Kidney International Reports | 2021
POS-248 Hemophagocytic lymphohistiocytosis:a retrospective study from the nephrology department of Monastir
Abstract
Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening condition due to inappropriate survival of histiocytes and cytotoxic T cells, leading to a cytokine storm, haemophagocytosis and multi-organ damage It can be inherited and thus termed familial HLH or secondary (sHLH) triggered in such a way by malignancy, infection or autoimmunity It has a wide range of symptoms and biological features leading to diagnosis and management challenges sHLH patient’s prognosis is poor especially in those with multiple chronic disease The aim of our work is to describe clinical and biological features of nephrology patients with sHLH We will also report the results of our proposed therapeutic protocol Methods: A retrospective descriptive study was carried out on 13 patients admitted in our nephrology department between 2017 and 2020 The diagnosis of HLH was accomplished according to H-score Results: There were 13 patients included, 61 5 % men and 38 5 % women The median age of the patients was 53 years with extremes ranging from 17 to 83 only one patient had normal renal function but proteinuria For the rest: 38 % were dialyzed, 23 % had altered chronic renal function and 30 % had acute renal failure The median H-score was 221 23 with extremes ranging from 140 to 282 The onset of symptoms was brutal in all patients The clinical presentation was dominated by fever and deterioration of general state Hepatosplenomegaly was found in 30 7% of cases and peripheral lymph nodes in 8 % of patients Biological findings constantly revealed an increase of inflammatory markers In complete blood count, there were pancytopenia in 30% of patients and bicytopenia in 69% of patients Hyperferritinemia was constant in all patients as well as hypertriglyceridemia Cytological examination of bone marrow revealed hemophagocytosis in 92% of cases sHLH was predominantly associated with infectious diseases: 92% of cases;infections were bacterial in 50 % of cases, Cytomegalovirus infection in 16 %, COVID-19 in 16 %, Epstein Barr virus infection in 8 % and systemic candida albicans infection in 8 % of cases We also noted one case of lymphoma In addition to antibacterial drugs in infected patients and chemotherapy for the patient with lymphoma, all patients were administered immunoglobulin at a dose of 2g per kilogram and 15 % of patients received Etoposide as second line therapy The evolution was favorable in 61 % of patients with total regression of clinical and biological abnormalities The rest of our patients died due to multiple organ failure Conclusions: sHLH in adults is a rare disease, its mimicry with other conditions leads to its under-recognition sHLH has a broad range of triggers including infection, malignancy and autoimmunity The diagnosis of such a fatal condition is lying at the boundaries of multiple specialties, so clinicians must be aware of the possibility of sHLH in at-risk patients under their care Prompt treatment is the key factor to improve the mortality and morbidity associated with this disease No conflict of interest