Physical growth in thalassemic children of 2-12 years with multiple transfusions

The hemoglobinopathies are the most common single-gene defect in man. The thalassemia syndromes are a heterogeneous group of hereditary disorder due to decreased synthesis of either α or β globin chain of Hb A. There are 3 phases of growth disturbances and have three different etiologies. First phase: growth disturbances is mainly due to hypoxia, anaemia, ineffective erythropoiesis and nutritional factors; the Second phase: During late childhood, growth retardation is mainly due to iron overload affecting the GH-IGF-1 axis and other endocrinal complications. Third phase: after the age of 10-11 years, delayed or arrested puberty is an important contributory factor to growth failure in adolescents thalassemic who does not show any growth spurt. Cross-sectional, observational, single-centre, tertiary hospital-based study. Children of thalassemia major of 2-12 years with multiple transfusions was taken over 1 year. Study population was divided into 2 groups: Group1-irregularly transfused; Group 2-regularly transfused. Clinical settings, anthropometry, laboratory tests like serum ferritin, pre-transfusion haemoglobin, total leucocyte count etc. were taken into consideration. Thalassemia children with other comorbidities like tuberculosis, chronic kidney disease, chronic heart diseases etc. were excluded from the study.Among the 200 children, 143 (71.5%) were taking regular (2-4 weekly) transfusion therapy and 57 (28.5%) were taking irregular transfusion (>4weekly). Mean age of diagnosis was 18.66 ± 7.443months in Group 1 (Irregularly transfused) and 18.93 ± 7.218 months in Group 2 (Regularly transfused). Among the regularly transfused thalassemic 17.7% children had W/A < 3 percentile and among the irregularly transfused children it was 15%. Among the irregularly transfused children, 27. 1% and among the regularly transfused children 21.6% had H/A <3rd percentile. In the present study children 61% had normal BMI and only 5.4 % had BMI less than 3rd percentile overall. Among irregularly transfused thalassemic children >10years of age, 86.7% have not attained puberty yet. Among the regularly transfused thalassemic children 96.7% have not attained puberty yet. US and LS individually affected resulting in stunting but it was proportionate innature so US: LS ratio was according to age. A positive correlation between pre-transfusion haemoglobin and W/A and H/A suggested that with decreasing pre-transfusion haemoglobin concentration more child had growth retardation. Mean value of serum Ferritin was 941 ± 608.490 ng/ml in Group 1(Irregularly transfused) and Mean value of serum Ferritin was 1403 ± 685.584ng/ml in Group 2(Regularly transfused). MUAC in the present study was 12.44cm suggesting mild-moderate malnutrition. Extremely variable clinical and haematological findings were observed in these patients. Growth retardation has found in both regularly and irregularly transfused patients. These findings are almost comparable to other Indian studies. Appropriate knowledge regarding prenatal counselling, early diagnosis, regular transfusions and overall treatment can help better management of this group of patients.

The Use of Convalescent Plasma (CP) Transfusion Therapy in Moderate to Severe COVID-19 Patients: a Single Center Experience in Indonesia

Background: Coronavirus disease 2019 (COVID-19) was declared as a world pandemic since early 2020. There was no specific antiviral agent that appeared to be active against the virus, and antiviral agent such as remdesivir, favipiravir were in limited supply. We evaluated the use of convalescent plasma (CP) administered as adjuctive treatment to standard of care in moderate to severe COVID-19 patients. Methods: We conducted a series of 9 moderate to severe patients of COVID-19 older than 18 years received CP transfusion from 9 recovered donors at a single institution (Sulianti Saroso Infectious Disease Hospital, Jakarta, Indonesia) from January 2021 to June 2021. Results: Out of 9 patients (age range 30-81 years, 6 males and 3 female), and all patients received at least 1 or 2 unit of 200 mL of CP from 9 recovered donors. There were 4 patients (age range 30-71 years, 4 male) that were not treated with antiviral therapy. Of the 9 patients, 2 severe cases were died, while all of moderate cases survived and they were discharged from the hospital (length of stay: 8-22 days). Conclusion: Our experience showed that CP transfusion in moderate COVID-19 patients might provide clinical benefit and it was well-tolerated. However, further development clinical trials with better designs and greater power is needed to evaluate the efficacy and safety of this treatment.

