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- malignant disease and immunosuppression
- malignant and benign haematology
- oncology
- haematology (incl blood transfusion)
Description
A 60-year-old Caucasian man with untreated chronic lymphocytic leukaemia presented with a 3-month history of recurrent arthralgia and joint swelling, predominantly affecting the knee, wrist and DIP joints. He subsequently developed painful, dusky fingers and was referred to the haematology department at the Royal Free Hospital for investigation (figure 1).
On admission, a full blood count revealed: Hb 92 g/L, white cell count 86.7×109/L with a lymphocyte differential of 77.2×109/L and platelets 332. Renal, liver and bone profiles within normal ranges. Cryoglobulins were detected, and subsequent immunofixation showed the presence of a type 1, monoclonal IgM lambda cryoglobulin.
Before treatment.
The patient was managed with a continuous iloprost infusion, high dose corticosteroid therapy, plasmapharesis, rituximab and subsequently a dose of cyclophosphamide. On discharge, he was referred to University College Hospital for long-term management of CLL. He was referred to plastic surgery for amputation of affected digits (figure 2).
After treatment, showing halting of necrosis.
Type 1 cryoglobulinaemia is a rare disorder characterised by monoclonal production of immunoglobulins that precipitate at cold temperatures and can cause microvascular damage. The damage in this case led to end tissue damage and significant changes to the patient’s quality of life.1 It can manifest in a number of ways, and it is therefore important to appreciate the accompanying underlying disorders that can drive it.
Learning points
Type 1 cryoglobulinaemia is a rare disorder characterised by monoclonal production of immunoglobulins that precipitate at cold temperatures and can cause microvascular damage.
It makes up 10%–15% of cryoglobulinaemias and is typically associated with lymphoproliferative disorders.
Response to treatment is often monitored using cryocrit percentage or paraprotein quantification.
Footnotes
Contributors JM: senior house officer. JS: foundation year 1 doctor. All research undertaken at The Royal Free Hospital. Both authors contributed equally to all aspects of planning, writing and designing the attached report.
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.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.