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Histiocytosis X (Langerhans Cell Histiocytosis)

It consist of group of clinical pathologic entities

  1. Eosinophilic granuloma of bone
  2. Hand Schuller Chritian disease
  3. 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) :


Clinical features:

  1. Diagnosed in 5-10 years of age.
  2. Presents with localizing pain acompained by swelling and low grate fever.
  3. Skull is the most common site followed by femur.
  4. Others site pelvis, ribs and spine


  1. Mixture of eosinophils, plasma cells, histiocytes & mononuclear giant cells (langerhans cells).
  2. Cytoplasm of langerhans cells contain TENNIS RACKET Shaped BIRBECK GRANULES.


  1. Lytic lesion appear as punched out or moth eaten.
  2. Vertebra plana


  1. Spontenous remission and favorable outcome over period of months to years.
  2. Curettage and bone grafting in weight bearing bones.
  3. 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.



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