Transfusional iron overload in heavily transfused patients: Real-life data from a 10-year retrospective study of 611 cases managed in a French general hospital

ObjectivesEpidemiological studies on transfusional iron overload (TIO) in the general population of heavily transfused patients are scarce. The aim of this work was to provide a picture on the distribution and management of this complication within the context of unselected individuals attending a general hospital.MethodsWe retrospectively assessed the characteristics of 611 patients from a single institution having received at least 20 red blood cell (RBC) units over a 10-year period.ResultsAbout two-thirds of these individuals were males and their median age at the 20th RBC was 72 years (range: 10–98). Myelodysplastic syndromes (MDS) and acute myeloid leukemia represented the most frequent underlying conditions (53%) but lymphoid malignancies and solid malignancies accounted for 13.6 and 7.3% respectively. In the vast majority of cases various comorbidities (range: 1–6 per patient) were registered including especially cardiovascular disorders. The highest cumulative RBC numbers were observed in MDS patients. Serum ferritin was assessed in 451 patients (73.8%) and ≥ 1000 μg/L in 250 cases, ≥ 2000 μg/L in 100 cases and ≥ 2500 μg/L in 71 cases. Only 97 patients (15.9%) received a treatment for TIO using either a chelator (n = 93) or phlebotomy (n = 4).ConclusionTIO is not limited to MDS or hemoglobin disorders. Its assessment and management are suboptimal in clinical practice. The ratio of patients receiving iron chelation is markedly lower than theoretically expected mainly because of comorbidities or drug intolerance.     

Warm red blood cell autoantibodies and clinical diagnoses in patients with or without autoimmune hemolysis

ObjectivesRed blood cell autoantibodies (RBC autoAbs) of IgG class are found in the majority of patients with warm autoimmune hemolytic anemia (wAIHA) but sometimes also during the pretransfusion testing of patients with different diagnoses but without hemolysis. The aim of the study was to identify the main differences between these two groups of patients according to age, gender, subclass and titer of IgG RBC autoAbs and diagnosis.Material and methodsIn the 9-year retrospective study, data were collected from records of 291 patients with IgG RBC autoAbs detected by gel technique, from which 111 with wAIHA.ResultsMore than 85% of patients in both groups were over 40 years old, with male to female ratio 1:1.9 in wAIHA vs 1:1.3 in patients without hemolysis (P = 0.0916). The main characteristics of patients with wAIHA vs patients without hemolysis were: IgG only 38% vs 70%, IgG + Complement 62% vs 30%, total IgG1 79% vs 55%, IgG1 + IgG3 35% vs 11%, titer of 100 for IgG1 + IgG3 17% vs 3% (P < 0.0001), respectively, while titer of 100 for IgG1 18% vs 9% (P = 0.0241). The underlying diagnosis in wAIHA vs patients without hemolysis: hematologic disorders 41% vs 22% (P = 0.0006), autoimmune disorders 12% vs 13% (P = 0.8033), solid tumors 5% vs 14% (P = 0.0154) and surgery procedures 6% vs 26% (P < 0.0001).ConclusionWe observed more wAIHA patients with high titer of IgG1 and high prevalence of IgG1 + IgG3 and consider that patients without hemolysis having identical results might be interesting to find out how they are protected from damage by RBC autoAbs.     

Transfusions érythrocytaires au cours des syndromes myélodysplasiques (SMD)

PRBC transfusion remains the mainstay of treatment of anemia in MDS after failure of erythropoiesis stimulating agents. The most common transfusion trigger in transfusion-dependent MDS patients is 80 g/L. This level is based only on expert consensus; a randomized controlled trial comparing restrictive against liberal policy is required to evaluate potential impact of transfusion policy on with QoL and survival. Prophylactic antigen matching for RhCE and K must be used in order to reduce the risk of red blood cell (RBC) alloimmunization. Transfusion associated circulatory overload (TACO) is the first fatal complication of transfusion in MDS patients. Prevention, in this high risk group (older people with cardiac comorbidities) requires slow transfusion rates and rigorous monitoring of systolic blood pressure. Long-term transfusion in low risk MDS patients could also induce iron overload complications that could be prevent by iron chelating agents. These latter are usually not very well tolerated, however, a new formulation of Deferasirox^® seems to be able to improve patient satisfaction.     

L’âge des culots globulaires transfusés influence-t-il toujours le pronostic des patients en réanimation ?

