57. Jahrestagung der Deutschen Gesellschaft für Transfusionsmedizin und Immunhämatologie

A case report: positive clinical development of a rare disease (GPCR-autoantibodies (AABs) mediated microangiopathy with idiopathic hypoxemia) with immunadsorption (IA)

Hemotherapy and Patient Blood Management

Ein case report: Positive klinische Entwicklung einer seltenen Erkrankung (GPCR-Autoantikörper (AABs), vermittelte Mikroangiopathie mit idiopathischer Hypoxämie) durch Immunadsorption (IA)

About 3 years ago, the 67-year old male patient complained of CHD typical symptoms, but more likely at the beginning or after exercise. Cardiological and pulmonological examinations showed no pathological features.In the meantime, clinical signs of hypoxemia (lip cyanosis, acrocyanosis) and evidence of strongly fluctuating decreased oxygen saturation up to 90% were observed.In addition, there were also new symptoms of ME/CFS and GPCR-AABs were detected in Bioassay and Elisa technologies.After screening for eligibility the patient received 5 IA-treatments on 5 consecutive days (primary device: Spectra Optia, Terumo BCT; secondary device: ADAsorb medicap clinic with TheraSorb Ig omni 5 Adsorber, Miltenyi Biotec B.V.&amp;Co. KG). The primary endpoint was the removal of AABs. The treatment resulted in lowering of plasma IgG to levels below 2 g/l with focus on the elimination of AABs.The removal of IgG was successfully high (IgG lowering about 90%). The control immediately after the IA showed no more AABs detectable.The first therapy with 5 sessions in January 2023 resulted without improvement of symptoms. After the 2nd series of IA in May 2023 (5 treatments on 5 consecutive days) the patient recognized a slow improvement and no more AABs were detected in Bioassay.The general condition has improved quite significantly: still dependent on oxygen, but with oxygen administration hardly any pain, no more continuous bedriddenness quite acceptable at rest, but almost no resilience and significantly less stenocardia and less dyspnea.Based on the highly probable classic diagnosis of idiopathic hypoxemia, there is a suspicion that the condition is not idiopathic, but an AAB-mediated microangiopathy. The AABs would then also be responsible for the other symptoms described at the beginning.IA resulted in a significant reduction of symptoms of our patient and none worsened.In conclusion this clinical picture is unknown, because idiopathic hypoxemia itself is extremely rare, and AABs have never been discussed in this context.None.

Poster

Development of a concept to reduce the discard of erythrocyte concentrates

Entwicklung eines Konzepts zur Reduzierung des Verfalls von Erythrozytenkonzentraten

Due to an imbalance between blood donations and demand, there are shortages of erythrocyte concentrates. Therefore, it is essential that all parties involved in using blood as a resource work as effectively as possible. This includes optimizing logistical processes to minimize discard, particularly at the end of use. The aim of this work is to define factors influencing discard and derive measures for expiration reduction applicable to hospitals.The immunohematologic statistics from 2008 to 2023 for 18 hospitals and their departments were evaluated. These statistics include the number of cross-matches, transfusions, and discarded erythrocyte concentrates. Based on this data, rejection rates and the transfused/cross-matched ratio were calculated for each hospital and department.Anomalies in the statistics were discussed with the respective transfusion managers in separate interviews, and influencing factors were defined.Furthermore, a profile was created for each hospital, detailing case numbers and bed capacity, to facilitate comparability later on.The most notable influencing factors were observed in maximum care hospitals. Hospital Q reduced expiration from 6.32% to 3.29% by revising indication lists and further improved discard rates to 1.16% by using type and screen instead of cross matches.Hospital H decreased expiration from 16.58% to 7.29% by switching to AB0-compatible transfusions.In Hospital K, expiration rates increased from 7.43% to 21.64% due to changes in laboratory and clinical structure. Reasons for higher discard are the implementation of patient blood management without reduction of blood bag stock, closing the lab in the hospital, an increase of orders as a panic reaction as well as personal engagement of the transfusion specialists.Based on the defined influencing factors, a catalogue of measures could be created. This catalogue of measures is an individually usable tool for hospitals, enabling them to identify factors they can optimize without significant additional financial expenses, thereby contributing to an improved supply situation of erythrocyte concentrates.No conflict of interest.

