Apheresis for babesiosis: Therapeutic parasite reduction or removal of harmful toxins or both?

Babesiosis is a potentially life‐threatening illness caused by intraerythrocytic protozoan parasites of the genus Babesia that are transmitted most commonly by Ixodes ticks, and rarely from blood transfusion or congenitally. Clinical presentations of babesiosis include asymptomatic infection, mild to moderate disease, or severe disease. Antibiotics such as atovaquone plus azithromycin or clindamycin and quinine can be used effectively to treat this disease in most cases, however in high risk populations, the mortality rate can be as high as 20% despite therapy. Therapeutic exchange transfusion has been used in severe babesiosis and is of apparent therapeutic benefit. It is not entirely clear through what mechanism therapeutic exchange transfusion may help patients. Data suggests that in addition to parasite load reduction, it is possible that therapeutic exchange transfusion removes toxins generated by babesia infection. There are many remaining questions that need to be addressed regarding exchange transfusion for babesiosis. J. Clin. Apheresis 31:454–458, 2016. © 2015 Wiley Periodicals, Inc.     

The unusual case of babesiosis causing disseminated intravascular coagulation with hemophagocytic lymphohistiocytosis

Babesiosis is increasing in the elderly due to an age‐related decline in immunity. Prompt diagnosis with blood smear and PCR prevent life‐threatening complications, like DIC and HLH. Studies focusing on pathophysiology and risk factors are needed.     

Therapeutic plasma exchange in the management of acute complications of sickle cell disease: A single centre experience

Sickle cell disease results in systemic inflammation even at steady state and this is accentuated during acute crises. The plasma of affected patients contains several proinflammatory cytokines as well as adhesion molecules and prothrombotic factors. This environment promotes further red cell sickling while many of these substances can cause direct tissue toxicity and end-organ damage. Even though red cell transfusion, whether simple or exchange, is the mainstay of treatment of severe acute complications, addition of therapeutic plasma exchange could potentially provide additional benefit by removing such harmful substances. Here, we describe two cases where therapeutic plasma exchange was used. The first involved a patient with the acute chest syndrome who despite adequate red cell exchange remained significantly hypoxic and in severe pain. We therefore proceeded to perform plasma exchange; this led to rapid clinical improvement and resolution of his symptoms. The second case involved a patient with intractable chest wall pain and impending acute chest syndrome; this patient also had a past history of hyperhaemolysis. The patient underwent therapeutic plasma exchange with very rapid resolution of the pain, avoidance of any respiratory deterioration and full recovery. We also give a brief summary of our previous experience using plasma exchange in patients with sickle cell disease. Plasma exchange was well tolerated with no adverse events in all cases we have treated, led to rapid resolution of pain irrespective of primary indication and in the majority of cases to a favourable clinical outcome.     

Transfusion support for patients with sickle cell disease

Red blood cell (RBC) transfusion is an essential treatment for many patients with sickle cell disease (SCD), whose RBCs express hemoglobin S (HbS), a mutated form of hemoglobin A (HbA). Transfusion goals include increasing blood oxygen carrying capacity and decreasing the relative amount of HbS to HbA to mitigate vaso-occlusion in small blood vessels. In situations where correction of severe anemia and reduction in HbS may be achieved without removal of RBCs, simple transfusion may be utilized. Partial manual RBC exchange, which removes blood containing HbS by phlebotomy and replaces with donor blood transfusion sequentially allows for larger changes in the ratio of HbS to HbA when compared to simple transfusion. Automated RBC exchange by apheresis is useful in situations where a rapid and drastic HbS reduction is indicated. Vascular access is an important consideration for transfusion. Although peripheral access may be sufficient, central venous catheters and implantable venous access devices may be necessary for adequate access over time. Blood bank considerations include adequate RBC antigen matching to mitigate the risk of RBC alloimmunization, of which patients with SCD are at risk of developing. Transfusion may be utilized in efforts to intervene in the evolution of potentially life-threatening complications of SCD such as acute stroke, severe acute anemia and acute chest syndrome. Transfusion is also useful in several non-acute settings, such as stroke prevention, pregnancy, pre-surgery, and transfusion support for curative therapies. Individualized treatment plans are an essential component of patient care. Continuous evaluation of clinical indications and evolution of guidelines will continue to optimize care for patients with SCD.     

