Hemophagocytic lymphohistiocytosis: pouring gasoline on the cytokine storm


Abstract views: 155 / PDF downloads: 100

Authors

  • Ola ABUZAID Erciyes University, Faculty of Medicine, Kayseri, Turkey
  • Şefika AKYOL Erciyes University, Faculty of Medicine, Department of Pediatrics, Division of Pediatric Hematology Oncology & Hematopoietic Stem Cell Transplant Unit, Kayseri, Turkey
  • Said Can ALCALI
  • Ekrem UNAL Erciyes University

DOI:

https://doi.org/10.51271/KMJ-0036

Keywords:

Hemophagocytic lymphohistiocytosis, hyperferritinemia, macrophage activation syndromes

Abstract

Hemophagocytic lymphohistiocytosis should be considered in patients with persistent fever, hepatosplenomegaly, pancytopenia. Hypercytokinemia originated from genetic disorder effecting the cellular defects of cytotoxic T and natural killer lymphocyte activity is the underlying pathophysiology of the disorder. In this review we summarized the recent advances in the management of hemophagocytic lymphohistiocytosis.

References

-Fışgın T, Patıroğlu T, Özdemir A, et al. Clinical and laboratory data of primary hemophagocytic lymphohistiocytosis: A retrospective review of the Turkish Histiocyte Study Group. Primer hemofagositik lenfohistiositozisli hastaların klinik ve labarotuvar verileri; Türk Histiosit Çalışma Grubunun retrospektif derlemesi. Turk J Haematol 2010; 27: 257-62.

Simon AC, Delhi Kumar CG, Basu D, Ramesh Kumar R. Hemophagocytic lymphohistiocytosis in children: clinical profile and outcome. J Pediatr Hematol Oncol 2020; 42: 281-5.

Esteban YM, de Jong JLO, Tesher MS. An Overview of hemophagocytic lymphohistiocytosis. Pediatr Ann 2017; 46: 309-13.

Kardas F, Patiroglu T, Unal E, Chiang SC, Bryceson YT, Kendirci M. Hemophagocytic syndrome in a 4-month-old infant with biotinidase deficiency. Pediatr Blood Cancer 2012; 59: 191-3.

Cansever M, Zietara N, Chiang SCC, et al. A rare case of activated phosphoinositide 3-kinase delta syndrome (APDS) presenting with

hemophagocytosis complicated with Hodgkin lymphoma. J Pediatr Hematol Oncol 2020; 42: 156-9.

Si SJ, Tasian SK, Bassiri H, et al. Diagnostic challenges in pediatric hemophagocytic lymphohistiocytosis. J Clin Immunol 2021; 41: 1213-8.

Trottestam H, Horne A, Aricò M, et al. Chemoimmunotherapy for hemophagocytic lymphohistiocytosis: long-term results of the HLH-94 treatment protocol. Blood 2011; 118: 4577-84.

Ponnatt TS, Lilley CM, Mirza KM. Hemophagocytic lymphohistiocytosis. Arch Pathol Lab Med 2022; 146: 507-19.

Niece JA, Rogers ZR, Ahmad N, Langevin AM, McClain KL. Hemophagocytic lymphohistiocytosis in Texas: observations on ethnicity

and race. Pediatr Blood Cancer 2010; 54: 424-8.

Henter JI, Elinder G, Soder O, Ost A. Incidence in Sweden and clinical features of familial hemophagocytic lymphohistiocytosis. Acta Paediatr Scand 1991; 80: 428-35.

Al-Samkari H, Berliner N. Hemophagocytic lymphohistiocytosis. Annu Rev Pathol 2018; 13: 27-49.

Schram AM, Comstock P, Campo M, et al. Haemophagocytic lymphohistiocytosis in adults: a multicentre case series over 7 years. Br J Haematol 2016; 172: 412-9.

11.Parikh SA, Kapoor P, Letendre L, Kumar S, Wolanskyj AP. Prognostic factors and outcomes of adults with hemophagocytic lymphohistiocytosis. Mayo Clin Proc 2014; 89: 484-92.

Stepp SE, Dufourcq-Lagelouse R, Le Deist F, et al. Perforin gene defects in familial hemophagocytic lymphohistiocytosis. Science 1999; 286: 1957-9.

Canna SW, Marsh RA. Pediatric hemophagocytic lymphohistiocytosis. Blood 2020; 135: 1332-43.

Akyol S, Ozcan A, Sekine T, et al. Different clinical presentation of 3 children with familial hemophagocytic lymphohistiocytosis with 2 novel mutations. J Pediatr Hematol Oncol 2020; 42: 627-9.

