EBV+ B-cell Lymphoproliferative Disorder Associated with Subsequent Development of Burkitt Lymphoma in a Patient with Idiopathic CD4+ T-lymphocytopenia

Abstract

We report here a case of idiopathic CD4 + T-lymphocytopenia (ICL) associated with Epstein-Barr virus (EBV) + lymphoproliferative disorder (LPD) terminating in Burkitt lymphoma (BL). A 33-year-old Japanese male was admitted to the hospital showing severe CD4 + lymphocytopenia and neutropenia that was diagnosed as ICL in 1993. Twenty months after the onset of disease, right cervical lymphadenopathy was detected. Biopsy of the specimen showed reactive lymph node hyperplasia and interfollicular B-cell hyperplasia. Ninety-one months later, polypoid tumors were resected from the bilateral nasal cavities and were diagnosed as BL. Immunohistological studies suggested the reactive nature of the initial lymph node biopsy specimen.

Idiopathic CD4 Lymphocytopenia: Spectrum of opportunistic infections, malignancies, and autoimmune diseases

“…Only English language papers were reviewed. We found 258 cases diagnosed with ICL in 143 published papers[134567891011121314151617181920212223242526272829303132333435363738394041424344454647484950515253545556575859606162636465666768697071727374757677787980818283848586878889909192939495969798…”

Administration of interleukin-7 increases CD4 T cells in idiopathic CD4 lymphocytopenia

“…[36][37][38][39][40][41][42] IL-7 plays an essential role in T-cell homeostasis and T-cell survival and has been found to be well tolerated in humans with various lymphopenic conditions including HIV [8][9][10] and cancer, 11 as well as in stem cell transplant recipients. 12 These previous studies showed that administration of IL-7 expands CD4 and CD8 T cells, decreases Tregs, and can help restore CD4 T cells in the gut mucosa of lymphopenic HIV patients as a result of increased T-cell homing and possibly local expansion.…”

Humanized mouse models reveal an immunologic classification of idiopathic CD4 lymphocytopenia subtypes

“…3), autoimmunity (4-6), and HPV-or EBV-related neoplasias (7,8) that in some cases can be fatal (9,10). Twenty-five years after the CDC defined ICL, its etiology remains elusive, and there is currently no efficacious treatment, other than the use of antibiotics for prophylaxis or treatment of opportunistic infections (11).…”