While packed red blood cell (PRBC) transfusion therapy is a mainstay in the treatment of certain patients with sickle cell disease (SCD) and the standard of care for preoperative management, there are associated risks. Delayed hemolytic transfusion reaction (DHTR) is a risk of PRBC transfusion occurring 2 to 20 days from transfusion and typically presents with severe pain characteristic of vaso-occlusive crisis, fever, and hemolytic anemia. DHTRs are uncommon, occurring in only 4% to 11% of transfused patients with SCD, but may be catastrophic in nature with progression to multiorgan failure within hours. Here, we describe a case of a 20-year-old female with sickle cell SS disease who developed a severe DHTR 5 days following an elective preoperative PRBC transfusion, and rapidly progressed to multiorgan failure and death. This is the first reported case of a catastrophic DHTR in a patient with SCD without any detectable known or new alloantibodies.

Download full-text PDF

Source
http://dx.doi.org/10.1097/MPH.0000000000001307DOI Listing

Publication Analysis

Top Keywords

sickle cell
12
cell disease
12
prbc transfusion
12
delayed hemolytic
8
hemolytic transfusion
8
transfusion reaction
8
multiorgan failure
8
transfusion
6
catastrophic delayed
4
reaction patient
4

Similar Publications

Background: Microalbuminuria, an early indicator of kidney damage in Sickle Cell Disease (SCD) patients, is linked to a heightened risk of chronic kidney disease (CKD) in adulthood. This study investigates the determinants of microalbuminuria in paediatric SCD patients in South-South Nigeria.

Methodology: This cross-sectional study was conducted over six months at the Rivers State University Teaching Hospital, Nigeria, involving 60 children with [HbSS genotype, SCD] in a steady state.

View Article and Find Full Text PDF

This scoping review aims to assess the literature on genetic modifiers of leg ulcers in sickle cell disease, evaluating available evidence, methodologies, and research gaps. A major morbidity in sickle cell disease is the development of leg ulcers. This clinical syndrome of SCD leg ulcers (SLU) has continued to be an enigma due to its multifactorial evolution, dearth of promising guidelines on treatment, and generally unsatisfactory response to treatment.

View Article and Find Full Text PDF

Introduction: Sickle cell disease (SCD) is a global public health priority due to its high morbidity and mortality. In the Democratic Republic of the Congo (DRC), effective care for this disease depends on the availability of resources and the level of knowledge of healthcare workers (HCWs). However, in Bukavu, there is limited data available on these two crucial aspects, which are vital for enhancing the care of patients with SCD.

View Article and Find Full Text PDF

Unlabelled: Sickle cell disease (SCD) is a global health problem causing premature deaths and preventable severe chronic complications. A priority goal to improve outcomes both in the short and long term is the screening for early diagnosis and access to specialized care. In Italy, as in other countries, no systematic national screening program is available.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!