Cytomegalovirus infections, kidney transplantation, and secondary hemophagocytic lymphohistiocytosis

Hemophagocytic lymphohistiocytosis (HLH) is an uncommon and life-threating condition characterized by major immune activation and massive cytokine production by mononuclear inflammatory cells, due to defects in cytotoxic lymphocyte function. It is even more unusual in renal transplant recipients, in which it is often associated with uncontrolled infection. The mortality is high in HLH and differential diagnosis with sepsis is a challenge. The approach and management depend on the underlying trigger and comorbidities. We report a case of a 50-year-old renal transplant female admitted with fever and malaise 3 months post-transplant and presenting anemia, fever, hypertriglyceridemia, high levels of serum ferritin, and positive CMV antigenemia. Urine was positive for decoy cells and BKV-DNA. Graft biopsy showed CMV nephritis. Both blood and urine cultures where positive for E. coli. Hemophagocytosis was confirmed by bone marrow aspiration. Immunosuppression was reduced, and the patient received high-dose intravenous immunoglobulin and dexamethasone, with complete response after 3 weeks. We highlight the importance of early diagnosis and proper management of a rare and serious condition in a renal transplant patient, which can allow a favorable clinical course and improve survival rate.

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“…Although the strategy might be successful in most cases, the best choice in this setting is still a matter of controversy. 9 , 14 …”

Section: Discussionmentioning

confidence: 99%

Hemophagocytic lymphohistiocytosis, a rare condition in renal transplant - a case report

et al. 2018

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“…Some examples of bacterial etiologies are mycobacteria, Brucella, Rickettsia, Hemophilus influenzae and Serratia sp. The most common parasites are Leishmania and Toxoplasma [22][23][24][25][26][27][28][29][30].…”

Section: Discussionmentioning

confidence: 99%

Hemophagocytic lymphohistiocytosis in a patient with Sjögren’s syndrome: case report and review

et al. 2016

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Hemophagocytic lymphohistiocytosis (HLH) is a very rare syndrome with a mortality up to 95% of cases if not treated. It is characterised by an excessive activation of the immune system that leads to a disproportionate and destructive inflammatory response. The high mortality rates are in part due to a delay in the diagnosis, and therefore clinicians must maintain a high index of suspicion. When the treatment is started early, the survival rate reaches around 55% of cases. HLH usually presents with persistent fever, pancytopenia, and organomegaly and is associated with very high levels of serum ferritin. In this manuscript, we present the case of a patient with primary Sjögren's syndrome who developed HLH after an acute infection by Cytomegalovirus. We will describe and discuss the pathogenesis, differential diagnosis and a pragmatic approach to the treatment for this critically important and, when diagnosed early, potentially curable syndrome.

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Rapidly Fatal Hemophagocytic Lymphohistiocytosis Developing Within Six Days Following Deceased-Donor Renal Transplantation: Case Report

Filippone

Singh

Frank

et al. 2016

Transplantation Proceedings

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Cytomegalovirus infections, kidney transplantation, and secondary hemophagocytic lymphohistiocytosis

Cited by 4 publications

References 21 publications

Hemophagocytic lymphohistiocytosis, a rare condition in renal transplant - a case report

Hemophagocytic lymphohistiocytosis, a rare condition in renal transplant - a case report

Hemophagocytic lymphohistiocytosis in a patient with Sjögren’s syndrome: case report and review

Rapidly Fatal Hemophagocytic Lymphohistiocytosis Developing Within Six Days Following Deceased-Donor Renal Transplantation: Case Report

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