Multivariate analysis of prognosis for patients with natural killer/T cell lymphoma-associated hemophagocytic lymphohistiocytosis

Jin, Zhili; Wang, Yini; Wang, Jingshi; Lin, Wei; Pei, Ruijun; Lai, Wenyuan; Zhao, Wei‐Li

doi:10.1080/10245332.2017.1385191

Cited by 28 publications

(25 citation statements)

References 29 publications

(47 reference statements)

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…This result suggests that the worse prognosis of T/NK-cell LAHS may be related with that EBV infection is more common in T/NK cell lymphoma. EBV itself can cause HLH, and whether it is EBV-HLH or EBV (+) NK/T-LAHS [3,30], combined with positive EBV is an independent prognostic factor, as also observed in our study (Fig. 3).…”

Section: Discussionsupporting

confidence: 88%

Requirement for Containing Etoposide in the Initial Treatment of Lymphoma Associated Hemophagocytic Lymphohistiocytosis

Song

Wang

et al. 2020

Preprint

Self Cite

View full text Add to dashboard Cite

Abstract Background: Hemophagocytic lymphohistiocytosis (HLH) is a severe or even fatal inflammatory status caused by a hereditary or acquired immunoregulatory abnormality. Lymphoma associated hemophagocytic lymphohistiocytosis (LAHS) is a kind of secondary HLH (sHLH). It has the worst prognosis among sHLH. However, there’s still no standard treatment strategy. The argument mainly focuses on whether an HLH-directed or malignancy-directed approach should initially be adopted. Etoposide is one of the key drugs in HLH treatment, also effective in lymphomas. We sought to identify the importance of containing etoposide in initial treatment, comparing with the chemotherapy which directed at lymphoma but without etoposide. Methods: 66 patients diagnosed as LAHS in our center between Jan 1 2015 and Dec 31 2017 were divided into two groups according to weather the initial treatment containing etoposide, or other lymphoma-directed chemotherapy without etoposide. Results: The remission rate of the initial etoposide group (52 patients) is significantly better than that of no initial etoposide group (14 patients) (73.1% vs. 42.9%, p = 0.033). The relapse occurrence between two groups shows difference (26.3% vs. 50%) but not significant (p = 0.339). There were 28 deaths in patients with initial etoposide (mortality rate 53.8%) and 12 deaths in the without-initial-etoposide group (mortality rate 85.7%). A significant difference in mortality was noted between the two groups (p = 0.030). The 2-month survival (79.8% vs. 46.8%, p = 0.019) and overall survival (median survival time 25.8w vs. 7.6w, p = 0.048) of the initial etoposide contained group is also better. Multivariate cox analysis revealed that for patients without EBV infection (37 cases), initial treatment with etoposide could significantly improve prognosis (p = 0.010, Exp(B) = 0.183), but for patients with positive EBV, it’s not significant. Conclusions: We concluded that containing etoposide is essential in the initial treatment of LAHS, weather using the HLH directed or lymphoma directed strategy. It provides higher response rate, lower mortality rate and better survival, especially for EBV negative patients.

show abstract

Section: Discussionsupporting

confidence: 88%

Requirement for Containing Etoposide in the Initial Treatment of Lymphoma Associated Hemophagocytic Lymphohistiocytosis

Song

Wang

et al. 2020

Preprint

Self Cite

View full text Add to dashboard Cite

show abstract

“…Another study of 22 patients with NK/T-LAHLH revealed a 2-year OS rate of 30% (calculated from the diagnosis of ENKTL), with an ORR of 17.4%, and a median survival time of only 26 days from the HLH diagnosis (Jia et al 2016 ). Moreover, a study of 42 NK/T-LAHLH patients revealed OS rates of 48.9% at 1 month, 36.7% at 2 months, 28.8% at 3 months, 23.0% at 6 months, and 15.4% at 1 year (Jin et al 2018 ). Interestingly, in a study of 295 ENKTL patients, 21 patients developed HLH and the OS among all patients did not exceed 3 months after the HLH occurred, with a median survival of only 35 days (Li et al 2017 ).…”

Section: Discussionmentioning

confidence: 99%

Using etoposide + dexamethasone-based regimens to treat nasal type extranodal natural killer/T-cell lymphoma-associated hemophagocytic lymphohistiocytosis

