Autoinflammatory constrictive pericarditis and chronic myelomonocytic leukaemia: when one speciality is not enough.
BMJ Case Rep
; 12(3)2019 Mar 07.
Article
em En
| MEDLINE
| ID: mdl-30850566
ABSTRACT
We present a case of constrictive pericarditis with concomitant blood and bone marrow appearances of chronic myelomonocytic leukaemia (CMML). Despite surgical treatment with pericardiectomy, the patient deteriorated into multiorgan failure. Pericardial histology disclosed a typical inflammatory picture with no evidence of monocytic or malignant infiltrate. Following intensive collaboration between cardiologists, haematologists and rheumatologists via daily email exchanges, a diagnosis was reached of autoinflammatory constrictive pericarditis with a non-infiltrative coexisting CMML. The key to achieving a rapid and sustained response was a trial of high-dose steroids followed by intravenous immunoglobulins. This achieved restoration of cardiac function, resolution of symptoms and near normalisation of inflammatory markers. A diagnosis of concurrent CMML was confirmed at 3 months. The patient remains well, taking colchicine and steroids.
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Base de dados:
MEDLINE
Assunto principal:
Pericardite Constritiva
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Pericardiectomia
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Leucemia Mielomonocítica Crônica
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Doenças Hereditárias Autoinflamatórias
Idioma:
En
Ano de publicação:
2019
Tipo de documento:
Article