Some troubles and complications while using transfusion therapy

In the review the literary data on some troubles and complications o f blood transfusion are drawn.

mTOR Signaling Regulates the Development and Therapeutic Efficacy of PMN-MDSCs in Acute GVHD

Myeloid-derived suppressor cells (MDSCs) represent a population of heterogeneous myeloid cells, which are characterized by their remarkable ability to suppress T cells and natural killer cells. MDSCs have been proven to play a positive role in protecting acute graft-versus-host disease (aGVHD). Here, we aimed to describe the mechanism behind how mTOR signaling regulates MDSCs’ generation and explore its prophylactic and therapeutic potential in aGVHD. Reducing mTOR expression retains myeloid cells with immature characteristics and promotes polymorphonuclear MDSC (PMN-MDSC) immunosuppressive function through STAT3-C/EBPβ pathway. Prophylactic transfusion of mTORKO PMN-MDSCs could alleviate aGVHD while maintaining the graft-versus-leukemia (GVL) effect, which could downregulate the Th1/Th2 ratio, decrease serum proinflammatory cytokines, and increase the proportion of regulatory T cells (Tregs) in aGVHD models at the early stage after transplantation. Moreover, transfusion therapy could promote the reconstruction and function of donor-derived PMN-MDSCs. Not only the percentage and the absolute number of donor-derived PMN-MDSCs significantly increased but also the immunosuppressive ability was much more robust compared to other groups. Altogether, these findings indicated that mTOR is an intrinsic regulator for PMN-MDSCs’ differentiation and immunosuppressive function. Together, mTORKO PMN-MDSC transfusion can play a protective role in alleviating cytokine storm at the initial stage and promoting the quantitative and functional recoveries of donor-derived PMN-MDSCs in aGVHD.

Impact of transfusion of blood components on the recipient immune system

Transfusions of blood provide essential therapeutic measures in a number of pathological conditions. However, when carrying out blood component therapy, it is important to consider probability of post-transfusion complications. Most of them are immune-mediated side effects. The unfavorable consequences of blood transfusions can manifest at long-range time periods, and pathogenesis of these phenomena may be associated not only with the presence of alloantibodies. They may be caused by alloimmunization to HLA antigens, leukocyte factors, including cytokines, products of leukocyte degranulation, as well as storage-related erythrocyte damage («storage lesion»), immunomodulatory properties of extracellular vesicles or microparticles derived from blood components, and other factors. Despite significant number of publications on this issue, a lot of unresolved issues still remain, concerning transfusion-related effects of blood components on the immune system of recipients. The review article provides the results of current studies in this area. We present and discuss the results of current studies and the features of transfusion-mediated immunomodulation (TRIM) revealed over recent years, when transfusing different blood components. The role of plasma factors, microparticles, platelets and erythrocytes, HLA sensitization and microchimerism in the development of TRIM is highlighted, the data on occurrence and clinical features of TRIM in perioperative period are presented. A separate section of the review provides information about recent clinical studies, devoted to the issues of TRIM in different clinical cohorts, including newborns, patients with malignant neoplasms, immunocompromised patients after heart and vascular surgery. The data on TRIM incidence in the patients with exhausted immune system due to previous disease or treatment, severe comorbidity, extensive surgical thoracic/abdominal intervention and artificial circulation are also in scope. As based on the studies performed, the role of distinct measures, e.g., washing of erythrocyte concentrates, leukodepletion, and gamma irradiation are discussed in view of potential TRIM prevention. The results of published research do not allow us to draw definite conclusions about the effects of blood component transfusion on the immune system of recipients with respect to differences between the studied groups of patients, characteristics of the studied disorders and clinical situations, diversity of hemocomponents, as well as varying standards of transfusion therapy adopted in different countries. However, the systematic literature review may provide some guidance in transfusion-mediated immune modulation.

