Occupational Medicine Advance Access originally published online on March 21, 2008
Occupational Medicine 2008 58(3):219-221; doi:10.1093/occmed/kqn031
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Case Report |
Cold haemagglutinin disease misdiagnosed as hand–arm vibration syndrome
Correspondence to: Dr C. J. M. Poole, Occupational Health Service, Dudley NHS PCT, Health Centre, Cross Street, Dudley, West Midlands DY1 1RN, UK. Tel: +44 1384 366270; fax: +44 1384 366422; e-mail: jon.poole{at}dudley.nhs.uk
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Abstract |
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Abstract A patient with a diagnosis of hand–arm vibration syndrome was referred for a second opinion. He worked as a multi-skilled operative in the housing department of a local authority, a job not normally associated with high levels of exposure to hand-transmitted vibration (>2.5 m/s2 A(8)). He described blanching of his fingers and a blue discolouration of his extremities in cold weather. On examination, his fingertips, toes and pinnae were acrocyanotic, the fingers were patchily pale and sensation was subjectively impaired in all of the digits. Investigations revealed a haemolytic anaemia and haemagglutination. He was diagnosed with idiopathic cold haemagglutinin disease. Exposure to vibration may confound with exposure to cold in which case the differential diagnoses of cold haemagglutinin disease or cryoglobulinaemia should be excluded before diagnosing hand–arm vibration syndrome.
Keywords Acrocyanosis; cold haemagglutinin disease; hand–arm vibration syndrome
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Introduction |
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Hand–arm vibration syndrome (HAVS, vibration white finger) is currently the most commonly Prescribed Industrial Disease [1] and the subject of a considerable amount of civil litigation in the UK. The diagnosis of HAVS is based on specific criteria being met: (i) evidence of sufficient exposure to vibration; (ii) confirmation of episodic pallor of the digits and/or sensorineural effects and (iii) consideration of other causes of Raynaud's phenomenon or sensory changes [2].
Although help is provided for the calculation of vibration exposure [3], historical measurements of exposure may not be available in which case judgements about exposures in relation to exposure limit values will need to be made by a competent person. Photographic evidence of blanching, standardised testing with a thermal aesthesiometer, vibrometer, cold provocation and nerve conduction tests [4,5] will help to confirm the presence of digital sensorineural or vascular dysfunction, but will not identify a cause for the symptoms. These include primary and secondary Raynaud's phenomenon for which there is a wide range of causes to include hyperviscosity syndromes of the blood [6,7].
Cold haemagglutinin disease (CHD) is a haemolytic anaemia caused by autoantibodies, typically IgM, binding to erythrocytes and activating complement, particularly at cold temperatures to cause haemagglutination. It is most commonly associated with an infection such as infectious mononucleosis or mycoplasma pneumonia or with a lymphoproliferative disease such as lymphoma or leukaemia but may also be idiopathic [8]. It has also been associated with necrotic skin lesions in acral parts of the body [9].
Cryoglobulinaemia is due to immunoglobulins (IgG or IgM, or a mixture of the two) precipitating in the serum at cold temperatures and redissolving on rewarming, usually in association with a vasculitis, a lymphoproliferative disease or an infection such as hepatitis C [10]. Both CHD and cryoglobulinaemia may occur simultaneously in the same patient [8]. Vibrating tools are often used in cold temperatures, which may therefore be a confounding factor for any disease attributed to vibration.
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Case report |
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A 59-year-old multi-skilled local authority craftsman diagnosed with HAVS was referred for a second opinion. He had done this work for 18 years, having previously been a bricklayer and a lorry driver. His work involved the periodic use of powered drills, grinders, saws and concrete breakers but not for prolonged periods of time with acceleration greater than the exposure action value of 2.5 m/s2 A(8).
He described patchy blanching of his fingers and a blue discolouration of his extremities particularly in cold weather for 
2 years. Other than hypertension, for which he took amlodipine and irbesartan, and poor hearing his health had been good. He smoked 10 cigarettes per day. On examination in a warm consulting room in May his fingertips, toes and pinnae were acrocyanotic. His fingers were patchily blanched but without clear edges of demarcation. Sensation to light touch, pin prick and vibration was subjectively lost in all the fingers of both hands. Adson's test was negative. He wore a hearing aid. Cardiovascular and general examination was otherwise normal.
