Síndrome hemofagocítico en un trasplantado renal con síndrome de Alport

Vega, Jorge; Rodríguez, María de los Ángeles; Goecke, Helmuth; Santamarina, Mario

doi:10.4067/s0034-98872013000400014

Cited by 3 publications

(8 citation statements)

References 16 publications

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“…Hypertriglyceridemia 24,33,34 and hyperferritinemia 1,29 are commonly found in HLH. The hypertriglyceridemia occurs probably due to increased lipolytic activity induced by cytokines such as IL-1 and TNF.…”

Section: Resultsmentioning

confidence: 99%

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Hemophagocytic Lymphohistiocytosis in Autopsied Adults: Clinical, Laboratory and Immunohistochemical Evaluation for CD68 and CD57. Case Report and Literature Review

et al. 2019

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Hemophagocytic lymphohistiocytosis (HLH) is a rare, usually fatal and underdiagnosed autoimmune-activated disease. The present study aimed to perform a macroscopic, histopathological and immunohistochemical evaluation for CD68 and CD57 in organs of autopsied adults with HLH. A total of 604 autopsy reports were analyzed, and all the patients that filled the diagnostic criteria for HLH (n = 2) were selected. These patients were 18 and 37 years old. Were evaluated both clinical and autopsy reports and performed histopathological and immunohistochemical analysis of the liver and spleen. Both patients filled the diagnostic criteria for HLH, as well as presented common signs and symptoms of this disease, such as chills, abdominal pain, diaphoresis, and jaundice. Hemophagocytosis was observed in the spleen, bone marrow, and lymph nodes of the two patients at autopsy. Immunostaining in the liver and spleen of both patients was mainly severe for CD68, and predominantly mild for CD57, indicating a decrease in NKC numbers and an increase in the number of macrophages, respectively. This was the first study to evaluate CD57 and CD68 in autopsies of adults with HLH. Thus, more studies are required, not only to better elucidate the pathogenetic mechanisms involved in the secondary HLH, but also to disseminate the results in the clinical environment, contributing to the early diagnosis and treatment with consequent reduction of mortality rate. Keywords: Autoimmune Diseases. Histiocytosis. Biomarkers. ResumoA Linfohistiocitose Hemofagocítica (HLH) é uma doença autoimune rara, geralmente fatal e subdiagnosticada. Este estudo tem como objetivo realizar avaliação macroscópica, histopatológica e imunohistoquímica para CD68 e CD57 em órgãos de pacientes adultos com HLH submetidos a autópsia. Um total de 604 laudos de autópsias foram analisados e todos os pacientes que preencheram os critérios diagnósticos para HLH (n = 2) foram selecionados. Esses pacientes tinham 18 e 37 anos de idade. Foram analisados tanto os prontuários quanto os laudos de autópsia, bem como foram realizadas análises histopatológicas e imunohistoquímicas do fígado e baço dos pacientes. Ambos preencheram os critérios diagnósticos para HLH e apresentarem sinais e sintomas comuns da doença, como calafrios, dor abdominal, sudorese e icterícia. A hemofagocitose foi observada no baço, medula óssea e linfonodos dos dois pacientes na autópsia. A imunohistoquímica do fígado e do baço de ambos os pacientes demonstrou imunomarcação acentuada para CD68 e predominantemente discreta para CD57, que indicam diminuição do número de NKC e aumento do número de macrófagos, respectivamente. Este foi o primeiro estudo a avaliar o CD57 e CD68 em autópsias de adultos com HLH. Assim, mais estudos são necessários, não apenas para melhor elucidar os mecanismos patogenéticos envolvidos na HLH secundária, mas também para disseminar os resultados no ambiente clínico, contribuindo para o diagnóstico e tratamento precoces com consequente redução da taxa de mortalidade. Palavras-chave: Doenças Autoimunes. Histiocitose. Biomarcadores.

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Section: Resultsmentioning

confidence: 99%

“…Anemia is a frequent finding in HLH 23,24,33,36 being caused by erythrophagocytosis and enhanced by the inflammatory state. 32 In the present study, both P1 and P2 had severe anemia and increased levels of lactate dehydrogenase (LDH).…”

Section: Resultsmentioning

confidence: 99%

Hemophagocytic Lymphohistiocytosis in Autopsied Adults: Clinical, Laboratory and Immunohistochemical Evaluation for CD68 and CD57. Case Report and Literature Review

et al. 2019

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“…En los pacientes trasplantados esta condición se puede presentar especialmente en las primeras semanas postrasplante y se relaciona frecuentemente con infecciones, la presencia de malignidades y el uso de medicamentos depletores de linfocitos (22) . Existe una relación compleja entre los procesos infecciosos y la LHH, esto debido a las infecciones que pueden causar este síndrome en personas predispuestas genéticamente o a la leucopenia secundaria a la (11,12,14,15,18,19,21) LHH que puede favorecer el desarrollo de un proceso infeccioso. Los microorganismos más frecuentemente asociados son los virus, especialmente los herpesvirus, por infección primaria o reactivación.…”

