Cytokine nephropathy and multi-organ dysfunction in lymphoma

however, his liver extended 4 cm below the right costalmargin and the spleen was just palpable. Urine outputWe report two cases of renal, hepatic and respiratory was 20 ml/h with only red cells detected in his urine,failure occurring with the haemophagocytic syndrome of which <10% were dysmorphic. His biochemistry(HPS) in association with occult peripheral T-cell showed severely impaired renal function (urealymphomas. The haemophagocytic syndrome has 56.2 mmol/l, creatinine 692 mmol/l, sodium 112 mmol/lrecently been recognised to occur in association with potassium 4.0 mmol/l). Liver function tests demon-massive cytokine release, however there have been no strated markedly elevated enzymes but moderatelydocumented reports of hypercytokinaemia occurring preserved synthetic function (bilirubin 512 mmol/l,together with acute renal or hepatic failure. In both AST 406 U/l, ALT 260 U/l, ALP 310 U/l, albuminthese cases the renal failure was secondary to an acute 32 g/l, INR 1.0). His ESR was normal as was a fulltubular necrosis-like lesion, together with severe inter- liver screen including hepatitis viral serology. Serumstitial oedema, but in the absence of either glomerular iron was 27 mmol/l and TIBC 9 l but ferritin waspathology or cellular inltration. In addition there was markedly elevated at 21250 mg/l. In addition LDHno lymphomatous inltration of the liver to explain was high at 2801 U /l and triglycerides were 5.2 mmol l.the hepatic failure. These ndings occurred in the Clotting studies including brinogen and x-linkedsetting of very high cytokine levels and in the absence degradation products were within the normal range.of any obvious ischaemic or toxic insults. Blood cultures, urine, and throat swabs grew no organ-We suggest that the hypercytokinaemia may be a isms. In addition an extensive serological screen fordirect cause of the renal, hepatic and pulmonary fail- infections was negative including Epstein–Barr virusure, and present the two cases together with evidence (EBV) serology. In addition he had a negative myco-supporting this hypothesis. bacterial screen including Mantoux test, CSF, andbone marrow examinations. Autoantibodies were allnegative; however, both C3 and C4 were mildly reduced