Hyperhaemolytic transfusion reaction in two β‐thalassaemia major patients: The role of eculizumab

Abstract Background Typically minor ABO incompatible platelet products are transfused without any incident, yet serious hemolytic transfusion reactions occur. To mitigate these events, ABO ‘low titer’ products are used for minor ABO incompatible transfusions. We sought to understand the role of IgG and complement activation by anti-A on extravascular hemolysis. Samples evaluated: i) Group O plasma from a blood donor whose apheresis platelet product resulted in an extravascular transfusion reaction, ii) Group O plasma from 12 healthy donors with matching titers that activated complement (N = 6) or not (N = 6), and iii) Group O sera from 10 patients with anti-A hemolysin activity. Monocytes from healthy donors were co-incubated with anti-A-sensitized fluorescently-labeled Group A1+ RBCs with and without fresh Group A serum, as a source of complement C3, and phagocytosis was analyzed by flow cytometry. The plasma and sera had variable direct agglutinating (IgM) and indirect (IgG) titers. Results None of 12 selected samples showed monocyte-dependent erythrophagocytosis with or without complement activation. The donor sample causing a hemolytic transfusion reaction and 2 of the 10 patient sera with hemolysin activity showed significant erythrophagocytosis (>10%) only when complement C3 was activated. The single donor plasma and two sera demonstrating significant erythrophagocytosis had high IgM (≥128) and IgG titers (>1024). The donor plasma anti-A was IgG1, while the patient sera were an IgG3 and an IgG1 plus IgG2. Conclusion High anti-A IgM/IgG titers act synergistically to cause significant monocyte erythrophagocytosis by activating complement C3, thus engaging both Fcγ- and CR1-receptors.

Download Full-text

Role of non-invasive assessment in prediction of preclinical cardiac affection in multi-transfused thalassaemia major patients

Hematology ◽

10.1179/1607845413y.0000000140 ◽

2013 ◽

Vol 19 (7) ◽

pp. 380-387 ◽

Cited By ~ 4

Author(s):

Khaled M. Said Othman ◽

Shereen A. Elshazly ◽

Nihal M. Heiba

Keyword(s):

Thalassaemia Major ◽

Non Invasive

Download Full-text

The influence of faith and religion and the role of religious and community leaders in prenatal decisions for sickle cell disorders and thalassaemia major

Prenatal Diagnosis ◽

10.1002/pd.1507 ◽

2006 ◽

Vol 26 (9) ◽

pp. 801-809 ◽

Cited By ~ 53

Author(s):

Shenaz Ahmed ◽

Karl Atkin ◽

Jenny Hewison ◽

Josephine Green

Keyword(s):

Sickle Cell ◽

Community Leaders ◽

Thalassaemia Major ◽

Sickle Cell Disorders

Download Full-text

Prevalence of thromboembolic events among 8,860 patients with thalassaemia major and intermedia in the Mediterranean area and Iran

Thrombosis and Haemostasis ◽

10.1160/th06-05-0267 ◽

2006 ◽

Vol 96 (10) ◽

pp. 488-491 ◽

Cited By ~ 171

Author(s):

Ali Taher ◽

Hussain Isma’eel ◽

Ghassan Mehio ◽

Daniela Bignamini ◽

Antonis Kattamis ◽

...

Keyword(s):

Mediterranean Area ◽

Specific Information ◽

Thromboembolic Events ◽

Thalassaemia Major ◽

Tertiary Referral ◽

Transfusion Therapy ◽

Increased Risk ◽

Multiple Choice Questionnaire ◽

The Mediterranean

SummaryBeta-thalassaemia isa congenital haemolytic anaemia characterized by partial (intermedia, TI) or complete (major, TM) deficiency in the production of β-globin chains.The primary aim of this study was to determine the prevalence of thromboembolic events in patients with β-thalassaemia.To achieve this,a multiple-choice questionnaire was sent to 56 tertiary referral centres in eight countries (Lebanon, Italy, Israel, Greece, Egypt, Jordan, Saudi Arabia and Iran), requesting specific information on patients who had experienced a thromboembolic event.The study demonstrated that thromboembolic events occurred ina clinically relevant proportion (1.65%) of 8,860 thalassaemia patients (TI – 24.7% or TM – 75.3%) from the Mediterranean and Iran. Thromboembolism occurred 4.38 times more frequently in TI thanTM (p<0.001), with more venous events occurring inTI and more arterial events occurring in TM.Thrombosis in thalassaemia was also more common in females, splenectomized patients and those with profound anaemia (haemoglobin <9 g/dl). Due to the increased risk of thromboembolic events, the rationale for splenectomy should perhaps be re-assessed and the role of transfusion therapy for the prophylaxis of thrombosis, among other complications, be evaluated prospectively.

