雷公藤多甙致老年急性再生障碍性贫血3例分析

由于难以确定药源性再生障碍性贫血（再障）和药物之间的相关性及难以建立良好的实验动物模型，因此，对药源性再障的相关研究非常少。近几年不断有报告利用雷公藤多甙（GTW）的免疫抑制功能治疗急性再障并取得良好疗效的报告。自2003年以来我们收治了3例因服用GTW导致急性再障的病例，现分析其发病经过和治疗过程等临床资料并复习有关文献，以期探讨GTW引起急性再障的可能发病机制。     

雷公藤多甙治疗急性再生障碍性贫血的疗效及其对CD34+细胞增殖能力的影响

急性再生障碍性贫血(AAA)患者的预后极差，免疫抑制剂如抗人胸腺细胞球蛋白(ATG)或环孢霉素A(CsA)可改善患者的预后，但仍有部分患者对治疗无反应，而且ATG和CsA医疗费用较高，临床上难以常规应用，因此，寻找新的有效治疗方法仍有重要的临床意义。雷公藤多甙(GTW)是     

雷公藤多甙致重度贫血一例

患者,男性,39岁。外院诊断为轻度系膜增生性IgM肾病, 8个月前始服雷公藤多甙片治疗,起始剂量为20mg/次,1天3 次。7个月前曾自行将药物加量为30mg/次,1天3次,共用1 周。WBC3.8×109/L,余均正常。遂恢复起始剂量,之后每两 周复查1次血象均正常。2个月前查血常规显示:Hb9.8g/dl, WBC和PLT正常。药物减量为20mg/次,1天2次。1个月前 查血常规显示:Hb8.9g/dl,WBC,PLT正常,停用雷公藤多甙 片。半月前Hb7.6g/dl,10天前Hb6.3g/dl,1天前Hb5.9g/ dl。为进一步诊治收住院。既往体健,不偏食,家族史无特殊。 入院查体:血压130/65mmHg,营养良…     

再生障碍性贫血并发Sweet综合征1例报告并文献复习

目的:提高对再生障碍性贫血并发Sweet综合征的认识。方法:报告1例再生障碍性贫血并发Sweet综合征患者的临床资料,并作文献复习。结果:Sweet综合征可发生在未接受粒细胞集落刺激因子治疗的再生障碍性贫血患者中。结论:Sweet综合征并发再生障碍性贫血时粒细胞可减少,应用皮质类固醇治疗有效。     

大剂量环磷酰胺治疗重型再生障碍性贫血3例并文献复习

目的 探讨以大剂量环磷酰胺(HDCTX)治疗重型再生障碍性贫血(SAA)的可行性.方法 对3例SAA患者行HDCTX免疫抑制剂治疗,观察其疗效及不良反应,并复习相关文献.结果 SAA患者接受HDCTX治疗结束后,1例完全缓解,1例部分缓解,1例死亡.未见明显不良反应.随访至今(53.5个月)未见复发及继发克隆性疾病.结论 HDCTX可能是治疗SAA的有效方法.     

再生障碍性贫血并发滤泡性淋巴瘤病例报告及文献复习

正再生障碍性贫血(简称再障)的发病机制目前仍未完全清楚,多数患者是T-淋巴细胞介导的自身免疫性疾病,在长期免疫抑制剂治疗后有发生淋巴增殖性疾病(lymphoproliferative disorders,LPDs)危险[1],特别是合并EBV(Epstein-Barr virus,EBV)、HBV感染的患者~([2])。再障可先于、同时、或继发于LPDs。本文报道1例慢性再障患者免疫抑制剂治     

抗结核治疗致急性再生障碍性贫血3例报道并文献复习

抗结核治疗药物种类多,剂量足,疗程长,该类药物都是高毒性药物,药物的不良反应多,而引起急性再生障碍性贫血（再障）的报道少见,现将我院发现的3例予以报道.     

