Histiocytosis X (Langerhans Cell Histiocytosis)
It consist of group of clinical pathologic entities
- Eosinophilic granuloma of bone
- Hand Schuller Chritian disease
- Letterer Siwe disease.
These entities are the result of proliferation and dissemination of pathologic histiocyte cells or langerhans cells.
Eosinophilic granuloma (Solitary and multiple without extra skeletal involvement) :
- Diagnosed in 5-10 years of age.
- Presents with localizing pain acompained by swelling and low grate fever.
- Skull is the most common site followed by femur.
- Others site pelvis, ribs and spine
- Mixture of eosinophils, plasma cells, histiocytes & mononuclear giant cells (langerhans cells).
- Cytoplasm of langerhans cells contain TENNIS RACKET Shaped BIRBECK GRANULES.
- Lytic lesion appear as punched out or moth eaten.
- Vertebra plana
- Spontenous remission and favorable outcome over period of months to years.
- Curettage and bone grafting in weight bearing bones.
- Radiotherapy for lesion which can threaten neurological function.
Hand Schuller Chritian disease:
- It includes multiple eosinophylic granulomas involving bone diabetes insipidus (due to pitutary gland involvemnt) & exophthalmos (due to presence of retro or bital granuloma).
- Patient also have fever, hepato splenomegaly, lymph adenopathy & anemia.
- Treatment: Low dose irradiation and steroid.
Letterer Siwe disease:
Acute, disseminated progressive form of histiocytosis. Presents before 2 year of age. Visceral involvement is diffuse and severe patient may present with fever and debilitating infection due to marrow failure.
Hepatosplenomegaly, lymphadenopathy, pappular rash, bleeding diasthesis & anemia.
Treatment: Chemotherapy, Steroid and antibiotics.