A Case of leukemic Reticuloendotheliosis: A case report and literature review.
- Author:
Se Ho CHANG
1
;
Sun Ja LEE
;
Jong Hoon PARK
;
Dong Hyuk KUM
;
Jung Dal LEE
Author Information
1. Department of pediatrics, Korea General Hospital, Seoul, Korea.
- Publication Type:Original Article
- MeSH:
Child;
Cytoplasm;
Drug Therapy;
Hematopoietic System;
Humans;
Hypersplenism;
Leukemia, Hairy Cell*;
Lymphatic Diseases;
Male;
Splenectomy;
Splenomegaly
- From:Journal of the Korean Pediatric Society
1979;22(11):996-1002
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Leukemic reticuloendotheliosis (L.R.E.) was first described as a clinical and pathological entity by Ewald in 1923. LRE is a rare neoplastic disease of the hematopoietic system that is characterized cliniclly by chronic course with an insidious onset. marked splenomegaly with absence of substantial lymphadenopathy and predominant in male and characterized histologically by the presence of circulating abnormal mononuclear cells with many cytoplasmic projections, which have been refered to ??airy cells frequently. Splenectomy appeared to be the most beneficial treatment at present for those patients with massive splenomegaly and hypersplenism and chemotherapy is of little benefit in the treatment in LRE. Recently, the authors had the opportunity to observe a two year and eight old male child at this hospital whose clinical course and pathologid features were consistent with LRE. Splenectomy is performed and discharged with relatively good conditions.