a trilineage response of hematopoiesis was obtained after the first treatment, a chromosomal change [45XX, -7] was observed in 20 of the 20 metaphases examined. Later, we were able to show a monoclonal X inactivation pattern in the phosphoglycerate kinase (PGK) gene in the peripheral blood polymorphonuclear leukocytes and mononuclear cells, indicating the presence of clonal hematopoiesis regardless of the disappearance of the karyotype abnormality. We suggest that it is important to pay close attention to the appearance of clonal hematopoiesis during the administration of G-CSF to patients with idiopathic severe bone marrow aplasia.     

Recent advances in bone marrow transplantation

The major barriers to successful bone marrow transplantation (BMT) are graft-versus-host disease (GVHD), infection, rejection and relapse. The combination of methotrexate and cyclosporin is significantly better than either alone in controlling GVHD. Removal of T cells from donor marrow prior to BMT has also decreased GVHD significantly, but a 5–10% rejection rate occurs and an increased relapse risk is being reported by some centres. Cyclosporin is valuable in the treatment of both acute and chronic GVHD. Interstitial pneumonitis due to cytomegalovirus (CMV) Is a major cause of mortality. Protection can be provided with CMV hyperimmune globulin and also by the avoidance of blood donors who are CMV antibody positive. Fractionated total body irradiation is associated with decreased toxicity compared to single dose. There is a 75% 4 year disease-free survival following BMT for acute non-lymphoblastic leukaemia in first remission, a 50% survival for acute lymphoblastic leukaemia in second remission and an 88% survival for chronic myeloid leukaemia in chronic phase. BMT for β-thalassaemia major in young patients without organ dysfunction cures 80% of patients and identical results are achieved for severe aplastic anaemia when BMT is undertaken prior to blood product transfusion.