Secondary hemophagocytic lymphohistiocytosis: a case report

Secondary hemophagocytic lymphohistiocytosis: a case report


  • Lia Salvati University of L'Aquila
  • Anita Di Renzo University of L'Aquila
  • Sergio Moscardelli University of L'Aquila
  • Laura Natali University of L'Aquila
  • Ferri Claudio University of L'Aquila
  • Angelo Viscido University of L'Aquila


hemophagocytic syndrome; lymphoma; fever; hyperferritinemia


Hemophagocytic lymphohistiocytosis (HLH) is a rare hematologic syndrome presenting either as an inherited life-threatening inflammatory disorder in children or as a secondary disease in adults. Inherited HLH involves inborn defects in lymphocytes and includes autosomal recessive and X-linked disorders characterized by uncontrolled activation of T cells and macrophages and overproduction of inflammatory cytokines. Secondary or acquired HLH occurs in the settings of infections, systemic connective tissue disease and lymphoid malignancies, possibly due to underlying genetic predisposition to develop HLH. The mechanisms leading to secondary HLH have yet to be fully determined and the disease remains frequently undiagnosed and thereby untreated. Herewith we report the case of an 83-year old Caucasian male who referred to our Division of Internal Medicine and Nephrology due to marked asthenia associated with fever, mental confusion, drowsiness and hyporexia, who was ultimately diagnosed with HLH secondary to anaplastic B cell lymphoma. This case report illustrates the difficulties in the diagnostic workup of HLH, mainly related to early identification of the underlying disease and rapid instauration of appropriate therapy.


Saeed HM, Rakhshani Z F, Heidari E. “Hemophagocytic Lymphohistiocytosis in Children”. Arch Pediatr Infect Dis. 2019; 7(3): e84041.

La Rosée P, Horne A, Hines M, et al. “Recommendations for the management of hemophagocytic lymphohistiocytosis in adults”. Blood 2019; 133:2465-2477.

Tamura K, Kanazawa T, Tsukada S, et al. “Increased serum monocyte chemoattractant protein-1, macrophage inflammatory protein-1β, and interleukin-8 concentrations in hemophagocytic lymphohistiocytosis”. Pediatr Blood Cancer. 2008;51(5):662–8.

Voskoboinik I, Smyth MJ, Trapani JA. “Perforin-mediated target-cell death and immune homeostasis”. Nat Rev Immunol. 2006; 6(12):940–52.

Filipovich AH, Chandrakasan S. “Pathogenesis of Hemophagocytic Lymphohistiocytosis”. Hematol Oncol Clin North Am. 2015;29(5):895-902.

Usmani GN, Woda BA, Newburger PE. “Advances in understanding the pathogenesis of HLH”. Br J Haematol. 2013;161(5):609-22.

Miyahara M, Sano M, Shibata K, et al. (2000); “B-cell lymphoma-associated hemophagcytic syndrome: clinicopathological characteristics”. Ann Hematol 79: 378-388.

Del Pinto R, Ferri C. “Inflammation-Accelerated Senescence and the Cardiovascular System: Mechanisms and Perspectives”. Int J Mol Sci. 2018;19(12).






Case Reports: General Surgery and Miscellanea

How to Cite

Salvati L, Di Renzo A, Moscardelli S, Natali L, Claudio F, Viscido A. Secondary hemophagocytic lymphohistiocytosis: a case report. Acta Biomed [Internet]. 2021 Apr. 30 [cited 2024 Jul. 20];92(S1):e2021060. Available from: