CASE REPORT
Neuroblastoma presenting as haemophagocytic lymphohistiocytosis
1
Department of Paediatric Haematology and Oncology, Jurasz University Hospital 1, Collegium Medicum, Nicolaus Copernicus University Toruń, Bydgoszcz, Poland
Submission date: 2024-11-05
Final revision date: 2025-02-02
Acceptance date: 2025-02-17
Publication date: 2025-04-15
Corresponding author
Monika Pogorzala
Department of Paediatric Haematology and Oncology, Jurasz University Hospital 1, Collegium Medicum, Nicolaus Copernicus University Toruń, Bydgoszcz, Poland
Department of Paediatric Haematology and Oncology, Jurasz University Hospital 1, Collegium Medicum, Nicolaus Copernicus University Toruń, Bydgoszcz, Poland
Pediatr Pol 2025;61(1):109-116
KEYWORDS
TOPICS
ABSTRACT
Haemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening syndrome of excessive activation of the immune system, which can be primary (genetically determined) or secondary (acquired). Secondary forms are most often triggered by infections, but also by autoimmune processes, neoplastic diseases, drugs, and metabolic diseases. Secondary HLH associated with malignancy occurs more often in adults, mainly in the course of lymphomas and leukaemia. It is rarely induced by solid tumours but can develop during chemotherapy. The presented case of a 1.5-year-old child reflects a very rare situation when symptoms of haemophagocytic syndrome turned out to be the primary manifestation of a non-haematological proliferative disease.
REFERENCES (25)
1.
Griffin G, Shenoi S, Hughes GC. Hemophagocytic lympho histiocytosis: An update on pathogenesis, diagnosis, and therapy. Best Pract Res Clin Rheumatol 2020; 34: 101515.
2.
Benevenuta C, Mussinattto I, Orsi C, et al. Secondary hemophagocytic lymphohistiocytosis in children. Exp Ther Med 2023; 26: 423.
3.
Strenger V, Merth G, Lackner H, et al. Malignancy and chemotherapy induced hemphagocytic lymphohistiocytosis in children and adolescent – a single center experience of 20 years. Ann Hematol 2018; 97: 989-998.
4.
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.
5.
Henter JI, Horne A, Aricó M, et al. HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007; 48: 124-131.
7.
Debaugnies F, Mahadeb B, Ferster A, et al. Performances of the H score for diagnosis of hemophagocytic lymphohistiocytosis in adult and pediatric patients. Am J Clin Pathol 2016; 145: 862 870.
8.
Shakoory B, Geerlinks A, Wilejto M, et al. The 2022 EULAR/ACR points to consider at the early stages of diagnosis and management of suspected haemophagocytic lymphohistiocytosis/macrophage activation syndrome (HLH/ MAS). Ann Rheum Dis 2023; 82: 1271-1285.
9.
Bami S, Vegrecha A, Soberman D, et al. The use of anakinra in the treatment of secondary hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2020; 67: e28581.
10.
Eloseily EM, Weiser P, Crayne CB, et al. Benefit of anakinra in treating pediatric secondary hemophagocytic lymphohistiocytosis. Arthritis Rheumatol 2020; 72: 326-334.
11.
Gurunathan A, Boucher AA, Mark M, et al. Limitations of HLH 2004 criteria in distinguishing malignancy associated hemophagocytic lympho histiocytosis. Pediatr Blood Cancer 2018; 65: e27400.
12.
Lehmberg K, Nichols KE, Henter JI, et al. Consensus recommendations for the diagnosis and management of hemophagocytic lymphohistiocytosis associated with malignancies. Haematologica 2015; 100: 997 1004.
13.
Bergsten E, Horne A, Arico M, et al. Confirmed efficacy of etoposide and dexamethasone in HLH treatment: long-term results of the cooperative HLH-2004 study. Blood 2017; 130: 2728-2738.
14.
Henter JI, von Bahr Greenwood T. Etoposide therapy of cytokine storm syndromes. Adv Exp Med Biol 2024; 1448: 525-551.
15.
Gopalakrishnan M, Ramanathan A, Jayaraman D, et al. Malignancy-associated secondary hemophagocytic lymphohistiocytosis mimicking an infection: a case report and review of the literature. Cureus 2024; 16: e56738.
16.
O’Brien MM, Lee-Kim Y, George TI, et al. Precursor B-cell acute lymphoblastic leukemia presenting with hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2008; 50: 381-383.
17.
Cader FZ, Colmenero I, Mussai F. Hemophagocytic lymphohistiocytosis associated with 2 cases of pediatric lymphocyte depleted classic Hodgkin lymphoma. J Pediatr Hematol Oncol 2019; 41: e341 e345.
18.
Huang Z, Jia Y, Zuo Y, et al. Malignancy associated hemophagocytic lymphohistiocytosis in children: A 10 year experience of a single pediatric hematology center. Hematology 2020; 25: 389 399.
19.
Ćwiklińska M, Czogała M, Balwierz W, et al. Zespół hemofagocytarny u dzieci w różnych jednostkach chorobowych. Przeg Lek 2010; 67: 430-435.
20.
Murphy EP, Mo J, Yoon JM. Secondary hemophagocytic lymphohistiocytosis in a patient with favorable histology Wilms tumor. J Pediatr Hematol Oncol 2015; 37: e494 e496.
21.
Celkan T, Berrak S, Kazanci E, et al. Malignancy-associated hemophagocytic lymphohistiocytosis in pediatric cases: a multicenter study from Turkey. Turk J Pediatr 2009; 51: 207-213.
22.
Zhang WY, Zhang Y, Dong NN, et al. Clinical features and prognosis of malignancy-associated hemophagocytic lymphohistiocytosis in children: a clinical analysis of 24 cases. Zhongguo Dang Dai Er Ke Za Zhi 2018; 20: 295-297.
23.
Nascimento FA, Nery J, Trennepohl J, et al. Hemophagocytic lymphohistiocytosis after initiation of chemo therapy for bilateral adrenal neuroblastoma. J Pediatr Hematol Oncol 2016; 38: e13 e15.
24.
Kounami S, Nakayama K, Yoshiyama M, et al. Early-onset hemophagocytic lymphohistiocytosis after the start of chemotherapy for advanced neuroblastoma. Pediatr Hematol Oncol 2012; 29: 99-103.
25.
Nozawa A, Ozeki M, Yasue S, et al. Proinflammatory cytokine secretion in a patient with recurrent neuroblastoma related to the onset of malignancy-associated hemophagocytic lymphohistiocytosis. J Pediatr Hematol Oncol 2020; 42: e199-e201.
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