Reactive haemophagocytic lymphohistiocytosis: a frequent finding in immunosuppressed patients with multiple organ failure

Reactice haemophagocytic lymphohistiocytosis (HLH) is a highly underdiagnosed condition in Adult Intensive Care. It is characterized by a non-malignant proliferation of histiocytes with phagocytosis of haematopoietic cells, resulting in cytopenia of at least two cell lines. The acquired form of HLH has been associated with infections, neoplasms, autoimmune diseases and immunosuppression.

Methods

Retrospective review of 15 patients who developed reactive HLH during their stay in the Liver Intensive Care Unit between January and November 2000. Predictors for survival were calculated using a non-parametric Mann-Whitney U-test.

Results

Admission diagnosis was fulminant hepatic failure in six patients, two patients presented with sepsis and chronic liver disease and seven patients following liver transplantation. All patients were in multiple organ failure (MOF) and severely thrombocytopenic (median 32 × 10⁵), despite platelet support in 13 patients, at the time of diagnosis. Twelve patients received immunosuppressive medication. Among the post transplant patients, six tested positive for CMV DNA and were treated with Ganciclovir. Ten patients died, two are still requiring intensive care treatment and three have been discharged from ITU (two from hospital). Survivors had significant lower SOFA scores at the time of HLH diagnosis, a significant higher, unsupported platelet count 7 days after diagnosis and high dose gamma-globulin therapy and a trend towards a shorter stay on mechanical ventilation (Table - all parameters median and range).

Table

Conclusion

Reactive HLH should be suspected and excluded in all thrombocytopenic patients with MOF. This is especially true for the immunosuppressed post transplant population. The diagnosis of HLH in these patients is associated with a poor prognosis.