2010年与2013年北京协和医院住院患者红细胞输注情况

目的　初步了解2010年和2013年北京协和医院住院患者红细胞输注的概况和变化。方法　随机抽取2010年和2013年在北京协和医院住院期间行红细胞输注的患者资料，比较其性别、年龄、输血时所在科室、第1次红细胞输注前的血红蛋白值等临床信息。结果　本研究纳入住院期间行红细胞输注的患者2010年为174例，2013年为500例。住院患者红细胞输注前平均血红蛋白2010年非手术科室为（67.71±11.31）g/L，手术科室为（78.68±15.64） g/L；2013年非手术科室为（63.75±12.41）g/L，手术科室为（81.53±17.09）g/L；2010年和2013年非手术科室患者红细胞输注前血红蛋白检测率均为100%;手术科室患者2013年红细胞输注前血红蛋白检测率为68.6%，较2010年的47.7%提高，差异具有统计学意义（P＜0.001）。结论　北京协和医院非手术科室和手术科室启动红细胞输注的血红蛋白阈值均符合我国与国际输血指南所推荐的范围，体现本院对红细胞输注一贯严格把握输血指征，用血行为合理，并不断完善进步。     

More efficient exchange of sickle red blood cells can be achieved by exchanging the densest red blood cells: An ex vivo proof of concept study

Background

In sickle cell disease (SCD), red blood cells (RBCs) containing hemoglobin S can be denser than RBCs containing wild-type hemoglobin, especially when dehydrated. We hypothesize that targeting denser RBCs during red blood cell (RBC) exchange for SCD could result in more efficient removal of dehydrated, sickled RBCs and preservation of non-sickled RBCs.

With a simple calculation,the fraction of platelets remaining can be used to estimate the residual hemoglobin S percentage in sickle cell disease patients undergoing automated red blood cell exchange

Objectives

Automated red blood cell exchange (RBX) is an important treatment for patients with sickle cell disease (SCD). Although not specifically targeted for removal, platelets (PLTs) are collected along with red blood cells during RBX. We sought to determine whether the pre- and post- RBX PLT counts could be used to derive the post-procedure hemoglobin S% (HgbS%).

Prenatal cases with rare RIT1 variants causing severe fetal hydrops and death

We describe two clinical prenatal cases with rare de novo RIT1 variants, which showed more severe clinical manifestations than other Noonan Syndrome genotypes, resulting in fetal death. Extra attention is recommended when these variants are detected.     

Plasma and red cell exchange transfusions for erythropoietic protoporphyria: A case report and review of the literature

Erythropoietic protoporphyria (EPP) is a rare and usually autosomal dominant disorder characterized by ferrochelatase deficiency and accumulation of protoporphyrin in red blood cells (RBCs), skin, and liver. A small minority of patients develop severe liver dysfunction for which optimum treatment is lacking. Therapeutic plasma exchange (TPE) and RBC exchange (RCE) have been anecdotally reported to benefit patients with EPP and liver failure. A 50‐year‐old female with EPP developed severe liver dysfunction after knee replacement surgery and high‐dose acetaminophen use. Liver biopsy showed cholestatic liver injury without fibrosis. A total of 20 TPE procedures, six RCE procedures, and then 14 more TPE procedures were performed as adjunctive therapy with the purpose of preventing progression to end‐stage liver failure. After initial TPE, the plasma and RBC protoporphyrin levels decreased from 834.9 to 180.4 μg/dL (normal, ≤1 μg/dL), and from 3,905 to 2,879 μg/dL (normal, ≤80 μg/dL), respectively, without liver function improvement. RCE decreased RBC protoporphyrin levels from 2,879 to 1,225 μg/dL but plasma protoporphyrin increased from 180.4 to 1,044.1 μg/dL, and liver function failed to improve. Additional TPE again stabilized plasma protoporphyrin and improved RBC protoporphyrin levels but the patient ultimately died owing to end‐stage liver disease complications. This case illustrates that TPE and RCE may improve the plasma and RBC biochemical markers of EPP activity but liver function abnormalities may persist and patients may still progress to liver failure either because of irreversible liver injury or independent pathobiological factors unrelated to EPP‐induced hepatotoxicity. J. Clin. Apheresis, 2012. © 2012 Wiley Periodicals, Inc.     