Section: Factores De Riesgounclassified

“…Adicionalmente, se han reportado algunos casos de LHH en pacientes con trasplante de órgano sólido bajo terapia inmunosupresora, con una incidencia reportada de 0,4-0,5 % (14) y con una mortalidad del 80 %, especialmente cuando se asocia con una falla multisistémica (2,4,15,16) . Karras y col. observaron que la presencia de organomegalia, elevación de las aminotransferasas, prolongación del tiempo de protrombina y trombocitopenia, fueron factores de riesgo de muerte en pacientes con LHH posterior a un trasplante renal (15) .…”

Section: Introductionunclassified

“…Karras y col. observaron que la presencia de organomegalia, elevación de las aminotransferasas, prolongación del tiempo de protrombina y trombocitopenia, fueron factores de riesgo de muerte en pacientes con LHH posterior a un trasplante renal (15) . (3) En la actualidad existe poca literatura con respecto a la LHH en pacientes trasplantados de riñón, siendo en su mayoría proveniente de reportes o series de casos (Tabla 2) (11,12,14,(17)(18)(19)(20) . El poco conocimiento de la LHH postrasplante, sumado a su presentación clínica inespecífica, hace que su diagnóstico sea un reto.…”

Section: Introductionunclassified

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Linfohistiocitosis hemofagocítica en trasplante renal

Nieto-Ríos

Morales-Contreras

Chacón-Jaimes

et al. 2019

Iatreia

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La linfohistiocitosis hemofagocítica (LHH) posterior al trasplante renal hace referencia a un estado hiperinflamatorio grave, asociado a la activación no controlada de los linfocitos T citotóxicos y macrófagos por causa infecciosas y/o secundaria al tratamiento inmunosupresor. Las causas más prevalentes dentro de las infecciones son la histoplasmosis, la tuberculosis y las infecciones por virus herpes. Se caracteriza por fiebre, organomegalias, citopenias, hiperferritinemia, hipertrigliceridemia y/o hipofibrinogenemia; puede acompañarse con hemofagocitosis documentada en la médula ósea, el hígado u otros órganos. Su curso puede ser fulminante con progresión a falla multisistémica y la muerte.Eltratamiento va enfocado a controlar tempranamente la causa desencadenante, reducir la inmunosupresión y controlar la inflamación. En pocos casos es necesario el uso de otros inmunosupresores, quimioterapia o, en situaciones muy seleccionadas, se puede requerir el trasplante de médula ósea.

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Hemophagocytic syndrome triggered by donor‐transmitted toxoplasmosis as a complication in same‐donor recipients of renal transplantation: Case report and review of the literature

Franci

Flores

Candel

et al. 2021

Transplant Infectious Dis

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Background Hemophagocytic syndrome (HPS) is an infrequent complication of transplantation caused by an inflammatory response with a benign proliferation of macrophages and defective lytic capability of T lymphocytes and NK cells that can lead to multiorgan failure. Transplant patients are particularly exposed as a result of the increased risk of both infections and malignancies derived from immunosuppressive drugs. There is no consensus for therapy or immunosuppression; mortality is high. We report a case and present a review of all cases of HPS occurring in solid organ transplant recipients. Case report We report two cases of infection by Toxoplasma gondii transmitted by the kidney allograft. One of the recipients was seronegative before transplantation and developed disseminated primary toxoplasmosis. An immune reaction compatible with an HPS ensued. Both were treated with Trimethoprim/sulfamethoxazole, immunosuppression was tapered, and after a 2‐week period a complete response was obtained. Conclusion HPS presents therapeutic challenges in the context of transplantation. If HPS is suspected, the search of a very likely underlying infection should be central to the management.

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Síndrome hemofagocítico en un trasplantado renal con síndrome de Alport

Abstract: We report a 28-year-old male with a hereditary nephritis (Alport (Rev Med Chile 2013; 141: 519-524).

Cited by 3 publications

References 16 publications

Hemophagocytic Lymphohistiocytosis in Autopsied Adults: Clinical, Laboratory and Immunohistochemical Evaluation for CD68 and CD57. Case Report and Literature Review

Hemophagocytic Lymphohistiocytosis in Autopsied Adults: Clinical, Laboratory and Immunohistochemical Evaluation for CD68 and CD57. Case Report and Literature Review

Linfohistiocitosis hemofagocítica en trasplante renal

Hemophagocytic syndrome triggered by donor‐transmitted toxoplasmosis as a complication in same‐donor recipients of renal transplantation: Case report and review of the literature

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