scholarly journals Plastic Bronchitis and the Role of Bronchoscopy in the Acute Chest Syndrome of Sickle Cell Disease

CHEST Journal ◽  
2001 ◽  
Vol 120 (2) ◽  
pp. 608-613 ◽  
Author(s):  
Chuanpit Moser ◽  
Eliezer Nussbaum ◽  
Dan M. Cooper
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Acute Chest Syndrome ◽  
Plastic Bronchitis ◽  
Cell Disease

Role of Chlamydia pneumoniae in acute chest syndrome of sickle cell disease

1991 ◽  
Vol 118 (1) ◽  
pp. 30-33 ◽  
Author(s):  
Scott T. Miller ◽  
Margaret R. Hammerschlag ◽  
Keith Chirgwin ◽  
Sreedhar P. Rao ◽  
Patricia Roblin ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Chlamydia Pneumoniae ◽  
Acute Chest Syndrome ◽  
Cell Disease

Acute chest syndrome: the role of erythro-exchange in patients with sickle cell disease in Sicily

2003 ◽  
Vol 29 (1) ◽  
pp. 39-44 ◽  
Author(s):  
Turiddu Lombardo ◽  
Rosamaria Rosso ◽  
Alfio La Ferla ◽  
Maria Gabriella Ferro ◽  
Benedetta Ximenes ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Acute Chest Syndrome ◽  
Cell Disease

scholarly journals Sickle cell disease: when and how to transfuse

Hematology ◽  
2016 ◽  
Vol 2016 (1) ◽  
pp. 625-631 ◽  
Author(s):  
Jo Howard
Keyword(s):  
Sickle Cell Disease ◽  
Observational Data ◽  
Sickle Cell ◽  
Randomized Trials ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Chronic Complications ◽  
Advantages And Disadvantages

Abstract Blood transfusion remains an important therapeutic intervention in patients with sickle cell disease (SCD), aiming to both increase the oxygen carrying capacity of blood and to reduce the complications of vaso-occlusion. Simple, manual exchange and automated exchange can be effective in reducing the acute and chronic complications of SCD, and the advantages and disadvantages of each methodology mean they all have a role in different situations. Evidence for the role of emergency transfusion in the management of the acute complications of SCD, including acute pain and acute chest syndrome, comes from observational data. Several important randomized controlled trials have shown the efficacy of transfusion in primary and secondary stroke prevention in patients with SCD but, outside these areas, clinical practice lacks a clear evidence base. Evidence for the role of long-term transfusion in the prevention of the non-neurologic chronic complications of SCD comes from analysis of secondary outcomes of these randomized trials and from observational data. In view of the paucity of data, the risks and benefits of transfusion should be fully discussed with patients/families before a long-term transfusion program is commenced. Evidence is only available for the role of preoperative transfusion or for prophylactic transfusion through pregnancy in certain situations, and the role of transfusions outside these situations is discussed. Questions about when and how to transfuse in SCD remain and will need further randomized trials to provide answers.

Potential role of the ventilation and perfusion (V/Q) lung scan in the diagnosis of acute chest syndrome in adults with sickle cell disease

2004 ◽  
Vol 77 (4) ◽  
pp. 407-409 ◽  
Author(s):  
Navleen Kaur ◽  
Bharat Motwani ◽  
Devaki Sivasubramaniam ◽  
Lori Feldman ◽  
Sandra Allen ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Potential Role ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Lung Scan ◽  
Ventilation And Perfusion

scholarly journals Indications for transfusion in the management of sickle cell disease

Hematology ◽  
2021 ◽  
Vol 2021 (1) ◽  
pp. 696-703
Author(s):  
Hyojeong Han ◽  
Lisa Hensch ◽  
Venée N. Tubman
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Perioperative Complications ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Transfusion Therapy ◽  
Frequent Use ◽  
Clinical Scenarios ◽  
Conclusive Data

Abstract The transfusion of red blood cells (RBCs) is a crucial treatment for sickle cell disease (SCD). While often beneficial, the frequent use of transfusions is associated with numerous complications. Transfusions should be offered with specific guidelines in mind. Here we present updates to the indications for transfusion of RBCs in SCD. We review recent publications and include expert perspectives from hematology and transfusion medicine. For some clinical indications, such as ischemic stroke, the role of transfusion has been well studied and can be applied almost universally. For many other clinical scenarios, the use of transfusion therapy has less conclusive data and therefore must be tailored to individual needs. We highlight the roles of RBC transfusions in preventing or mitigating neurological disease, in reducing perioperative complications, in managing acute chest syndrome, and in optimizing pregnancy outcomes in SCD. We further highlight various transfusion techniques and when each might be considered. Potential complications of transfusion are also briefly discussed.