苯中毒致急性再生障碍性贫血3例

苯中毒可以引起再生障碍性贫血 (再障 )、白血病等 ,苯的骨髓毒性作用是其代谢产物——环氧化苯所致。环氧化苯作用于造血祖细胞 ,可抑制其 DNA合成。近年来 ,我们遇到苯中毒致急性再障 3例 ,现择其 1例总结如下。患者男 ,2 0岁。因乏力、手足麻木 2 0天 ,发热、鼻衄伴头晕、乏力 10余天入院。体检 :T39.2℃ ,四肢及躯干见散在出血点 ,鼻腔出血。心率 12 0次 / min,律齐 ;肝脾未及肿大。化验 :Hb33g/ L,WBC0 .8× 10 9/ L,中性粒细胞 0 .12× 10 9/ L,淋巴细胞占 6 8% ,PL T1.1× 10 9/ L,网织红细胞为 0。骨髓穿刺涂片示三系增生重度…     

重型再生障碍性贫血合并急性脑梗死一例报道并文献复习

再生障碍性贫血以骨髓造血细胞增生降低和外周血全血细胞减少为特征[1].临床过程中发现一些再生障碍性贫血患者合并有急性脑梗死发生,但由于发生率低,目前相关机制的研究较少.娄底市中心医院神经内科收治1例重症再生障碍性贫血合并急性脑梗死患者,探讨控制血象的方式,现结合相关文献报道如下.     

重型再生障碍性贫血并结核感染诊断难点探讨——附3例报告并文献复习

正重型再生障碍性贫血(severe aplastic anemia,SAA)患者由于其疾病本身、免疫抑制剂的治疗使其免疫功能下降,增加结核感染风险。合并的结核感染症状、实验室检查及影像学表现不典型,给临床诊断增加困难。本文将报道3例SAA行免疫抑制治疗(immunosuppressive therapy,IST)或异基因造血干细胞移植(allogeneic hematopoietic stem cell transplantation,allo-HSCT)后并发结核感染的病例,探讨其诊断难点并进行文献复习。     

Aplastic anemia associated with intravenous chloramphenicol

A patient is presented who developed aplastic anemia 3 months after exposure to intravenous chloramphenicol. She died of this disease 4 years later. Other cases of marrow aplasia due to parenteral chloramphenicol are reviewed, in order to emphasize that this complication, although rare, is not restricted to the use of oral chloramphenicol.     

Aplastic anemia successfully treated with rituximab

An otherwise healthy 73-year-old female was admitted to our department in 1997 because of easy bruising and a platelet count of 12 x 10(9)/L. The patient was taking no medications. Bone marrow examination revealed erythroid hyperplasia, megakaryocytic hypoplasia, and no sign of malignancy. Chromosome analysis showed a normal karyotype. There was serological evidence of previous infection with parvovirus B19. No antibodies to HBV, HCV, CMV, or EBV were found. ANA and cardiolipin antibodies were not detected. Treatment with prednisolone was without effect, but 3 weeks after i.v. gamma-globulin therapy, the platelet count was normal, 233 x 10(9)/L. Two years later, the patient was readmitted with a platelet count of 11 x 10(9)/L. At this time, treatment with corticosteroids, azathioprine, and gamma-globulin had only a temporary effect, and further therapy was stopped because of side effects. During the next 3 years, the patient developed transfusion-dependent anemia, and her white blood cell count decreased to 1.8 x 10(9)/L. A new bone marrow examination showed aplastic anemia with bone marrow cellularity about 10%. After an intracerebral hemorrhage, the patient accepted treatment with rituximab and received 4 weekly doses of 375 mg/m2. This therapy was followed by an increase in the platelet count to 232 x 10(9)/L, white blood cell count to 6.8 x 10(9)/L, and no more need for blood transfusions. A bone marrow examination 5 months after treatment with rituximab showed hyperplastic myelopoiesis, normoblastic erythropoiesis, and slightly reduced megakaryopoiesis. The use of anti-CD20 monoclonal antibody in aplastic anemia warrants further investigation.     