ObjectiveFew studies have shown that aged packed red blood cells (RBC) transfusion negatively influenced the outcome of ICU patients, probably related to storage lesions which could be decreased by leukodepletion of RBC. The purpose of this study was to evaluate the impact of aged leukodepleted-RBC pack, on the outcome of ICU patients.DesignRetrospective, observational, cohort study in a Medical Intensive Care Unit.PatientsConsecutive patients admitted during the years 2005 and 2006, and requiring a transfusion. We recorded patient's demographic data, number of RBC unit and age of each RBC, length of ICU, mortality during ICU stay.ResultsFive hundred and thirty-four patients were included with global mortality was 26.6%, length of stay in ICU six days (3–14) and SAPS II 48 (35–62). RBC equaling to 5.9 were transfused per patients (22.7% < 14 days and 57.3% < 21 days). The number of RBC was significantly higher in the dead patients group, but the rate of RBC stored less than 21 days was not different (54% versus 60%; p = 0.21). In a multivariate logistic model, independent predictors of ICU death were SAPS II (OR = 1.02 per point, p < 0.001), number of RBC (OR = 1.08 per RBC, p < 0.001), length of stay in ICU (p < 0.001). Similar results were obtained while introducing the age of RBC as time dependent covariates in a multivariate Cox's model.ConclusionsRBC transfused in our ICU are old. The ICU outcome is independently associated with the number of leucodepleted RBC transfused, but not with their age.     

What is the optimal pretransfusion testing interval for multi-transfused patients? The University Hospital of Brest experience

The red cell allo-antibodies research is mandatory before transfusion. In France, pretransfusion testing intervals that are prescribed by regulatory and accrediting agencies are commonly 72 hours. In the University hospital of Brest, the interval for multi-transfused patients has been 24 hours. In this study we aim to analyse these practice and argue the delay.MethodsThis is a retrospective study of post-transfusional allo-immunizations from 2015 to 2020. For each patient, the time interval between the last negative research and the allo-immunization was investigated.Results189 patients developed allo-antibodies. In 16 patients (8,5%), the interval for allo-immunization was 24 hours, 48 hours and 72 hours in 4, 8 and 4 patients respectively. 12 patients were transfused after the discovery of the allo-antibodies. That means if we have chosen a delay of validity of 72 hours, then 9 patients would have been transfused with a negative result.ConclusionChecking for allo-antibodies before RBC transfusion with an interval of 24 hours (and not 72 hours) is pertinent in order to assure an optimal transfusion safety and to limit the risk of hemolytic transfusion reactions. A pretransfusion testing interval of 24 hours for multi-transfused patients should be considered.     

Associations of killer cell immunoglobulin-like receptors with acute myeloid leukemia in Chinese populations

Many studies have investigated the relationship between KIR, HLA and acute myeloid leukemia (AML), but the results were different in different laboratories, and the data in Chinese population were limited. In this study, the distribution of KIR gene, KIR genotypes, HLA-C groups, HLA-Bw4, and KIR-HLA interaction from 273 healthy participants and 253 AML patients (M0–M6) in southern Chinese Han were determined to investigate the relationships among KIR, HLA and AML. The results showed that the frequencies of 2DS4del in M5 patients were significantly higher than those of the controls (65.0% vs 46.5%, P = 0.0104, OR = 2.135, P < ɑ′). The frequency of KIR genotype BX13 in the healthy controls was significantly higher than that in AML patients (3.7% vs 0%, P = 0.0019, OR = 20.2, P < ɑ′). No other significant differences in the frequencies of KIR, HLA and KIR-HLA interaction were identified between AML patients and controls. Our study suggests that 2DS4del may conduct a susceptibility to AML, and genotype BX13 might conduct a protective effect on AML.     

Clinical characteristics and outcome of invasive fungal infections in pediatric acute myeloid leukemia patients in a medical center in Taiwan

Background

Invasive fungal infection (IFI) causes significant morbidity and mortality in patients with hematological malignancies, especially those with acute myeloid leukemia (AML), recurrent acute leukemia, high-risk acute lymphoblastic leukemia, and after allogeneic hematopoietic stem cell transplantation. The study aimed to investigate the clinical characteristics and outcome of IFIs in pediatric AML patients in a medical center in Taiwan.