Schram AM, Berliner N. How I treat hemophagocytic lymphohistiocytosis in the adult patient. Blood 2015; 125: 2908-14.

Hue SS, Oon ML, Wang S, Tan SY, Ng SB. Epstein-Barr virus-associated T-and NK-cell lymphoproliferative diseases: an update and diagnostic approach. Pathology 2020; 52: 111-27.

Sifers TM, Raje N, Dinakar C. Hemophagocytic lymphohistiocytosis: A concise review for the practicing physician. Allergy Asthma Proc 2016; 37: 256-8.

Rosado FGN, Kim AS. Hemophagocytic lymphohistiocytosis. Am J Clin Pathol 2013; 139: 713-27.

Janka GE, Lehmberg K. Hemophagocytic lymphohistiocytosis: pathogenesis and treat-ment. Hematology Am Soc Hematol Educ

Program 2013; 2013: 605-11.

Minoia F, Davì S, Horne A, et al. Clinical features, treatment, and outcome of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis: a multinational, multicenter study of 362 patients. Arthritis Rheumatol 2014; 66: 3160-9.

Griffin G, Shenoi S, Hughes GC. Hemophagocytic lymphohistiocytosis: an update on pathogenesis, diagnosis, and therapy. Best Pract Res Clin Rheumatol 2020; 34: 101515.

Ciraci S, Ozcan A, Ozdemir MM, et al. A case of familial hemophagocytic lymphohistiocytosis type 4 with involvement of the central nervous system complicated with infarct. J Pediatr Hematol Oncol 2017; 39: 321-4.

Çetin BŞ. SARS-CoV-2 Infection in children; what do we know so far? J Pediatr Acad 2020; 1: 76-85.

Özdemir Çiçek S, Paç Kısaarslan A. Pediatric rheumatologists’ perspective on corona virus disease 2019. J Pediatr Acad 2020; 1: 3-7.

Jordan MB, Allen CE, Greenberg J, et al. Challenges in the diagnosis of hemophagocytic lymphohistiocytosis: Recommendations from the North American Consortium for Histiocytosis (NACHO). Pediatr Blood Cancer 2019; 66: e27929.

Henter JI, Horne A, Aricó M, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007; 48: 124-31.

Fardet L, Galicier L, Lambotte O, et al. Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome. Arthritis Rheumatol 2014; 66: 2613-20.

Otterbein LE, Soares MP, Yamashita K, Bach FH. Heme oxygenase-1: unleashing the protective properties of heme. Trends Immunol 2003; 24: 449-55.

Tabata C, Tabata R. Possible prediction of underlying lymphoma by high sIL-2R/ferritin ratio in hemophagocytic syndrome. Ann Hematol 2012; 91: 63-71.

Hayden A, Park S, Giustini D, Lee AYY, Chen LYC. Hemophagocytic syndromes (HPSs) including hemophagocytic lymphohistiocytosis (HLH) in adults: a systematic scoping review. Blood Rev 2016; 30: 411-20.

Goel S, Polski JM, Imran H. Sensitivity and specificity of bone marrow hemophagocytosis in hemophagocytic lymphohistiocytosis. Ann Clin Lab Sci 2012; 42: 21-5.

Gars E, Purington N, Scott G, et al. Bone marrow histomorphological criteria can accurately diagnose hemophagocytic lymphohistiocytosis. Haematologica 2018; 103: 1635-41.

La Rosée P, Horne A, Hines M, et al Recommendations for the management of hemophagocytic lymphohistiocytosis in adults. Blood

; 133: 2465-77.

Machowicz R, Janka G, Wiktor-Jedrzejczak W. Similar but not the same: differential diagnosis of HLH and sepsis. Crit Rev Oncol Hematol 2017; 114: 1-12.

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: 3.

Aricò M, Janka G, Fischer A, et al. Hemophagocytic lymphohistiocytosis. Report of 122 children from the International Registry. Leukemia 1996; 10: 197-203.

Lehmberg K, Sprekels B, Nichols KE, et al. Malignancy-associated haemophagocytic lymphohistiocy

Downloads

Published

2022-06-21

How to Cite

ABUZAID , O. ., AKYOL, Şefika ., ALCALI, S. C. ., & UNAL, E. (2022). Hemophagocytic lymphohistiocytosis: pouring gasoline on the cytokine storm. Kastamonu Medical Journal, 2(2), 30–34. https://doi.org/10.51271/KMJ-0036