Challenges in the diagnosis of hemophagocytic lymphohistiocytosis: Recommendations from the North American Consortium for Histiocytosis (NACHO)

Jordan, Michael B.; Allen, Carl E.; Greenberg, Jay; Henry, M. M.; Hermiston, Michelle L.; Kumar, Ashish; Hines, Melissa; Eckstein, Olive S.; Ladisch, Stephan; Nichols, Kim E.; Rodríguez‐Galindo, Carlos; Wistinghausen, Birte; McClain, Kenneth L.

doi:10.1002/pbc.27929

Cited by 250 publications

(255 citation statements)

References 105 publications

(122 reference statements)

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“…HLH presents in a wide spectrum of clinical contexts, including fever of unknown origin, acute liver failure, sepsislike, Kawasaki-like, and neurologic abnormalities (8). Although a distinctive constellation of clinical and laboratory features has been described for HLH, diagnosis remains challenging as patients may have very different clinical manifestations associated with a variety of triggers (9). Atypical presentations involving mainly CNS or chronic pathologic inflammation manifestations are examples of such complexity (10)(11)(12).…”

Section: Introductionmentioning

confidence: 99%

“…To date, 4 genes (PRF1, UNC13D, STXBP2, and STX11) and 1 genomic region (9q21. [3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22] have been identified as candidate causes of FHL2 (13), FHL3 (14), FHL4 (15), FHL5 (16), and FHL1 (17), respectively (18). Other monogenic diseases that produce HLH are Chédiak-Higashi syndrome (LYST), Griscelli syndrome type 2 (RAB27A), Hermansky-Pudlak syndrome (AP3B1), Xlinked lymphoproliferative syndrome (XLP)-1 (SH2D1A), and XLP-2 (XIAP) (19).…”

Section: Introductionmentioning

confidence: 99%

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FHLdb: A Comprehensive Database on the Molecular Basis of Familial Hemophagocytic Lymphohistiocytosis

et al. 2020

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

FHLdb: A Comprehensive Database on the Molecular Basis of Familial Hemophagocytic Lymphohistiocytosis

et al. 2020

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“…Acute respiratory distress syndrome (ARDS) [19] was the most common systemic complication (35/202, 17.33%). Other less common complications included sepsis shock [20] (27/202, 13.37%), multiple organ dysfunction syndrome (MODS) [21](19/202, 9.41%), and hemophagocytic lymphohistiocytosis (HLH) [22](3/202, 1.49%). PIBO was the most common sequela after a severe adenovirus pneumonia ( Fig.…”

Section: Patients' Baseline Characteristicsmentioning

confidence: 99%

Role of Intravenous immunoglobulin in Non-Immunocompromised children With Severe Adenovirus Pneumonia: A Retrospective Observational Study

Fan¹,

Chen²,

Xu³

et al. 2020

Preprint

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Background: Adenovirus pneumonia is a pulmonary infectious disease commonly occurring in children, and severe cases can lead to death and sequelae. Here, we aimed to observe the therapeutic timing and dosage of intravenous immunoglobulin(IVIg) in non-immunocompromised children with severe adenovirus pneumonia.Methods: This retrospective observational study investigated severe adenovirus pneumonia treated with IVIg in non-immunocompromised pediatric patients at a tertiary hospital in 2019. Participants were classified as early presenters (5-10 days of illness course) and later presenters (11-15 days) according to the timing of IVIg treatment. Patients’ clinical data were then analyzed in terms of different dosages of IVIg administration. Results: Among 202 patients enrolled, 128 were early presenters and 74 were later presenters during the study period. The later presenters had longer fever duration, more incidences of fungal coinfections, more demands for mechanical ventilation, and higher incidence of bronchiectasis than early presenters (P<0.05). For early presenters, no statistically significant differences in demands for advanced life support, outcomes and sequelae were observed between the two different dosage groups (P>0.05). For later presenters, shorter fever duration and lower usage of extracorporeal membrane oxygenation (ECMO) were observed in the high-dosage group than that in the low-dosage group (P<0.05). The incidence of post-infectious bronchiolitis obliterans (PIBO) and bronchiectasis was not significantly different between the two groups (P>0.05). The incidence of adverse events was 6.62% during IVIg infusion, showing no significant difference between the two groups (P>0.05).Conclusions: Early medical care and treatment with IVIg are very important to improve the prognosis of non-immunocompromised children with severe adenovirus pneumonia. For later presenters, children with severe conditions may benefit from a high IVIg dosage.

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“…Coagulation disorders are common in sHLH patients and are reported in up to 60% of patients with sHLH [4]. Fibrinogen (FIB) less than or equal to 1.50 g/L, one of the most frequently reported coagulopathies and the hallmark of HLH, is also a diagnostic criterion in in HLH [5]. Several studies have also demonstrated that 50-80% of sHLH patients have hypo brinogenemia [6,7].…”

Section: Introductionmentioning

confidence: 99%

The prognostic role of plasma fibrinogen in adult secondary hemophagocytic lymphohistiocytosis

Yin

Man

Liao

et al. 2020

Preprint

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Abstract Background: Coagulation abnormality is common in hemophagocytic lymphohistiocytosis (HLH), but the more frequently reported anomaly is an isolated decrease in fibrinogen (FIB). Nonetheless, the prognostic value of FIB in adult secondary HLH (sHLH) remains uncertain. Methods: Circulating plasma levels of FIB were measured at diagnosis in 293 cases of adult sHLH. We categorized FIB levels into tertiles. Multivariable Cox proportional hazards models were conducted to evaluate the relationship between FIB and mortality. Restricted cubic spline models and two-piecewise Cox proportional hazards models were used to address the nonlinear association between FIB and mortality.Results: During a median follow-up of 52 (interquartile ranges, 18-221) days, 208 deaths occurred, with 137 deaths in MHLH and 71 deaths in non-MHLH. After multivariable adjustment, compared to the highest tertile of FIB, the hazard ratios (HRs) with 95% confidence intervals (CIs) of mortality for tertile 2 and tertile 1 were 0.88 (0.61-1.26) and 1.51 (1.05-2.18), respectively. The restricted cubic spline curve displayed a nonlinear and inverse relationship between FIB and mortality. Furthermore, threshold effect analysis demonstrated that the inflection point for the curve was at a FIB level of 1.76 g/L. The HRs (95% CIs) for mortality were 2.47 (1.52-4.04) and 0.83 (0.64 -1.09) on the left and right side of the inflection point, respectively. Conclusions: These results suggest that plasma fibrinogen is nonlinearly and inversely associated with the risk of mortality in adult secondary hemophagocytic lymphohistiocytosis.

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Challenges in the diagnosis of hemophagocytic lymphohistiocytosis: Recommendations from the North American Consortium for Histiocytosis (NACHO)

Cited by 250 publications

References 105 publications

FHLdb: A Comprehensive Database on the Molecular Basis of Familial Hemophagocytic Lymphohistiocytosis

FHLdb: A Comprehensive Database on the Molecular Basis of Familial Hemophagocytic Lymphohistiocytosis

Role of Intravenous immunoglobulin in Non-Immunocompromised children With Severe Adenovirus Pneumonia: A Retrospective Observational Study

The prognostic role of plasma fibrinogen in adult secondary hemophagocytic lymphohistiocytosis

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