Long-term red blood cell exchange in children with sickle cell disease: Manual or automatic?

Little information is available on erythrocytapheresis in children with sickle cell disease, and no comparison has ever been made with manual exchanges in a long-term blood exchange program. We matched a historical cohort of five patients who received 60 erythrocytapheresis procedures with five who received 124 manual exchanges. Long-term erythrocytapheresis was feasible and well-tolerated even in children of low weight. In a long-term approach, automated exchanges were more efficient in maintaining a low HbS level, and exchanges could be spaced out. This approach appears especially useful in the cases where the HbS level must be maintained below 30%.     

Red blood cells from donors with sickle cell trait: a safety issue for transfusion?

Sickle cell trait (SCT) affects approximately 8% of the population in Martinique (FWI) and about the same rate is found among the African Americans. In several regions of tropical Africa, up to 40% of individuals are also affected. SCT has been characterized as a benign condition and patients are currently asymptomatic or run a benign course. However, life-threatening complications may occur. SCT can be detected in patients hospitalized for various complaints, and SCT likely causes serious morbidity and mortality in some people. Moreover, these clinical observations have been supported by several in vitro studies in which haemoglobin AS red cells showed abnormalities of their filterability. These problems are revisited with the implementation of universal leucoreduction in several countries. The question of the screening of all blood donors for SCT and the use of their red blood cells (RBCs) has yet to be resolved.     

Plasma exchange—A useful adjunct therapy to red cell exchange in patients with sickle cell disease and multiorgan dysfunction

Background

Urgent red cell exchange (RBCx) is indicated for many complications of sickle cell disease (SCD), including acute chest syndrome, stroke, and hepatic/splenic sequestration. Many who receive RBCx remain hospitalized and develop further complications, including multiple organ dysfunction syndrome (MODS), a leading cause of death in intensive care units. Therapeutic plasma exchange (TPE) has been advocated as an effective treatment of MODS, but its role in SCD compared with RBCx alone is not well studied.

Methods

We identified all ICU encounters from 2013 to 2019 involving RBCx procedures for MODS or SCD crisis that progressed to MODS, a total of 12 encounters. Data regarding hospital length of stay (LOS), survival, number of TPE procedures following RBCx, and procedure characteristics were collected. Surrogate laboratory markers of end-organ damage and disease severity scores were recorded at the time of admission, post-RBCx, post-TPE, and at discharge.

Results

Eight encounters involved RBCx followed by TPE (TPE group) while four involved RBCx alone (RBCx group). The TPE group had a higher SOFA score at ICU admission (9.5 vs. 7.0), greater predicted mortality, and a statistical trend toward higher disease severity scores following RBCx relative to the RBCx group (p = 0.10). The TPE group showed a significantly greater decrease in SOFA score between RBCx and discharge (p = 0.04). No significant difference in mortality or hospital LOS was observed between the groups.

Conclusion

The findings suggest TPE may be considered as an adjunct treatment for patients with acute complications of SCD that progress to MODS, especially in cases where there is no significant improvement following RBCx.     

Guillain-Barré syndrome in a patient previously diagnosed with COVID-19

As the COVID-19 pandemic continues to progress, the medical community is rapidly trying to identify complications and patterns of disease to improve patient outcomes. In a recent systematic review, it has been reported that isolated cases of Guillain-Barre Syndrome (GBS) have occurred secondary to COVID-19 infection. GBS is defined as a rare, but potentially fatal, immune mediated disease of peripheral nerves and nerve roots that is usually triggered by infections. The incidence of GBS can therefore increase during outbreaks of infectious diseases, as was seen during the Zika virus epidemics in 2013 in French Polynesia and 2015 in Latin America. While several cases of GBS secondary to COVID-19 infection have been reported in Italy, only one case has been reported in the United States (US). The reported case in the US was a 54- year old male. We present a case of GBS secondary to a COVID-19 infection and believe this to be the first documented female case in the US and the second documented case in the US overall. The presented case aims to supplement the existing body of knowledge and to assist clinicians in managing complications of COVID-19.     

Are there phases to the vaso-occlusive painful episode in sickle cell disease?

The purpose of this study was to describe the pain experience of children with sickle cell disease who were hospitalized for vaso-occlusive painful episodes. The pain experience, and signs and symptoms prior to admission and during hospitalization, are presented in the context of whether there is evidence to support the existence of phases to a vaso-occlusive painful episode. Children were interviewed about the onset of the painful episode and were asked to describe their pain from the day of admission to the day of discharge from the hospital. They were also observed for the absence or presence of signs and symptoms associated with the painful vaso-occlusive episode. Findings from this study provide some evidence to support previous observations related to changes during the evolution of painful episodes that may be occurring in phases (e.g., evolving, inflammatory, resolving), as previously described in adults and children. These phases had different names, although the concepts were similar.     

Aortic valve replacement in a patient with sickle cell disease—Are we justified to perform surgery in the TAVI era?

Not all variants of SCD need the same management and this should be decided on a case‐by‐case basis. Heterozygous SCD patients can undergo cardiac surgery without the need for intraoperative exchange transfusions with good clinical outcomes.     

Red blood cell transfusion in non-bleeding critically ill patients with moderate anemia: is there a benefit?

Purpose

This study was undertaken to investigate the efficacy of red blood cell transfusion (RBCT) at reversing the deleterious effects of moderate anemia in critically ill, non-bleeding patients.