Investigations revealed haemoglobin 10.5 g/dl, a reticulocytosis of 158 x 109 per litre (6.8%), a positive direct Coombs test due to complement C3d with marked red cell agglutination, a thrombocytosis of 518 x 109 per litre and a raised serum IgM of 2.77 g/l of lambda type by electrophoresis. Titres of cold agglutinins were 1 : 64 at 37°C, 1 : 65 856 at room temperature (22°C) and 1 : 131 712 at 4°C. His serum bilirubin was raised at 81 µmol/l, all unconjugated, as was lactate dehydrogenase at 1015 IU/l. Cryoglobulins were not detected. Antinuclear antibodies were negative and rheumatoid factor was weakly positive. Dipstick testing for haem was negative. A chest X-ray was normal.
He was diagnosed with idiopathic CHD and advised to keep warm in cold weather. He was also redeployed to an inside storeman's job and given gloves to wear. Haemolysis and haemagglutination at low temperatures is notoriously difficult to treat; however, success with the monoclonal antibody rituximab has been reported [11]. Treatment was tried in this case but without success. The presumed reactive thrombocytosis was treated with low-dose aspirin. He remains under the care of a haematologist for monitoring of the anaemia and early identification of an aetiology for the CHD such as a lymphoproliferative disease.
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Discussion |
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Many jobs involving exposure to vibration are performed outside or in cold environments. Exposure to cold may aggravate or trigger secondary causes of Raynaud's phenomenon other than hand-transmitted vibration, such as CHD or cryoglobulinaemia. In such cases cold acts as a confounding factor that may result in a misdiagnosis of HAVS. The distinction is important not just for security of diagnosis but also for treatment. The anaemia in this case was mild with no effect on day-to-day capabilities, but it may be severe or even absent and vary according to the time of year. The infusion of cold fluids should also be avoided in these patients.
CHD and cryoglobulinaemia may be distinguishable from HAVS by the history of patchy blanching and acrocyanosis, in contrast to the well-demarcated edges and triphasic response (pallor, cyanosis and reactive hyperaemia) of Raynaud's phenomenon due to HAVS. The blue–purple discolouration of acrocyanosis is normally associated hypoxia from poor peripheral circulation but in this case it was due to haemagglutination in the peripheral capillaries.
Vibration exposure can now be calculated and should form part of the risk assessment under the Control of Vibration at Work Regulations. When exposure data are unavailable or exposure is historical, occupational physicians should have the competency to estimate whether the exposure action value is likely to have been exceeded for a particular job or jobs for a sufficient length of time to cause HAVS. It is possible that this patient had a degree of HAVS but any evaluation with standardised neurosensory or vascular testing would not have helped with diagnosis.
Raynaud's phenomenon has an incidence of 5 to 30% in the general population [6], so if the diagnosis of HAVS needs to be made with any degree of certainty, as opposed to on a balance of probability, then CHD and cryoglobulinaemia should be excluded in the workup of the patient. To do this it is recommended that samples of blood be taken from the patient for a full blood count with a film for haemolysis and agglutination identification, as well as a separate sample of plasma for cryoglobulin screening.
Key points
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None declared.
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References |
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- www.hse.gov.uk/statistics/causdis/vibration/index.htm.
- Aw TC, McCaig R. Vibration. In: Oxford Textbook of Medicine—Warrell DA, Cox TM, Firth JD, Benz EJ, eds. (2003) 4th edn. Oxford: Oxford University Press.
- www.hse.gov.uk/vibration.
- Lawson IJ, Nevell DA. Review of objective tests for the hand-arm vibration syndrome. Occup Med (Lond) (1997) 47:15–20.[CrossRef][Medline]
- Pelmear PL. The clinical assessment of hand-arm vibration syndrome. Occup Med (Lond) (2003) 53:337–341.[CrossRef][Medline]
- Faculty of Occupational Medicine Working Party. Hand-Transmitted Vibration: Part 1 (1993) London: The Royal College of Physicians of London.
- Dowd PM. Reactions to cold, Raynaud's phenomenon. In: Rook's Textbook of Dermatology—Burns T, Breathnachs S, Cox N, Griffiths C, eds. (2004) 2, 7th edn. Massachusetts: Blackwell Science Ltd. 12–16.
- Petz LD, Garratty G. Immune Hemolytic Anaemias (2004) 2nd edn. Philadelphia: Churchill Livingstone.
- Porras-Luque JI, Fernandez-Herrera J, Dauden E, et al. Cutaneous necrosis by cold agglutinins associated with glomeruloid reactive angioendotheliomatosis. Br J Dermatol (1998) 139:1068–1072.[CrossRef][Web of Science][Medline]
- Dammacco F, Sansonno D, Piccoli C, Tucci FA, Racanelli V. The cryoglobulins: an overview. Eur J Clin Invest (2001) 31:628–638.[CrossRef][Web of Science][Medline]
- Engelhardt M, Jakob A, Ruter B, et al. Severe cold haemagglutinin disease (CHD) successfully treated with rituximab. Blood (2002) 100:1922–1923.
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