Prof. Dr.

Prof. Dr. med.

Patient Blood Management under Duress – Interdisciplinary Longtime Approach for a Pregnant Woman with Placenta Accreta Spectrum (PAS) Disorder Complicated by Anti-U

Patient Blood Management unter Zugzwang – Interdisziplinäres Langzeitvorgehen bei Schwangerer mit Placenta Accreta Spectrum (PAS) Disorder Erschwert durch Anti-U

Prolonged blood supply for a bleeding patient with an antibody against a high frequent antigen is challenging, particularly if blood groups with high ethnic disparities are involved. Besides optimization of hematopoiesis and surgical blood saving techniques, availability of compatible blood units is essential during life-threatening clinical situations.A 38-year-old female (G5 P3, 30th week of gestation WG) of African ancestry was admitted with vaginal bleeding due to PAS disorder. Lab results: Hb 10,5g/dl; Hct 31%; MCV 79 fl; MCH 27 pg; MCHC 34 g/dl; BG: 0 ccD.ee; Kell neg.; S-, s-. Anti-U (known since 2022), titer 16 (IAT, tube technique). GYPB-Genotyping: most consistent with GYPB*05N, homozygous. Anti-U follow-up titers: max titer 32 (IAT, 34th WG). Antibody dependent cell-mediated cytotoxicity &lt; 10% predictive for no HDN-risk. The clinically unstable situation (bleeding risk, HDN-risk) made long-term PBM obligatory. The logistics had to last 4 weeks, up to operational delivery planned at 34th WG. Departments of obstetrics, prenatal medicine, anesthesiology, transfusion medicine and radiology were involved.The patient got parenteral iron substitution. The national rare donor panel (RDP) revealed 4 pRBC1 (frozen stored) and one walking donor2, both with Uvar-phenotype (1genotype GYPB*03N.04 homozygous, 2genotype not available). The frozen stored pRBC were crossmatch-negative (x-match), the blood of the walking donor was x-match positive (2-fold reaction, IAT, column agglutination technique). With the international RDP we obtained 4 totally compatible, U-neg. pRBC (liquid stored, 1 from NL, 3 from UK). At 34th WG the child was born by caesarean combined with hysterectomy and intraaortic balloon-insertion to prevent massive hemorrhage. The delivery was uneventful. Maternal blood loss was appr. 700 ml, therefore no pRBC were required.Interdisciplinary PBM is the key approach for clinical situations with prolonged high volume bleeding risk and limited blood resources. In our case, the situation was complicated due to an antibody (Anti-U) corresponding to a high frequent blood group antigen. The provision of U-negative pRBC was time consuming and only possible with the use of international rare donor panels. Due to the increasing migration to Germany, ethnic diversification of our blood donor population is necessary for such cases.We have no conflicts of interest to disclose.

Efficacy of therapeutic plasma exchange in neurological autoimmune diseases at a University Medical Center

Wirksamkeit des therapeutischen Plasmaaustausches bei neurologischen Autoimmunerkrankungen an einem Universitätsklinikum

Therapeutic plasma exchange (TPE) is used for the extracorporeal removal of plasma and its replacement by plasma derivatives or albumin solution and is a treatment option for autoimmune neurological disorders such as Guillain-Barré-Syndrome (GBS), Chronic inflammatory Demyelinating Polyneuropathy (CIDP), Multiple Sclerosis (MS), Neuromyelitis Optica Spectrum Disorders (NMOSD) and Myasthenia gravis (MG). The aim of this study is to describe the efficacy of TPE in neurological autoimmunity at our centre against the background of adverse effects.We analyzed 83 patients diagnosed with GBS (n=29), CIDP (n=10), MS (n=23), NMOSD (n=5) or MG (n=16) who received one or more TPE cycles at Leipzig University Hospital between 2018 and 2023. A total of 527 exchanges were performed in 103 exchange cycles. Patients were exchanged with albumin alone (20% of cycles) or albumin/plasma (80%).The median delay from hospital admission to the first TPE session was one day; the median duration of inpatient treatment was 10 days. In contrast, a median of 12 days elapsed until results of antibody testing were available. The majority of patient had "symptom improvement" in GBS, MS, NMOSD and MG. In contrast, the majority of patients with CIDP had "unchanged symptoms". Over all diagnostic groups, cranial nerve dysfunction improved in 77% and motor weakness in 61% of patients. In contrast, sensory loss improved in 23%, and combined ataxia and areflexia improved in 34% of affected patients. Only a small proportion of patients suffered symptom worsening during TPE treatment (1% to 5%). In 62 of 527 exchanges (11.8%), side effects occurred, consisting of citrate reactions (n=25), circulatory dysregulation (n=12), sensory disturbances (n=11), nausea and vomiting (n=7) and allergic reactions (n=7). None fulfilled GCP criteria for severe adverse event (SAE).In most autoimmune neurological disorders, TPE is considered as a second line immunotherapy, i.e. as an escalation in case glucocorticoids have failed. Given the observed risk-benefit profile in our cohort, TPE might be taken into account earlier in the treatment of autoimmune neurological disorders.Es wurde kein Interessenkonflikt erklärt.

