Abstract
IntroductionSystemic Epstein-Barr-virus-positive T cell lymphoproliferative disease of childhood is an extremely rare disorder, characterized by clonal proliferation of Epstein-Barr-virus-infected T cells with an activated cytotoxic phenotype. The disease is more frequent in Asia and South America, with only few cases reported in Western countries. A prompt diagnosis, though often difficult, is a necessity due to the very aggressive clinical course of the disease.Case presentationWe report the clinicopathological features of fulminant T cell lymphoproliferative disease that arose in the setting of acute primary Epstein-Barr virus infection. Our patient, a 23-year-old man, presented to our facility with persisting fever, hepatosplenomegaly and severe pancytopenia. On bone marrow biopsy, an abundant lymphoid infiltrate was observed. Immunophenotypic and molecular studies revealed that the atypical lymphoid cells displayed a CD8+, Epstein-Barr-encoded-RNA-positive T cell phenotype with clonal rearrangement of the T cell receptor genes, the final diagnosis being systemic Epstein-Barr-virus-positive T cell lymphoproliferative disease. On reviewing the literature we found only 14 similar cases, all presenting with very aggressive clinical courses and requiring extensive phenotyping and molecular techniques for final diagnosis.ConclusionThough extremely rare, this disease can occur in Europe, and a comprehensive diagnostic approach is thus recommended in all case of Epstein-Barr-virus-positive lymphoproliferative disorders. Unfortunately, at present no specific treatment is available; however, prompt administration of anti- Epstein-Barr virus treatment and rapid attempts to control the hemophagocytic syndrome are indicated.
Highlights
Systemic Epstein-Barr-virus-positive T cell lymphoproliferative disease of childhood is an extremely rare disorder, characterized by clonal proliferation of Epstein-Barr-virus-infected T cells with an activated cytotoxic phenotype
Primary infection of Epstein-Barr virus (EBV) is commonly asymptomatic, but some children, adolescents and young adults develop infectious mononucleosis [1] (IM), a benign febrile disease characterized by hepatosplenomegaly, lymphadenopathy, and increase of activated CD8 + T lymphocytes in peripheral blood [1,2]
EBV is implicated in the pathogenesis of different types of lymphoproliferative diseases (LPD), which are related to diverse immune alterations or peculiar clinical backgrounds [4]
Summary
Our case report underlines the importance of a comprehensive diagnostic approach in the management of atypical EBV+ LPDs. though, at present, specific therapies are not available, the correct. Unspecified Fever, generalized erythematous skin eruption, hepatosplenomegaly, pancytopenia, hypoplastic bone marrow, pulmonary infiltrates. Unspecified Fever, generalized lymphadenopathy, hepatosplenomegaly, pancytopenia, diarrhea, gastric pain. Unspecified Gluten enteropathy for 19 years; fever, persistent diarrhea, nodular erythematous skin lesion. Night sweats, weight loss, hepatosplenomegaly, pancytopenia, jaundice, generalized lymphadenopathy. At 14 years old developed hepatosplenomegaly and hemophagocytic syndrome. Fulminant EBV+ T cell TCR-b g EBV+, LPD (phenotype: not rearranged clonal reported). CAEBV = chronic active EBV infevtion; FIM = fatal infectious mononucleosis; HLA = human leukocyte antigen; IM = infectious mononucleosis; NOS = not otherwise specified; TCR = T cell receptor; VAHS = virus-associated hemophagocytic syndrome. A copy of the written consent is available for review by the Editor-in-Chief of this journal
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