Indications for transfusion in the management of sickle cell disease

The transfusion of red blood cells (RBCs) is a crucial treatment for sickle cell disease (SCD). While often beneficial, the frequent use of transfusions is associated with numerous complications. Transfusions should be offered with specific guidelines in mind. Here we present updates to the indications for transfusion of RBCs in SCD. We review recent publications and include expert perspectives from hematology and transfusion medicine. For some clinical indications, such as ischemic stroke, the role of transfusion has been well studied and can be applied almost universally. For many other clinical scenarios, the use of transfusion therapy has less conclusive data and therefore must be tailored to individual needs. We highlight the roles of RBC transfusions in preventing or mitigating neurological disease, in reducing perioperative complications, in managing acute chest syndrome, and in optimizing pregnancy outcomes in SCD. We further highlight various transfusion techniques and when each might be considered. Potential complications of transfusion are also briefly discussed.

Usage of 25% albumin solution in the intraoperative and early postoperative periods of liver transplantation from a cadaveric donor.

Введение. Терапевтическое использование раствора человеческого альбумина у пациентов в периоперационном периоде трансплантации печени (ТП) представляет интерес в контексте осложнений и исходов операции. Цель исследования: оценить влияние интраоперационной трансфузии 25% раствора человеческого альбумина на течение раннего послеоперационного периода при ортотопической ТП от посмертного донора. Материалы и методы. В исследование включены 47 пациентов, которым была выполнена трупная ТП. Были сформированы 2 группы: пациенты группы 1 (n = 21) получали трансфузию 25% раствора человеческого альбумина в конце операции ТП и через 24 ч после операции; пациенты группы 2 (n = 26) получали трансфузию 25% раствора человеческого альбумина в первые сутки послеоперационного периода. Осуществляли контроль лабораторных параметров крови пациента перед началом оперативного вмешательства, через 24 ч и через 48 ч после операции. Интраоперационно оценивали значения систолического (САД) и диастолического (ДАД) артериального давления, частоту сердечных сокращений (ЧСС), дозу вазопрессоров, объем инфузионно-трансфузионной терапии, кровопотерю и диурез. В послеоперационном периоде фиксировали возможные осложнения, проведенные сеансы заместительной почечной терапии (ЗПТ), а также количество дней в стационаре. Результаты. Оценка значений САД, ДАД и ЧСС в начале и конце операции показала достоверно лучшие показатели гемодинамики и снижение дозировок вазопрессорной поддержки в конце вмешательства в группе 1 по сравнению с группой 2 (p < 0,05). В послеоперационном периоде инфекционные осложнения зарегистрированы у одного пациента группы 1 и у трех пациентов группы 2. Проведение ЗПТ потребовалось двум пациентам из группы 2. Все пациенты обеих групп были выписаны из стационара, при этом число дней госпитализации в группе 2 было статистически значимо больше по сравнению с пациентами группы 1: 26,9 ± 3,9 против 17,2 ± 4,3 (p < 0,05). Заключение. Интраоперационная инфузия 25% раствора альбумина позволяет стабилизировать показатели гемодинамики в конце операции ТП, снизить потребность в кардиотонической поддержке и сократить время госпитализации у пациентов после ТП. Background. The therapeutic use of human albumin solution in patients in the perioperative period of liver transplantation (LT) is of interest in the context of complications and outcomes of surgery. Objectives: to assess the effect of intraoperative transfusion of 25% human albumin solution on the early postoperative period in orthotopic LT from a postmortem donor. Patients/Methods. The study included 47 patients who underwent cadaveric LT. Two groups were formed: patients in group 1 (n = 21) received transfusion of 25% human albumin solution at the end of LT and 24 hours after surgery; patients in group 2 (n = 26) received transfusion of 25% human albumin solution on the first day of the postoperative period. The laboratory parameters of the patient’s blood were monitored before surgery, 24 hours later, and 48 hours after surgery. Intraoperatively, the values of systolic (SBP) and diastolic (DBP) blood pressure, heart rate (HR), dose of vasopressors, volume of infusion- transfusion therapy, blood loss and diuresis were assessed. In the postoperative period, possible complications, renal replacement therapy (RRT) sessions performed, and the number of days in the hospital were recorded. Results. Assessment of SBP, DBP and HR values showed significantly better hemodynamic parameters and reduced dosages of vasopressor support at the end of surgery in group 1 compared to group 2 (p < 0.05). In the postoperative period, infectious complications were registered in one patient of group 1 and in three patients of group 2. Two patients from group 2 required RRT. All patients were discharged from the hospital, while the number of hospitalization days in group 2 was statistically significantly greater than in patients in group 1: 26.9 ± 3.9 versus 17.2 ± 4.3 (p < 0.05). Conclusions. Intraoperative infusion of 25% albumin solution allows stabilizing hemodynamic parameters at the end of LT surgery, reducing the need for cardiotonic support and shortening the hospitalization time in patients after LT.

