Pulmonary Thromboembolism in a Child with Sickle Cell Hemoglobin D Disease in the Setting of Acute Chest Syndrome

Introduction. Sickle cell hemoglobin D disease (HbSD) is a rare variant of sickle cell disease (SCD). Incidence of pulmonary thromboembolism (PE) and deep venous thrombosis (DVT) in children with HbSD is unknown. PE and DVT are known complications of SCD in adults but have not been reported in the l...

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Main Authors: Hazel Villanueva, Sandeepkumar Kuril, Jennifer Krajewski, Aziza Sedrak
Format: Article
Language:English
Published: Wiley 2013-01-01
Series:Case Reports in Pediatrics
Online Access:http://dx.doi.org/10.1155/2013/875683
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author Hazel Villanueva
Sandeepkumar Kuril
Jennifer Krajewski
Aziza Sedrak
author_facet Hazel Villanueva
Sandeepkumar Kuril
Jennifer Krajewski
Aziza Sedrak
author_sort Hazel Villanueva
collection DOAJ
description Introduction. Sickle cell hemoglobin D disease (HbSD) is a rare variant of sickle cell disease (SCD). Incidence of pulmonary thromboembolism (PE) and deep venous thrombosis (DVT) in children with HbSD is unknown. PE and DVT are known complications of SCD in adults but have not been reported in the literature in children with HbSD. Case Report. We are reporting a case of a 12-year-old boy with HbSD with acute chest syndrome (ACS) complicated by complete thrombosis of the branch of the right pulmonary artery and multiple small pulmonary artery emboli seen on computed tomography (CT) pulmonary angiogram and thrombosis of the right brachial vein seen on Doppler ultrasound. Our patient responded to treatment with anticoagulant therapy. Conclusion. There are no cases reported in children with HbSD disease presenting as ACS with pulmonary thromboembolism. We suggest that PE should be suspected in patients presenting with ACS who do not show improvement with standard management. CT pulmonary angiogram should be utilized for early diagnosis and appropriate management as there is no current protocol for management of PE/DVT in pediatric patients with SCD.
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spelling doaj-art-f970c5eec236462f9f14c9ad4dd082f32025-02-03T01:03:29ZengWileyCase Reports in Pediatrics2090-68032090-68112013-01-01201310.1155/2013/875683875683Pulmonary Thromboembolism in a Child with Sickle Cell Hemoglobin D Disease in the Setting of Acute Chest SyndromeHazel Villanueva0Sandeepkumar Kuril1Jennifer Krajewski2Aziza Sedrak3Pediatrics, The Brooklyn Hospital Center, Brooklyn, NY 11201, USAPediatrics, The Brooklyn Hospital Center, Brooklyn, NY 11201, USAPediatric Hematology-Oncology, The Brooklyn Hospital Center, Brooklyn, NY 11201, USAPediatric Hematology-Oncology, The Brooklyn Hospital Center, Brooklyn, NY 11201, USAIntroduction. Sickle cell hemoglobin D disease (HbSD) is a rare variant of sickle cell disease (SCD). Incidence of pulmonary thromboembolism (PE) and deep venous thrombosis (DVT) in children with HbSD is unknown. PE and DVT are known complications of SCD in adults but have not been reported in the literature in children with HbSD. Case Report. We are reporting a case of a 12-year-old boy with HbSD with acute chest syndrome (ACS) complicated by complete thrombosis of the branch of the right pulmonary artery and multiple small pulmonary artery emboli seen on computed tomography (CT) pulmonary angiogram and thrombosis of the right brachial vein seen on Doppler ultrasound. Our patient responded to treatment with anticoagulant therapy. Conclusion. There are no cases reported in children with HbSD disease presenting as ACS with pulmonary thromboembolism. We suggest that PE should be suspected in patients presenting with ACS who do not show improvement with standard management. CT pulmonary angiogram should be utilized for early diagnosis and appropriate management as there is no current protocol for management of PE/DVT in pediatric patients with SCD.http://dx.doi.org/10.1155/2013/875683
spellingShingle Hazel Villanueva
Sandeepkumar Kuril
Jennifer Krajewski
Aziza Sedrak
Pulmonary Thromboembolism in a Child with Sickle Cell Hemoglobin D Disease in the Setting of Acute Chest Syndrome
Case Reports in Pediatrics
title Pulmonary Thromboembolism in a Child with Sickle Cell Hemoglobin D Disease in the Setting of Acute Chest Syndrome
title_full Pulmonary Thromboembolism in a Child with Sickle Cell Hemoglobin D Disease in the Setting of Acute Chest Syndrome
title_fullStr Pulmonary Thromboembolism in a Child with Sickle Cell Hemoglobin D Disease in the Setting of Acute Chest Syndrome
title_full_unstemmed Pulmonary Thromboembolism in a Child with Sickle Cell Hemoglobin D Disease in the Setting of Acute Chest Syndrome
title_short Pulmonary Thromboembolism in a Child with Sickle Cell Hemoglobin D Disease in the Setting of Acute Chest Syndrome
title_sort pulmonary thromboembolism in a child with sickle cell hemoglobin d disease in the setting of acute chest syndrome
url http://dx.doi.org/10.1155/2013/875683
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AT jenniferkrajewski pulmonarythromboembolisminachildwithsicklecellhemoglobinddiseaseinthesettingofacutechestsyndrome
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