Hemophagocytic syndromes - An update

Gritta Janka-Schaub; Kai Lehmberg

doi:10.1016/j.blre.2014.03.002

Hemophagocytic syndromes - An update

Standard

Hemophagocytic syndromes - An update. / Janka-Schaub, Gritta; Lehmberg, Kai.

In: BLOOD REV, Vol. 28, No. 4, 22.03.2014, p. 135-142.

Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review

Harvard

Janka-Schaub, G & Lehmberg, K 2014, 'Hemophagocytic syndromes - An update', BLOOD REV, vol. 28, no. 4, pp. 135-142. https://doi.org/10.1016/j.blre.2014.03.002

APA

Janka-Schaub, G., & Lehmberg, K. (2014). Hemophagocytic syndromes - An update. BLOOD REV, 28(4), 135-142. https://doi.org/10.1016/j.blre.2014.03.002

Vancouver

Janka-Schaub G, Lehmberg K. Hemophagocytic syndromes - An update. BLOOD REV. 2014 Mar 22;28(4):135-142. https://doi.org/10.1016/j.blre.2014.03.002

Bibtex

@article{2261b8a92699495bbf106f5a36d6ce7d,

title = "Hemophagocytic syndromes - An update",

abstract = "Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome and not an independent disease. HLH represents the extreme end of a severe uncontrolled hyperinflammatory reaction that can occur in many underlying conditions. Genetic forms of HLHs are due to defects in transport, processing and function of cytotoxic granules in natural killer cells and cytotoxic T lymphocytes, and are not restricted to manifestation in childhood. Acquired forms of HLH are encountered in infections, autoinflammatory and autoimmune diseases, malignancies, acquired immune deficiency. Functional tests allow for differentiation between genetic and acquired HLH. Treatment aims at suppressing hypercytokinemia and eliminating activated and infected cells. It includes immunomodulatory and immunosuppressive agents, cytostatics, T-cell and cytokine antibodies. In genetic HLH cure can only be achieved with hematopoietic stem cell transplantation. Reduced-intensity conditioning regimens have considerably improved survival.",

author = "Gritta Janka-Schaub and Kai Lehmberg",

year = "2014",

month = mar,

day = "22",

doi = "10.1016/j.blre.2014.03.002",

language = "English",

volume = "28",

pages = "135--142",

journal = "BLOOD REV",

issn = "0268-960X",

publisher = "Churchill Livingstone",

number = "4",

}

RIS

TY - JOUR

T1 - Hemophagocytic syndromes - An update

AU - Janka-Schaub, Gritta

AU - Lehmberg, Kai

PY - 2014/3/22

Y1 - 2014/3/22

N2 - Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome and not an independent disease. HLH represents the extreme end of a severe uncontrolled hyperinflammatory reaction that can occur in many underlying conditions. Genetic forms of HLHs are due to defects in transport, processing and function of cytotoxic granules in natural killer cells and cytotoxic T lymphocytes, and are not restricted to manifestation in childhood. Acquired forms of HLH are encountered in infections, autoinflammatory and autoimmune diseases, malignancies, acquired immune deficiency. Functional tests allow for differentiation between genetic and acquired HLH. Treatment aims at suppressing hypercytokinemia and eliminating activated and infected cells. It includes immunomodulatory and immunosuppressive agents, cytostatics, T-cell and cytokine antibodies. In genetic HLH cure can only be achieved with hematopoietic stem cell transplantation. Reduced-intensity conditioning regimens have considerably improved survival.

AB - Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome and not an independent disease. HLH represents the extreme end of a severe uncontrolled hyperinflammatory reaction that can occur in many underlying conditions. Genetic forms of HLHs are due to defects in transport, processing and function of cytotoxic granules in natural killer cells and cytotoxic T lymphocytes, and are not restricted to manifestation in childhood. Acquired forms of HLH are encountered in infections, autoinflammatory and autoimmune diseases, malignancies, acquired immune deficiency. Functional tests allow for differentiation between genetic and acquired HLH. Treatment aims at suppressing hypercytokinemia and eliminating activated and infected cells. It includes immunomodulatory and immunosuppressive agents, cytostatics, T-cell and cytokine antibodies. In genetic HLH cure can only be achieved with hematopoietic stem cell transplantation. Reduced-intensity conditioning regimens have considerably improved survival.

U2 - 10.1016/j.blre.2014.03.002

DO - 10.1016/j.blre.2014.03.002

M3 - SCORING: Journal article

C2 - 24792320

VL - 28

SP - 135

EP - 142

JO - BLOOD REV

JF - BLOOD REV

SN - 0268-960X

IS - 4

ER -