Pulmonary capillary hemangiomatosis: a lesson learned
Autops. Case Rep
; 9(3): e2019111, July-Sept. 2019. ilus
Artigo
em Inglês
| LILACS
| ID: biblio-1016910
Biblioteca responsável:
BR26.7
ABSTRACT
Pulmonary capillary hemangiomatosis (PCH) is a rare and controversial entity that is known to be a cause of pulmonary hypertension and is microscopically characterized by proliferation of dilated capillary-sized channels along and in the alveolar walls. Clinically, it is mostly seen in adults. Clinical features are characterized by nonspecific findings such as shortness of breath, cough, chest pain, and fatigue. It can be clinically indistinguishable from pre-capillary pulmonary arterial hypertension disorders such as primary pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension. However, the diagnostic distinction, which usually requires a multidisciplinary approach, is crucial in order to avoid inappropriate treatment with vasodilator medications usually used for PAH treatment. Prognosis of PCH remains poor with lung transplant being the only definitive treatment. We report an autopsy case of pulmonary capillary hemangiomatosis unmasked at autopsy that was treated with a prostacyclin analog, usually contraindicated in such patients. We emphasize that this entity should always be on the differential diagnosis in a patient with pulmonary hypertension and requires great vigilance on the part of the clinician, radiologist and pathologist to make the diagnosis and guide appropriate management.
Texto completo:
Disponível
Coleções:
Bases de dados internacionais
Base de dados:
LILACS
Assunto principal:
Hemangioma Capilar
Tipo de estudo:
Estudo diagnóstico
/
Estudo prognóstico
Limite:
Idoso
/
Feminino
/
Humanos
Idioma:
Inglês
Revista:
Autops. Case Rep
Assunto da revista:
Anatomia
/
Patologia Cl¡nica
/
Patologia Legal
Ano de publicação:
2019
Tipo de documento:
Artigo
País de afiliação:
Israel
/
Paquistão
/
Estados Unidos
Instituição/País de afiliação:
Cedars Sinai Medical Center/US
/
Foundation University Medical College/PK
/
Tel Aviv University/IL