Characteristics and predictors of post-transplant-associated hemophagocytic lymphohistiocytosis in adults

Hattori, Norimichi; Sato, Misuzu; Uesugi, Yuka; Nakata, Ayaka; Sasaki, Yohei; Shimada, Shotaro; Watanuki, Megumi; Fujiwara, Shun; Kawaguchi, Yukiko; Arai, Nana; Uto, Yui; Matsui, Tomoharu; Yanagisawa, Kouji; Tahara, Sachiko; Koeffler, H. Phillip; Iezumi, Keiichi; Nakamaki, Tsuyoshi

doi:10.1007/s12185-020-03067-6

Cited by 5 publications

(4 citation statements)

References 27 publications

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“…In this context, different sets of criteria have been suggested for helping clinicians diagnose the disease (1,21). The identification of a simple model to make a differential diagnosis has been regarded as a necessary advance since HLH may be difficult to distinguish from an underlying disease (9,(22)(23)(24)(25); this model would allow doctors to make adequate treatment decisions at the earliest opportunity.…”

Section: Discussionmentioning

confidence: 99%

A predictive model for identifying secondary underlying diseases of hemophagocytic lymphohistiocytosis

Gao

et al. 2023

Front. Immunol.

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BackgroundSecondary hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening disease of immune hyperactivation that arises in the context of infectious, inflammatory, or neoplastic triggers. The aim of this study was to establish a predictive model for the timely differential diagnosis of the original disease resulting in HLH by validating clinical and laboratory findings to further improve the efficacy of therapeutics for HLH.MethodsWe retrospectively enrolled 175 secondary HLH patients in this study, including 92 patients with hematologic disease and 83 patients with rheumatic disease. The medical records of all identified patients were retrospectively reviewed and used to generate the predictive model. We also developed an early risk score using multivariate analysis weighted points proportional to the β regression coefficient values and calculated its sensitivity and specificity for the diagnosis of the original disease resulting in HLH.ResultsThe multivariate logistic analysis revealed that lower levels of hemoglobin and platelets (PLT), lower levels of ferritin, splenomegaly and Epstein−Barr virus (EBV) positivity were associated with hematologic disease, but young age and female sex were associated with rheumatic disease. The risk factors for HLH secondary to rheumatic diseases were female sex [OR 4.434 (95% CI, 1.889-10.407), P =0.001], younger age [OR 6.773 (95% CI, 2.706-16.952), P<0.001], higher PLT level [OR 6.674 (95% CI, 2.838-15.694), P<0.001], higher ferritin level [OR 5.269 (95% CI, 1.995-13.920), P =0.001], and EBV negativity [OR 27.656 (95% CI, 4.499-169.996), P<0.001]. The risk score included assessments of female sex, age, PLT count, ferritin level and EBV negativity, which can be used to predict HLH secondary to rheumatic diseases with an AUC of 0.844 (95% CI, 0.836~0.932).ConclusionThe established predictive model was designed to help clinicians diagnose the original disease resulting in secondary HLH during routine practice, which might be improve prognosis by enabling the timely treatment of the underlying disease.

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Section: Discussionmentioning

confidence: 99%

A predictive model for identifying secondary underlying diseases of hemophagocytic lymphohistiocytosis

Gao

et al. 2023

Front. Immunol.

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“…In some cases the triggering factor may be the residual disease ( 171 ). PT-HLH diagnosis is extremely challenging because of the complex clinical and laboratory picture of the affected patients, for whom specific diagnostic criteria do not exist yet, and a high suspicion rate is essential ( 180 ).…”

Section: Classificationmentioning

confidence: 99%

Secondary hemophagocytic lymphohistiocytosis in children (Review)

et al. 2023

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Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening condition characterized by hyperinflammation in an uncontrolled and ineffective immune response. Despite great improvement in diagnosis and treatment, it still represents a challenge in clinical management, with poor prognosis in the absence of an aggressive therapeutic approach. The present literature review focuses on secondary HLH at pediatric age, which represents a heterogeneous group in terms of etiology and therapeutic approach. It summarizes the most recent evidence on epidemiology, pathophysiology, diagnosis, treatment and prognosis, and provides a detailed description and comparison of the major subtypes of secondary HLH. Finally, it addresses the open questions with a focus on diagnosis and new treatment insights.

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“…Indeed, EBV, CMV, and human herpesvirus-8 account for >50% of cases of HLH after solid organ transplantation. [11][12][13][14] On the other hand, toxoplasmosis-associated HLH after renal transplantation is rare, with only eight cases reported until 2021. The median time of diagnosis was 30 days post-transplant (range 9-4000) and three of the eight patients died.…”

Section: Triggers Of Hlh Following Solid Organ Transplantation (Marcio)mentioning

confidence: 99%

“…HLH occurring after solid organ transplantation is uncommon (0.4%–2% of cases of HLH) and the most frequent triggers are infectious agents, especially viruses. Indeed, EBV, CMV, and human herpesvirus‐8 account for >50% of cases of HLH after solid organ transplantation 11–14 . On the other hand, toxoplasmosis‐associated HLH after renal transplantation is rare, with only eight cases reported until 2021.…”

Section: Triggers Of Hlh Following Solid Organ Transplantation (Marcio)mentioning

confidence: 99%

Complex considerations – Fever and pancytopenia after solid organ transplantation

Franci

Adekunle

Nucci

et al. 2023

Transplant Infectious Dis

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This case describes a 42‐year‐old man who underwent kidney transplantation and developed fevers, pancytopenia, and elevated liver function tests starting on post‐operative day 9. An extensive microbiologic and molecular workup was performed, ultimately leading to a diagnosis of donor‐derived toxoplasmosis with associated hemophagocytic lymphohistiocytosis in the recipient. This case highlights the potential for post‐transplant toxoplasmosis in high‐risk mismatch (D+/R‐) recipients, as well as the role of Toxoplasma‐targeted prophylaxis in such patients.

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Characteristics and predictors of post-transplant-associated hemophagocytic lymphohistiocytosis in adults

Cited by 5 publications

References 27 publications

A predictive model for identifying secondary underlying diseases of hemophagocytic lymphohistiocytosis

A predictive model for identifying secondary underlying diseases of hemophagocytic lymphohistiocytosis

Secondary hemophagocytic lymphohistiocytosis in children (Review)

Complex considerations – Fever and pancytopenia after solid organ transplantation

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