Treatment of pure red-cell aplasia following major ABO-mismatched T-cell-depleted bone marrow transplantation: Two case reports with successful response to plasmapheresis

Abstract. Major ABO-mismatched bone marrow transplantation (BMT) may be accompanied by red-cell haemolysis, but pure red-cell aplasia following BMT is a rare complication. Two cases of transient pure red-cell aplasia following T-lymphocyte-depleted BMT for a period of > 20 weeks are described, both of which responded to one cycle of plasmapheresis. The prompt response of the two patients described with red-cell aplasia with no evidence of haemolysis suggests that plasmapheresis may be considered in such clinical situations as a first treatment of choice before attempting more complex modes of therapy.     

In vitro assessment of red-cell concentrates in SAG-M filtered through the MacoPharma™ P-CAPT prion-reduction filter

This study investigated whether filtration of leucodepleted red cells in SAG-M through the P-CAPT™ filter in order to prevent the potential risk of vCJD infection associated with prion transmission through transfusion has any deleterious effect on red-cell quality. Bottom-and-top SAG-M leucodepleted red-cell concentrates (24 units) were prion-reduction filtered on the day following collection, with half of the units undergoing irradiation on day 14. A control group (12 units) was not prion filtered. Units were sampled at 7-day intervals up to day 35 and tested using standard measures of red-cell quality as well as prothrombin content (to examine prion filter efficacy). Haemoglobin loss per unit was ∼9 g and in some cases levels were below standard specification (40 g). Haemolysis increased significantly after filtration [0.01 (0.00-0.05) vs. 0.23 (0.07-0.52, p<0.001)]. Prothrombin levels were reduced 41.6-fold compared to leucodepleted red-cell units. Product specifications were within or close to routine acceptable levels. Owing to the reduction in haemoglobin levels below those specified, it may be preferable to reduce haemoglobin specification levels and transfuse more prion-filtered units rather than transfuse potentially unsafe blood product. The risk of transfusing more units with less haemoglobin should be offset against the risk of transfusing unfiltered blood.     

环孢素A治疗纯红细胞再生障碍性贫血15例

目的探讨环孢素A治疗纯红细胞再生障碍性贫血（PRCA）的疗效。方法采用回顾性分析的方法，对我院2000．1-2007．12月门诊和住院收治的15例采用环孢素A（CsA）治疗的15例PRCA患者进行研究。结果治疗总有效率达73．3％，其中7例患者外周血象明显进步（46．7）％；4例患者外周血象改善（36．7％）。4例病人治疗无效疗（36．7％）。结论CsA治疗PRCA效果肯定，可以用作PRCA的一线治疗。     

氨磷汀联合红系生成素治疗高龄单纯红系再生障碍性贫血近期疗效观察

本研究旨在观察氨磷汀（AMF）联合重组人β红系生成素（rhβ-EPO）对单纯红系再生障碍性贫血（PEA）患者的近期疗效及副作用。采用AMF联合rhβ-EPO治疗2例高龄PEA患者。治疗方案为AMF0．4g静脉滴注，5天为1疗程，休息2天，连续3疗程为1个治疗周期；rhβ-EPO 6000U，皮下注射，1周3次。结果显示：2例PEA患者外周血的红细胞数、血红蛋白、网织红细胞在治疗后明显上升，骨髓中红系比例增高，治疗后输血间隔逐渐延长，输血量较治疗前明显下降，改善了患者的生活质量？而AMF副作用主要表现为胃肠道反应，患者均可耐受。结论：氨磷汀联合rhβ-EPO可能是高龄单纯红系再生障碍性贫血患者一种新型、安全、有效的方法，其远期疗效及作用机制尚需进一步探讨．     

Eltrombopag for the Treatment of Refractory Pure RBC Aplasia after Major ABO Incompatible Hematopoietic Stem Cell Transplantation

Pure RBC aplasia (PRCS) is a well-recognized complication after allogeneic hematopoietic stem cell transplantation (HSCT). Many therapeutic options are available to treat this condition, including erythropoietin, rituximab, bortezomib, plasma exchange, immunoadsorption, donor lymphocyte infusion, mesenchymal stem cells, antithymocyte globulin, and high-dose steroids; however, treatment outcomes are often variable and can sometimes lead to disappointing results. In this brief article we report our experience with 2 patients with PRCA after major ABO-incompatible HSCT who were resistant to multiple therapeutic interventions and who eventually benefited from treatment with eltrombopag, a thrombopoietin mimetic approved by the US Food and Drug Administration for the treatment of patients with immune thrombocytopenic purpura or severe aplastic anemia refractory to immunosuppressive agents or not eligible for HSCT. Data from these 2 patients show that eltrombopag was effective in treating erythroid aplasia and transfusion dependence after HSCT in patients who did not benefit from multiple previous treatments. Moreover, eltrombopag was well tolerated, with only a transient thrombocytosis requiring dose adjustment and no evidence of clonal evolution. Based on the positive results obtained in these 2 patients, we suggest that eltrombopag may have a favorable effect on unilineage cytopenias such as PRCA. Further studies in a large proportion of patients are mandatory to confirm these preliminary results.     

