مرض كثرة النوسجات (لانغرهانس)‎ (LCH)‎ في مستشفى الأطفال-جامعة دمشق

الملخص EN

Background &Objective: Langerhans cell histiocytosis (LCH) is a heterogeneous disease, characterized by accumulation of dendritic cells with features similar to epidermal Langerhans cells in various organs.

The clinical course may vary from a self-limiting disease to a rapidly progressive one that might lead to death.

The Histiocyte Society has made a major contribution to the management of LCH.

The society has categorized LCH into two major groups with significantly different prognoses, to help with treatment and diagnosis.

The diagnosis of LCH is based on histological and immunophenotypic examination of lesional tissue .Treatment options vary depending on the extent of the disease and the severity at onset.

Response to front-line treatment is an important information to adapt the therapeutic strategy.1,2 the objective of this descriptive analysis study of patients with Langerhans cell histiocytosis (LCH) diagnosed at Damascus University Children Hospital during the period 1/1/2000- 31/12/2012 ‘ natural history, management, and outcome, specially those who were treated with LCH II protocol.

Materials & Methods: 79 patients with histologically proven (LCH) less than 14 years age, were categorized into multisystem disease and those with single system .Clinical features ,treatment and outcome were determined from the case history notes.

Treatment were include chemotherapy (62 patients), surgery (in two cases), the end point were disease free survival, active disease or death.

The median time follow up was 58 months (1mo-120 months).

Results: 47 males 32 females patients, ages ranged from 2 months -12 years, the median age was 19 months.

most patients (63%)were less than 2 years old range from (0-24)months, The most common complains were fever (56%) ,skin rashes 37%.

pale is was the most common clinical findings (64%),skin rashes (44%) ,hepatosplenomegaly (39%.).

2 patients with single bone lesions (Eosinophilic Granuloma) were surgery treated (curettage), 1 of them was completely recover (CR), the other relapsed and chemotherapy treated with no response (NR).

62 patients multisystem disease were chemotherapy treated with LCH II protocol, (15) of them treated for 6 months ,(36 ) for12 months, 11 were lost .Complete Remission (CR) occurred in 24 patients, Partial remission (PR) in 10, No Response (NR) in17, Relapse (Re) in10 cases of thos.

Death were happened in 12 cases with a various causes of death.

Conclusion: Langerhans cell histiocytosis is a rare disease with male predominance, occurrence, decreased incidence during age with a very different types of clinical phenotypes.

Chemotherapy Complete Recover may occur with a high percentage of death especially of the multisystem type, high risk types.