Hanane ZAHIR, Jihane BELKHIR, Mustapha AIT AMEUR, Hanane MOUHIB, Mohammed CHAKOUR

Hemophagocytic lymphohistiocytosis: epidemiological, clinical and biological profile

Background/aim: Hemophagocytic lymphohistiocytosis (HLH) is a clinical, biological, and pathological entity that is rare but has certainmorbidity that may be life-threatening. This work aims to establish a focus on the hemophagocytic lymphohistiocytosis and analyzedifferent aspects of diagnosis while emphasizing the biological data.Materials and methods: We report the results of a retrospective study conducted in the hematology department of Avicenna Hospital inMarrakesh. Thirty-one patients with hemophagocytic lymphohistiocytosis were enrolled.Results: The clinical presentation was dominated by fever and deterioration of the general state for almost all our patients. Splenomegalywas objectified in 90% of the patients. Hepatomegaly, lymphadenopathy, and hemorrhagic manifestations were observed in almost50% of the patients. Biological assessments revealed bi- or pancytopenia in 96% of the patients, and coagulation disorders in 51% ofthe patients. On the other hand, hyperferritinemia was found in 84% of the patients, and hepatic cytolysis and hypertriglyceridemia inhalf of the patients. Hemophagocytosis was observed in all bone marrow samples taken from our patients. Concerning the evolution ofpatients, in 38.5% of the patients, the evolution was favorable with regression of clinical and biological signs. Twenty six percent of thepatients had died, mainly from multiple organ failure and disseminated intravascular coagulation.Conclusion: HLH is a diverse condition with many causes and is likely to be under-recognized, which contributes to its high morbidityand mortality. Clinicians need to be able to recognize the signs and symptoms commonly seen in HLH and actively pursue this diagnosisin the cases of undiagnosed febrile illness with multiorgan dysfunction. Early recognition is crucial for any reasonable attempt at curativetherapy to be made.

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1. Henter JI, Horne A, Aricó M, Egeler RM, Filipovich AH et al. HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatric Blood & Cancer 2007; 48 (2): 124-131. doi: 10.1002/pbc.21039
2. Murohashi I, Yoshida K, Ihara N, Wakao D, Yagasaki F et al. Serum levels of Thl/Th2 cytokines, angiogenic growth factors, and other prognostic factors in young adult patients with hemophagocytic syndrome. Laboratory Hematology 2006; 12 (2): 71-74. doi: 10.1532/LH96.05035
3. Mazodier K, Marin V, Novick D, Farnarier C, Robitail S et al. Severe imbalance of IL-18/IL-18BP in patients with secondary hemophagocytic syndrome. Blood 2005; 106 (10): 3483-3489. doi: 10.1182/blood-2005-05-1980
4. Ramos-Casals M, Brito-Zerón P, López-Guillermo A, Khamashta MA, Bosch X. Adult haemophagocytic syndrome. Lancet 2014; 383 (9927): 1503-1516. doi: 10.1016/ S0140-6736(13)61048-X
5. Melissa R George. Hemophagocytic lymphohistiocytosis: review of etiologies and management. Journal of Blood Medicine 2014; 5: 69-86. doi: 10.2147/JBM.S46255
6. Papo T. Syndromes hémophagocytaires. Syndrome d’activation des macrophages. AKOS (Traité de Médecine) 2011; 6: 1-8 (in French). doi: 10.1016/S1634-6939(11)56293-3
7. Rivière S, Galicier L, Coppo P, Marzac C, Aumont C et al. Reactive hemophagocytic syndrome in adults: A multicenter retrospective analysis of 162 patients. American journal of medicine 2014; 127 (11): 1118-25. doi: 10.1016/j. amjmed.2014.04.034
8. Créput C, Galicier L, Oksenhendler E, Azoulay E. Pathophysiology of organ dysfunction in the macrophage activation syndrome. Reanimation 2005; 14: 604-613 (in French with an abstract in English). doi: 10.1016/j.reaurg.2005.10.007
9. Larroche C. Syndrome d’activation macrophagique de l’adulte: état des connaissances en 2003. Sang Thrombose Vaisseaux 2003; 15 (3): 135-142 (in French).
10. Li J, Wang Q, Zheng W, Ma J, Zhang W, Wang W et al. Hemophagocytic Lymphohistiocytosis: Clinical Analysis of 103 Adult Patients. Medicine (Baltimore) 2014; 93(2): 100-105. doi: 10.1097/MD.0000000000000022
11. Costello R, Mercier C, Mazodier K, Kaplanski G. Lymphohistiocytose hémophagocytaire. Hématologie 2014; 9 (2): 1-11 (in French). doi: 10.1016/S1155-1984(13)62411-5
12. Karras A, Hermine O. Hemophagocytic syndrome. La Revue de Médecine Interne 2002; 23 (9): 768-778 (in French with an abstract in English). doi: 10.1016/S0248-8663(02)00673-2
13. Pradalier A, Teillet F, Molitor JL, Drappier JC. Macrophage activation syndrome, hemophagocytic syndrome. Pathologie Biologie 2004; 52 (7): 407-414 (in French with an abstract in English). doi: 10.1016/j.patbio.2003.12.001
14. Morimoto A, NakazawaY, Ishii E. Hemophagocytic lymphohistiocytosis: Pathogenesis, diagnosis, and management. Pediatrics International 2016; 58 (9): 817-825. doi: 10.1111/ped.13064.