DRESS complicated by Haemophagocytic lymphohistiocytosis. Is screening required?

Abstract

A 49-year-old man was transferred to the Intensive Care Unit (ICU) at our centre from a local hospital, having suffered a spontaneous intracranial bleed requiring neurosurgical intervention. There was no past medical history and he was not taking any regular medication. The patient subsequently developed refractory seizures and received levetiracetam, phenytoin, sodium valproate, midazolam, and clobazam. The admission was complicated by a pneumonia, requiring vancomycin and piperacillin with tazobactam, later switching to meropenem. On Day 25 of admission he developed a morbilliform rash on the torso and limbs, generalised oedema, and persistent fevers of up to 42°C. Laboratory investigations demonstrated\xa0a rising eosinophil count (peaking at 1.3 x 109 /L; 10% of total leucocyte count), rising alanine aminotransferase (ALT) and declining renal function. Computed tomography revealed prominent, mildly enlarged mediastinal, axillary, pelvic and inguinal lymph nodes, up to 16mm in diameter. A diagnosis of drug reaction with eosinophilia and systemic symptoms (DRESS) was suspected, with the RegiSCAR scoring system (Table 1) giving our patient 7 points, indicating a definite case. This was supported by skin biopsy findings of a perivascular lymphocytic inflammatory infiltrate with interstitial eosinophils and intraluminal neutrophils (Figure 1a,b). A viral reactivation screen (which included EBV, HHV-6, HHV-7, HIV, HBV and HCV) was negative.