J Med Cases

Journal of Medical Cases, ISSN 1923-4155 print, 1923-4163 online, Open Access

Article copyright, the authors; Journal compilation copyright, J Med Cases and Elmer Press Inc

Journal website https://www.journalmc.org

Case Report

Volume 15, Number 11, November 2024, pages 347-353

A Rare Case of Systemic Epstein-Barr Virus-Positive Diffuse Large B-Cell Lymphoma With Hemophagocytic Lymphohistiocytosis in an Immunocompetent Young Man: Potential Diagnostic Pitfall and Therapeutic Challenge

Figures

Figure 1. Nasopharynx biopsy showing diffuse infiltration of atypical lymphoid cells (a: H&E, × 100 magnification). IHC stains showing CD20 positivity (b: × 100 magnification) and a high Ki-67 index (c: × 100 magnification). ISH for EBERs was positive (d: × 100 magnification). H&E: hematoxylin and eosin; IHC: immunohistochemistry; ISH: in situ hybridization; EBER: Epstein-Barr virus-encoded small RNA.

Figure 2. Lymph nodes biopsy showing diffuse proliferation of lymphoid cells accompanied by necrosis (a: H&E, × 100 magnification). IHC stains showing CD20 positivity (b: × 100 magnification) and a high Ki-67 index (c: × 100 magnification). ISH for EBERs was positive (d: × 100 magnification). H&E: hematoxylin and eosin; IHC: immunohistochemistry; ISH: in situ hybridization; EBER: Epstein-Barr virus-encoded small RNA.

Figure 3. Endoscopic characteristic of case. Scattered mucosal swellings of varying sizes were found (a). The largest lesion was about 2.5 × 3.0 cm (b).

Figure 4. Intestinal mucosa biopsy showing diffuse infiltration of large atypical lymphocytes (a: H&E, × 100 magnification). IHC stains showing CD20 positivity (b: × 100 magnification) and high Ki-67 index (c: × 100 magnification). ISH for EBERs was positive (d: × 100 magnification). H&E: hematoxylin and eosin; IHC: immunohistochemistry; ISH: in situ hybridization; EBER: Epstein-Barr virus-encoded small RNA.

Table

**Table 1.** Differential Diagnosis Among IM, EBV⁺ LPD with IDD, and EBV⁺ DLBCL
	IM	EBV⁺ LPD with IDD	EBV⁺ DLBCL
IM: infectious mononucleosis; EBV⁺ LPD: EBV-positive lymphoproliferative disorder; IDD: immune deficiency/dysregulation; EBV⁺ DLBCL: EBV-positive diffuse large B-cell lymphoma; GI: gastrointestinal; CNS: central nervous system; LyG: lymphomatoid granulomatosis; CHL: classic Hodgkin lymphoma.
Age	Children, young adults	Any	Any
IDD clinical setting	-	+	+
Clinical presentation	Mostly self-limited	Ranges from isolated to generalized symptoms	> 50% advance stage
Nodal lesion	+	+	+
Extranodal lesion	+, esp. upper respiratory	+, extranodal destructive mass, involve lung, GI tract, CNS, skin, some mimicking LyG	+, extranodal destructive mass
Cell proliferation	Reactive, mixed cell spectrum, foci of immunoblasts	Polymorphic infiltrate with full range of muturation	Monomorphology, polymorphology, some mimicking CHL or EBV⁺ LPD
Necrosis	Seldom	Seldom	Often
Phenotype	B-cell derivation	B-, T-, and natural killer-cell derivation	B-cell derivation
Ki-67 index	Variable, usually low	Variable	Usually high
EBV latency	3	2 or 3	2, few 3
Clonality	Seldom monoclonality	< 50% monoclonality	Usually monoclonality