Calciphylaxis, occurring 10 weeks after hypercalcaemia, in apatient with multiple myeloma

A 52-year-old male patient presented to the emergency

department with a history of sudden onset back pain and

increasing confusion. Routine blood tests showed urea

31Æ5 mmol/l, creatinine 226 lmol/l, calcium 4Æ99 mmol/l,

phosphate 1Æ88 mmol/l and raised inflammatory markers

(white cell count 19Æ8 · 109/l, C-reactive protein 127 mg/l).

The patient was treated with intravenous antibiotics, high dose

corticosteroids and pamidronate but became increasingly

unwell and acidotic. He was transferred to the intensive

therapy unit for haemofiltration and required ventilation for

8 d. His calcium level returned to normal after 2 d. He was

found to have light chain myeloma with 1Æ98 g of kappa light

chains in a 24 h urine collection and the serum free light chain

(FLC) assay showed kappa chains >198 mg/l, lambda chains

8Æ9 mg/l. Following two courses of CTD (cyclophosphamide,

thalidomide and dexamethasone) chemotherapy, the urinary

protein had disappeared and the serum FLC assay was normal

(ratio = 0Æ75).

More than 10 weeks after the initial presentation, the patient

developed an exquisitely tender rash which began as violaceous

mottling on the lower back and progressed to a confluent

stellate purpura. Areas of necrosis developed in central areas

after several days (left). A clinical diagnosis of calciphylaxis was

made and intravenous sodium thiosulphate therapy was

initiated along with opiates for the pain. His condition

deteriorated and the patient died from metabolic complica-

tions and sepsis. Post-mortem skin biopsy confirmed the

diagnosis of calciphylaxis. An arteriole with a ring of purple

calcification and intimal hyperplasia is shown in the histolog-

ical sample (right, haematoxylin and eosin stain).

Calciphylaxis is a rare syndrome of calcification of small

arterioles leading to skin necrosis and, in some patients, organ

failure. It is frequently fatal. It is usually seen in patients with

end-stage renal failure, although it has been described in

hyperparathyroidism, breast cancer, cirrhosis, Crohn disease

and collagen diseases. This case is unusual in that the

calciphylaxis occurred 12 weeks after resolution of the hyper-

calcaemia and hyperphosphataemia. There is no specific

treatment but tissue plasminogen activator and chelation with

sodium thiosulphate have been tried with variable success.

Emily Sullivan1

Christine Hoyle2

1Renal Medicine, Wrexham Maelor Hospital, Betsi Cadwaladr University

Hospital Trust, Wrexham, Clwyd, and 2Department of Haematology,

Glan Clwyd Hospital, Betsi Cadwaladr University Hospital Trust, Rhyl,

Denbighshire, UK.

E-mail: Christine.Hoyle@wales.nhs.uk

images in haematology

First published online 24 May 2011doi:10.1111/j.1365-2141.2011.08751.x ª 2011 Blackwell Publishing Ltd, British Journal of Haematology, 155, 136