Secondary hemophagocytic lymphohistiocytosis: a case report

Secondary hemophagocytic lymphohistiocytosis: a case report

Authors

  • 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

Keywords:

hemophagocytic syndrome; lymphoma; fever; hyperferritinemia

Abstract

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.

References

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Published

30-04-2021

Issue

Section

Case Reports: General Surgery and Miscellanea

How to Cite

1.
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: https://www.mattioli1885journals.com/index.php/actabiomedica/article/view/8836