Évaluation de la prévalence de l’hémosidérose post-transfusionnelle, étude SFVTT-01 : résultats préliminaires du groupe de travail de la SFVTT

RationalAlthough notification of post-transfusion hemosiderosis is mandatory since 1994 among the French hemovigilance network, it is so far largely under reported.Patients and methodsWe screened 42,443 patients hospitalized for blood diseases in France in 2009 and 2010 and determined which patients had received more than 20 PRC. Among them, we selected those having at least one measure of serum ferritin, and subsequently those which ferritin was greater than or equal to 1000 ng/mL.ResultsThree thousand eight hundred and twelve patients (9%) received more than 20 PRC, 1935 (4.5%) had a ferritin assay, which was increased in 1216 patients (2.9%). Eight hundred and eighty-one patients underwent an hemovigilance report form. Forty-nine percent had low-risk myelodysplasia or acute leukemia, 7% hemoglobinopathies. Hemosiderosis was asymptomatic for 680 patients (77%), serious 188 (88%) and life-threatening for 11 (1%). Two patients died of terminal heart failure. The most severe hemosiderosis (≥ grade 2) were low-risk myelodysplasia and idiopathic aplastic anemia. Ninety-two percent of thalassemia patients and 46% of sickle cell anemia patients received an iron chelator. For low-risk myelodysplastic syndromes and idiopathic aplastic anemia, 228 of the 317 patients whose treatment is known and who could benefit from iron chelation (72%) have not received it.ConclusionThese results encourage seeking optimal transmission of information (over 20 CGR) to the clinician, and prolonging hemovigilance action towards a more comprehensive statement of post-transfusion hemochromatosis.     

Extracorporeal photochemotherapy in mycosis fungoides

Objectives

Extracorporeal photo-chemotherapy (ECP, photopheresis) is an approved treatment modality for mycosis fungoides (MF). Our aim is to present our ECP data for MF.

Abnormal hemoglobin H band in myelodysplastic syndromes (MDS): A case report

Myelodysplastic syndrome (MDS) is a group of heterogeneous diseases derived from hematopoietic stem cells characterized by hemolytic anemia and high risk of conversion to acute leukemia. MDS is an age-related disease in which approximately 80% of patients are over 60 years of age, male and female. Anemia is the most common clinical condition, and many patients are also associated with infection and bleeding. When the amount of α globin synthesis is insufficient, the remaining β chain forms tetramer β4, i.e. HbH. The latter forms a precipitate in red blood cells, leading to hemolytic anemia, called HbH disease, the majority of which is congenital, a small number of patients with myelodysplastic syndrome and acute myeloid leukemia may appear HbH (called acquired HbH disease). We reported a 71 years old male patient diagnosed as myelodysplastic syndromes (MDS) in our hospital. The patient has a negative α-thalassemia gene test. The H band is detected by hemoglobin electrophoresis. This article analyzed and discussed this case with MDS, as well reviewed MDS.     

Compliance with the European trauma guidelines: An observational single centre study

Objectives

The European trauma guidelines were developed to assist clinicians in the early phase of trauma management to diagnose and treat coagulopathy and bleeding. This study aimed to determine compliance with these European trauma guidelines in a French referral trauma centre.

Profil clinico-biologique et immunohématologique des patients atteints de β-thalassémie en Tunisie : à propos de 26 cas

Aim of the studyTo study the clinical and biological profile of β-thalassemic patients in our region, reflecting the quality of their care.Patients and methodsA retrospective study (2010–2011) on 26 β-thalassemic patients followed in the pediatrics service at CHU Farhat Hached Sousse, Tunisia. Epidemiological, clinical and biological data were collected from medical records and transfusion files of patients. The transfusion protocol adopted was to maintain a hemoglobin level > 10 g/dL by regular transfusions every 3–4 weeks. Iron chelation therapy, in order to maintain serum ferritin < 1500 ng/mL, was introduced when serum ferritin exceeded 800–1000 ng/mL.ResultsThe mean age of patients at diagnosis was 15 months. The clinical impact of anemia had resulted in failure to thrive in 54% of patients and facial dysmorphism in 23%. The average transfusion requirement was estimated at 311.02 mL/kg/year with 6 cases of hyperconsumption. The immunohaematological monitoring showed the appearance of anti-RBC alloimmunization in one patient and 4 cases of autoimmunization. Poor adherence of chelation therapy was 62% and causing 5 cases of cardiac complications, 4 cases of liver injury and 14 cases of endocrine complications.ConclusionImproving the therapeutic care of β-thalassemic children requires better monitoring of transfusion recovery and improved adherence to chelation therapy.     

