Secondary hemophagocytic syndrome after renal transplantation: two case-reports

Abstract

Hemophagocytic 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.

Hemophagocytic lymphohistiocytosis secondary to virus infection and followed by lupus nephritis recurrence in a renal transplantation pediatric recipient: a case report

“…If the infection is induced by viruses such as EBV, CMV, or B19 virus, the treatment will generally begin with a reduction of immunosuppression. Meanwhile short-term (usually about 3 days) and high-dose regimen (accumulated dose of 1.6-2 g/kg body weight) are mostly used to control the virus in a short period of time [ 8 – 10 ], Nevertheless, several cases of death due to recurrence have been reported [ 5 ]. According to our experience and literature review, multiple viruses, especially B19 virus, were not so easy to be controlled in such a short term (less than 7 days) in transplant cohort [ 11 ].…”

Secondary Hemophagocytic Lymphohistiocytosis Complicated by a Cavitary Lung Lesion in a Kidney Transplant Recipient

“…According to the available literature, sHLH has been most commonly associated with malignancies in about half of the reported cases – followed by the systemic infections and autoimmune diseases [ 6 ]. It has been much less prevalent in patients on immunosuppressive therapy [ 5 , 12 ]. In a population of kidney transplant recipients, the most commonly identifiable cause of sHLH was viral infections, mainly EBV and CMV [ 6 ].…”

“…We present the development and clinical course of sHLH in a kidney transplant recipient. The literature on the subject has been scarce [ 5 , 6 , 7 , 8 ], with the biggest series involving 17 cases of sHLH among a cohort of 4,230 kidney graft recipients [ 9 ]. Our case was complicated by the pulmonary lesion different from the typical complications observed in HLH.…”