CHARACTERISTICS OF HEMOPHAGOCYTIC SYNDROME PROGRESSION
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Gadabadze, M., Kandashvili, T., Gadabadze, Q., Geldiashvili, M., & Kiphshidze, S. (2023). CHARACTERISTICS OF HEMOPHAGOCYTIC SYNDROME PROGRESSION. Collection of Scientific Works of Tbilisi State Medical University, 56, 26–29. Retrieved from https://journals.4science.ge/index.php/CSW/article/view/2266

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is frequently associated with multiorgan damage. HLH most commonly occurs in the context of malignancies, infectious, and rheumatic diseases, with approximately 20% of cases having an unknown etiology (63). This study discusses the first HLH treatment protocol developed in 1994, referred to as “HLH-94,” and its subsequent revision in 2004, referred to as the “HLH-2004” protocol. The authors of this study also discuss the essential components of initial, specific therapy for HLH. The authors describe the clinical anamnesis of patients with secondary hemophagocytic syndrome, the course of the disease, the treatment administered, and the outcome of the condition in patients seen at the TSMU First University Clinic over the past year. In the cases presented, the diagnosis of hemophagocytic syndrome was delayed, resulting in poor patient outcomes. A complete retrospective analysis of the data was not possible due to the patients’ short follow-up period. In conclusion, a timely diagnosis of HLH and the initiation of appropriate treatment are crucial in determining the outcome of the disease.

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References

Kenneth Kaushanski, Marschall A. Lichtman, Josef T. Prchal, Marcel M. Levi, Oliver W. Press, Linda J. Burns, Michael A. Caligiuli: WILLIAMS Hematology, Hemophagocytic Lymphohistiocytosis, 2016, 9th edition, 1113-1114

Henter JI, Horne A, Aricó M, et al.. HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007;48(2):124-131.

Kenneth L McClain, M. P. (2020). Treatment and prognosis of hemophagocytic lymphohistiocytosis. uptodate.

Yu Ri Kim1, D.-Y. K. (2021). Current status of the diagnosis and treatment of hemophagocytic lymphohistiocytosis in adults. blood research, 5. [20]

Paul La Rosée, A. H.-H. (2019). Recommendations for the management of hemophagocytic lymphohistiocytosis in adults. American Society of Hematology - ASH Publications. [21]

Jordan MB, Allen CE, Weitzman S, Filipovich AH, McClain KL. How I treat hemophagocytic lymphohistiocytosis. Blood. 2011;118:4041–52.

Helena Trottestam, c. a.-I. (2011). Chemoimmunotherapy for hemophagocytic lymphohistiocytosis: long-term results of the HLH-94 treatment protocol. PubMed Central®(PMC).

Trottestam H, Horne A, Aricò M, Egeler RM, Filipovich AH, Gadner H, et al. Chemoimmunotherapy for hemophagocytic lymphohistiocytosis: Long-term results of the HLH-94 treatment protocol. Blood. 2011;118:4577–84

Horne A, Wickström R, Jordan MB, et al.. How to treat involvement of the central nervous system in hemophagocytic lymphohistiocytosis? Curr Treat Options Neurol. 2017;19(1):3.

Sunitha PazhaniyandiRamamoorthi Lenin ShobhanaSivathanu; Hemophagocytic lymphohistiocytosis with a leukemoid reaction in an infant with scrub typhus; Department of Paediatrics, ESIC Medical College & PGIMSR, K.K. Na-gar, Chennai 600 078, Tamil Nadu, India; Journal of Infection and Public Health Volume 8, Issue 6, November–December 2015, Pages 626-629

Elisabet Bergsten, A. H. (2017). Confirmed efficacy of etoposide and dexamethasone in HLH treatment: long-term results of the cooperative HLH-2004 study. American Society of Hematology ASH Publications.

Renal involvement as a unique manifestation of hemophagocytic syndrome. Roccatello D, Sciascia S, Barreca A, Naretto C, Alpa M, Quattrocchio G, Radin M, Fenoglio R. Front Med (Lausanne). 2022 Oct 5;9:796121.

Farquhar JW, Claireaux AE. Familial haemophagocytic reticulosis. Arch Dis Child. 1952;27(136):519–525.

Kurosawa S, Sekiya N, Fukushima K, et al. Unusual manifestation of disseminated herpes simplex virus type 2 infection associated with pharyngotonsilitis, esophagitis, and hemophagocytic lymphohisitocytosis without genital involvement. BMC Infect Dis. 2019;19(1):65.

Yamaguchi K, Yamamoto A, Hisano M, Natori M, Murashima A. Herpes Simplex Virus 2–Associated Hemophagocytic Lymphohistiocytosis in a Pregnant Patient. Obstet Gynecol. 2005;105(5):1241-4.

Nasser MF, Sharma S, Albers E, Sharma S, Duggal A. Pregnancy-related Hemophagocytic Lymphohistiocytosis Associated with Herpes Simplex Virus-2 Infection: A Diagnostic Dilemma. Cureus. 2018;10(3).

Ikumi K, Ando T, Katano H, et al. HSV-2–related hemophagocytic lymphohistiocytosis in a fingolimod-treated patient with MS. Neurol Neuroinflammation. 2016;3(4):e247.

Tanya SajanPonnatt, M., Cullen M. Lilley, M., & Kamran M. Mirza. (2021). Hemophagocytic Lymphohistiocytosis. Archives of Pathology & Laboratory Medicine.

Jan-Inge Henter, M. A. (1997). HLH-94: A Treatment Protocol for Hemophagocytic Lymphohistiocytosis. Medical and Pediatric Oncology.

K. Goudarzipour, 1. M. (2013). Epstein-Barr Virus-Induced Hemophagocytic Lymphohistiocytosis. pubmed central.

BindiyaSalunke, 1. S. (2019). Hemophagocytic Syndrome-An Approach to the Management. pubmed central.

Henter JI, Samuelsson-Horne A, Arico M, et al. Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation, Blood, 2002, vol. 100 (pg. 2367-2373)

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