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Practice Guidelines

Management of an acute painful sickle cell episode in hospital: summary of NICE guidance

BMJ 2012; 344 doi: https://doi.org/10.1136/bmj.e4063 (Published 27 June 2012) Cite this as: BMJ 2012;344:e4063

Re: Management of an acute painful sickle cell episode in hospital: summary of NICE guidance

Sir,

Gillis et al’s summary of NICE guidance on sickle cell crises offers a very holistic approach to this medical emergency. In addition to the expected advice about clinical management, especially analgesia, the importance of “psychological and/or social support” is emphasised.

However, despite addressing the wide range of issues this challenging condition poses, NICE guidance appears to have neglected an important infectious cause of a “sickle cell crisis”. In particular, one should be mindful of the possibility of Parvovirus B19 infection, also known as “Slapped Cheek Disease” or erythema infectiosum.

This single-stranded DNA virus targets erythroid progenitor cells. Thus, in an acute sickle cell crisis, the clue is a low, or inappropriately low-normal reticulocyte count in the face of the typically profound anaemia of the sickle cell crisis.

Whilst “normal” children usually manifest the “slapped cheek appearance” which gives this condition its common name, adults will often have variable combinations of a non-specific erythematous rash, arthralgia, myalgia and flu-like symptoms.

Parvovirus B19 is a well-known precipitant of aplastic crises in patients with sickle cell disease patients (and patients with other haemoglobinopathies)[2,3,4]. This may seem like a small-print condition, but in fact the UK is currently experiencing a period of high Parvovirus B19 activity[5].

When patients with sickle cell disease and other hereditary haemoglobinopathies present with an acute aplastic crises e.g. a sickle cell crisis with no or a surprisingly low reticulocytosis, Parvovirus B19 infection should be considered. Appropriate serological investigations should be carried out.

If, in such a scenario, Parvovirus B19 infection is not suspected, there is a risk of nosocomial transmission of Parvovirus B19[6]. This is an avoidable risk; as the famous microbiologist Louis Pasteur (1822-1895) once said: “fortune favours the prepared mind”.

References:

1. Gillis VL et al. Management of an acute painful sickle cell episode in hospital: summary of NICE guidance. BMJ 2012;344 doi: 10.1136/bmj.e4063
2. Saarinen UM et al. Human Parvovirus B19-induced epidemic acute red cell aplasia in patients with hereditary hemolytic anemia. Blood 1986;67:1411-1417
3. Setubai S et al. Aplastic crisis caused by parvovirus B19 in an adult patient with sickle cell disease. Rev Soc Bras Med Trop 2000;33(5):477-481
4. Regaya F et al. Parvovirus b19 infection in Tunisian patients with sickle cell anemia and acute erythroblastopenia. BMC Infect Dis 2007;7:123 doi :10.1186/1471-2334-7-123
5. Health Protection Report HPA vol 6, No. 11. 16 June 2012 http://www.hpa.org.uk/Topics/InfectiousDiseases/InfectionsAZ/ParvovirusB19/ (link to pdf, accessed 24/7/12)
6. Bell LM et al. Human Parvovirus B19 infection amongst hospital staff members after contact with infected patients. NEJM 1989;321(8):485-91

Competing interests: No competing interests

24 July 2012
Gee Yen Shin
Consultant Virologist
St. George's Healthcare NHS Trust
Department of Microbiology, St. George's Hospital, Blackshaw Road, London SW17 0QT