Infusion-transfusion therapy in the treatment of acute massive surgical blood loss in cancer surgery

Введение. Расширенные комбинированные вмешательства являются основным методом лечения пациентов с местнораспространенными солидными злокачественными опухолями. Основным следствием масштабной онкохирургии является риск развития острой массивной кровопотери (ОМОК). Цель исследования: оценка переносимости ОМОК у онкологических больных. Материалы и методы. В одноцентровое ретроспективное исследование с января 1999 г. по декабрь 2018 г. были включены 4236 больных, оперированных в радикальном объёме по поводу злокачественных опухолей различных локализаций, течение операции у которых осложнилось ОМОК. Критерием ОМОК являлась потеря ≥ 50% от расчётного объёма циркулирующей крови (ОЦКр) в течение 3 ч операции. Пациенты, вошедшие в исследование, были разделены на 4 группы в зависимости от объёма кровопотери: от 51 до 100%, от 101 до 200%, от 201 до 300% и свыше 300% ОЦКр. Результаты. Пациенты исследованных групп не различались ни по общим показателям, ни по исходному соматическому статусу. Про анализированы качественный и количественный состав инфузионно-трансфузионной терапии (ИТТ), показатели баланса жидкости интраоперационно, продолжительность пребывания пациентов в отделении реанимации и интенсивной терапии, летальность. Заключение. Соблюдение определенного протокола ИТТ при онкологических операциях, осложнённых ОМОК, является залогом успешного лечения и позволяет снизить интраоперационную летальность до 0,8% и госпитальную летальность до 6,45%. Метод аппаратной реинфузии аутоэритроцитов продемонстрировал высокую эффективность. Background. Cancer surgery remains the backbone of treatment approaches in patients with locally advanced solid malignancies. Risk of acute massive blood loss (AMBL) remains the main complication of such large-scale cancer surgery. Objectives: assessment of AMBL tolerance in cancer patients. Patients/Methods. A single- center retrospective study from January 1999 to December 2018 included 4,236 patients who underwent radical surgery for malignant tumors of various localizations, whose course of surgery was complicated with AMBL. The AMBL criterion was the loss of ≥ 50% of the calculated circulating blood volume (СBVс) within 3 hours of the operation. The patients included in the study were divided into 4 groups depending on the volume of blood loss: from 51 to 100%, from 101 to 200%, from 201 to 300% and over 300% of СBVс. Results. Patients of the studied groups did not differ either in general parameters or in baseline somatic status. The qualitative and quantitative composition of infusion-transfusion therapy (ITT), intraoperative fluid balance, patients stay in the intensive care unit, and mortality were analyzed. Conclusions. Compliance with a specific ITT protocol during cancer surgery complicated by AMBL is the key to successful treatment and allows to reduce intraoperative mortality to 0.8% and hospital mortality to 6.45%. The method of intraoperative red cell salvage and autologus transfusion has demonstrated high efficiency.