Dr. med.

Univ. Prof. Dr.

Therapeutic plasma exchange of neuroimmune disorders

Therapeutischer Plasmaaustausch bei neuroimmunologischen Erkrankungen

We present a single-center experience of therapeutic plasma exchange (TPE) for various neuroimmune disorders. TPE is an established option for therapy-refractory multiple sclerosis (MS), myasthenia gravis (MG), and neuromyelitis optica spectrum disorders (NMOSD). Patients present with symptoms ranging from minor visual impairment and muscle weakness to blindness and complete paralysis. Although currently available therapies are extensive, chronic symptoms remain a major burden.We performed a systematic retrospective data analysis of patients with neuroimmune disorders who received TPE between January 2017 and April 2024 at our department. Our study includes 33 patients (MS n=21, MG n=6, NMOSD n=6) who were admitted for TPE due to unresponsive major flare to current medication and/or high dose corticosteroid therapy. Medical history, clinical symptoms, relevant past and current medication, number of TPE sessions, and clinical outcome parameters have been analyzed to assess the significance of TPE as an escalation therapy at a major academic center.The time between diagnosis and first TPE was 3.5 (mean; ±1.7 SD) years. The most common symptoms were vision impairment (85 %), paralysis (61 %), dysesthesia (56 %) and pain (28 %). The average number of flare ups per year was 0.8 (±1.6 SD). The average number of TPE sessions per year and patient was 2.3 (±4.1 SD), respectively. Forty-eight percent of patients have received two or more approved medications besides corticosteroids before TPE. Seventy-two percent of patients achieved an alleviation of symptoms after TPE. Regarding the current clinical status, sixty-one percent of patients can be classified with a stable disease, whereas thirty percent are still facing aggravating symptoms unresponsive to current therapy.We observed that a significant proportion of patients referred to TPE is clinically progressive and may benefit from therapy escalation and TPE maintenance. A novel approach is anti-CD19 chimeric antigen receptor (CAR) T-cell therapies, which is currently examined in clinical trials for treatment of autoimmune disorders, including patients with chronic neuroimmune diseases. The results of these studies are eagerly awaited.I declare that I have no potential conflicts of interest to disclose that relate to the research described.

Therapeutic leukocytapheresis is more efficient in patients with AML compared to CML – a single center experience

Die Therapeutische Leukozytapherese ist effektiver in Patienten mit AML als in Patienten mit CML – die Single-Center Erfahrung

