NDLI: Proliferation through activation: hemophagocytic lymphohistiocytosis in hematologic malignancy

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Proliferation through activation: hemophagocytic lymphohistiocytosis in hematologic malignancy

Content Provider	Scilit
Author	Vick, Eric J. Patel, Kruti Prouet, Philippe Martin, Mike G.
Copyright Year	2017
Description	Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of cytokine-driven immune activation. Cardinal features include fever, hemophagocytosis, hepatosplenomegaly, lymphocytic infiltration, and hypercytokinemia that result in multisystem organ dysfunction and failure. Familial HLH is genetically driven, whereas secondary HLH (SHL) is caused by drugs, autoimmune disease, infection, or cancer. SHL is associated with worse outcomes, with a median overall survival typically of less than 1 year. This reflects difficulty in both diagnostic accuracy and in establishing reliable treatments, especially in cases of malignancy-induced SHL, which have significantly worse outcomes. Malignancy-induced HLH is seen almost exclusively with hematologic malignancies, constituting 97% of cases in the literature over the past 2 years. In these situations, the native immune response driven by CD8 T cells produces an overabundance of T helper 1 cytokines, notably interferon-γ, tumor necrosis factor-α, and interleukin-6, which establish a positive feedback loop of inflammation, enhancing replication of hematologic malignancies while leaving the host immune system in disarray. In this paper, we present 2 case studies of secondary HLH driven by HM, followed by a review of the literature discussing the cytokines driving HLH, diagnostic criteria, and current treatments used or undergoing investigation.
Related Links	http://www.bloodadvances.org/content/bloodoa/1/12/779.full.pdf
Ending Page	791
Page Count	13
Starting Page	779
DOI	10.1182/bloodadvances.2017005561
Journal	Blood advances
Issue Number	12
Volume Number	1
Language	English
Publisher	American Society of Hematology
Publisher Date	2017-05-09
Access Restriction	Open
Subject Keyword	Hematology Survival Treatments Malignancy Hematologic Malignancies Worse Lymphohistiocytosis Secondary Hlh Journal: Blood advances (Vol- 118, Issue- 12)
Content Type	Text
Resource Type	Article

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