Similar but not the same: Differential diagnosis of HLH and sepsis

Rafał Machowicz; Gritta Janka; Wieslaw Wiktor-Jedrzejczak

doi:10.1016/j.critrevonc.2017.03.023

Similar but not the same

Standard

Similar but not the same : Differential diagnosis of HLH and sepsis. / Machowicz, Rafał; Janka, Gritta; Wiktor-Jedrzejczak, Wieslaw.

In: CRIT REV ONCOL HEMAT, Vol. 114, 06.2017, p. 1-12.

Research output: SCORING: Contribution to journal › SCORING: Review article › Research

Harvard

Machowicz, R, Janka, G & Wiktor-Jedrzejczak, W 2017, 'Similar but not the same: Differential diagnosis of HLH and sepsis', CRIT REV ONCOL HEMAT, vol. 114, pp. 1-12. https://doi.org/10.1016/j.critrevonc.2017.03.023

APA

Machowicz, R., Janka, G., & Wiktor-Jedrzejczak, W. (2017). Similar but not the same: Differential diagnosis of HLH and sepsis. CRIT REV ONCOL HEMAT, 114, 1-12. https://doi.org/10.1016/j.critrevonc.2017.03.023

Vancouver

Machowicz R, Janka G, Wiktor-Jedrzejczak W. Similar but not the same: Differential diagnosis of HLH and sepsis. CRIT REV ONCOL HEMAT. 2017 Jun;114:1-12. https://doi.org/10.1016/j.critrevonc.2017.03.023

Bibtex

@article{50ee8e293bf945ffbaa39ab791fef5c9,

title = "Similar but not the same: Differential diagnosis of HLH and sepsis",

abstract = "Differential diagnosis of hemophagocytic lymphohistiocytosis (HLH; hemophagocytic syndrome) and sepsis is critically important because the life-saving aggressive immunosuppressive treatment, required in the effective HLH therapy, is absent in sepsis guidelines. Moreover, HLH may be complicated by sepsis. Hyperinflammation, present in both states, gives an overlapping clinical picture including fever and performance status deterioration. The aim of this review is to provide aid in this challenging diagnostic process. Analysis of clinical features and laboratory results in multiple groups of patients (both adult and pediatric) with either HLH or sepsis allows to propose criteria differentiating these two conditions. The diagnosis of HLH is supported by hyperferritinemia, splenomegaly, marked cytopenias, hypofibrinogenemia, low CRP, characteristic cytokine profile and, only in adults, hypertriglyceridemia. In the presence of these parameters (especially the most characteristic hyperferritinemia), the other HLH criteria should be assessed. Genetic analyses can reveal familial HLH. Hemophagocytosis is neither specific nor sensitive for HLH.",

keywords = "Diagnosis, Differential, Humans, Lymphohistiocytosis, Hemophagocytic, Sepsis, Journal Article, Review",

author = "Rafa{\l} Machowicz and Gritta Janka and Wieslaw Wiktor-Jedrzejczak",

year = "2017",

month = jun,

doi = "10.1016/j.critrevonc.2017.03.023",

language = "English",

volume = "114",

pages = "1--12",

journal = "CRIT REV ONCOL HEMAT",

issn = "1040-8428",

publisher = "Elsevier Ireland Ltd",

}

RIS

TY - JOUR

T1 - Similar but not the same

T2 - Differential diagnosis of HLH and sepsis

AU - Machowicz, Rafał

AU - Janka, Gritta

AU - Wiktor-Jedrzejczak, Wieslaw

PY - 2017/6

Y1 - 2017/6

N2 - Differential diagnosis of hemophagocytic lymphohistiocytosis (HLH; hemophagocytic syndrome) and sepsis is critically important because the life-saving aggressive immunosuppressive treatment, required in the effective HLH therapy, is absent in sepsis guidelines. Moreover, HLH may be complicated by sepsis. Hyperinflammation, present in both states, gives an overlapping clinical picture including fever and performance status deterioration. The aim of this review is to provide aid in this challenging diagnostic process. Analysis of clinical features and laboratory results in multiple groups of patients (both adult and pediatric) with either HLH or sepsis allows to propose criteria differentiating these two conditions. The diagnosis of HLH is supported by hyperferritinemia, splenomegaly, marked cytopenias, hypofibrinogenemia, low CRP, characteristic cytokine profile and, only in adults, hypertriglyceridemia. In the presence of these parameters (especially the most characteristic hyperferritinemia), the other HLH criteria should be assessed. Genetic analyses can reveal familial HLH. Hemophagocytosis is neither specific nor sensitive for HLH.

AB - Differential diagnosis of hemophagocytic lymphohistiocytosis (HLH; hemophagocytic syndrome) and sepsis is critically important because the life-saving aggressive immunosuppressive treatment, required in the effective HLH therapy, is absent in sepsis guidelines. Moreover, HLH may be complicated by sepsis. Hyperinflammation, present in both states, gives an overlapping clinical picture including fever and performance status deterioration. The aim of this review is to provide aid in this challenging diagnostic process. Analysis of clinical features and laboratory results in multiple groups of patients (both adult and pediatric) with either HLH or sepsis allows to propose criteria differentiating these two conditions. The diagnosis of HLH is supported by hyperferritinemia, splenomegaly, marked cytopenias, hypofibrinogenemia, low CRP, characteristic cytokine profile and, only in adults, hypertriglyceridemia. In the presence of these parameters (especially the most characteristic hyperferritinemia), the other HLH criteria should be assessed. Genetic analyses can reveal familial HLH. Hemophagocytosis is neither specific nor sensitive for HLH.

KW - Diagnosis, Differential

KW - Humans

KW - Lymphohistiocytosis, Hemophagocytic

KW - Sepsis

KW - Journal Article

KW - Review

U2 - 10.1016/j.critrevonc.2017.03.023

DO - 10.1016/j.critrevonc.2017.03.023

M3 - SCORING: Review article

C2 - 28477737

VL - 114

SP - 1

EP - 12

JO - CRIT REV ONCOL HEMAT

JF - CRIT REV ONCOL HEMAT

SN - 1040-8428

ER -