Wei

Yang

Cong

et al. 2020

J Cancer Res Clin Oncol

View full text Add to dashboard Cite

Purpose Nasal type extranodal natural killer/T-cell lymphoma (ENKTL) can be associated with hemophagocytic lymphohistiocytosis (NK/T-LAHLH), which is a rare and fatal disease with no effective therapy. We evaluated whether etoposide + dexamethasone-based chemotherapy regimens might be useful for treating NK/T-LAHLH. Methods This retrospective single-center study evaluated clinical data from 37 patients with NK/T-LAHLH who were treated between May 2008 and January 2020. Results Among 363 patients with ENKTL, the cumulative incidence of HLH was 11.9%. Among 43 patients with NK/T-LAHLH, 37 patients received etoposide + dexamethasone-based chemotherapy regimens, with an overall response rate of 45.9% for the HLH. The overall response rate was substantially higher for newly diagnosed NK/T-LAHLH than it was for relapsed or refractory NK/T-LAHLH (66.7% vs. 18.8%). The median overall follow-up time was 4 months, with overall survival rates of 81.1% at 1 month, 62.2% at 2 months, 56.8% at 3 months, and 34.4% at 6 months. Significantly better overall survival (all P < 0.05) was observed for patients with newly diagnosed NK/T-LAHLH (vs. relapsed/refractory disease), stage I/II disease (vs. stage III/IV disease), and nasal disease (vs. non-nasal disease). Patients who responded to the ENKTL treatment also experienced response in their HLH; 8 patients experienced continued complete response for both HLH and ENKTL. Multivariate analysis revealed that a poor prognosis among patients with NK/T-LAHLH was independently related to relapsed/refractory ENKTL and non-nasal disease. Conclusion Although patients with NK/T-LAHLH generally experienced poor outcomes, etoposide + dexamethasone-based chemotherapy regimens were associated with good outcomes among select patients with newly diagnosed or stage I/II NK/T-LAHLH.

show abstract

“… 5 This compares with mortality rates of more than 80% in HLH-related lymphoma, 13 and the figure may be even higher in patients with HLH-related NK/T-cell lymphoma. 27 We also analyzed the role of therapeutic interventions in survival, and found better trends in patients treated with GCs plus other agents (IDs, IVIGs, or biologics) and those treated with etoposide. In contrast, patients treated with GC monotherapy and those who did not receive specific HLH therapy had the worst survival curves.…”

Section: Discussionmentioning

confidence: 99%

Prognostic Factors of Death in 151 Adults With Hemophagocytic Syndrome: Etiopathogenically Driven Analysis

Brito-Zerón¹,

Kostov

Moral-Moral

et al. 2018

Mayo Clinic Proceedings: Innovations, Quality & Outcomes

View full text Add to dashboard Cite

ObjectiveTo characterize the etiologies and clinical features at diagnosis of patients with hemophagocytic lymphohistiocytosis (HLH) and correlate these baseline features with survival using an etiopathogenically guided multivariable model.Patients and MethodsThe Spanish Group of Autoimmune Diseases HLH Study Group, formed in 2013, is aimed at collecting adult patients with HLH diagnosed in internal medicine departments between January 3, 2013, and October 28, 2017.ResultsThe cohort consisted of 151 patients (91 men; mean age, 51.4 years). After a mean follow-up of 17 months (range, 1-142 months), 80 patients died. Time-to-event analyses for death identified a worse survival curve for patients with neoplasia (P<.001), mixed microbiological infections (P=.02), and more than 1 infection (P=.01) and glucocorticoid monotherapy (P=.02). According to univariate analyses, platelets of less than 100,000/mm3 (hazard ratio [HR], 3.39; 95% CI, 1.37-8.40), leukopenia (HR, 1.81; 95% CI, 1.01-3.23), severe hyponatremia (HR, 1.61; 95% CI, 1.02-2.54), disseminated intravascular coagulation (HR, 1.87; 95% CI, 1.05-3.34), bacterial infection (HR, 1.99; 95% CI, 1.09-3.63), mixed microbiological infections (HR, 3.42; 95% CI, 1.38-8.46), and 2 or more infectious triggers (HR, 2.95; 95% CI, 1.43-6.08) were significantly associated with death. In contrast, peripheral adenopathies (HR, 0.63; 95% CI, 0.40-0.98) and the immunosuppressive drug/intravenous immunoglobulin/biological therapies (HR, 0.44; 95% CI, 0.20-0.96) were protective against all-cause mortality. Multivariable Cox proportional hazards regression analysis identified 2 or more infectious triggers (HR, 3.14; 95% CI, 1.28-7.68) as the only variable independently associated with death.ConclusionThe mortality rate of adult patients diagnosed with HLH exceeds 50%. Infection with more than 1 microbiological agent was the only independent variable associated with mortality irrespective of the underlying disease, epidemiological profile, clinical presentation, and therapeutic management.

show abstract

Multivariate analysis of prognosis for patients with natural killer/T cell lymphoma-associated hemophagocytic lymphohistiocytosis

Cited by 28 publications

References 29 publications

Requirement for Containing Etoposide in the Initial Treatment of Lymphoma Associated Hemophagocytic Lymphohistiocytosis

Requirement for Containing Etoposide in the Initial Treatment of Lymphoma Associated Hemophagocytic Lymphohistiocytosis

Using etoposide + dexamethasone-based regimens to treat nasal type extranodal natural killer/T-cell lymphoma-associated hemophagocytic lymphohistiocytosis

Prognostic Factors of Death in 151 Adults With Hemophagocytic Syndrome: Etiopathogenically Driven Analysis

Contact Info

Product

Resources

About