Effects of ABO incompatibility in allogeneic hematopoietic stem cell transplantation

IntroductionThe impact of ABO mismatch on outcomes following allo-HSCT remains controversial. In this study, our aim is to define the effect of ABO mismatch on post-transplant outcomes, engraftment kinetics and complications in a large cohort.Patients and methodsWe retrospectively identified 1000 patients who underwent allo-HSCT from either bone marrow or peripheral blood stem cells at our center between 1988 and 2016. P < 0.05 was considered statistically significant.ResultsFive hundred and ninety (59%) patient-donor pairs were ABO matched, 164 (16.4%) were ABO major mismatched (MM), 191 (19.1%) were ABO minor MM, and 55 (5.5%) were ABO bi-directionally MM. ABO matched pairs were more common in transplants from related donors (P < 0.001) and using bone marrow as a stem cell source (P < 0.001). In minor ABO MM transplantations, mild delayed hemolytic reaction occurred more frequently compared to major and bidirectional ABO MM transplantations (47% vs 35% and 18%, P < 0.001). Neutrophil engraftment was slightly delayed in ABO MM patient-donor pairs when compared ABO matched donor pairs according to median engraftment time in all group (167/410, 41% vs 204/590, 35%, P = 0.046). Pure red cell aplasia was diagnosed in 6 patients (1%). Higher risk of death was shown in ABO MM transplants compared to ABO matched transplants in overall survival (OS) analysis (HR:1.201, 95% CI:1.004–1.437, P = 0.045). The non-relapse mortality (P = 0.546) and cumulative incidences of acute graft versus host disease (aGVHD) and chronic (c) GVHD were comparable between ABO MM and ABO matched patient-donor pairs (for aGVHD, P = 0.235; for cGVHD, P = 0.137).ConclusionABO MM transplants were associated with decreased OS and slightly delayed neutrophil engraftment. NRM and the risk of GVHD were not related to ABO incompatibility.     

Preleukemic phase of chronic myelogenous leukemia: morphologic and immunohistochemical characterization of 7 cases

Patients with chronic myelogenous leukemia (CML) present typically with an elevated white blood cell count (WBC) and cytogenetic or molecular genetic evidence of t(9;22)/BCR-ABL1 fusion gene. Rarely, CML patients may present with a normal or mildly elevated WBC and are asymptomatic, and we describe 7 patients in this study. The WBC in these patients ranged from 3.6 to 14.3 K/mm³ with 50% to 73% granulocytes and 0% blasts. In all patients, t(9;22)(q34;q11.2) was detected by conventional cytogenetics, and BCR-ABL1 fusion was shown, supporting the diagnosis of preleukemic CML (pre-CML). We compared these patients with a group of 5 cases of CML in chronic phase (CML-CP) and 5 bone marrow specimens with a leukemoid reaction (n = 5). Reticulin, CD34, and CD61 immunostains were performed on all bone marrow biopsy specimens. Peripheral blood absolute basophilia (≥200/mm³) was noted in only 4 of 7 pre-CML cases, whereas it was present in all CML-CP cases and absent in leukemoid reaction cases. The mean ±SD of microvascular density of pre-CML cases (10.0 ± 4.3 vessels/200× field) was twice that of leukemoid reaction cases (5.0 ± 1.0) (P = .02; Student t test) but similar to that of CML-CP cases (12.5 ± 3.6). Microvessels in pre-CML, highlighted by CD34, were tortuous with abnormal branching, although to a lesser extent than those found in CML-CP. Microvessels in leukemoid reaction were generally straight. The percentage of small, hypolobated megakaryocytes, highlighted by CD61 in pre-CML, was 40%, 3 times that found in leukemoid reaction cases (13%) but less than that of CML-CP cases (86%). We conclude that pre-CML should be suspected in patients with a normal to mildly elevated WBC and absolute basophilia. Bone marrow examination can usually distinguish pre-CML from a leukemoid reaction based on the percentage of small, hypolobated megakaryocytes; microvascular density; and morphologic features.     