Download Full-text

Left ventricular torsional mechanics and myocardial iron load in beta-thalassaemia major: a potential role of titin degradation

BMC Cardiovascular Disorders ◽

10.1186/1471-2261-14-49 ◽

2014 ◽

Vol 14 (1) ◽

Cited By ~ 4

Author(s):

Mei-pian Chen ◽

Shu-na Li ◽

Wendy WM Lam ◽

Yuen-chi Ho ◽

Shau-yin Ha ◽

...

Keyword(s):

Potential Role ◽

Left Ventricular ◽

Myocardial Iron ◽

Thalassaemia Major ◽

Beta Thalassaemia ◽

Iron Load

Download Full-text

Predictive Role of Blood Group, Rh Factor and Gestational Age in Transfusion Reaction in Neonates

Acta Medica Saliniana ◽

10.5457/ams.236.11 ◽

2011 ◽

Vol 40 (1) ◽

Author(s):

Aida Zabic ◽

Fahrija Skokic ◽

Olivera Batić-Mujanović ◽

Sunita Ćustendil-Delić ◽

Azra Žugić ◽

...

Keyword(s):

Blood Group ◽

Gestational Age ◽

Transfusion Reaction ◽

Predictive Role

Download Full-text

Independent role of interleukin interleukin-6 and interleukin-8 in the etiology of transfusion reactions to platelet concentrates in children

Vojnosanitetski pregled ◽

10.2298/vsp160721241s ◽

2018 ◽

Vol 75 (4) ◽

pp. 390-397 ◽

Cited By ~ 1

Author(s):

Olivera Serbic-Nonkovic ◽

Milos Kuzmanovic ◽

Maja Zivotic ◽

Svetlana Zunic ◽

Dragana Jovicic-Gojkov ◽

...

Keyword(s):

Interleukin 6 ◽

Pediatric Population ◽

Buffy Coat ◽

Transfusion Reaction ◽

Platelet Concentrates ◽

Leukocyte Antigen ◽

Platelet Antibodies ◽

Platelet Antigen ◽

Transfusion Reactions

Background/Aim. Transfusion reaction is an adverse event which manifests during or after administration of blood components to the patient. We aimed to show less known aspects of most common transfusion reactions (allergic and febrile non-hemolytic transfusion reactions ? FNHTR) in the pediatric population at the platelet concentrates. The aim of this study was to determine the role of the accumulated cytokines interleukin-6 (IL-6), interlekin- 8 (IL-8) and presence of anti-platelet antibodies in the etiology of transfusion reaction in children. Methods. The study included 239 pediatric patients, who received platelet concentrates. Data of reported transfusion reaction were collected and evaluated prospectively. The levels of IL-6 and IL-8 were determined using an immunoassay. Antihuman leukocyte antigen antibodies (anti-HLA) and antihuman platelet antigen antibodies (anti-HPA) were identified by Luminex flow cytometry. Results. Toral of 70 transfusion reactions were recorded 52 patients. Allergic reactions occurred in most of the cases (74.3%), followed by FNHTR (17.1%). Platelets derived from buffy coat caused the majority of reactions (73.5%). Patients with infection after platelet transfusion with FNHTR had the highest levels of IL-6, 483.30 ? 1,041.79 pg/mL (p = 0.020). Respectively, the febrile patients had IL-6, 302.52 ? 720.04 pg/mL (p = 0.004). The level of IL-8 in platelet units that caused transfusion reactions was 95.66 ? 319.10 pg/mL, which was significantly higher (p = 0.001) compared to the control platelet units. Conclusion. The predominant etiologic mechanism for FNHTR in our study was leukocyte derived cytokine accumulation during storage. Etiopathogenesis of FNHTR induced by IL-6 and IL- 8 presented differently. We concluded that significant factors in the etiology of FNHTR by IL-6 were the factors related to the pediatric patient (infection, inflammation).

Download Full-text