Clinical features of 47 secondary hemophagocytic lymphohistiocytosis patients complicated with capillary leak syndrome

Man, Changfeng; Wang, Mengmeng; Yin, Guangli; Huang, Jiayu; Cheng, Wanying; Wu, Xiaoshu; Liu, Lingling; Gao, Xin; Wang, Jujuan; Tian, Tian; Duan, Limin; Xu, Jianping; Qiu, Hai-Rong

doi:10.1007/s12185-020-03011-8

Cited by 4 publications

(1 citation statement)

References 28 publications

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“…HLH is a complex disease that may affect many different organs. Therefore, the signs and symptoms may be extremely diverse, and no finding is pathognomonic for this condition (4,19,20). In this context, different sets of criteria have been suggested for helping clinicians diagnose the disease (1,21).…”

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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Capillary leak syndrome: State of the art in 2021

Bichon

Bourenne

Gainnier

et al. 2021

La Revue de Médecine Interne

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HLH-Like Syndrome and Rhabdomyolysis in an Adolescent Patient

Maloney¹,

Baz²,

Hazra³

2021

Pediatrics

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Hemophagocytic lymphohistiocytosis (HLH) is a rare heterogeneous group of disorders characterized by immune overactivation. It can occur because of primary genetic mutations or secondary to almost any inflammatory or infectious process. The clinical manifestations of this syndrome are varied and life-threatening and resemble those of many malignancies, infections, sepsis, and multisystem inflammatory syndrome in children. Laboratory abnormalities often are not diagnostic for HLH until late in the disease course, and the laboratory studies are send-out tests at most institutions. Thus, quickly and accurately diagnosing pediatric patients with HLH presents significant challenges to the clinician. Furthermore, there has been recent discussion in the literature regarding the use of diagnostic criteria for HLH. In this case report, we detail an adolescent male individual who developed persistent unexplained fever, rhabdomyolysis, and regional ischemic immune myopathy. To our knowledge, there is no previous report of a pediatric patient with this rare myopathy or HLH presenting with persistent rhabdomyolysis in the literature. The patient was hospitalized for a total of 61 days, with multiple treatments attempted throughout during his course of illness. In this report, we highlight the importance of using diagnostic flexibility when HLH is suspected in pediatric patients and provide insight into the unique challenges of identifying this condition.

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Clinical features of 47 secondary hemophagocytic lymphohistiocytosis patients complicated with capillary leak syndrome

Cited by 4 publications

References 28 publications

A predictive model for identifying secondary underlying diseases of hemophagocytic lymphohistiocytosis

A predictive model for identifying secondary underlying diseases of hemophagocytic lymphohistiocytosis

Capillary leak syndrome: State of the art in 2021

HLH-Like Syndrome and Rhabdomyolysis in an Adolescent Patient

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