HLH M&M Sources

FOAM Sources (Free open access medical education):

Maryland CC Project – Jennie Law MD : Hemophagocytic Lymphohistiocytosis, a critical care conundrum

PulmCrit- Sepsis 4.0: Understanding sepsis-HLH overlap syndrome



Clinical characteristics, prognostic factors, and outcomes of  adult patients with hemophagocytic lymphohistiocytosis Zaher K. Otrock and Charles S. Eby  American Journal of Hematology, Vol. 90, No. 3, March 2015


Prognostic factors and outcomes of adults with hemophagocytic lymphohistiocytosis.  Mayo Clin Proc. 2014 Apr;89(4):484-92.

Prognostic factors and outcomes of adult-onset hemophagocytic lymphohistiocytosis:
a retrospective analysis of 34 cases  Hematology Reports 2015; volume 7:5841

Histoplasmosis-Induced Hemophagocytic Syndrome: A Case Series and Review of the Literature  Open Forum Infect Dis. 2015 Apr; 2(2):


Reactive hemophagocytic syndrome in adults: A multicenter retrospective analysis of 162 patients Am J Med. 2014 Nov;127(11):1118-25


Infection is the major trigger of hemophagocytic syndrome in adult patients treated with biological therapies. Brito-Zerón P, et al. Semin Arthritis Rheum. 2016.


Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome. Fardet L, et al. Arthritis Rheumatol. 2014.
Highly elevated ferritin levels and the diagnosis of hemophagocytic lymphohistiocytosis. Allen CE, et al. Pediatr Blood Cancer. 2008.

Marked hyperferritinemia does not predict for HLH in the adult population. Schram AM, et al. Blood. 2015.

The role of the initial bone marrow aspirate in the diagnosis of hemophagocytic lymphohistiocytosis Pediatr Blood Cancer 2008;51:402–404

Marrow Assessment for Hemophagocytic Lymphohistiocytosis Demonstrates Poor Correlation With Disease Probability Am J Clin Pathol (2014) 141 (1)62-71


Interleukin-1 Receptor Blockade Is Associated With Reduced Mortality in Sepsis Patients With Features of Macrophage Activation Syndrome: Reanalysis of a Prior Phase III Trial. Crit Care Med. 2016 Feb;44(2):275-81

Prognostic factors of early death in a cohort of 162 adult haemophagocytic syndrome: impact of triggering disease and early treatment with etoposide Arca, M., et. al. 2015 Br J Haematol, 168: 63–68



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