Methods

This was a retrospective, pair-matched (ratio 1:1) cohort study. Non-bleeding critically ill patients with moderate anemia (nadir hemoglobin level between 70 and 95 g/l), admitted to the ICU over a 27-month period, were included. Anemic patients were included upon meeting five matching criteria of having the same nadir hemoglobin (±5 g/l), APACHE II score (±5), SOFA score (±2), admission diagnostic group, and age (±5 years). Outcome events occurring over the whole ICU stay and after RBCT were collected. After hospital discharge, all patients had a 2-year follow-up period.

Results

Two hundred fourteen non-transfused anemic patients (NTAPs) were successfully matched with 214 transfused anemic patients (TAPs). In addition to the matching criteria, at baseline, both groups were homogenous with respect to multiple comorbidities. Compared with TAPs, NTAPs showed significantly lower rates of hospital mortality (21 vs.13 %, respectively; p < 0.05) and ICU re-admission (7.4 vs. 1.9 %, respectively; p < 0.05). Additionally, NTAPs had significantly lower rates of nosocomial infection (12.9 vs. 6.7 %, respectively; p < 0.05) and acute kidney injury (24.8 vs. 16.7 %, respectively; p < 0.05). Similar results were obtained in subgroup analysis where only more anemic patients (68 matched pairs) or patients with cardiovascular comorbidities (63 matched pairs) were considered.

Conclusions

RBCT does not improve the clinical outcome in non-bleeding critically ill patients with moderate anemia.     

Does a febrile reaction to platelets predispose recipients to red blood cell alloimmunization?

BACKGROUND: A variable effect of inflammation on alloimmunization to transfused red blood cells (RBCs) in mice has been recently reported. We investigated whether RBC alloimmunization in humans was affected by transfusion of blood products in temporal proximity to experiencing a febrile transfusion reaction (FTR) to platelets (PLTs), an event predominantly mediated by inflammatory cytokines.
STUDY DESIGN AND METHODS: Blood bank databases were used to identify patients who experienced an FTR or possible FTR to PLTs from August 2000 to March 2008 (FTR group). The control group of patients received a PLT transfusion on randomly selected dates without experiencing an FTR. The event was defined as the PLT transfusion that caused the FTR in the FTR group or the index PLT transfusion in the control group. The number of transfused blood products and their proximity to the event were recorded along with other recipient data. The primary endpoint was the rate of RBC alloimmunization between the two groups.
RESULTS: There were 190 recipients in the FTR group and 245 in the control group. Overall, the recipients in the control group were younger and received more blood products on the day of their event and over the subsequent 10 days. The alloimmunization rate among recipients in the FTR group was higher than in the control group (8% vs. 3%, respectively; p = 0.026).
CONCLUSIONS: These preliminary data support our hypothesis that recipient inflammation may affect RBC alloimmunization in humans; however, a more detailed understanding of the pathophysiologic association between inflammation and alloimmunization is required before definitive conclusions can be reached.     

Additional red blood cell alloantibodies after blood transfusions in a nonhematologic alloimmunized patient cohort: is it time to take precautionary measures?

BACKGROUND: Red blood cell (RBC) alloimmunization is common in transfused patients. Most studies report on the rate of alloimmunization in chronically transfused patients, which can be as high as 60 percent. Less is known on the incidence of clinically relevant antibodies in accidentally transfused patients. Because the probability of repeat transfusion increases with longer life expectancy, it was wondered to which extend non-chronically transfused alloimmunized patients are prone to form additional antibodies after repeat transfusion events. STUDY DESIGN AND METHODS: A 20-year retrospective multicenter study was performed analyzing additional alloantibody formation, against the RH, KEL, FY, JK, and MNS blood group systems. RESULTS: After additional transfusions, 21.4 percent of 653 patients produced additional antibodies, resulting in 157 new antibody specificities. At the end of the study 33.4 percent of patients had multiple antibodies. Eighty of 140 patients (57%) who formed additional antibodies did so after one transfusion episode of a median of 2 units of RBCs. Based on the antigen profile of 316 patients, 83 percent of antibodies could have been prevented by extended matching for the C, E, c, K, Fy(a), and Jk(a) antigens. Considering the current available donors in our region, 1 to 10 percent of potential donors would be available for 39 percent of patients and greater than 10 percent of potential donors for 61 percent of patients. CONCLUSION: It has been shown that nonhematooncologic alloimmunized patients are high antibody responders, with a more than 20 times increased risk to form antibodies compared to first-time alloimmunization risk. If extended matching for C, c, E, K, Fy(a), and Jk(a) antigens in the future is considered, this group should be taken into account.     

How would you like your COVID-19? From a host with mild course disease,or from a severe one?

The clinical course of COVID-19 presents a broad spectrum, being asymptomatic in some individuals while following a severe course and resulting in mortality in others. It is known that such factors as age and chronic diseases can result in a different clinical courses in individuals, however, variable clinical courses among the similar individuals in terms of age and chronic diseases are also seen. Other possible factors affecting the course of the disease that are mostly speculative or under investigation are genetic factors and the origin of transmission or possible subtype of novel coronavirus. Whether the source of transmission is important in the clinical course of the disease is unknown.A case series composed of seven individuals in a similar age group, with different lines of descent and different genetic structures, but who were infected from the same source is presented here. The similar and different clinical, laboratory and radiological findings of the cases residing in the same nursing home, who presented to the hospital altogether, were evaluated. The aim of the study was to analyze whether the source of transmission is influential in the clinical course of the disease.