Langerhans cell histiocytosis in children less than 2 years of age.

Abstract:

OBJECTIVE:To study the clinical profile and outcome of langerhans cell histiocytosis in children upto 2 years of life. DESIGN:Retrospective analysis. METHODS:Medical records of Children upto 2 years of age with a diagnosis of langerhans cell histiocytosis (LCH) were analyzed. Their clinical pattern, treatment modalities and outcome were studied. The patients Were categorized into 2 groups according to their clinical presentation: (i) Subject without organ dysfunction; and (ii) cases with organ dysfunction. Treatment considered of surgical intervention, radiotherapy, chemotherapy or combination of all these modalities depending upto the extent of disease. RESULTS:There were 20 children upto 2 years of age with histiocytosis during the 12 year period (January 1983 - December 1994). The median age at diagnosis was 18 months (range 52 days - 24 months). Of the twenty patients,13 patients didn't have organ dysfunction and 7 had organ dysfunction. Out of the 13 children without organ dysfunction eleven patients received treatment and all of them are alive free of disease with a median follow up of 62 months. But all children with organ dysfunction succumbed to disease within a few weeks. CONCLUSION:Children under 2 years of age with localised and or multifocal LCH without organ dysfunction have a good prognosis and they should not be exposed to aggressive form of treatment. All children with organ dysfunction require multi-agent chemotherapy.

journal_name

Indian Pediatr

journal_title

Indian pediatrics

authors

Kusuma Kumary P,Priyakumari T,Chellam VG,James FV,Nair MK

keywords:

subject

Has Abstract

pub_date

1999-01-01 00:00:00

pages

29-36

issue

1

eissn

0019-6061

issn

0974-7559

journal_volume

36

pub_type

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