重型再生障碍性贫血免疫抑制治疗后并发淋巴瘤2例报告及文献复习

目的:通过报告2例重型再生障碍性贫血(再障)进行抗人T细胞免疫球蛋白(ATG)加环孢素(CsA)为主的免疫抑制治疗后并发非霍奇金淋巴瘤的病例,结合文献复习,提高对重型再障免疫抑制治疗后并发淋巴增殖性疾病的认识。方法:针对此2例患者进行了病例分析及相关文献复习。结果:2例患者分别在行ATG加CsA为主的免疫抑制治疗后6个月及8个月并发肠道B细胞非霍奇金淋巴瘤,出现不全肠梗阻。其中1例有免疫抑制治疗后病毒感染史,并发淋巴瘤后行CHOP方案化疗,病情好转;另1例无病毒感染史,手术后死于肺部感染。结论:重型再障经免疫抑制治疗后可并发淋巴增殖性疾病,其发生与免疫抑制应用及病毒感染有关,病因及病理与移植后淋巴增殖性疾病相似,是重型再障进行免疫抑制治疗的一种严重并发症。     

Aplastic anemia occurring as amegakaryocytic thrombocytopenia with and without an inhibitor of granulopoiesis

Two patients with aplastic anemia evolving from cellular bone marrows with severely diminished megakaryocytes are reported. During this evolution a plasma inhibitor of in vitro granulocyte-macrophage colony formation was demonstrated associated with non-A, non-B hepatitis in one patient. The second patient had abnormal liver function that corrected after the delivery of a normal newborn but there was persistence of pancytopenia without evidence of a plasma inhibitor.     

Aplastic anemia in Brazil: incidence and risk factors

This study is the first large-scale epidemiological investigation of acquired aplastic anemia (AAA) in South America. The objective was to estimate the incidence and to identify risk factors for AAA in Brazil. A national case-control study was conducted to investigate the risk factors for the disease. One hundred twenty-five cases and 129 controls were included. Multiple logistic regression was used in the estimation of odds ratios (OR) to control confounding. The size of Brazil made it unfeasible to estimate the incidence of AAA in the whole country, and we limited the calculation to the state of Parana. The annual incidence of AAA in Parana was 2.4 cases/10(6) inhabitants. There was no positive association between chloramphenicol use and AAA (OR 0.4; 95% CI: 0.1-2.9). The OR of AAA associated with household pesticides that include organophosphates in their composition was 2.7 (1.0-8.4). The OR for the usage of unspecified thinner and/or acetone for at least 7 days was 3.0 (1.2-7.3). Cases of AAA in Brazil seem to be associated with some factors traditionally related to this disease, such as certain solvents and the incidence is similar to what has been reported from Europe.     

Aplastic anemia successfully treated with rituximab: the possible role of aplastic anemia-associated autoantibodies as a marker for response

A 1-yr-old Japanese male infant developed hepatitis-associated aplastic anemia (AA), and anti-thymocyte globulin (ATG) plus cyclosporine A (CsA) was administered without any appreciable effects. Laboratory examination of the patient's serum obtained before therapy revealed various autoantibodies, such as PA-IgG, anti-platelets, anti-single-stranded DNA (ssDNA), and anti-double-stranded DNA (dsDNA) antibodies (Abs) in addition to anti-DRS-1 Abs and anti-moesin Abs, both of which are known to be detectable in approximately 40% of all patients presenting with AA. He was therefore treated with 17.5 mg/kg/d rituximab 5.5 months after ATG/CsA therapy. The same rituximab therapy was repeated three times once a month thereafter. His neutrophil counts started to increase 50 d after the first rituximab therapy and he achieved a complete remission at 16 months after the last rituximab administration. All of the autoantibodies including anti-ssDNA, dsDNA, DRS-1, and moesin became undetectable when he attained the remission. Anti-CD20 monoclonal antibody therapy may be effective in a subset of patients with AA characterized by the presence of autoantibodies.     