Therapeutic leukocytapheresis is used to rapidly decrease excessive white blood cell (WBC) counts in patients with acute or chronic leukemia and leukostasis syndrome. This study aims to compare the effectiveness of leukocytapheresis among different patient groups and also evaluates the role of hydroxyethyl starch (HES) in apheresis efficiency. Specifically, our research focuses on comparison between myeloid and lymphoblastic diseases.We retrospectively analyzed first leukocytapheresis procedures in 58 consecutive patients, performed between January 2016 and April 2024. Patients were diagnosed with acute myeloid (AML, n=38), chronic myeloid (CML, n=10), acute lymphoblastic (ALL, n=6), chronic myelomonocytic (CMML, n=1), chronic lymphocytic (CLL, n=1), and T prolymphocytic leukemia (T-PLL, n=2). We assessed the median WBC reduction rate and analyzed the influence of HES as sedimentation aid on the depletion efficiency (Table 1 and 2).Statistical significance was determined using the Mann-Whitney U test, with a threshold p-value ofLeukocyte depletion was tolerated well in all patients. Comparing myeloid versus lymphoblastic leukemia patients, no significant difference was found in median WBC precounts (192 vs. 308.5; p=0.17) and WBC reduction rates (49.2 vs. 47.7%; p=0.89). However, patients with AML showed higher WBC reduction rates compared to CML (54.4 vs. 30.3%, p=0.002) and CML patients had higher WBC precounts (459 vs. 175; p&lt;0.001). Patients with multiple leukocytaphereses had higher WBC precounts (276.1 vs. 163.3; p&lt;0.001), and the use of HES (45% of CML and 18% of AML cases) had a significant effect on the WBC reduction in AML but not in CML patients. However, HES did not influence the need for a second leukocytapheresis, irrespective of disease entity.Our study highlights the efficacy of therapeutic leukocytapheresis in managing hematological disorders, demonstrating a better WBC reduction in AML patients compared to CML patients. AML patients also benefited more from the use of HES than CML patients. Cases with multiple leukocytaphereses had higher WBC precounts, emphasizing the need for repeated interventions in excessive hyperleukocytosis.None.

Professor

Prof.

PD Dr.

Serum Eye drops – survey on manufacturing

Umfrage zur Herstellung von Serum-Augentropfen

Serum eye drops (SED) are an experimental therapy for severe forms of dry eyes, increasingly in use worldwide. SED are only mentioned briefly in the Cross-Sectional Guidelines for Therapy with Blood Components and Plasma Derivatives (&quot;Querschnitts-Leitlinien&quot;). Until now standards for manufacturing have not been defined by German Guidelines, &quot;Richtlinien[RS1] Hämotherapie&quot;. In the context of European regulations (SOHO[RS2] ) this will be required.A small pilot survey was conducted to evaluate manufacturing of SED in Germany in two steps, informal interviews and a formal pilot questionnaire. Data of donation, manufacturing details and side effects observed were evaluated according to this standardized questionnaire.In different regions of Germany regulatory requirements differed. In contrast to previous surveys serum eye drops [RS1] were not manufactured by departments of ophthalmology but increasingly by or in collaboration with departments of transfusion medicine / blood centers. The majority of manufacturers offer autologous serum eye drops [RS2] only. Few departments also provide allogenous serum eye drops[RS3] . A closed system for single day use, ophthiols, is mostly used for packaging. One center manufactures eye drops in sterile bottles filled in clean room conditions. In the formal pilot survey 6 centers reported results on over 8.500 donations in up to 12 years. Sterile controls are performed from each preparation. In comparison to other methods for manufacturing blood products a larger variability between the manufacturers seems to be the case. No serious adverse event related to the product was observed. In two patients[RS4] , bacterial contaminations during SED use were reported: one associated with longer use than prescribed (&gt;&gt; 24h), one with contamination of one single day use ophthiol with 2 eyelash hairs. In both cases the other single day use ophthiols were tested sterile.In this large cohort of patients, safety of SED production in more than 10 years could be shown. A detailed survey on manufacturing of serum eye drops [RS1] in a larger group of manufacturers is proposed. This will provide additional support for setting scientific standards for manufacturing, which should then become part of the national guidelines in the context of the European SoHO regulations.Kein Interessenskonflikt

Temperature at the end of the line: How warm are cold erythrocytes really transfused?

Die Temperatur am Ende des Schlauchs: Wie warm werden kalte Erythrozyten wirklich transfundiert

