Transfusion prevents acute chest syndrome predicted by elevated secretory phospholipase A2

Styles, Lori; Abboud, Miguel R.; Larkin, Sandra; Lo, Margaret C.; Kuypers, Frans A.

doi:10.1111/j.1365-2141.2006.06409.x

Cited by 73 publications

(57 citation statements)

References 7 publications

(8 reference statements)

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“…Activated sPLA2 converts marrow fat into inflammatory free fatty acids that cause lung injury. Based on these observations, a small study evaluated the efficacy of prophylactic blood transfusions in patients with sPLA2 elevation during VOC (41). This therapeutic approach eliminated ACS in this very small cohort, suggesting that larger confirmatory trials are indicated.…”

Section: Acute Chest Syndromementioning

confidence: 99%

Pulmonary Complications of Sickle Cell Disease

Gladwin

Vichinsky²

2008

N Engl J Med

414

398

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Sickle cell disease (SCD) is a common monogenetic disorder with high associated morbidity and mortality. The pulmonary complications of SCD are of particular importance, as acute chest syndrome and pulmonary hypertension have the highest associated mortality rates within this population. This article reviews the pathophysiology, diagnosis, and treatment of clinically significant pulmonary manifestations of SCD, including acute chest syndrome, asthma, and pulmonary hypertension in adult and pediatric patients. Clinicians should be vigilant in screening and treating such comorbidities to improve patient outcomes.

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Section: Acute Chest Syndromementioning

confidence: 99%

Pulmonary Complications of Sickle Cell Disease

Gladwin

Vichinsky²

2008

N Engl J Med

414

398

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“…One small prospective study demonstrated that RBC transfusion provided to patients hospitalized for pain who had elevated secretory phospholipase A2 levels helped to prevent ACS. 25 However, a subsequent clinical trial within the SCD Clinical Research Network designed to confirm these findings was closed due to inadequate subject accrual and poor predictive performance of secretory phospholipase A2.…”

Section: Acute Chest Syndromementioning

confidence: 99%

Transfusion therapy for sickle cell disease: a balancing act

Chou

2013

Hematology

103

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Transfusion therapy is a key intervention in decreasing morbidity and mortality in patients with sickle cell disease (SCD). Current indications for acute and chronic transfusion therapy have significantly increased the number of RBC units transfused to patients with SCD worldwide. This review summarizes transfusion management for the treatment or prevention of neurologic and perioperative complications, acute chest syndrome, and acute anemia associated with SCD. Despite the recognized benefits of transfusion therapy, it is not without the risks of iron overload, alloimmunization, and delayed hemolytic transfusion reactions. Transfusional iron overload management includes automated RBC exchange, noninvasive imaging to monitor iron burden, and iron chelation with parenteral or oral agents. Although limited and extended RBC antigen matching reduces antibody formation, the prevalence of RBC alloimmunization in patients with SCD remains high. Recent studies demonstrate that RH genetic diversity in patients with SCD contributes to Rh alloimmunization, suggesting that even more refined RBC matching strategies are needed. Advances in molecular blood group typing offer new opportunities to improve RBC matching of donors and recipients and can be of particular benefit to patients with SCD.

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“…In a study of 14 adolescents, secretory phospholipase A 2 was used as a biomarker for ACS in patients hospitalized for vaso-occlusive crises. Early red blood cell transfusion prevented the development of ACS in these patients [55]. …”

Section: Sickle Cell Diseasementioning

confidence: 99%

Thalassemia Major and Sickle Cell Disease in Adolescents and Young Adults

Yacobovich

Tamary

2014

Acta Haematol

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The increased longevity of patients with thalassemia and sickle cell disease (SCD) introduces new clinical challenges due to the accumulation of disease-related morbidity, psychosocial issues and health care adjustments. Patients with thalassemia major now live into adulthood without suffering heart failure but must confront delayed puberty, impaired fertility and progressive bone disease. The increased survival in SCD brings to the front previously unrecognized complications including pulmonary hypertension, silent cerebral infarcts and also reproductive dysfunction. Adolescents and young adults (AYAs) have age-related psychosocial needs in their transition from the pediatric health care environment to the adult system. In this review we present the uniquely age-related medical issues facing the AYA thalassemia and SCD cohort in their transition into adulthood.

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Transfusion prevents acute chest syndrome predicted by elevated secretory phospholipase A2

Cited by 73 publications

References 7 publications

Pulmonary Complications of Sickle Cell Disease

Pulmonary Complications of Sickle Cell Disease

Transfusion therapy for sickle cell disease: a balancing act

Thalassemia Major and Sickle Cell Disease in Adolescents and Young Adults

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