重症中暑患者在院期间成分输血情况分析

目的分析重症中暑患者临床资料及住院期间输血情况,为重症中暑患者合理有效输血治疗提供参考。方法对本院自2015年6月—2018年8月期间采用成分输血和/或血浆置换救治的21名重症中暑患者的临床资料特征、部分相关血液检验结果及血液成分输注情况进行回顾性分析。结果本研究共21名患者中,用血时间为3.8±2.86 d,住院天数为27.29±17.04 d。患者中6例输注悬浮红细胞RBC共计52.4 U;20例输注冰冻血浆FP共计35 840 mL,14例输注冷沉淀CRYO共计466 U,10例输注血小板APC共计18个治疗量;3例完成8次血浆置换用量为7 720.00±1 500.20 mL共计23 160 mL。按21例输血情况计算4种血液成分的输注量分别为RBC:2.50±6.28 U,FP:1 706.67±1 240.68 mL,CRYO:22.19±21.62 U,APC:0.86±1.08个治疗量。3名行血浆置换患者,置换后血清丙氨酸氨基转移酶ALT、血清天门冬氨酸氨基转移酶AST两项指标均明显降低;血清总胆红素TBil、血清直接胆红素DBil两项指标变化趋势没有特异性差别。1名患...     

非血缘脐血移植术后患者输血情况相关影响因素分析

目的探讨行非血缘脐血移植术(UCBT)后患者成分输血及剂量的相关影响因素。方法回顾29例行非血缘脐血移植的血液病患者移植后60天内输血信息,将患者分为低剂量输注组:红细胞和血小板输注量总计<18 U;高剂量输注组:红细胞和血小板输注量总计≥18 U。分析患者的移植前临床资料、移植后植入情况、移植后急性移植物抗宿主病(aGVHD)发生以及一年内生存率与临床输血情况的关系。结果29例患者移植后60天内平均红细胞输注量为(10.97±11.80)U,平均血小板输注量为(7.12±5.06)U,血小板与红细胞的输入量呈显著正相关;高低剂量输注组移植前两组患者临床资料无显著差异,移植后中性粒细胞植入时间与血小板输注量呈正相关(P<0.05);移植后发生Ⅱ~Ⅳ级aGVHD的患者输注红细胞与血小板显著高于0~I级aGVHD的患者;低剂量输注组一年生存率显著高于高剂量输注组(P<0.05)。结论非血缘脐血移植患者移植后60天内红细胞和血小板输注量与供体植入,移植后aGVHD发生以及移植后生存期显著相关。     

Anti-Co(a) implicated in severe haemolytic disease of the foetus and newborn

summary .  The Colton (Co^a) antigen is of high frequency; its incidence in Caucasians is about 99.8%. Reports on haemolytic transfusion reactions and haemolytic disease of the foetus/newborn (HDFN) due to anti-Co^a are rare. We report a severe HDFN due to anti-Co^a. The first child of the mother was healthy. The second died a few hours after delivery because of hydrops fetalis, likely due to HDFN; anti-Co^a in the maternal serum, the father typed as Co(a+). The third pregnancy was followed up by the measurements of anti-Co^a titre (additional antibodies were excluded), its functional activity by the chemiluminescence test (CLT) and the Doppler flow in the middle cerebral artery of the foetus. Increased values of antibody titre up to 128, the CLT to 30% and multiplex of median of the peak systolic velocity to 1·71 indicated haemolytic disease and the necessity for an intrauterine transfusion. The foetus received the maternal red blood cells (RBCs). Delivery had to be by Caesarean section for obstetrical reasons at 34-week gestation. The newborn (anti-Co^a on red cells and in plasma, the rise of the bilirubin concentration up to 333 μmol L⁻¹) had four exchange transfusions: the first of maternal RBCs, the remaining of donor's Co(a+) cells and one top-up transfusion. The baby was discharged in good health. Anti-Co^a was responsible for severe HDFN. Proper monitoring during pregnancy and antenatal and post-natal therapy were successful. This is the second severe published HDFN due to anti-Co^a.     

