J Korean Pediatr Soc.  1979 Nov;22(11):996-1002.

A Case of leukemic Reticuloendotheliosis: A case report and literature review

Affiliations
  • 1Department of pediatrics, Korea General Hospital, Seoul, Korea.
  • 2Department of Pathology, Korea General Hospital, Seoul, Korea.

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.


MeSH Terms

Child
Cytoplasm
Drug Therapy
Hematopoietic System
Humans
Hypersplenism
Leukemia, Hairy Cell*
Lymphatic Diseases
Male
Splenectomy
Splenomegaly
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