Epstein-Barr virus-associated T-lymphoproliferative disease with hemophagocytic syndrome, followed by fatal intestinal B lymphoma in a young adult female with WHIM syndrome. Warts, hypogammaglobulinemia, infections, and myelokathexis.
A rare association of Epstein-Barr virus-associated T- and B-lymphoproliferative disease (EBV(+) T- and EBV(+) B-LPD) in a patient with WHIM (warts, hypogammaglobulinemia, infections, and myelokathexis) syndrome is reported. A 26-year-old Japanese female, who had been treated for WHIM syndrome since early childhood, developed hemophagocytic syndrome associated with EBV(+) T-LPD at the lymph nodes and spleen. The disease rapidly resolved in response to prednisolone therapy. However, 6 weeks later, fatal EBV(+) B lymphoma unresponsive to chemotherapy occurred in the intestine and other organs. Caution must be exercised that the patient with WHIM syndrome may be at risk for EBV-LPD.
Imashuku
S
S
Kyoto City Institute of Health and Environmental Sciences, 1-2 Higashi-Takada-cho, Mibu, Nakagyo-ku, Kyoto 604-8845, Japan. shinim95@mbox.kyoto-inet.or.jp
Miyagawa
A
A
Chiyonobu
T
T
Ishida
H
H
Yoshihara
T
T
Teramura
T
T
Kuriyama
K
K
Imamura
T
T
Hibi
S
S
Morimoto
A
A
Todo
S
S
D000328:Adult; D017809:Fatal Outcome; D005260:Female; D004854:Herpesvirus 4, Human; D015616:Histiocytosis, Non-Langerhans-Cell / Q000097:blood / Q000150:complications*; D006801:Humans; D007414:Intestinal Neoplasms / Q000097:blood / Q000473:pathology*; D016393:Lymphoma, B-Cell / Q000097:blood / Q000473:pathology*; D008232:Lymphoproliferative Disorders / Q000097:blood / Q000150:complications* / Q000821:virology; D009504:Neutrophils / Q000473:pathology*; D013601:T-Lymphocytes / Q000276:immunology / Q000473:pathology