Paediatric Haemophagocytic Lymphohistiocytosis: Clinical Presentation and Outcome of 20 Patients at a Single Institution

Overview

Journal Sudan J Paediatr

Specialty Pediatrics

Date 2024 Feb 21

PMID 38380413

Authors

Haifa Ali Bin Dahman

Ali Omer Aljabry

Affiliations

Soon will be listed here.

Abstract

Paediatric haemophagocytic lymphohistiocytosis (pHLH) is a potentially life-threatening condition with significant diagnostic and therapeutic difficulties. The purpose of this study was to describe the clinical presentation, the diagnostic challenges, and the outcomes of haemophagocytic lymphohistiocytosis (HLH) in children assessed at Mukalla Hospital, Yemen. Data from 20 medical records of HLH patients admitted between January 2010 and May 2022 were retrospectively analysed. The median age at presentation was 3.5 ± 5.1 years. Male: female ratio was 1:1. The median time for referral to the hospital was 30 ± 64 days. The most common clinical manifestations were fever and pallor in 95% of cases, and splenomegaly (85%). Hepatomegaly, chest, renal and neurological manifestations were detected in 80%, 45%, 15% and 20% of cases, respectively. Bone marrow haemophagocytosis was detected in 60% of cases. Sixteen patients fulfilled the HLH diagnostic criteria, and 11 patients (55%) received the HLH 2004 protocol. Out of the 20 patients, three (15%) patients are alive. Fourteen patients died, with overall mortality of 82.35%. All mortalities were due to HLH disease with multi-organ failure. Relapse was noticed in five patients either during treatment or after full recovery. pHLH is a challenging emergency with a high mortality rate. High clinical suspicion is essential for early detection and intervention to improve the prognosis.

References

Ishii E, Ohga S, Tanimura M, Imashuku S, Sako M, Mizutani S . Clinical and epidemiologic studies of familial hemophagocytic lymphohistiocytosis in Japan. Japan LCH Study Group. Med Pediatr Oncol. 1998; 30(5):276-83. DOI: 10.1002/(sici)1096-911x(199805)30:5<276::aid-mpo3>3.0.co;2-c. View

Luo Z, Chen Y, Xu X, Zhao N, Tang Y . Prognostic factors of early death in children with hemophagocytic lymphohistiocytosis. Cytokine. 2017; 97:80-85. DOI: 10.1016/j.cyto.2017.03.013. View

Ellis E, Sharp T, Perez-Padilla J, Gonzalez L, Poole-Smith B, Lebo E . Incidence and Risk Factors for Developing Dengue-Associated Hemophagocytic Lymphohistiocytosis in Puerto Rico, 2008 - 2013. PLoS Negl Trop Dis. 2016; 10(8):e0004939. PMC: 4996422. DOI: 10.1371/journal.pntd.0004939. View

Mahlaoui N, Ouachee-Chardin M, de Saint Basile G, Neven B, Picard C, Blanche S . Immunotherapy of familial hemophagocytic lymphohistiocytosis with antithymocyte globulins: a single-center retrospective report of 38 patients. Pediatrics. 2007; 120(3):e622-8. DOI: 10.1542/peds.2006-3164. View

Weitzman S . Approach to hemophagocytic syndromes. Hematology Am Soc Hematol Educ Program. 2011; 2011:178-83. DOI: 10.1182/asheducation-2011.1.178. View

Filipovich A . Hemophagocytic lymphohistiocytosis (HLH) and related disorders. Hematology Am Soc Hematol Educ Program. 2009; :127-31. DOI: 10.1182/asheducation-2009.1.127. View

Meeths M, Horne A, Sabel M, Bryceson Y, Henter J . Incidence and clinical presentation of primary hemophagocytic lymphohistiocytosis in Sweden. Pediatr Blood Cancer. 2014; 62(2):346-352. DOI: 10.1002/pbc.25308. View

Singh S, Chandrakasan S, Ahluwalia J, Suri D, Rawat A, Ahmed N . Macrophage activation syndrome in children with systemic onset juvenile idiopathic arthritis: clinical experience from northwest India. Rheumatol Int. 2011; 32(4):881-6. DOI: 10.1007/s00296-010-1711-z. View

Cancado G, Freitas G, Faria F, Vaz de Macedo A, Nobre V . Hemophagocytic lymphohistiocytosis associated with visceral leishmaniasis in late adulthood. Am J Trop Med Hyg. 2013; 88(3):575-7. PMC: 3592544. DOI: 10.4269/ajtmh.12-0563. View

10.

Henter J, Elinder G, Soder O, Ost A . Incidence in Sweden and clinical features of familial hemophagocytic lymphohistiocytosis. Acta Paediatr Scand. 1991; 80(4):428-35. DOI: 10.1111/j.1651-2227.1991.tb11878.x. View

11.

Ishii E . Hemophagocytic Lymphohistiocytosis in Children: Pathogenesis and Treatment. Front Pediatr. 2016; 4:47. PMC: 4865497. DOI: 10.3389/fped.2016.00047. View

12.

Chan J, Shing M, Lee V, Li C, Yuen P . Haemophagocytic lymphohistiocytosis in Hong Kong children. Hong Kong Med J. 2008; 14(4):308-13. View

13.

Ramachandran B, Balasubramanian S, Abhishek N, Ravikumar K, Ramanan A . Profile of hemophagocytic lymphohistiocytosis in children in a tertiary care hospital in India. Indian Pediatr. 2010; 48(1):31-5. DOI: 10.1007/s13312-011-0020-2. View

14.

Rodrigues J, Nasr B, Cypriano M . Hemophagocytic lymphohistiocytosis: presentation and outcome of twenty-one patients at a single institution. Hematol Transfus Cell Ther. 2021; 44(4):485-490. PMC: 9605882. DOI: 10.1016/j.htct.2021.05.003. View

15.

Zhang Z, Wang J, Ji B, von Bahr Greenwood T, Zhang Y, Wang Y . Clinical presentation of hemophagocytic lymphohistiocytosis in adults is less typical than in children. Clinics (Sao Paulo). 2016; 71(4):205-9. PMC: 4825195. DOI: 10.6061/clinics/2016(04)05. View

16.

Tristano A, Casanova-Escalona L, Torres A, Rodriguez M . Macrophage activation syndrome in a patient with systemic onset rheumatoid arthritis: rescue with intravenous immunoglobulin therapy. J Clin Rheumatol. 2006; 9(4):253-8. DOI: 10.1097/01.rhu.0000081259.61370.eb. View

17.

Astigarraga I, Gonzalez-Granado L, Allende L, Alsina L . [Haemophagocytic syndromes: The importance of early diagnosis and treatment]. An Pediatr (Engl Ed). 2018; 89(2):124.e1-124.e8. DOI: 10.1016/j.anpedi.2018.05.003. View

18.

Rouphael N, Talati N, Vaughan C, Cunningham K, Moreira R, Gould C . Infections associated with haemophagocytic syndrome. Lancet Infect Dis. 2007; 7(12):814-22. PMC: 7185531. DOI: 10.1016/S1473-3099(07)70290-6. View

19.

Nandhakumar D, Loganatha A, Sivasankaran M, Sivabalan S, Munirathnam D . Hemophagocytic Lymphohistiocytosis in Children. Indian J Pediatr. 2020; 87(7):526-531. DOI: 10.1007/s12098-020-03190-6. View

20.

Henter J, Horne A, Arico M, Egeler R, Filipovich A, Imashuku S . HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2006; 48(2):124-31. DOI: 10.1002/pbc.21039. View