Congenital and acquired single-lung patients: long-term follow-up reveals high mortality risk

Purpose

Single-lung patients are uncommon. Case reports suggest that these patients have significant morbidity and mortality risks because of associated anomalies, airway obstruction as well as decreased pulmonary reserve. This study was designed to review morbidity and mortality of single-lung patients.

Methods

A retrospective review of single-lung patients admitted to our hospital from January 1988 to June 2007.

Results

Twelve single-lung patients were treated in our hospital with mean follow-up of 22.9 months. Six congenital lung aplasia patients presented as neonates, with 2 diagnosed antenatally. Of 4 patients with respiratory distress, 3 required surgical interventions to correct airway symptoms. Three patients died before 9 months of age because of respiratory failure, sepsis, or cardiac arrest. The remaining survivors have minimal respiratory distress. There were 6 pneumonectomy patients as follows: 5 for malignancies and 1 for bronchiectasis. There were 2 deaths from recurrent disease. Four patients had respiratory symptoms with one patient requiring tissue expander insertion for the correction of airway torsion from mediastinal shift.

Conclusions

The morbidity and mortality of infant single-lung patients are significant. Respiratory distress from decreased pulmonary reserve must be distinguished from airway obstruction because of mediastinal shift. Bronchoscopic assessment was invaluable to the diagnosis and management of these patients.     

ABO血型不合异基因造血干细胞移植患者ABO血型抗体的监测

目的:监测ABO血型不合造血干细胞移植患者IgM型和IgG型ABO血型抗体的变化,研究这些变化在移植患者血型转变中的作用。方法:用凝聚胺法和微柱凝胶法监测32例移植患者ABO血型,IgM型和IgG型ABO血型抗体的变化。结果:32例ABO血型不合患者血型全部转变为供者型,造血重建时间结果分析表明血型不合对于粒系和血小板的恢复无影响;患者血型转为供者血型后,其血清中缺乏相对应的抗体;32例ABO血型不合患者中有6例供受者移植模式为A→O,其中有3例发生PRCA,且均检出IgG型抗A抗体,其余29例未检测到IgG血型抗体;3例PRCA患者红系造血恢复时间明显延长。结论:IgG型ABO血型抗体可能会抑制ABO血型不合骨髓移植患者的红系分化成熟,在PRCA的发生中可能起重要作用。监测ABO血型不合移植患者体内的ABO血型抗体变化可指导输血治疗,避免溶血性输血反应。     

Pure red cell aplasia due to parvovirus B19 infection after liver transplantation： A case report and review of the literature

Pure red cell aplasia （PRCA） due to parvovirus B19 （PVB19） infectiori after solid organ transplantation has been rarely reported and most of the cases were renal transplant recipients, Few have been described after liver transplantation. Moreover, little information on the management of this easily recurring disease is available at present. We describe the first case of a Chinese liver transplant recipient with PVB19-induced PRCA during immunosuppressive therapy. The patient suffered from progressive anemia with the lowest hemoglobin level of 21 g/L. Bone marrow biopsy showed selectively inhibited erythropoiesis with giant pronormoblasts. Detection of PVB19-DNA in serum with quantitative polymerase chain reaction （PCR） revealed a high level of viral load. After 2 courses of intravenous immunoglobulin （IVIG） therapy, bone marrow erythropoiesis recovered with his hemoglobin level increased to 123 g/L. He had a lowlevel PVB19 load for a 5-too follow-up period without recurrence of PRCA, and finally the virus was cleared. Our case indicates that clearance of PVB19 by IVIG in transplant recipients might be delayed after recovery of anemia.     

A case of pure red cell aplasia and systemic lupus erythematosus caused by human parvovirus B19 infection

Human parvovirus B19 (B19) rarely induces pure red cell aplasia (PRCA) in healthy hosts. Meanwhile B19 infection is often clinically similar to systemic lupus erythematosus (SLE), and several cases have been reported wherein B19 actually stimulated SLE exacerbation in an immunocompetent subject. An 82-year-old healthy woman was diagnosed to have complicated with B19 infection and PRCA. Four weeks later, she had high fever, polyarthritis, and oral ulcers, additionally diagnosed with SLE, and subsequently, 15 mg of prednisone was started. This is the first case wherein B19 infection caused both PRCA and SLE in a healthy patient as far as our investigations are concerned.