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Case Reports
. 2020 Mar;42(1):118-123.
doi: 10.1590/2175-8239-JBN-2018-0246. Epub 2019 Jul 18.

Secondary hemophagocytic syndrome after renal transplantation: two case-reports

[Article in Portuguese, English]
José Narciso Júnior  1 , Beatriz de Oliveira Neri  1 , Gilberto Loiola de Alencar Dantas  2 , Lara de Holanda Jucá Silveira  2 , Maria Luiza de Mattos Brito Oliveira Sales  1 , Tainá Veras de Sandes Freitas  1 , Ronaldo de Matos Esmeraldo  1
Affiliations
Case Reports

Secondary hemophagocytic syndrome after renal transplantation: two case-reports

[Article in Portuguese, English]
José Narciso Júnior et al. J Bras Nefrol. 2020 Mar.

Abstract
in English, Portuguese

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.

A síndrome hemofagocítica (SHF) ou linfo-histiocitose hemofagocítica é uma condição infrequente e subdiagnosticada que tem por base a ativação excessiva da resposta imune, resultando em fagocitose das células do sangue. Após o transplante renal (TxR), a SHF é habitualmente secundária (ou reativa) a processos infecciosos e neoplásicos, culminando em elevadas taxas de mortalidade. Não há evidências quanto ao tratamento ideal dessa condição. Além de investigação e tratamento da patologia desencadeante do processo de desregulação do sistema imune, há descrições do uso de imunoglobulina humana (IVIG), esteroides em altas doses e plasmaférese. Não há evidências quanto à melhor forma de delinear a imunossupressão de manutenção. Este artigo apresenta dois casos de SHF reativa pós-TxR que realizaram tratamento adjuvante com IVIG, obtendo bons resultados clínicos. Apesar da elevada morbimortalidade associada à SHF reativa após o TxR, o diagnóstico ágil e preciso, associado à instituição de terapia com IVIG adjuvante ao tratamento da doença desencadeante, foi uma estratégia eficaz em conter o processo.

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Figures

Figure 1
Figure 1. Bone marrow aspirate showing marked hemophagocytosis (case 2).
See this image and copyright information in PMC

References

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