Document Detail

Acute Chest Syndrome: the role of erythro-exchange in patients with sickle cell disease in Sicily.
MedLine Citation:
PMID:  12877891     Owner:  NLM     Status:  MEDLINE    
Acute Chest Syndrome (ACS) describes a syndrome characterized by the presence of a new pulmonary infiltrate on a chest X-ray, fever, and respiratory symptoms and is the leading cause of death and hospitalization in sickle cell disease (SCD). We studied 21 patients affected by SCD (13 HbSbeta+, 4 HbS beta(o), 4 HbSS, mean age 38.2 years). Six out of the 21 patients developed one episode of ACS (two patients had positive blood cultures, for Mycoplasma pneumoniae and Haemophilus influenzae respectively). The aim of our study was to evaluate the therapeutic efficacy of red cell-exchange during ACS. This procedure decreases HbS levels. The patients who underwent erythro-exchange showed a dramatic clinical and radiographic improvement with stabilized HbS levels between 20% and 30%. During follow up (14-32 months), none of the 6 patients developed viral complications related to transfusion therapy, alloimmunization or recurrence of ACS. In conclusion, in regard to the pre- and post-red cell-exchange clinical and laboratory data, we can say that red cell-exchange provides a dramatic resolution of the episode of ACS, minimizes the development of iron overload, and rapidly decreases HbS and hematocrit levels. In light of our results, we hypothesize that ACS episodes are secondary to pulmonary damage and to a gradual worsening related to age, and that there is some evidence that individuals affected by SCD in the third to fourth decade of life are more susceptible to ACS and/or other severe disease-related complications, needing repeated and strict clinical follow up.
Turiddu Lombardo; Rosamaria Rosso; Alfio La Ferla; Maria Gabriella Ferro; Benedetta Ximenes; Viviana Frontini; Salvatore Pennisi
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis     Volume:  29     ISSN:  1473-0502     ISO Abbreviation:  Transfus. Apher. Sci.     Publication Date:  2003 Aug 
Date Detail:
Created Date:  2003-07-24     Completed Date:  2003-12-05     Revised Date:  2006-06-07    
Medline Journal Info:
Nlm Unique ID:  101095653     Medline TA:  Transfus Apher Sci     Country:  England    
Other Details:
Languages:  eng     Pagination:  39-44     Citation Subset:  T    
Servizio di Thalassemia, Ospedale Santo Bambino, via Tindaro 1, 95124 Catania, Italy.
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MeSH Terms
Anemia, Sickle Cell / complications*
Erythrocytes / cytology*
Follow-Up Studies
Haemophilus influenzae / metabolism
Hemoglobin, Sickle / biosynthesis
Lung Diseases / genetics
Middle Aged
Mycoplasma pneumoniae / metabolism
Time Factors
Reg. No./Substance:
0/Hemoglobin, Sickle

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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