A brief review of common mathematical calculations in therapeutic apheresis

Apheresis is defined as the removal of blood from the body, its separation into constituent components, and removal or manipulation of one of these components prior to intravascular return with or without the addition of replacement fluid. Patients undergoing therapeutic apheresis often have multiple comorbidities, potentially affecting their hemodynamic status. Thus, a thorough understanding of apheresis principles and calculations is required for the performance of safe, efficacious, and successful procedures. The performance of simple transfusions or red blood cell exchange procedures is additionally complicated by the difficulties inherent in the procurement of compatible blood products, and the emphasis on minimizing exposure to unnecessary blood products. It is essential that apheresis physicians be able to accurately evaluate the risks/benefits inherent in the procedural options and efficiently stratify patients to the optimal therapeutic modality. The formulas requisite for performing therapeutic apheresis calculations are herein reviewed.     

Transfusion of Blood Products in Trauma: An Update

Background: Blood transfusion in the management of severely injured patients can be lifesaving. These patients are susceptible to developing early coagulopathy, thus perpetuating bleeding. Objectives: This article presents recent advances in both the civilian and military clinical arena to improve the treatment of trauma patients with severe hemorrhage, the use of agents to support coagulation, perspectives on restrictive transfusion strategies, and transfusion-related risks. Discussion: Massive blood transfusion is an adjunct to surgical care. The volume of blood products transfused and the ratio of blood components have been associated with increased morbidity and mortality rates. The adverse clinical effects of transfusion and the limited supply of blood products have resulted in modern resuscitation protocols to limit the volume of blood transfused. Conclusion: A restrictive blood transfusion strategy and the use of hemostatic agents may decrease morbidity and mortality in trauma patients, but insufficient data are available for their use in trauma patients. Massive transfusion should reflect an equal ratio of packed red cells and plasma to limit coagulopathy. Prospective randomized trials are needed to standardize an effective protocol.     

miRNA96 expression level within red blood cells is probably associated with RSL indicators during the storage of red blood cell units

Background and objectivesMany biochemical and hematological changes occur during the storage of RBC units. Collectively, these changes are known as RSLs. Previous studies found miRNA96 as non-coding RNA that its expression level changed during RBC storage. However, its correlation with mechanical and biochemical RSL indicators is not yet determined. Therefore, this study aimed to assess possible correlations between miRNA96a and some RSLs indicators to clarify its biomarker capability for evaluating the storage quality of RBC units.Materials and methodsSamples were collected from ten leuko-reduced RBC units on days 0, 14, 28, and 42 of storage. miRNA96 gene expression level and RSLs indicators including hemolysis, mechanical fragility index (MFI), total antioxidant capacity (TAC), lipid peroxidation (TBARs), thiol groups, and RBC indices were measured on the days mentioned above.ResultsSignificant correlations were found between the changes in miRNA96 expression level and the levels of hemolysis, TAC, TBARs, and MFI indices (p values < 0.05). The donors were classified into the high risk group and low risk group, according to four important characteristics and lifestyle habits (smoking, physical activity, age, and BMI). The high risk group had a significantly lower rate of hemolysis, free hemoglobin, MFI, TAC, and a higher rate of lipid peroxidation compared to low risk group (p values < 0.05).ConclusionThe finding suggested that upregulation of miRNA96 could prevent hemolysis of RBCs, despite the accumulation of oxidative injuries in them. The miRNA96 expression level was probably a potential predictor for mechanical and biochemical RSL indicators.