Perceptions when to mandate warmed transfusions in patients with cold antibodies vary widely. This is influenced by personal experience and sometimes plainly by the availability of technical heating devices. But with what temperature (T) does the blood really arrive at the patient"s arm? Finding no fitting publication of actual measurements in our quick literature search, we devised our own experimental setup.Transfusion was simulated with expired red cell units, stored at T of 4-5°C and transfusion sets (Codan Med. Geräte GmbH, Ref 45.4424, 175 cm). T was recorded every minute in the blood bag (TESTO 110) and with a T probe (TESTO 440dP) using a hemodialysis Y-single needle adapter ((VMP/Effeemme Y-112), placed at the line"s end. A scale (Sartorius LC 12001P) helped to monitor flow speed, which was difficult to set. We measured T after resting 0-30" at RT 22-23°C and various "flow" timings (30-60").Flow speed and time strongly influenced exit temperature. Directly from storage with flow time of 30" (average: 28,6", SD 2,5, min.: 24" max.: 31"), we had an average T(core) 14,6°C, min. 6,9°C, max 22,6°C and av. T(flow): 18,1°C, SD 1,78, min. 16,1°C max. 21,5°C. With 15" rest before 30" flow (av. 27,8" SD 2,23): Tcore 13,4°C min 8,2°C max 21,4°C Tline 18,1°C SD 1,13, min 16,1°C max 21,5°C. With 30" rest and 30" flow (av. 29" SD 0,82), Tcore 15,72°C, SD 2,51, min 12,6°C max 21,9°C. no rest and 60" flow (61,4" SD 7,5) Tcore 14,6°C min 6,9°C max. 22,6°C Tflow 21,6°C SD 0,9, min 18,7°C max 22,9°C. With rest 30" flow 60" (av. flow 54" SD 1,0): Tcore 16,7°C SD 2,9, min 12,5°C, max 22,6°C and Tflow 21,3°C SD 0,7, min 19,9°C max 22,7°C.Blood bags were emptied via normal transfusion sets and T were recorded in standardized conditions. Directly from the refrigerator at the exit of the line the blood reached nearly room temperature when flowing slowly. This data helps avoiding unnecessary use of blood heating devices and may lower concerns regarding transfusion reactions.Additionally, one practical hint resulted: It is better to transfuse slowly than waiting for the blood bag to acclimatize to room temperature.No conflict of interests.

Single needle access for automated red cell exchange in patients with sickle cell disease (SCD)

Single-needle-Verfahren für therapeutische Erythrozytapherese bei Patienten mit Sichelzellkrankheit

Automated red blood cell exchange (aRBCX) is a guideline-based treatment for patients with SCD after severe events (e.g. stroke), but often impeded by difficult venous access (VA). In July 2022, a single needle protocol (SN) for aRBCX became available for Spectra Optia (Fa. Terumo BCT). Using intermittent flow technique, portions of ca. 30 ml blood are sequentially drawn and returned until the procedure is complete. We present our experience with this protocol in two patients with SCD.To date, patient 1 and 2 were treated with SN-aRBCX 12 and 10 times respectively. Both patients received a high-flow single lumen TidalPortTM with 9,6F catheter (Fa. Norfolk Medical). A 18G non-coring port needle (Fa. PakuMed) is used for VA.Prior to treatment, the ports routinely are declotted with Alteplase (Actilyse CathfloR 2mg). Spectra Optia is fitted with the RBCX set and the SN adapter and primed with saline and citrate according to the manufacturs instructions. The procedure usually starts with a depletion phase of up to 3% hematocrit with saline substitution, then switches to exchange mode with substitution of packed red cell concentrates (pRBC).Venous access for treatment with double needle aRBCX (DN-aRBCX) for patient 1 was usually difficult, painful and often resulted in vasovagal reactions. Patient 2 was not eligible for DN-aRBCX because of very poor veins. Since treatment start with SN-aRBCX, venous access via port needle only has been easy and mostly pain free for both patients. Mean maximum flow rate (frmax) for patient 1 increased from 39ml/min (DN-aRBCX) to 69ml/min (SN-aRBCX), enabling higher exchange volumes/time and leading to reduced mean FRC from 32% to 24%. Mean exchange time for SN-aRBCX is 154 min. Mean frmax for patient 2 ist 56ml/min with a mean exchange time of 156 min. Because of three-weekly exchange intervals, the resulting mean FCR of 40% is sufficient to keep HbS below 30%.The SN-procedure is a game changer for the aRBCX-treatment of patients with SCD, especially when combined with a high flow port. It is well tolerated, facilitates a sufficient exchange volume in a reasonable exchange time and alleviates the problem of painful and often insufficient venous access.There is no conflict of interest.

