This consensus statement is directed to intensivists, hematologists, and oncologists caring for critically ill cancer patients and focuses on the management of these patients.Electronic supplementary materialThe online version of this article (10.1007/s00277-018-3312-y) contains supplementary material, which is available to authorized users.

We aimed to compare the characteristics and outcome of patients treated within the multi-centre German Primary CNS Lymphoma Study Group 1 trial (G-PCNSL-SG-1; TRIAL group) and patients treated outside this clinical trial ("real-life" setting, R-LIFE group). Therefore, we conducted a retrospective single-centre study in order to analyse all patients with newly diagnosed primary CNS lymphoma (PCNSL) treated consecutively in our institution between November 2000 and June 2015. Altogether, 86 patients were analysed (median 68 years). Twenty patients were treated within (TRIAL) and 66 patients outside the clinical trial (R-LIFE), respectively. The majority (n = 75; 87 %) received high-dose methotrexate as the first-line treatment. Thirty-eight of 66 patients (57.6 %) responded to the first-line therapy. The R-LIFE patients were older (median age 70 vs. 62 years; p = 0.005) and had more frequently a worse performance status (ECOG score 2-4: 59.1 vs. 20.0 %; p = 0.004; median Karnofsky index 70 vs. 80 %; p = 0.003) and less frequently a low prognostic score (IELSG score 0-1: 19.7 vs. 45.0 %; p = 0.038), than the TRIAL patients. Median overall survial (OS) was shorter for the R-LIFE patients (9.3 months [95 % CI 1.9-16.7] vs. 33.4 months [95 % CI 17.6-49.2]; p = 0.065). Median progression-free survival (PFS) was significantly inferior for the R-LIFE patients (3.4 months [95 % CI 2.4-4.4] vs. 24.8 months [95 % CI 4.6-45.0]; p = 0.037). Our data indicate that the outcome of PCNSL patients treated outside, but about analogous to the G-PCNSL-SG-1 trial, was poor. This is likely explained by more unfavourable prognostic factors in patients being treated off trial.

Background With Sepsis-3, the increase in sequential organ failure assessment (SOFA) as a clinical score for the identification of patients with sepsis and quickSOFA (qSOFA) for the identification of patients at risk of sepsis outside the intensive care unit (ICU) were introduced in 2016. However, their validity has been questioned, and their applicability in different settings and subgroups, such as hematological cancer patients, remains unclear. We therefore assessed the validity of SOFA, qSOFA, and the systemic inflammatory response syndrome (SIRS) criteria regarding the diagnosis of sepsis and the prediction of in-hospital mortality in a multicenter cohort of hematological cancer patients treated on ICU and non-ICU settings. Methods We retrospectively calculated SIRS, SOFA, and qSOFA scores in our cohort and applied the definition of sepsis as “life-threatening organ dysfunction caused by dysregulated host response to infection” as reference. Discriminatory capacity was assessed using the area under the receiver operating characteristic curve (AUROC). Results Among 450 patients with hematological cancer (median age 58 years, 274 males [61%]), 180 (40%) had sepsis of which 101 (56%) were treated on ICU. For the diagnosis of sepsis, sensitivity was 86%, 64%, and 42% for SIRS, SOFA, and qSOFA, respectively. However, the AUROCs of SOFA and qSOFA indicated better discrimination for sepsis than SIRS (SOFA, 0.69 [95% CI, 0.64–0.73] p < 0.001; qSOFA, 0.67 [95% CI, 0.62–0.71] p < 0.001; SIRS, 0.57 [95% CI, 0.53–0.61] p < 0.001). In-hospital mortality was 40% and 14% in patients with and without sepsis, respectively ( p < 0.001). Regarding patients with sepsis, mortality was similar in patients with positive and negative SIRS scores (39% vs. 40% ( p = 0.899), respectively). For patients with qSOFA ≥ 2, mortality was 49% compared to 33% for those with qSOFA < 2 ( p = 0.056), and for SOFA 56% vs. 11% ( p < 0.001), respectively. SOFA allowed significantly better discrimination for in-hospital mortality (AUROC 0.74 [95% CI, 0.69–0.79] p < 0.001) than qSOFA (AUROC 0.65 [95% CI, 0.60–0.71] p < 0.001) or SIRS (AUROC 0.49 [95% CI, 0.44–0.54] p < 0.001). Conclusions An increase in SOFA score of ≥ 2 had better prognostic accuracy for both diagnosis of sepsis and in-hospital mortality in this setting, and especially on ICU, we observed limited validity of SIRS criteria and qSOFA in identifying hematological patients with sepsis and at high risk of death. Electronic supplementary material The online version of this article (10.1186/s40560-019-0396-y) contains supplementary material, which is available to authorized users. ...

The patient population investigated was older than participants in published large multicenter trials. The majority of patients were categorized as 'high risk.' Age was the main risk factor. Molecular status was unavailable in the majority of patients diagnosed prior to 2008. The physician-based survey reported on significantly lower rates of constitutional symptoms than patient-based surveys in the literature. Consistent with previously published reports, hydroxyurea is the main agent used for PV therapy in an outpatient setting resulting in efficient control of hematopoietic parameters. Constitutional symptoms and splenomegaly, however, may not be reduced efficiently, which could be improved by the use of JAK inhibitor treatment for high-risk patients in the future.

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