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==[[Hemothorax pathophysiology|Pathophysiology]]==
==[[Hemothorax pathophysiology|Pathophysiology]]==
=== Pathogenesis ===
Three mechanisms of bleeding in haemothorax:
* a torn adhesion between the parietal and visceral pleurae.
* rupture of neovascularized bullae as a complication of subpleural emphysematous blebs.
* torn congenital aberrant vessels branching from the cupola and distributed in and around the bulla in the apex of the lung.
=== Genetics ===
* EDS forms part of a spectrum of genetically based connective tissue disorders that includes osteogenesis imperfect. Vascular Ehlers-Danlos syndrome (EDS IV) is characterized by an alteration in the COL3A1 gene. This gene encodes type III collagen and The alteration of this type of collagen produces aneurisms and ruptures of vessels and organs than can lead to haemothorax.
* Osler–Weber–Rendu disease, also known as hereditary hemorrhagic telangiectasia (HHT), is an autosomal dominant disease characterized by multiple cutaneous, systemic, and/or pulmonary arteriovenous malformations (AVMs). Bleeding from pulmonary lesions usually occur as haemoptysis and rarely as Spontaneous Haemothorax.
* there are other hereditary entities such as Loeys–Dietz syndrome, familial thoracic aortic aneurysm syndrome, or Shprintzen–Goldberg syndrome that are predisposed to aortic dissection and haemothorax.
* Costal exostosis or Osteochondroma is an autosomal dominant hereditary abnormality and the most common benign thoracic bone tumor. It often presents singly or in multiple sites that can cause laceration of the lung and hemothorax.
* Hemophilia A is a X-linked hereditary disorder of blood clotting that caused by the development of an inhibitor against coagulation factor VIII (FVIII). Hemophilia A manifests with early muscle and subcutaneous bleeding and rarely with haemothorax.
* Glanzmann thromboastenia is an autosomal-recessive bleeding disorder characterized by a lifelong bleeding tendency due to abnormalities of the platelet integrin αΠbβ3 [glycoprotein (GP) IIb; CD41/IIIa; CD61]. Glanzmann thromboastenia usually presents with mucocutaneous bleeding such as easy bruising, purpura, gingival bleeding, epistaxis, menorrhagia, haemarthrosis, haematuria, intracranial and visceral hemorrhage are rare but even rarer is Spontaneous Haemothorax.
* Type I neurofibromatosis (NF-1) or Von recklinghausen disease (VRD) is an autosomal dominant disease. This entity can affect any organ system, and is characterized by skin tumors and abnormal cutaneous pigmentation. Pathogenetic mechanisms for vasculopathy associated with VRD are: (I) direct vascular invasion from adjacent tumors; and (II) vascular dysplasia with thickening and concomitant reduced strength of the vessel wall and aneurysm formation.


==[[Hemothorax causes|Causes]]==
==[[Hemothorax causes|Causes]]==

Revision as of 20:18, 8 March 2018


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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: Hematothorax; haemothorax

Overview

Hemothorax as a clinico-pathological entity can be defined in two ways. Morphologically, it is a pathologic collection of blood within the pleural cavity, between the lung surface and inner chest wall. Clinically , hemothorax is defined as a pleural fluid with a hematocrit ranging from at least 25–50% of peripheral blood. In cases of long standing haemothorax due to haemodilution, hemothorax can appear with lower levels of hematocrit. massive hemothorax is defined as the drainage of more than 1500 cc of blood upon chest tube insertion.

Historical Perspective

Classification

Spontaneous haemothorax (SH) is a subcategory of haemothorax.

Pathophysiology

Pathogenesis

Three mechanisms of bleeding in haemothorax:

  • a torn adhesion between the parietal and visceral pleurae.
  • rupture of neovascularized bullae as a complication of subpleural emphysematous blebs.
  • torn congenital aberrant vessels branching from the cupola and distributed in and around the bulla in the apex of the lung.

Genetics

  • EDS forms part of a spectrum of genetically based connective tissue disorders that includes osteogenesis imperfect. Vascular Ehlers-Danlos syndrome (EDS IV) is characterized by an alteration in the COL3A1 gene. This gene encodes type III collagen and The alteration of this type of collagen produces aneurisms and ruptures of vessels and organs than can lead to haemothorax.
  • Osler–Weber–Rendu disease, also known as hereditary hemorrhagic telangiectasia (HHT), is an autosomal dominant disease characterized by multiple cutaneous, systemic, and/or pulmonary arteriovenous malformations (AVMs). Bleeding from pulmonary lesions usually occur as haemoptysis and rarely as Spontaneous Haemothorax.
  • there are other hereditary entities such as Loeys–Dietz syndrome, familial thoracic aortic aneurysm syndrome, or Shprintzen–Goldberg syndrome that are predisposed to aortic dissection and haemothorax.
  • Costal exostosis or Osteochondroma is an autosomal dominant hereditary abnormality and the most common benign thoracic bone tumor. It often presents singly or in multiple sites that can cause laceration of the lung and hemothorax.
  • Hemophilia A is a X-linked hereditary disorder of blood clotting that caused by the development of an inhibitor against coagulation factor VIII (FVIII). Hemophilia A manifests with early muscle and subcutaneous bleeding and rarely with haemothorax.
  • Glanzmann thromboastenia is an autosomal-recessive bleeding disorder characterized by a lifelong bleeding tendency due to abnormalities of the platelet integrin αΠbβ3 [glycoprotein (GP) IIb; CD41/IIIa; CD61]. Glanzmann thromboastenia usually presents with mucocutaneous bleeding such as easy bruising, purpura, gingival bleeding, epistaxis, menorrhagia, haemarthrosis, haematuria, intracranial and visceral hemorrhage are rare but even rarer is Spontaneous Haemothorax.
  • Type I neurofibromatosis (NF-1) or Von recklinghausen disease (VRD) is an autosomal dominant disease. This entity can affect any organ system, and is characterized by skin tumors and abnormal cutaneous pigmentation. Pathogenetic mechanisms for vasculopathy associated with VRD are: (I) direct vascular invasion from adjacent tumors; and (II) vascular dysplasia with thickening and concomitant reduced strength of the vessel wall and aneurysm formation.

Causes

Differentiating Hemothorax from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | Chest X Ray | CT | Echocardiography or Ultrasound | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1

Related Chapters

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