Severe Painful Vaso-Occlusive Crises and Mortality in a Contemporary Adult Sickle Cell Anemia Cohort Study

Darbari, Deepika S.; Wang, Zhengyuan; Kwak, Minjung; Hildesheim, Mariana; Nichols, James; Allen, Darlene; Seamon, Catherine; Peters-Lawrence, Marlene; Conrey, Anna; Hall, Melanie; Kato, Gregory J.; Taylor, James G.

doi:10.1371/journal.pone.0079923

Cited by 101 publications

(111 citation statements)

References 31 publications

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“…In two prospective studies sickle cell patients were selected for HC treatment based on their greater vaso-occlusive severity (Voskaridou et al, 2010;Lobo et al, 2013). Long term follow up showed that these patients survived longer than their non-HC treated counterparts, even though a higher mortality (Platt et al, 1994;Darbari et al, 2013) would have been predicted by their greater vaso-occlusive severity.…”

Section: Discussionmentioning

confidence: 92%

Hydroxycarbamide treatment in sickle cell disease: estimates of possible leukaemia risk and of hospitalization survival benefit

2014

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SummaryUsing health insurance claims databases we compared the frequency/ incidence of acute myeloid leukaemia (AML) and inpatient mortality in sickle cell disease (SCD) subjects taking (n = 1051), or not taking (n = 9203) hydroxycarbamide (HC). Patients taking HC were older (median 19 vs. 17 years of age), had a higher proportion of males (53% vs. 38%), and their median hospitalizations per year was five times greater than in SCD patients not on HC (all P < 0Á001). No new AML cases occurred in HC-treated paediatric SCD patients. For adults, the new AML incidence with HC exposure was 10Á7/10 000 patient years, vs. 4Á0/10 000 patient years in subjects not on HC (P = 0Á2), a possible AML risk ratio of 3Á18. Adjustment for a probable database bias for AML diagnosis/ascertainment lowered the risk ratio to 0Á94 (95% confidence interval = 0Á16-5Á47). Despite their greater disease severity, the inpatient mortality in SCD adults prescribed HC (0Á29%) was lower than that of patients not taking the drug (0Á42%, P = 0Á032). In this SCD population we find no increased risk for AML with HC treatment. If such a risk is eventually proven, it will probably be lower than that for drugs with known AML association. By contrast, HC treatment appears to confer a survival benefit.

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Section: Discussionmentioning

confidence: 92%

Hydroxycarbamide treatment in sickle cell disease: estimates of possible leukaemia risk and of hospitalization survival benefit

2014

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“…Wound healing is a complex, dynamic process that can be simplified in three phases: There are a few treatment principles that guide chronic wound therapy by the practicing sickle cell clinician (Table I): (1) Debride to remove dead tissue and biofilm; (2) open or excise closed wound edges to stimulate proliferation; (3) identify and treat infections; (4) absorb excess exudate to avoid maceration; (5) maintain moist wound surface ("a dry cell is a dead cell"); (6) protect healing wound from infection/trauma; and, most importantly, (7) use some type of compression to avoid venostasis.…”

Section: Principles Of Wound Healing and Wound Care For The Practicinmentioning

confidence: 99%

“…In the 21st century the majority of patients reach adulthood [1][2][3][4] As patients become older, the challenge of end organ damage has risen and among those, chronic ulcers represent a particularly debilitating and poorly understood complication. Their incidence has remained steady despite successful preventative strategies against bacterial infection and stroke [5], and improved supportive care.…”

Section: Introductionmentioning

confidence: 99%

Critical Reviews: How we treat sickle cell patients with leg ulcers

Minniti

Kato

2015

American J Hematol

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The past five decades have seen an improvement in the mortality and morbidity of sickle cell disease (SCD) because of prophylaxis against infectious complications, improved and expanded red cell transfusions, implementation of hydroxyurea therapy, and advances in supportive care. Now that the majority of patients in the western hemisphere reaches adulthood, end organ diseases are frequent, which include vasculopathic complications such as chronic leg ulcers. The management of patients with leg ulcers requires the hematologist to lead a team of health care professionals, and investigates the presence of associated, but potentially still occult signs of vasculopathy, such as pulmonary hypertension, renal disease, priapism and retinopathy. These complications may be asynchronous, and long term careful screening is indicated, in order to ensure early diagnosis and intervention. It is crucial to address both the immediate consequences of pain, infection and disability, and long term effects on quality of life, employment and stigma associated with chronic ulceration. Recent insights into their pathophysiology may have practical implications. We propose a holistic approach to the management of patients' physical and emotional problems and mechanisms of ulcers formation and delayed healing. An overview of topical and systemic therapies for chronic ulcers is given, with the understanding that wound care therapy is best left to the wound specialists, medical and surgical, with whom the hematologist must keep an open line of communication. In the absence of evidence-based guidelines, our opinion is based on both a critical review of the literature and our personal clinical and research experience. Am. J. Hematol. 91:22-30, 2016. V C 2015 Wiley Periodicals, Inc. Clinical CasesCase 1. A 49 year old African American woman with sickle cell anemia, bilateral hip replacements for avascular necrosis of the femoral head, pulmonary arterial hypertension documented by pulmonary artery catheterization, and history of laser treatment and vitrectomy for proliferative retinopathy and vitreous hemorrhage, presented with a very painful ulcer at the right malleolus. She has had three previous ulcers, on both legs, the first at age 32 years, attributed to excessive standing at her job, no trauma, which had responded to topical care and healed within few months of presentation. At that time she was not taking hydroxyurea, but has been taking it now for longer than 10 years with good fetal hemoglobin response: 17%. This current ulcer developed while she was experiencing high personal stress but no physical trauma. She rarely experiences vaso-occlusive pain crisis and has very infrequent hospitalizations. She was referred to the wound care service and after 7 months of unfruitful therapies, including surgical debridement, MIST ultrasound therapy, antibiotics, and compressions, she was started on monthly transfusions and her ulcer healed 5 months later. She has had consistently elevated serum lactate dehydrogenase (LDH) (>600 IU/L), serum dire...

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“…[1][2][3][4][5] The strongest genetic association with chronic kidney disease (CKD) in the general African-American population is homozygosity or compound heterozygosity for two common variants of APOL1, G1 (S342G and I384M substitutions) and G2 (N388 and Y389 deletions), [6][7][8][9][10][11] which is observed in 10-15% of African-Americans. 6,12 Similar to the genetic persistence of the Hb S mutation, which protects against mortality from malaria, the APOL1 G1 and G2 variants are believed to have been selected by affording protection from Trypanosoma brucei rhodesiense infection.…”

Section: Introductionmentioning

confidence: 99%