Patient blood management: desire and reality

Patient Blood Managment: Wunsch und Realität

Major surgery in patients refusing blood transfusion poses a maximum challenge for Patient Blood Management (PBM). At the same time kinetics of erythropoietic regeneration as influenced by therapeutic measures can be studied without parameters being changed by transfusion. We have analysed the anemia development and erythropoetic regeneration in a Jehova"s witness requiring urgent surgery.A 77yrs old male was admitted with respiratory decompensation due to tracheal compression (giant struma nodosa) requiring tracheotomy and hypertensive pulmonary edema. In need of clinical stabilisation thyreoidectomy was postponed. The course was complicated by pneumonia. Anemia management was implemented only stepwise: hemoglobin (Hb) monitoring; diagnostic anemia workup; substitution of documented deficits and (iv) erythropoetin stimulation with i.v. iron support. Routine methods were applied for clinical chemistry, iron parameters, folate, vitamin B12 and erythropoetin. Erythrocyte parameters were assessed with an Sysmex XN-9000. Due to comorbidities a Hb-concentration of ≥8g/dl was considered desirable.At admission renal failure was manifest; Hb-concentration (Hb-c) was 13,2g/dl [13,5-16,9] dropping at day+8 to 9,2 and 7,5 after surgery. At this time reticulocytopenia (38/nl) was manifest. Folate deficiency was objectivated (2,2ng/ml [3,9-26,9]) and substitution implemented. Reticulocytes only slightly increased (73/nl). The Hb-c was unstable (fluctuating fluid balance). Epoetin alfa (Erypo®FS 100IU/kg b.w. 3 times weekly) together with Iron-sucrose (FerMed®) were started. Within 5 days, reticulocyte numbers increased. The Hb-c dropped further to 7,1g/dl and then started to rise (7,9 at day +11 after start epoetin; 9,7 day+16; 13,0 day+27) while reticulocytosis was operative (124/nl day+5, 213 day+16, 287 day+27). Our case illustrates the dynamic of anemia development with delayed erythropoiesis support, as well as the kinetics of regeneration upon adequate utilisation of therapeutic tools. Reticulocyte counts are most valuable for estimating how anemia develops. In face of upcoming surgery erythropoiesis support has to be established forward-looking to the expactable development or aggravation of anemia. Therapeutic pragmatism is preferable to diagnostically based step-by-step decision making.The authors declare, that there are no conflicts of interest in relation to this work.

Patient Blood Management: Desire and Reality

Transfusing physician: "He must have the necessary knowledge and sufficient experience" (RiLi Hämotherapie, 2023) – training needs in hemotherapy

Transfundierender Arzt: &quot;Er muss die dafür erforderlichen Kenntnisse und ausreichende Erfahrung besitzen&quot; (RiLi HAT 2023) – Schulungsbedarf in der Hämotherapie

Every physician who uses blood products must have the necessary knowledge and sufficient experience and must have been instructed by a transfusion officer in the facility-specific processes and organizational structures in a documented manner. The instructions are to be updated in annual training courses under the responsibility of the transfusion officer. Knowledge of blood transfusion also applies to nurses if they support (delegation takers) the physician during a blood transfusion.It is examined and shown which regulations on the subject of instruction, training, and knowledge refresher in hemotherapy come into play. The study focuses also on job-specific aspects such as authorization and permission. The calculated scope and effort of the required training measures are quantified, checked for practicability, and critically commented on. A clear division of tasks and delimitation of powers as well as the definition of measures, including documentation, appear to be particularly important.The uniform instruction of all physicians (old stock, new entrants, etc.) is generally a difficult but feasible undertaking that requires a high degree of discipline, good organization, smooth cooperation with the Department of Human Resources Development, room management, and personnel administration, as well as with the lecturers. The results are different, but hardly flawless in hemotherapeutic practice. Improvement in practice and documentation is possible.Teaching following regulations in various healthcare facilities is complex and time-consuming, a permanently challenging task, and requires well thought out organization. Therefore, new and promising solutions, such as the use of digital and online education and training measures, are available to ensure legally compliant and up-to-date knowledge as well as practical experience among staff and to maintain them in the long term.Der Autor erklärt, keine Interessenkonflikte zu bestehen.

Posterausstellung 8
Hemotherapy and Patient Blood Management

Termin

Programm

Posterausstellung 8Hemotherapy and Patient Blood Management

Termin

Programm

Posterausstellung 8
Hemotherapy and Patient Blood Management