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Autoinflammatory constrictive pericarditis and chronic myelomonocytic leukaemia: when one speciality is not enough
  1. Frances Varian1,
  2. Harpreet Kaur2,
  3. Stuart Carter3 and
  4. Julian Gunn4
  1. 1 Department of Cardiology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK
  2. 2 Department of Haematology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK
  3. 3 Department of Rheumatology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK
  4. 4 Cardiovascular Research Unit, Sheffield Teaching Hospitals and University of Sheffield, Sheffield, UK
  1. Correspondence to Dr Frances Varian, fvarian{at}


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.

  • cardiovascular medicine
  • heart failure
  • pericardial disease
  • chronic myeloid leukaemia

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  • Contributors FLV wrote the first draft and prepared the figures. HK was the haematologist who performed the genetic tests for the diagnosis of CMML, and reviewed the manuscript and the figures and provided investigation results. SC was the rheumatologist involved in the diagnosis and acute and ongoing treatment of the condition and reviewed the manuscript. JG was the cardiologist and supervising consultant for FLV and oversaw the entire process and the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Patient consent for publication Obtained.