Hemophagocytic lymphohistiocytosis is a rare disorder characterized by a proliferation of phagocytic histiocytes in hematopoietic organs. It is accompanied by systemic manifestations and frequently has an abrupt onset with a fulminant clinical course and high mortality. Awareness of this condition is important since early diagnosis and initiation of treatment is critical for a successful outcome. The authors report a patient with hemophagocytic lymphohistiocytosis associated with angioimmunoblastic lymphoma, describe the clinical and histological features of hemophagocytic lymphohistiocytosis, and review the literature on this condition.
HenterJIHorneAAricóM. HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007;48:124-131.
3.
ChimCSHuiPK. Reactive hemophagocytic syndrome and Hodgkin’s disease. Am J Hematol. 1997;55:49-50.
4.
OhnoHTakimotoK. Gastric mucosa-associated lymphoid tissue lymphoma complicated with hemophagocytic syndrome in an elderly woman. Ann Hematol. 2010;89:1175-1176.
5.
CzuchlewskiDROupadiaSLZhangQY. Diffuse large B-cell lymphoma with florid hemophagocytosis. Int J Hematol. 2009;89:1-2.
6.
KrenovaZSterbaJBlatnyJKrenLSlanyJ. A case of anaplastic large cell lymphoma-induced hemophagocytic lymphohistiocytosis in an adolescent female. Pediatr Blood Cancer. 2007;49:1056.
7.
BlomABeylot-BarryMD’IncanMLarocheL. Lymphoma-associated hemophagocytic syndrome (LAHS) in advanced-stage mycosis fungoides/Sézary syndrome cutaneous T-cell lymphoma. J Am Acad Dermatol. 2011;65:404-410.
