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.
References
Henter JI, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007; 48:124.
Disclosure of Interest
None Declared
Background
With the arrival of the SARS-CoV-2 pandemic in 2020, it was proposed to make the change from intravenous (IV) tocilizumab (TCZ) to its subcutaneous formulation, in order to avoid rheumatological patients having to go to the day hospital and guarantee enough IV TCZ for those critical patients with COVID who needed it. The aim of this study was to describe the rate and reasons for switching back to IV TCZ from subcutaneous TCZ.
Methods
We included patients from the rheumatology service that were on treatment with IV TCZ in February 2020 and were followed up until March 2021. Patients that remained on subcutaneous TCZ were compared with those who switched back to IV TCZ (switch-back group). A subgroup analysis according to rheumatic disease was performed.
Results
Fifty-five patients switched to subcutaneous TCZ: 28 rheumatoid arthritis, 19 giant cell arteritis, 4 polymyalgia rheumatica, 2 juvenile idiopathic arthritis, and 2 systemic sclerosis. Seventeen patients switched back to IV TCZ due to ineffectiveness (n = 8), patient preference (n = 4), adverse events (n = 4), and difficulty with the SC administration route (n = 1). In the analysis by disease, 4 of 23 patients switched back to IV TCZ in giant cell arteritis/polymyalgia rheumatica group due to ineffectiveness (n = 2), injection site reaction (n = 1), or patient preference (n = 1). In rheumatoid arthritis group, 11 of 28 patients switched back to IV TCZ: ineffectiveness (n = 5), patient preference (n = 3), headache (n = 1), injection site reaction (n = 1), and due to difficulty with the SC administration route (n = 1).
Conclusions
Mass switch from IV to subcutaneous TCZ during the SARS-CoV-2 pandemic has been safe, effective, and well tolerated after 1 year of follow-up.
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