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.
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.
Henter JI, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007; 48:124.
Disclosure of Interest None Declared
Statistics from Altmetric.com
If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.