Systemic Epstein-Barr-virus-positive T cell lymphoproliferative childhood disease in a 22-year-old Caucasian man

A case report and review of the literature

Valentina Tabanelli, Claudio Agostinelli, Elena Sabattini, Anna Gazzola, Francesco Bacci, Saveria Capria, Claudia Mannu, Simona Righi, Maria Teresa Sista, Giovanna Meloni, Stefano A. Pileri, Pier Paolo Piccaluga

Research output: Contribution to journalArticle

12 Citations (Scopus)

Abstract

Introduction. 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. 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 presentation. We 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. Conclusion: Though 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.

Original languageEnglish
Article number218
JournalJournal of Medical Case Reports
Volume5
DOIs
Publication statusPublished - 2011

Fingerprint

Human Herpesvirus 4
T-Lymphocytes
Phenotype
Hemophagocytic Lymphohistiocytosis
T-Cell Receptor Genes
Pancytopenia
Epstein-Barr Virus Infections
Lymphoproliferative Disorders
South America
Rare Diseases
Fever
Bone Marrow
Lymphocytes
RNA
Biopsy
Therapeutics

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Systemic Epstein-Barr-virus-positive T cell lymphoproliferative childhood disease in a 22-year-old Caucasian man : A case report and review of the literature. / Tabanelli, Valentina; Agostinelli, Claudio; Sabattini, Elena; Gazzola, Anna; Bacci, Francesco; Capria, Saveria; Mannu, Claudia; Righi, Simona; Sista, Maria Teresa; Meloni, Giovanna; Pileri, Stefano A.; Piccaluga, Pier Paolo.

In: Journal of Medical Case Reports, Vol. 5, 218, 2011.

Research output: Contribution to journalArticle

Tabanelli, V, Agostinelli, C, Sabattini, E, Gazzola, A, Bacci, F, Capria, S, Mannu, C, Righi, S, Sista, MT, Meloni, G, Pileri, SA & Piccaluga, PP 2011, 'Systemic Epstein-Barr-virus-positive T cell lymphoproliferative childhood disease in a 22-year-old Caucasian man: A case report and review of the literature', Journal of Medical Case Reports, vol. 5, 218. https://doi.org/10.1186/1752-1947-5-218
Tabanelli, Valentina ; Agostinelli, Claudio ; Sabattini, Elena ; Gazzola, Anna ; Bacci, Francesco ; Capria, Saveria ; Mannu, Claudia ; Righi, Simona ; Sista, Maria Teresa ; Meloni, Giovanna ; Pileri, Stefano A. ; Piccaluga, Pier Paolo. / Systemic Epstein-Barr-virus-positive T cell lymphoproliferative childhood disease in a 22-year-old Caucasian man : A case report and review of the literature. In: Journal of Medical Case Reports. 2011 ; Vol. 5.
@article{785c39d9750f4118b8d52778913b6a07,
title = "Systemic Epstein-Barr-virus-positive T cell lymphoproliferative childhood disease in a 22-year-old Caucasian man: A case report and review of the literature",
abstract = "Introduction. 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. 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 presentation. We 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. Conclusion: Though 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.",
author = "Valentina Tabanelli and Claudio Agostinelli and Elena Sabattini and Anna Gazzola and Francesco Bacci and Saveria Capria and Claudia Mannu and Simona Righi and Sista, {Maria Teresa} and Giovanna Meloni and Pileri, {Stefano A.} and Piccaluga, {Pier Paolo}",
year = "2011",
doi = "10.1186/1752-1947-5-218",
language = "English",
volume = "5",
journal = "Journal of Medical Case Reports",
issn = "1752-1947",
publisher = "BioMed Central",

}

TY - JOUR

T1 - Systemic Epstein-Barr-virus-positive T cell lymphoproliferative childhood disease in a 22-year-old Caucasian man

T2 - A case report and review of the literature

AU - Tabanelli, Valentina

AU - Agostinelli, Claudio

AU - Sabattini, Elena

AU - Gazzola, Anna

AU - Bacci, Francesco

AU - Capria, Saveria

AU - Mannu, Claudia

AU - Righi, Simona

AU - Sista, Maria Teresa

AU - Meloni, Giovanna

AU - Pileri, Stefano A.

AU - Piccaluga, Pier Paolo

PY - 2011

Y1 - 2011

N2 - Introduction. 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. 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 presentation. We 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. Conclusion: Though 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.

AB - Introduction. 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. 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 presentation. We 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. Conclusion: Though 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.

UR - http://www.scopus.com/inward/record.url?scp=79957932645&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=79957932645&partnerID=8YFLogxK

U2 - 10.1186/1752-1947-5-218

DO - 10.1186/1752-1947-5-218

M3 - Article

VL - 5

JO - Journal of Medical Case Reports

JF - Journal of Medical Case Reports

SN - 1752-1947

M1 - 218

ER -