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A patient with rheumatoid arthritis, cryoglobulinaemia, and an “accidental” finding

BMJ 2014; 348 doi: https://doi.org/10.1136/bmj.g2701 (Published 14 April 2014) Cite this as: BMJ 2014;348:g2701
  1. Konstantinos Liapis, clinical fellow in haematology1,
  2. Yooyun Chung, foundation year 2 doctor2,
  3. Waqas Akhtar, core medical trainee year 21,
  4. David Taussig, consultant haematologist1
  1. 1Department of Haematological Oncology, St Bartholomew’s Hospital, Barts and the London NHS Trust, London EC1A 7BE, UK
  2. 2Trauma Centre, Royal London Hospital, Barts and the London NHS Trust, London, UK
  1. Correspondence to: K Liapis kosliapis{at}hotmail.com

A 60 year old woman, originally from Greece, was admitted after burning herself while trying to warm her legs on an electric heater. She had rheumatoid arthritis treated with methotrexate and adalimumab.

She had been well until four months earlier, when pain and stiffness developed in her metacarpophalangeal and interphalangeal joints, right ankle, and both knees. Six weeks before admission, she presented with severe arthralgia, a temperature of 37.6°C, a net-like rash on her legs compatible with livedo reticularis, and purpuric lesions around the malleoli. With the warming of her legs, the rash temporarily improved. Laboratory examinations at that time showed haemoglobin 109 g/L (reference range 130-170), leucocytes 2.9×109/L (4-10×109), neutrophils 1.2×109/L (2-7×109), platelets 170×109/L (150-410×109), rheumatoid factor 183 IU/mL (<20), hypergammaglobulinaemia, antinuclear antibodies 1:640 (<1:80), complement C4 0.04 g/L (0.14-0.54), anti-double stranded DNA antibody 8.6 IU/mL (0.0-7.0), negative antibodies to extractable nuclear antigens, and a positive test for cryoglobulins. Additional tests showed that the cryoglobulins were composed of polyclonal IgM, IgG, and IgA. Serological tests for HIV, hepatitis B, and hepatitis C viruses were negative.

On admission, fever (38.7°C) and pancytopenia (leucocytes 1.8×109/L, neutrophils 0.5×109/L, haemoglobin 90 g/L, platelets 126×109/L) were noted. She had no hepatosplenomegaly or lymphadenopathy. A blood film was unremarkable. Broad spectrum antibiotics were started empirically because of the possibility of neutropenic sepsis, but no improvement was seen. Cultures of blood, urine, and sputum; computed tomography of the chest, abdomen, and pelvis; a polymerase chain reaction assay of the peripheral blood for cytomegalovirus, and a tuberculin skin test were negative. A detailed history identified that she had travelled to Athens seven months earlier and had stayed with relatives who kept several dogs. For diagnostic evaluation of pancytopenia, she underwent bone marrow …

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