Aplastic anemia following administration of a tumor necrosis factor-alpha inhibitor

Upregulation of tumor necrosis factor-alpha (TNF-alpha) has been implicated in the pathogenesis of several inflammatory conditions, including rheumatoid arthritis. Therapeutic agents such as antibodies or soluble TNF-alpha receptor analogs, which block TNF-alpha activity are a recent addition to the therapeutic armamentarium for the conditions. We describe a patient who developed aplastic anemia complicated by sepsis after receiving etanercept, a TNF-alpha receptor analog, for the treatment of rheumatoid arthritis. Pancytopenia resolved within 3 wk of discontinuing etanercept. To our knowledge, this is the first report of aplastic anemia associated with TNF-alpha blockade.     

Relapse of aplastic anemia in children after immunosuppressive therapy: a report from the Japan Childhood Aplastic Anemia Study Group

Background

Although the therapeutic outcome of acquired aplastic anemia has improved markedly with the introduction of immunosuppressive therapy using antithymocyte globulin and cyclosporine, a significant proportion of patients subsequently relapse and require second-line therapy. However, detailed analyses of relapses in aplastic anemia children are limited.

Design and Methods

We previously conducted two prospective multicenter trials of immunosuppressive therapy for children with aplastic anemia: AA-92 and AA-97, which began in 1992 and 1997, respectively. In this study, we assessed the relapse rate, risk factors for relapse, and the response to second-line treatment in children with aplastic anemia treated with antithymocyte globulin and cyclosporine.

Results

From 1992 to 2007, we treated 441 children with aplastic anemia with standard immunosuppressive therapy. Among the 264 patients who responded to immunosuppressive therapy, 42 (15.9%) relapsed. The cumulative incidence of relapse was 11.9% at 10 years. Multivariate analysis revealed that relapse risk was significantly associated with an immunosuppressive therapy regimen using danazol (relative risk, 3.15; P=0.001) and non-severe aplastic anemia (relative risk, 2.51; P=0.02). Seventeen relapsed patients received additional immunosuppressive therapy with antithymocyte globulin and cyclosporine. Eight patients responded within 6 months. Seven of nine non-responders to second immunosuppressive therapy received hematopoietic stem cell transplantation and five are alive. Eleven patients underwent hematopoietic stem cell transplantation directly and seven are alive.

Conclusions

In the present study, the cumulative incidence of relapse at 10 years was relatively low compared to that in other studies mainly involving adult patients. A multicenter prospective study is warranted to establish optimal therapy for children with aplastic anemia.     

Aplastic anemia complicating systemic lupus erythematosus: response to androgens in two patients

We describe two female patients with systemic lupus erythematosus (SLE) who developed severe aplastic anemia. Although each patient had received multiple medications including diphenylhydantoin, the relationship to these drugs to the development of marrow aplasia was unclear. After administration of an oral androgen (oxymethalone) and corticosteroids, there was complete hematologic recovery. Both patients relapsed when oxymethalone was withdrawn, and both recovered when androgen therapy was reinstituted, with or without high-dose prednisone. In both patients, there was complete reversal of pancytopenia despite the presence of initially severe marrow aplasia (less than 10% cellularity). However, in both cases, prolonged androgen therapy (2 months) was required before hematologic improvement occurred. Androgens are known to stimulate hematopoiesis in man, and they appear to influence immune function in a mouse model of SLE. Thus androgens may be particularly useful in the treatment of SLE-associated aplastic anemia.     

Agranulocytosis and mixed‐type autoimmune hemolytic anemia in primary sjögren's syndrome: a case report and review of the literature

Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease that presents with sicca symptoms of the main mucosal surfaces. Patients with pSS have a broad spectrum of laboratory features, such as cytopenias and hypergammaglobulinemia. Although hematological abnormalities are usually seen in pSS patients, agranulocytosis and autoimmune hemolytic anemia (AIHA) are rare. Here we describe a 40‐year‐old woman with pSS who developed both agranulocytosis and mixed‐type AIHA. An increased risk of malignancies has also been reported in pSS patients with hematological changes. Although there is no evidence of malignancies, this patient should be closely followed up in case of developing lymphoma.