Chronic active Epstein-Barr virus infection associated with hemophagocytic syndrome and extra-nodal natural killer/T-cell lymphoma in an 18-year-old girl: A case report

Abstract

Rationale: Chronic active Epstein-Barr virus infection (CAEBV) associated with hemophagocytic syndrome (HPS) and extra-nodal natural killer (NK)/T-cell lymphoma (ENKL) is a rare life-threatening disorder. This disease is easily misdiagnosed because of its varied presentations.

Patient concerns: An 18-year-old girl was admitted to our hospital with a history of edema in the lower limbs and intermittent fever lasting for more than 1 month. At admission, she had severe liver injury of unknown etiology. Laboratory test results revealed pancytopenia, hyperferritinemia, hypertriglyceridemia, and hypofibrinogenemia. Results of serologic tests for EBV were positive. Results of a skin biopsy indicated EBV-positive NK/T-cell lymphoma, and bone marrow aspiration revealed focal hemophagocytosis and atypical lymphoid cells.

Diagnosis: On the basis of these findings, we diagnosed the case as extra-nodal NK/T-cell lymphoma-associated HPS (natural killer/T-cell lymphoma-associated hemophagocytic syndrome), which is commonly induced by CAEBV.

Interventions: Treatment consisted of general management of hepatitis, supplemented with albumin and empirical antibiotic therapy.

Outcomes: The patient died from massive gastrointestinal hemorrhage a week after she was discharged from the hospital.

Lessons: ENKL and HPS present with varied features and are generally fatal; therefore, clinicians should proceed with caution in suspected cases. HPS should be considered when the patient presents with fever, hepatosplenomegaly, pancytopenia, and liver failure. When HPS is suspected, clinicians should determine the underlying cause, such as severe infection, including infection with viruses such as EBV; genetic predisposition; or underlying malignancies, especially lymphoma because of its strong association with HPS.

Figure 1

(A) Computed tomography vein imaging showed compressed constrictive inferior vena cava (arrow). (B) Decreased liver density and hepatosplenomegaly, strengthen intrahepatic nodules (arrows). (C) Lymphadenectasis around the mesenteric root (arrows).

Figure 2

(A) Upper abdominal wall varicose veins (arrow). (B) The skin lesions of the patient (arrows).

Figure 3

(A) Hematoxylin and eosin staining result of the skin biopsy with lymphocytic infiltration (H&E × 200). (B–J) Immunohistochemical stainings of cluster of differentiation 2 (CD2), CD3, CD4, CD7, CD8, CD56, Ki-67, T-cell restricted intracellular antigen 1, and Epstein–Barr virus-encoded early small RNA in cutaneous nodules were positive (×200).

Figure 4

(A) Diffuse steatosis, sinusoidal dilatation with lymphocytic infiltration (H&E × 200). (B) Liver steatosis (H&E × 400).

Figure 5

(A) Bone marrow aspiration showed evidence of hemophagocytosis. Arrow showing a macrophage with phagocytosed red blood cells and platelets (arrow) (×400). (B) Atypical lymphocytes (arrow) in bone marrow (×400).