Korean J Pediatr Hematol Oncol.  1998 Apr;5(1):194-201.

A Case of Angioimmunoblastic Lymphadenopathy with Dysproteinemia (AILD) Like Peripheral T Cell Lymphoma

• Hahn H
• Choi HS
• Yoo ES
• Sung KW
• Shin HY
• Kim CW
• Ahn HS

Abstract

Angioimmunoblastic lymphadenopathy with dysproteinemia(AILD), first described at early seventies, is a clinico-pathologic syndrome which has common features of lymphadenopathy, hepatosplenomegaly, fever, rash and dysproteinemia. It is a lymphoproliferative disease, differs from malignant lymphoma by the high frequency of constitutional symptoms and by the presence of generalized lymph node enlargement, hepatosplenomegaly, cutaneous menifestations and polyclonal hypergammaglobulinemia at the time of clinical onset. Peripheral T cell lymphomas(PTCL) are morphologically and immunologically heterogeneous group of lymphoproliferative disorders that are composed of postthymic lymphocytes. There are group of cases showing features similar to AILD, and these cases are classified as AILD like PTCL. We report a rare case of AILD like PTCL in an 8 year old boy, who visited Seoul National University Hospital with fever, hepatosplenomegaly and cervical lymphadenopathy, and diagnosed as AILD like PTCL by lymph node biopsy. He did not respond to conventional chemotherapy and died 1 year after clinical onset during chemotherapy, showing feature of multiple brain infarction of unknown etiology.

Keyword

Angioimmunoblastic lymphadenopathy with dysproteinemia; Angioimmunoblastic lymphadenopathy with dysproteinemia like peripheral T cell lymphoma; Polyclonal gammopathy; Monoclonal gammopathy

MeSH Terms

Biopsy
Brain Infarction
Child
Drug Therapy
Exanthema
Fever
Humans
Hypergammaglobulinemia
Immunoblastic Lymphadenopathy*
Lymph Nodes
Lymphatic Diseases
Lymphocytes
Lymphoma
Lymphoma, T-Cell, Peripheral*
Lymphoproliferative Disorders
Male
Paraproteinemias
Seoul