Sickle Cell Disease and the Pulmonary Circulation

Kirkpatrick, Michael B.; Haynes, Johnson

doi:10.1055/s-2007-1006393

Cited by 16 publications

(11 citation statements)

References 56 publications

(115 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Finally, patients with SCD have a propensity to develop thrombotic complications. In situ thrombosis of small pulmonary vessels is a common finding during autopsy studies (Oppenheimer & Esterly, ; Kirkpatrick & Haynes, ; Adedeji et al , ). The incidence of inpatient pulmonary embolism is higher in the SCD population than in the non‐SCD population (Novelli et al , ).…”

Section: Hypercoagulation and Thrombosis In Sickle Cell Diseasementioning

confidence: 99%

Interplay between coagulation and vascular inflammation in sickle cell disease

2013

View full text Add to dashboard Cite

Sickle cell disease is the most common inherited hematologic disorder that leads to the irreversible damage of multiple organs. Although sickling of red blood cells and vaso-occlusion are central to the pathophysiology of sickle cell disease the importance of hemolytic anemia and vasculopathy has been recently recognized. Hypercoagulation state is another prominent feature of sickle cell disease and is mediated by activation of both intrinsic and extrinsic coagulation pathways. Growing evidence demonstrates that coagulation may not only contribute to the thrombotic complications, but also to vascular inflammation associated with this disease. This article summarizes the role of vascular inflammation and coagulation activation, discusses potential mechanisms responsible for activation of coagulation and reviews recent data demonstrating the crosstalk between coagulation and vascular inflammation in sickle cell disease.

show abstract

Section: Hypercoagulation and Thrombosis In Sickle Cell Diseasementioning

confidence: 99%

Interplay between coagulation and vascular inflammation in sickle cell disease

2013

View full text Add to dashboard Cite

show abstract

“…The most common cause of stroke in SCD patients is large vessel narrowing with superimposed thrombosis, as demonstrated by histopathology studies [15,16]. Additionally, postmortem evaluations of patients with SCD often reveal old and new thrombi in the pulmonary vasculature [26], and thrombosis in sickle cell patients is also associated with avascular necrosis of large joints, leg ulcers, and certain pregnancy complications [15,16]. The hypercoaguable state associated with SCD is thought to be due to abnormalities in the cell membrane of RBCs.…”

Section: Discussionmentioning

confidence: 99%

Oocyte cryopreservation in a patient with sickle cell disease prior to hematopoietic stem cell transplantation: first report

Dovey

Krishnamurti

Sanfilippo

et al. 2012

J Assist Reprod Genet

View full text Add to dashboard Cite

Purpose To report the first occurrence of successful ovarian stimulation, oocyte retrieval and oocyte cryopreservation for fertility preservation in an adolescent with severe sickle cell disease scheduled to undergo a hematopoietic stem cell transplant Methods Case report Results A 19 year old female with severe sickle cell disease presented for fertility preservation counseling prior to hematopoietic stem cell transplantation. She ultimately underwent ovarian stimulation using a minimal stimulation GnRH antagonist protocol resulting in the successful banking of oocytes prior to transplant. The unique hazards associated with ovarian stimulation in patients with sickle cell disease, such as thrombosis and vaso-occlusive events, are discussed and the methods undertaken to minimize these risks are described. Conclusions Controlled ovarian hyperstimulation and oocyte banking for fertility preservation is feasible in young women with sickle cell disease requiring hematopoietic stem cell transplant and deserves further investigation. Given the elevated risk of thrombosis and predisposition to painful vaso-occlusive events, controlled ovarian hyperstimulation in patients with sickle cell disease is not straightforward and requires a multi-disciplinary team approach to adequately address and minimize the risks in this unique patient population.

show abstract

“…Although these organisms tend to cause mild respiratory illnesses in normal hosts, they have been reported to result in severe, sometimes fatal ACS in sickle cell patients. In addition, local tissue infarction in the lung may result in impaired bacterial clearance, thus allowing enhanced proliferation of even nonvirulent organisms [25].…”

Section: Pathophysiologymentioning

confidence: 99%

“…In one study there was a strong correlation between the development of ACS and a thoracic bone scan which demonstrated rib or sternal infarction [44]. Although intrapulmonary vaso‐occlusion with attendant infarction has been suspected to occur in ACS, few studies have been able to document the existence of this [25,47‐49]. Recently pulmonary scintigraphy was used to demonstrate temporary occlusion of the segmental pulmonary arteries in a patient during an episode of ACS [50].…”

Section: Pathophysiologymentioning

confidence: 99%

Acute Chest Syndrome in Sickle Cell Disease: Pathophysiology and Management

Claster¹,

Vichinsky²

2000

J Intensive Care Med

View full text Add to dashboard Cite

Acute chest syndrome (ACS) is defined as the development of a new pulmonary infiltrate and respiratory symptoms in a patient with sickle cell disease (SCD). One of the most serious complications of SCD, ACS is the leading cause of mortality in patients with SCD. ACS is age dependent, with children having milder disease that often is infectious. Adults often have more severe disease, with pulmonary fat embolism secondary to preceding long bone infarction frequently as a contributing factor. Rapid diagnosis and a high index of suspicion are crucial since this syndrome may have a high mortality rate. A high white blood cell count and a falling hemoglobin tend to be associated with this illness. Patients are often febrile, but may not have positive blood or sputum cultures. Appropriate therapy includes judicious fluids, close attention to respiratory care, antibiotics, and transfusion therapy. Use of the drug, hydroxyurea, has been shown to decrease the incidence of ACS. Patients with repeated episodes are at risk for the development of chronic lung disease and pulmonary hypertension. New treatment strategies such as inhibitors of cytokines and pulmonary vasodilators such as nitric oxide may reduce the high mortality of ACS.

show abstract

Sickle Cell Disease and the Pulmonary Circulation

Cited by 16 publications

References 56 publications

Interplay between coagulation and vascular inflammation in sickle cell disease

Interplay between coagulation and vascular inflammation in sickle cell disease

Oocyte cryopreservation in a patient with sickle cell disease prior to hematopoietic stem cell transplantation: first report

Acute Chest Syndrome in Sickle Cell Disease: Pathophysiology and Management

Contact Info

Product

Resources

About