Changes In Cell-Derived Plasma Microparticles During The Acute Chest Syndrome In Children With Sickle Cell Disease

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 2239-2239 ◽  
Author(s):  
Christopher M McKinney ◽  
Marguerite R Kelher ◽  
Christopher C Silliman
Keyword(s):  
Sickle Cell Disease ◽  
Sample Size ◽  
Sickle Cell ◽  
Systemic Inflammation ◽  
Small Sample ◽  
Acute Chest Syndrome ◽  
Separate Analysis ◽  
Cell Disease ◽  
Larger Sample

Abstract Introduction Microparticles (MP) are small (less than 1 µm), membrane-derived extracellular vesicles produced following cellular activation or apoptosis. They have been implicated as causative in a wide variety of pro-inflammatory diseases. Sickle cell disease is a hemoglobinopathy characterized by chronic hemolysis and systemic inflammation which can be complicated by vaso-occlusive pain crises (VOC) or the acute chest syndrome (ACS). Increasingly there is evidence that microparticles may play an important role in the pathophysiology of sickle cell disease. We hypothesize that plasma microparticles are elevated during ACS compared to VOC and baseline (resting). Methods Whole blood was obtained under an approved consent from the Colorado Multiple Institution Review Board at the University of Colorado Denver, from three subjects with homozygous sickle cell disease (ages 4-18) at three distinct time points: baseline (resting) at routine clinic visits and upon hospitalization for VOC and ACS. The samples underwent differential centrifugation: plasma was isolated (5,000xg for 7 min at RT) and then cell free supernatant was separated (12,500xg for 6 min at 4°C). MPs were isolated by centrifugation at 17,000xg for 60 minutes at 4°C, and the MP pellet was resuspended in an equal volume of 1.25% human serum albumin. The samples were incubated with CD41 (PE)-, CD45 (PerCP-Cy5)-, and CD235 (FITC)-antibodies that are specific for MPs derived from platelets, leukocytes, and erythrocytes, respectively, and analyzed by flow cytometry. Statistics were performed using ANOVA with post-hoc Newman-Keuls test. Results The amount of leukocyte-derived MP (WBC) measured during ACS was significantly higher (almost two-fold) than levels measured during baseline (resting) (p < 0.05), but not VOC. The erythrocyte (RBC) MP levels were also significantly elevated during ACS, compared to resting and VOC (p<0.05). There is a trend toward higher platelet (Plt) MP levels during ACS compared to both baseline and VOC, however this does not reach statistical significance likely due to the small sample size (n=3) (Fig. 1). In a separate analysis, the MPs from two patients admitted for VOC with evolution to ACS during the same hospitalization exhibited a three-fold increase in WBC MP levels during VOC that preceded their progression to ACS by 24-72 hours. Discussion Microparticles may play a significant role in the pathophysiology of ACS in sickle cell disease. Leukocyte-derived MPs are elevated in ACS at higher levels than baseline. This likely reflects the important role of white blood cells (WBCs) in systemic inflammation and the importance of WBC-endothelial interactions in ACS. While platelet- and erythrocyte-derived MPs may be involved in ACS, a larger sample size will be needed to detect this effect. In addition, while hospitalized, two patients who had a similar magnitude increase in WBC MPs during VOC eventually progressed to ACS; thus, serial MP measurements could potentially serve as a biomarker to identify patients at risk for imminent development of ACS. Further prospective studies with larger sample sizes will be needed for validation. Future directions of study should also evaluate the role of endothelial-derived MPs and the role of ICAM-1. Disclosures: No relevant conflicts of interest to declare.

Plastic Bronchitis: An Unusual Complication Associated With Sickle Cell Disease and the Acute Chest Syndrome

PEDIATRICS ◽  
1997 ◽  
Vol 100 (1) ◽  
pp. 139-142 ◽  
Author(s):  
N. Raghuram ◽  
R. Pettignano ◽  
A. A Gal ◽  
A. Harsch ◽  
T. V. Adamkiewicz
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Acute Chest Syndrome ◽  
Unusual Complication ◽  
Plastic Bronchitis ◽  
Cell Disease
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