Langerhans cell histiocytosis true is all except aEUR'
Gonadal involvement occurs The working group of the histiocyte society has divided histiocytic disorders into 3 groups I. Dendritic cell histiocytosis Marcophage related disorders Malignant histiocytosis LCH belongs in group 1 and encompasse a number of diseases The clinical spectrum of LCH includes :? Eosinophilic granulomas (indolent and chronic lesion of bone and other organs. Hand-Schuller Christian disease (intermediate fonn, multifocal chronic involvement) Letterer siewe disease acute fulininant disseminated disease Clainical features Age LCH affects patients from neonatal period to childhood The age at onset varies according to the variant of LCH as follows - letterer siewe disease occurs predominantly in children younger than 2 years - Hand schuller Christian disease in children (2-10 years) - Eosinophilic granuloma (5-15 years) The clinical features vary according to the type of LCH Chronic unifocal LCH (eosirzophilic granuloma of bone) Solitary calvarial lesion in young adults Other sites involved are - Veebra - Rib - Mandible - Femur - Ilium - Scapula Lesions are usually asymptomatic but may produce following symptoms due to involvement of bone - Otitis media - Proptosis - Loose teeth - Pituitary dysfunction - Spontaneous .fracture Classic multifocal LCH (Hand schulller Christian disease) Presents with classic triad of :- Diabetes insipidus Exophthalmos Bony defect Acute disseminated (LCH) litterer siewe disease Cutaneous abnormalities - Present in 80% of patients - Frequently the first sign - Involve, scalp, .face, trunk, buttock - "Mimicks seborrhic dermatitis" Fever Anemia Thrombocytopenia Pulmonary infiltrates Spleen and liver involvement .* Enlargement of lymph nodes Laneherhans cells demonstrate following characteristics :? Abundant often vacuolated cytoplasm and vesicular nuclei Birbeck granules Characteristic of langherhans cells - "Tennis racket appearance" - Contain the protein langerin - Typically express - HLA -- DR - S -- 100 - CD/a Management of Histiocytosis The management is based on disease severity Single system disease Solitary bone lesion Curettage or excision Intralesional steroid injection Biphosphonates Indomethacin (polyostic lesions) Localized skin lesion Topical steroids Topical nitrogen mustards Acitretin Psoralen plus ultraviolet A (PUVA) Single lymph node infiltration Excision is the treatment of choice Multisystem disease Systemic chemotherapy - Combination of cytotoxic drugs and systemic steroids is generally effective. Drugs used are:- - Vinblastine - Etoposide - Prednisolone - Mercaptopurine Treatment of refractory disease Cladirabine (2 chlorodeoxyadenosine 2 - CDA) Cytosine arabinoside (Ara - c) Bone marrow transplant in patients with very poor prognosis
Peak incidence less than 3 years of age
Radiosensitive
Diffuse form is known as litter-sewe-disease
Gonadal involvement occurs