Abnormal hemostasis in children with vascular anomalies, part I: Thrombocytopenias among different vascular anomalies

Tole, Soumitra; Price, Victoria; Pope, Elena; Powell, Julie; David, Michèle; Zwicker, Kelley; Kendrick, Victoria; Malic, Claudia; John, Philip; Somers, Gino R.; Dubois, Josée; Brandão, Leonardo R.

doi:10.1016/j.thromres.2019.06.004

Cited by 6 publications

(3 citation statements)

References 74 publications

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“…Surgery can shorten treatment time, reduce recurrence rate, and reduce drug-related side effects and complications [20]. Therefore, surgical treatment needs to be considered when the following conditions occur: 1) When the lesion is small, and the surgical removal does not cause organ damage or functional impairment; 2) when the lesion is expected to cause damage, ulcers, hemorrhage or deformity to the body [21]; 3) those that have failed medical treatment or are imminently life threatening [22]. Accurate diagnosis, complete surgical preparation, and precise and moderate resection of the lesion are key factors to the success of the procedure.…”

Section: Discussionmentioning

confidence: 99%

Effective surgical treatment of life-threatening huge vascular anomalies associated with thrombocytopenia and coagulopathy in infants unresponsive to drug therapy

Wu¹,

Qiu²,

Zeng³

et al. 2020

BMC Pediatr

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Background: Systemic drug therapy is generally recommended for infant huge vascular anomalies associated with thrombocytopenia and coagulopathy, but some patients are not suitable due to drug unresponsiveness or life threatening conditions before the drug works, who will need to receive surgical treatment. This study retrospectively analyzed the clinical features, imaging features, and surgical outcomes of these patients. Methods: The clinical data of 4 infants with huge vascular anomalies (2 vein malformations (VMs) and 2 kaposiform hemangioendothelioma (KHE)) associated with thrombocytopenia and coagulopathy treated from June 2016 to December 2017 were retrospectively analyzed. All patients received glucocorticoids, propranolol, vincristine or sirolimus treatment before admission, but the treatment was ineffective. Skin petechia, thrombocytopenia and coagulopathy were present at the time of admission. CT scanning was performed before operation. The patient's general clinical data, hematological examination results, operation time, surgical bleeding volume, blood transfusion volume and surgical complications were collected for analysis. The patients were followed up for 10-26 months. Results: CT scanning results of 2 patients showed special CT features without detectable enhancement within the lesion after CT enhanced scanning and multiple phleboliths formation. Four patients underwent surgical treatment successfully. Two patients underwent complete resection of the lesion, and 2 underwent cytoreductive surgery. Preoperative clinical symptoms such as skin petechia, thrombocytopenia and coagulopathy were normal at 1 week after surgery. Postoperative pathological results showed 2 cases of KHE and 2 cases of VMs. All patients were discharged from hospital without physical dysfunction, recurrence, or death.

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Section: Discussionmentioning

confidence: 99%

Effective surgical treatment of life-threatening huge vascular anomalies associated with thrombocytopenia and coagulopathy in infants unresponsive to drug therapy

Wu¹,

Qiu²,

Zeng³

et al. 2020

BMC Pediatr

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“…Thrombosis can lead to thromboembolic disease such as pulmonary embolism and phlebitis 3 . Severe LIC may even progress to disseminated intravascular coagulopathy (DIC), marked by systemic consumption of platelets and coagulation factors, which can be life‐threatening and result in severe bleeding, especially during invasive surgical procedures 1,4,5 …”

Section: Introductionmentioning

confidence: 99%

“…3 Severe LIC may even progress to disseminated intravascular coagulopathy (DIC), marked by systemic consumption of platelets and coagulation factors, which can be lifethreatening and result in severe bleeding, especially during invasive surgical procedures. 1,4,5 Current guidelines suggest that patients with slow-flow vascular malformations and associated elevated D-dimers and/or low fibrinogen may be at an increased risk of thrombotic or bleeding complications. These patients may be treated with a short course of anticoagulation around the time of invasive procedures when disturbances in the coagulation cascade and thrombosis is at highest risk.…”

mentioning

confidence: 99%

Analyzing coagulation dynamics during treatment of vascular malformations with thromboelastography

Mack

Pierce

Richter

et al. 2020

Pediatric Blood & Cancer

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Background/Objectives: Slow-flow vascular malformations are abnormal vessels that can lead to activation and consumption of coagulation factors and thrombosis, known as localized intravascular coagulopathy (LIC). Most clinical and research evidence of vascular malformation hemostasis relies on conventional coagulation studies, which may not provide a complete picture. Thromboelastograpy (TEG) is a tool that can provide real-time assessment of a patient's coagulation dynamics, and may allow for a more individualized treatment approach. We hypothesized that patients with slowflow vascular malformations will have changes in TEG parameters peri-procedure that will help predict blood product or medication administration. Design/Methods: Institutional Review Board approved prospective study of patients with slow-flow vascular malformations undergoing a sedated, minor procedure. TEG and conventional coagulation studies were obtained preprocedure, 15 min, and when possible, at 30 min after the start of the procedure. Results: Twenty-five patients were enrolled. Median age was 15 years (range 3-47 years). Procedures included laser and/or sclerotherapy. There were no changes in TEG parameters from baseline to 15 min or 30 min. The following decreased from baseline to 15 min: fibrinogen 313 to 287 mg/dL (P = .001), D-dimer 1.3 to 1.1 mg/L (P = .02), hemoglobin 12.8 to 11.8 g/dL (P = .001), and platelet count 272 000 to 256 000 (P = .006). No patient had a bleeding/thrombotic complication during or within 1 week postprocedure. Conclusion: We saw no change in TEG parameters or bleeding or clotting complications despite significant numerical changes in conventional coagulation profiles, suggesting that conventional studies may not be as useful in determining risks of bleeding or thrombotic complications peri-procedure for minor procedures.

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Pediatric Vascular Anomalies: Opportunities in Primary Care

Wolfe

2020

Benign Hematologic Disorders in Children

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Abnormal hemostasis in children with vascular anomalies, part I: Thrombocytopenias among different vascular anomalies

Cited by 6 publications

References 74 publications

Effective surgical treatment of life-threatening huge vascular anomalies associated with thrombocytopenia and coagulopathy in infants unresponsive to drug therapy

Effective surgical treatment of life-threatening huge vascular anomalies associated with thrombocytopenia and coagulopathy in infants unresponsive to drug therapy

Analyzing coagulation dynamics during treatment of vascular malformations with thromboelastography

Pediatric Vascular Anomalies: Opportunities in Primary Care

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