Hemophagocytic Lymphohistiocytosis (HLH) Explained #50

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Welcome to the new HLH episode. I have redone this one as the sound was so bad on the previous episode. Hope you find this useful.
Hemophagocytic lymphohistiocytosis (HLH) is a rare disease that can be present in HIV positive patients.
Usually in the adult, HLH is an acquired disease and there is usually an underlying driver such as EBV infection or a lymphoma (there are many other infections or diseases that can drive HLH forward).
While the prognosis of HLH is generally quite poor, advances are being made on a regular basis and this video may very well be out of date within a few months of being published.
You can gain further resources from the Histiocytosis Association.
Easy read from: primaryimmune.org/disease/hem...
References from the video are:
1. Familial hemophagocytic lymphohistiocytosis. Genetics Home Reference. November 2014; ghr.nlm.nih.gov/condition/fami....
2. Kenneth L McClain, MD, PhD. Clinical features and diagnosis of hemophagocytic lymphohistiocytosis. UpToDate. July 29, 2015;
3. George MR.. Hemophagocytic lymphohistiocytosis: review of etiologies and management.. J Blood Med. June 2014; 5:69-86. www.ncbi.nlm.nih.gov/pmc/arti...
4. Kejian Zhang, MD, MBA, Alexandra H Filipovich, MD, Judith Johnson, MS, Rebecca A Marsh, MD, and Joyce Villanueva, MT, MBA.. Hemophagocytic Lymphohistiocytosis, Familial. GeneReviews. January 17, 2013; www.ncbi.nlm.nih.gov/books/NBK....
5. Kenneth L McClain, MD, PhD. Treatment and prognosis of hemophagocytic lymphohistiocytosis. UpToDate. October 15, 2015;
6. Zhang K, Wakefield E, and Marsh R. Lymphoproliferative Disease, X-Linked. GeneReviews. June 30, 2016; www.ncbi.nlm.nih.gov/books/NB....
7. Janka GE, Lehmberg K.. Hemophagocytic lymphohistiocytosis: pathogenesis and treatment.. Hematology Am Soc Hematol Educ Program. 2013; 2013:605-611. www.ncbi.nlm.nih.gov/pubmed/2....
8. Zhang L, Zhou J & Sokol L. Hereditary and acquired hemophagocytic lymphohistiocytosis. Cancer Control. October 2014; 21(4):301-312.
9. Schwartz RA. Lymphohistiocytosis (Hemophagocytic Lymphohistiocytosis). Medscape. 2016; emedicine.medscape.com/article...
10. Chellapandian D, Das R, Zelley K, et al. Treatment of Epstein Barr virus-induced haemophagocytic lymphohistiocytosis with rituximab-containing chemo-immunotherapeutic regimens. Br J Haematol. 2013;162(3):376-382. doi:10.1111/bjh.12386
11. Catherine M. Bollard, et al. How I treat T-cell chronic active Epstein-Barr virus disease. Blood. 2018;131(26):2899-2905
12. Christos Stefanou, et al. Combining an antiviral with rituximab in EBV-related haemophagocytic lymphohistiocytosis led to rapid viral clearance; and a comprehensive review. BMJ Case Reports 2016 Jan. casereports.bmj.com/content/2...
Take care and have great sexual health.
Bret

[@DrBretPalmer]

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