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Case History

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Image not available. A 25-year-old man is admitted via Accident and Emergency complaining of a 2-week history of skin rash, headache and palpitations. A full blood count reveals him to have a haemoglobin level of 13 g/dl, a raised white cell count of 28 × 109/l, with 25 × 109/l eosinophils, and platelet count of 175 × 109/l.

What should be your approach to investigation and management?

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Background

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Image not available. This patient presents with fairly non-specific symptoms but a marked peripheral blood eosinophilia. In considering causes of eosinophilia, one first has to consider reactive causes. These may be relatively common, such as:

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  • a drug reaction, particularly to gold, sulphonamides and antibiotics;

  • parasitic infections, including hookworm, filariasis, amoebiasis and schistosomiasis;

  • atopy – asthma, eczema, etc.

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Less common causes would be:

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  • skin diseases such as pemphigus and dermatitis herpetiformis;

  • connective tissue diseases such as polyarteritis nodosa;

  • lymphomas including Hodgkin lymphoma.

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However, there are a number of primary causes of overproduction of eosinophils that are relatively rare but probably relevant to this case by dint of the high eosinophil count. These include:

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  • hypereosinophilic syndrome (HES);

  • chronic eosinophilic leukaemia (CEL);

  • acute myeloid leukaemia with eosinophilia (M4Eo);

  • chronic myeloid leukaemia.

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A full history and examination is required, particularly concentrating on drug history, history of allergic conditions and, to assess the risk of parasite infection, potential exposure during foreign travel. Clinical examination should identify evidence of dermatological disorders, connective tissue disease and lymphoproliferative disorders. Investigations should include blood film examination, stool analysis to exclude parasitic infection, skin biopsy in dermatological conditions and lymph node biopsy or bone marrow examination in suspected lymphoproliferative disorders.

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The level of eosinophil count in this case means that HES or an eosinophilic leukaemia should be considered. The former can be defined as:1

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  • persistent eosinophilia of >1.5 × 109/l for at least 6 weeks;

  • lack of evidence of parasitic, allergic or other known cause;

  • signs and symptoms of organ involvement.

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In cases of HES, the male to female ratio is 9:1 with occurrence at a peak age of 20–50 years. The condition may be associated with significant end-organ damage particularly cardiac abnormalities. His symptoms of palpitations may be relevant in this regard.

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Organ involvement in hypereosinophilic syndrome

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Clinical history and examination should identify some of the more common organ-specific syndromes associated with HES, notably cardiovascular involvement including cardiomyopathy, restrictive pericarditis, endomyocarditis and occasional mural thrombi and endomyocardial fibrosis. Patients may also give a history of dermatological abnormalities with angio-oedema, urticaria and occasionally papules, nodules and plaques. Neurological abnormalities reported include peripheral neuropathy, encephalopathy and eosinophilic meningitis; pulmonary involvement (which needs to be distinguished from asthma-related complications in reactive eosinophilia) includes pulmonary infiltrates, effusions and fibrosis. A number of ...

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