Langerhans cell histiocytosis in children less than 2 years of age.
OBJECTIVE
To study the clinical profile and outcome of langerhans cell histiocytosis in children upto 2 years of life.


METHODS
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.


CONCLUSIONS
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.
Kusuma Kumary
P
P
Division of Pediatric Oncology and Radiation Oncology, Regional Cancer Center, Trivandrum, Kerala, India.
Priyakumari
T
T
Chellam
V G
VG
James
F V
FV
Nair
M K
MK
D000367:Age Factors; D000972:Antineoplastic Agents, Phytogenic / Q000627:therapeutic use; D001855:Bone Marrow Diseases / Q000503:physiopathology / Q000628:therapy; D002675:Child, Preschool; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000627:therapeutic use; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000627:therapeutic use; D006646:Histiocytosis, Langerhans-Cell / Q000188:drug therapy / Q000503:physiopathology* / Q000628:therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D008107:Liver Diseases / Q000503:physiopathology / Q000628:therapy; D008171:Lung Diseases / Q000503:physiopathology / Q000628:therapy; D008297:Male; D011239:Prednisolone / Q000627:therapeutic use; D011379:Prognosis; D012008:Recurrence; D012074:Remission Induction; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome; D014747:Vinblastine / Q000627:therapeutic use
D000972:Antineoplastic Agents, Phytogenic; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D014747:Vinblastine; D003520:Cyclophosphamide; D011239:Prednisolone