Fatal pulmonary thromboembolism associated with hemoglobin SC disease in a 15 year-old boy

Amancio, Thiago Trolez; Costa, Antônio Gadelha da; Borsato, Marcela Longo; Marins, Leandro; Bacchi, Lívia Moscardi; Zerbini, Maria Cláudia Nogueira

doi:10.4322/acr.2011.004

Cited by 3 publications

(3 citation statements)

References 15 publications

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“…A study by Amancio et al reported a case of hemoglobin SC complicated by fatal pulmonary thromboembolism diagnosed at autopsy [11]. In our patient, there was a high risk of critical comprise of pulmonary function; therefore, anticoagulation with heparin was started followed by maintenance anticoagulation therapy with enoxaparin.…”

Section: Discussionmentioning

confidence: 91%

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

Villanueva

Kuril

Krajewski

et al. 2013

Case Reports in Pediatrics

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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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Section: Discussionmentioning

confidence: 91%

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

Villanueva

Kuril

Krajewski

et al. 2013

Case Reports in Pediatrics

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“…Pulmonary hypertension, with a prevalence of 20-30% can be related to chronic hemolysis, nitric oxide deficiency, chronic hypoxemia, endothelial dysfunction and proliferative vasculopathy, or vaso-occlusive events due to sickling erythrocytes and hyperviscosity 8 - 10. In addition, SCD patients are at increased risk of developing large vessels thrombosis and more rarely intracardiac thrombus 11 - 13…”

Section: Discussionmentioning

confidence: 99%

“… 8 - 10 In addition, SCD patients are at increased risk of developing large vessels thrombosis and more rarely intracardiac thrombus. 11 - 13 …”

Section: Discussionmentioning

confidence: 99%

Bone marrow necrosis and fat embolism: an autopsy report of a severe complication of hemoglobin SC disease

Campos¹,

Ferreira²,

Felipe-Silva³

2014

ACR

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Sickle Cell Disease encompasses a group of disorders related with the hemoglobin S and other hemoglobin genotypes. The clinical manifestation and the severity of symptoms are dependent on the specific genotype. In this setting, homozygous genotype (HbSS) presents an early onset of symptoms and a low expectancy of lifetime. However, the SC genotype (HbSC), which apparently shows a less severe clinical course, may exhibit the same complications of HbSS. These complications are usually manifested late in the course of life, when compared with the HbSS patients. It is noteworthy that HbSC may present a normal hematocrit, and therefore stays unknown until the first complication, that may be disastrous. The authors report a case of an African-Descendant woman, aging 65 years, with no previous diagnosis of anemia who sought medical attention because of a thoracic back pain followed by fever and altered mental status. The clinical picture deteriorated very fast with multiple organ failure and death. The autopsy findings concluded by generalized vaso-occlusive crisis, bone marrow necrosis and bone marrow and fat embolism, mainly to the lungs and kidney. The authors call attention for the knowledge of this severe life threatening complication, mainly in a country with a high Afro-Descendant population.

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Embolie pulmonaire bilatérale mimant un syndrome thoracique aigu chez un adolescent drépanocytaire

Mornand

Chalard

Romain

et al. 2017

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Fatal pulmonary thromboembolism associated with hemoglobin SC disease in a 15 year-old boy

Cited by 3 publications

References 15 publications

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

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

Bone marrow necrosis and fat embolism: an autopsy report of a severe complication of hemoglobin SC disease

Embolie pulmonaire bilatérale mimant un syndrome thoracique aigu chez un adolescent drépanocytaire

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