How I treat hemophagocytic lymphohistiocytosis in the adult patient.

Blood
Alison M Schram, Nancy Berliner

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a devastating disorder of uncontrolled immune activation characterized by clinical and laboratory evidence of extreme inflammation. This syndrome can be caused by genetic mutations affecting cytotoxic function (familial HLH) or be secondary to infectious, rheumatologic, malignant, or metabolic conditions (acquired HLH). Prompt recognition is paramount and, without early treatment, this disorder is frequently fatal. Although HLH is well described in the pediatric population, less is known about the appropriate work-up and treatment in adults. Here, we review the clinical characteristics, diagnosis, and treatment of HLH in adults.

References

Apr 1, 1991·Acta paediatrica Scandinavica·J I HenterA Ost
Feb 1, 1986·The Journal of Pediatrics·A FischerC Griscelli
Jun 1, 1983·European Journal of Pediatrics·G E Janka
Oct 1, 1995·European Journal of Haematology·K Shirono, H Tsuda
Jan 20, 1999·European Journal of Cancer : Official Journal for European Organization for Research and Treatment of Cancer (EORTC) [and] European Association for Cancer Research (EACR)·K R Hande
May 3, 2000·British Journal of Haematology·E GrunebaumC M Roifman
Jun 3, 2000·Nature Genetics·G MénaschéG de Saint Basile
Oct 23, 2001·Archives of Disease in Childhood·S SawhneyK J Murray
May 31, 2003·Pediatric Hematology and Oncology·A GürgeyG Hiçsönmez
Dec 1, 1952·Archives of Disease in Childhood·J W FARQUHAR, A E CLAIREAUX
Aug 29, 2006·Pediatric Blood & Cancer·Jan-Inge HenterGritta Janka
Nov 3, 2006·Nature·Stéphanie RigaudSylvain Latour
Nov 25, 2006·Nature Reviews. Immunology·Ilia VoskoboinikJoseph A Trapani
Nov 30, 2007·The Lancet Infectious Diseases·Nadine G RouphaelCarolyn Gould
Dec 19, 2007·Pediatric Blood & Cancer·Carl E AllenKenneth L McClain
Jun 5, 2008·Pediatric Blood & Cancer·Abha GuptaMohamed Abdelhaleem
Apr 21, 2010·Nature Reviews. Clinical Oncology·Matthew P StroutNancy Berliner
Jul 17, 2010·Nature Reviews. Immunology·Geneviève de Saint BasileAlain Fischer
Aug 11, 2011·Blood·Michael B JordanKenneth L McClain

❮ Previous
Next ❯

Citations

Nov 6, 2015·British Journal of Haematology·Alison M SchramNancy Berliner
Dec 29, 2015·Clinical Lymphoma, Myeloma & Leukemia·Marco PizziAmy Chadburn
Apr 22, 2016·International Urology and Nephrology·Edward J Filippone, John L Farber
Jun 19, 2016·The American Journal of Medicine·Kai Rou TeyArchita Desai
Jun 29, 2016·Hematology/oncology and Stem Cell Therapy·Ernesto AyalaMohamed A Kharfan-Dabaja
Aug 27, 2016·Case Reports in Hematology·Ghulam Rehman Mohyuddin, Heather J Male
Sep 16, 2016·Lancet·Josh T CoatsSudhir Tauro
Dec 8, 2015·Hematology·Paul La Rosée
Aug 9, 2016·Transplant Infectious Disease : an Official Journal of the Transplantation Society·I RomiopoulosE Roilides
Oct 12, 2016·Medicina clínica·Pilar Hernández-JiménezCarlos Lumbreras
Oct 19, 2016·Critical Care Medicine·Lawrence Rice, Jose Pacheco
Nov 18, 2016·Rheumatology International·L García-MontoyaE Naredo
May 20, 2017·Acta Clinica Belgica·Kelly De Schuyter, Tom Lodewyck
May 21, 2016·Journal of Investigative Medicine High Impact Case Reports·Tiffany Y Shaw, Michael Schivo
Oct 13, 2017·Journal of the European Academy of Dermatology and Venereology : JEADV·S TokoroH Yokozeki
Sep 20, 2017·Nature Reviews. Clinical Oncology·Sattva S NeelapuElizabeth J Shpall
Jul 4, 2018·Leukemia & Lymphoma·Neel S BhattJulie An Talano
Aug 9, 2018·Praxis·Grégoire StalderMichel André Duchosal
Feb 27, 2018·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Sidharth Kumar SethiShyam Bihari Bansal
Jun 4, 2019·AACN Advanced Critical Care·Janice SkinnerBrenda K Shelton
Sep 19, 2019·AJR. American Journal of Roentgenology·Alice C ShiehNikhil H Ramaiya
Dec 31, 2019·PloS One·Jacqueline De GottardiAnnalisa Berzigotti
Jun 20, 2019·Journal of Obstetrics and Gynaecology : the Journal of the Institute of Obstetrics and Gynaecology·Jie ChengWen Di
Feb 25, 2020·Acta Clinica Belgica·Astrid MuyldermansMarijke Reynders
Mar 10, 2020·Blood Advances·Peter G MillerBenjamin L Ebert
Mar 17, 2017·Journal of General Internal Medicine·Arjun GuptaJesse Jaso
Sep 30, 2017·Leukemia & Lymphoma·Prajwal BodduTapan M Kadia
Sep 22, 2017·Annual Review of Pathology·Hanny Al-Samkari, Nancy Berliner
Nov 10, 2018·Blood·Kenneth L McClain, Caridad A Martinez
Mar 10, 2019·International Journal of Hematology·Oren PasvolskyAvi Leader
Aug 12, 2019·BMJ Case Reports·Pawan Kumar SinghPrashant Sharma

❮ Previous
Next ❯

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Blastomycosis

Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.

Related Papers

Indian Journal of Hematology & Blood Transfusion : an Official Journal of Indian Society of Hematology and Blood Transfusion
A M V R NarendraV R Srinivasan
Medicine and Health, Rhode Island
Peter Rintels, Sundarsesan Sambandam
© 2022 Meta ULC. All rights reserved