Abstract
ABSTRACTHemophagocytic syndrome or hemophagocytic lymphohistiocytosis (HLH) is an infrequent and underdiagnosed condition caused by an overactive immune response, resulting in blood cells phagocytosis. After kidney transplantation (KTx), HLH is usually secondary (or reactive) to infectious and neoplastic processes and has a high mortality rate. No effective treatment is available for this condition. Usual procedures include detecting and treating the pathology triggering the immune system dysregulation, other than administration of intravenous human immunoglobulin (IVIG) and high doses of steroids, and plasmapheresis. The best protocol for maintenance immunosuppressive therapy is also unknown. This article presents two cases of post-KTx reactive HLH that underwent adjuvant IVIG treatment and obtained good clinical results. Despite the high morbidity and mortality associated with reactive HLH after KTx, the early and precise diagnosis and the administration of IVIG therapy along with the treatment of the triggering disease, was an effective strategy to control HLH.
Highlights
IntroductionHemophagocytic lymphohistiocytosis (HLH) syndrome is a rare, underdiagnosed, and severe condition caused by an overactive immune response, resulting in phagocytosis of blood cells
Hemophagocytic syndrome or hemophagocytic lymphohistiocytosis (HLH) is an infrequent and underdiagnosed condition caused by an overactive immune response, resulting in blood cells phagocytosis
Hemophagocytic lymphohistiocytosis (HLH) syndrome is a rare, underdiagnosed, and severe condition caused by an overactive immune response, resulting in phagocytosis of blood cells
Summary
Hemophagocytic lymphohistiocytosis (HLH) syndrome is a rare, underdiagnosed, and severe condition caused by an overactive immune response, resulting in phagocytosis of blood cells. EGNB, a 41-year-old male with end-stage renal disease (ESRD) of unknown cause, was submitted to deceased donor KTx in 2010 He received antithymocyte globulin induction immunosuppression at a dose of 6 mg/kg of body weight and maintenance with tacrolimus and mycophenolate sodium, progressing uneventfully until hospital discharge. GBSA, a female of 61 years old with ESRD due to hypertensive nephrosclerosis, underwent deceased donor KTx in December 2015 She received antithymocyte globulin induction immunosuppression at a total dose of 6 mg/ kg body weight, 3 plasmapheresis sessions, and IVIG at total dose of 2 g/kg, as well as maintenance therapy with tacrolimus, everolimus, and prednisone, progressing without complications until hospital discharge. After 40 days of hospitalization, the patient had a massive pulmonary aspiration, clinical worsening, and death one week after the event
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