Background Hemophagocytic Lymphohistiocytosis (HLH) is a severe and acute inflammatory syndrome, underdiagnosed, difficult to treat and can occur at any stage of life. The macrophage activation syndrome is a variant of secondary HLH occurs in autoimmune diseases.¹

Objectives Describe the clinical, laboratory, treatment and outcome of patients diagnosed with secondary HLH, identifying probable etiological and triggers causes.

Methods A retrospective study was performed between 2008 and 2012 at the Donostia University Hospital, Spain. Inclusion criteria were to met diagnostic criteria for HLH and had a bone marrow biopsy with hemophagocytics cells. Mains endpoints were: demographics, diagnostic criteria, probable etiology, triggers causes and treatments. Secondary endpoints were: time delay in diagnosis, days of hospitalization, need for admission to the Intensive Care Unit (I.C.U.) and the cause, and overall mortality.

Results We recruited 11 patients (7 men and 4 women) with a mean age of 48.9 years (16 - 78 years). The below table describes the probable etiologies and triggers of secondary HLH. As no data in the literature described, we found as etiologies and triggers causes of secondary HLH: Campylobacter jejuni in a previously healthy patient without comorbidities; Pneumocystis jirovecii in a patient with Human immunodeficiency virus (H.I.V.); and patient with grade IV glioblastoma multiforme after starting chemotherapy with temozolomide. Hemophagocytics cells were found in ascitic fluid in one patient with Adult Still´s Disease. The mean delay in diagnosis was 14.5 days (3 – 31 days) and average time of hospitalization was 46.7 days (10 – 130 days). The 45% of patient required admission in I.C.U, the leading cause was the multiple organ failure (MOF). The overall mortality rate was 36.4% (4 MOF and 1 massive hemoptysis). Treatment given was steroids, synthetic immunosuppressants and biological drugs.

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Conclusions The secondary HLH should be suspected in any patient with prolonged fever unresponsive to broad-spectrum antibiotics, hepatosplenomegaly, cytopenias, coagulation and liver disorders. Hemophagocytics cells might be found in pathological body fluids before a bone marrow biopsy had been done or when there are doubts in the result of it. Meet all the diagnostic criteria is not necessary to start treatment when you have a high clinical suspicion and a bone marrow biopsy with hemophagocytics cells. Mortality can be influenced by etiology, trigger cause, and diagnosis and treatment delay.

References

1. Henter JI, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007; 48:124.

Disclosure of Interest None Declared