Clinico-biologic profile of Langerhans cell histiocytosis: a single institutional study.
BACKGROUND
Langerhans cell histiocytosis (LCH) is a rare atypical cellular disorder characterized by clonal proliferation of Langerhans cells leading to myriad clinical presentations and highly variable outcomes. There is a paucity of Indian studies on this subject.


OBJECTIVE
To present the experience of management of LCH at a single institution.


METHODS
This is a retrospective observational study of patients with LCH who presented at the Tata Memorial Hospital between January 1987 and December 2002.


METHODS
Fifty-two patients with LCH were treated in the study period. Due to the long observation period and variability in diagnostic and therapeutic protocols, the patients were risk-stratified based on present criteria. The disease pattern, management approaches and treatment outcomes of patients were recorded.


METHODS
Statistical analyses were done using Student's 't' test, test for proportion and survival estimates based on the Kaplan-Meier method.


RESULTS
The median age at presentation was 3 years and more than 48% of the patients had Group I disease. Skeleton, skin and lymphoreticular system were the commonly involved organs. Majority (80%) required some form of therapy. The projected overall survival is 63% at 10 years and mean survival is 118 months. Seventeen percent of surviving patients developed long-term sequelae.


CONCLUSIONS
The clinico-biologic profile of LCH patients in India is largely similar to international patterns except a higher incidence of lymphoreticular involvement. Majority of the patients respond favorably to therapy and have a good outcome, except a subset of Group I patients who warrant enrollment in clinical trials with innovative therapeutic strategies to improve outcome.
Narula
G
G
Department of Medical Oncology, Tata Memorial Hospital, Parel, Mumbai - 400 012, Maharashtra, India.
Bhagwat
R
R
Arora
B
B
Banavali
Sd
S
Pai
Sk
S
Nair
Cn
C
Seth
T
T
Laskar
S
S
Muckaden
Ma
M
Kurkure
Pa
P
Parikh
Pm
P
D000293:Adolescent; D000328:Adult; D000970:Antineoplastic Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D011878:Radiotherapy; D012189:Retrospective Studies; D012307:Risk Factors; D016896:Treatment Outcome
D000970:Antineoplastic Agents