La leishmaniose cutanée à l’hôpital militaire Moulay Ismaïl de Meknès (Maroc) : à propos de 49 cas diagnostiqués entre 2005 et 2011

An increase in the number of cases of cutaneous leishmaniasis has been observed, in recent years, in Morocco. We tried, through a series of 49 cases collected from the dermatology department at the military hospital Moulay Ismail of Meknes, to clarify the epidemiological, clinical and evolutionary aspects of the reported cases. We recorded for each patient: age, sex, geographic origin and the period before consultation. We have also noted the number of lesions, their location, and their clinical aspects. Finally, we recorded results of direct examination in search of the parasite, treatment and post treatment evolution. The sex ratio was 11. The average age was 35.6 years. The average period of consultation was 3.8 months. Eighty-six percent of cases (n = 42) were from the region extending from south to south-east of the atlas. The average number of lesions per patient was 4.46 (from 1 to 40). Thirty-seven percent of cases (n = 18) had single lesions and 63% (n = 31) multiple lesions. There was a polymorphism of clinical aspects of lesions with predominant of ulcerative crusting aspects observed in 55.1% of cases (n = 27). Face localization was observed in 20.4% of cases (n = 10). Ninety-two percent of cases (n = 45) had involvement of the upper limbs and/or lower. Direct examination was positive in 65% of cases (n = 32). The treatment was based on meglumine antimoniate associated in three quarters of cases with cryotherapy using liquid nitrogen. The outcome was favorable for all patients. We observed an increase in the number of cases in 2011 and especially those with multiple lesions followed by a decline in 2011.     

Apparition d’anticorps irréguliers anti-érythrocytaires chez les patients transfusés âgés de 80 ans et plus : résultats du suivi par l’hémovigilance sur trois ans

PurposeToday most transfusions are given to people over 70. In order to evaluate the production and the circumstances of the appearance of red blood cells (RBC) allo-antibodies (Ab), a three-year study was performed in transfused patients aged 80 and over.Material and methodsBased on the Adverse Event Reports (AER) on RBC Ab from 2007 to 2009 in the Rhône-Alpes area, the prevalence and specificity of the RBC Ab, the type of blood component involved, the imputability and the previous transfusion and obstetrical history were studied.ResultsOf 2,169 AER, 240 (11.1%) related to the appearance of RBC Ab; they included 150 females (F) patients (62.5%) and 90 males (M) (37.5%). The Rhesus (RH) blood group was most involved (75 AER) and anti-E was the most frequent Ab (52 cases; 69.3%). Packed RBC were predominantly involved (233 cases; 97,1%). Absolute imputability could be established in 120 cases only (50.0%). Previous transfusion history was observed in 85 F patients (56.7%) and 52 M (57.8%). Pretransfusion Ab was noted in 18 F patients (12.0%) and five M (5.6%). Seventy-three F patients (48.7%) had had a pregnancy but the number of unknown data was high (71 F patients; 47.3%).ConclusionIn the transfused patient population aged 80 and over, RBC Ab are common and in most cases are due to RBC transfusions. On the contrary, pretransfusion RBC Ab are not frequent.     

L’association fer intraveineux et acide tranexamique permet-elle de réduire le besoin transfusionnel ? Une étude comparative non randomisée

Objectives

To evaluate if the association of injectable iron and tranexamic acid allows a significant saving in transfusion, in cases of myomectomies and hysterectomies.

The detection,treatment of parvovirus B19 infection induced anemia in solid organ transplants: A case series and literature review of 194 patients

BackgroundThere are no optimal diagnostic, treatment and post-infection surveillance strategies for parvovirus B19 infection in solid organ transplantation (SOT) recipients.MethodsWe conducted a retrospective review of all PVB19 infected cases confirmed by qPCR among SOT recipients at our institution over a 3-year period and reviewed the literature from 1990 to 2021.ResultsEight kidney and two heart transplant patients with refractory anemia had PVB19 infection. The viral DNA load in peripheral blood ranged from 2.62 × 10² to 8.31 × 10⁶ copies/mL. Two patients with the lowest PVB19 DNA load only reduced the use of immunosuppressants and anemia was relieved. Eight received intravenous immunoglobulin (IVIG) (ranging from 0.25 to 0.5 g/kg/day). The median time to anemia improvement (hemoglobulin > 100 g/L) was 16 days (8–70 days) after treatment. One patient had a PVB19 relapse and viral DNA load > 1.00 × 10⁸ copies/mL at diagnosis. A total of 86 studies involving 194 SOTs were screened from the literature, and the most common symptom was anemia and low reticulocyte count. PVB19 DNA was detected in all cases. Of that, 91.4% of cases received IVIG, 53.8% received IVIG and immunosuppression reduction, 6.5% of cases showed reduced immunosuppression without IVIG, and 2.1% did not receive any special treatment. The recurrence rate was 17.5%.ConclusionPVB19 infection is a cause of anemia after SOT, and treatment mainly relies on IVIG and/or immunosuppression reduction.