Langerhans Cell Histiocytosis by savannah hettman

Causes:

  • Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by the presence of cells with characteristics similar to bone marrow.
  • The working group of the Histiocyte Society has divided histiocytic disorders into 3 groups: (1) dendritic cell histiocytosis, (2) macrophage-related disorders, and (3) malignant histiocytosis.
  • LCH belongs in group 1 and encompasses a number of diseases. On one end, the clinical spectrum includes an acute, fulminant, disseminated disease called Letterer-Siwe disease.

Symptoms:

  • In adults symptoms include:
  • Rash
  • Fever
  • Cough
  • Chest pain
  • Weight loss
  • Pain in bones
  • Shortness of breath
  • Increased urination
  • Thirst and an increased consumption of fluids
  • General uneasiness, discomfort, and ill feeling or malaise
  • In infants symptoms include:
  • Fever
  • Thirst
  • Limping
  • Seizures
  • Jaundice
  • Dizziness
  • Vomiting
  • Irritability
  • Headache
  • Bone pain
  • Weight loss
  • Loss of teeth
  • Night sweats
  • Short stature
  • Swollen gums
  • Failure to thrive
  • Abdominal pain
  • Vision problems
  • Delayed puberty
  • Protrusion of eyes
  • Swelling of eyelids
  • Frequent urination
  • Mental deterioration
  • Swollen lymph glands
  • Multiple ear infections
  • Long-term ear drainage
  • Seborrheic dermatitis of scalp
  • Rashes (purpura or petechiae)

Diagnosis:

  • tissue biopsy
  • CT scans
  • MRI's
  • bone marrow biopsy

Treatment:

  • LCH was considered to be a cancerous condition and it was treated by using chemotherapy and radiotherapy.
  • other treatment methods for LCH vary widely and depend on the extent of the disease.
  • The disorder may vanish without any treatment in a process known as spontaneous remission.
  • Management of LCH is also not necessary if the disease affects a non-harmful area of the body such as a bone that bears no weight.
  • Medical intervention is essential only if symptoms like fever and pain affect the patient or if organs like lungs, liver or bone marrow are negatively affected.
  • If LCH is restricted to a single organ, then local treatment is administered to that organ.
  • LCH can be treated by using corticosteroids that help in suppressing the immune function. Drugs that may be administered to children include:
  • Cyclophosphamide
  • Methotrexate
  • Etoposide
  • Vinblastine
  • Vincristine
  • VP16
  • Steroids
  • Other forms of treatment for LCH include the following:
  • Physical therapy
  • Radiation therapy
  • Hormone replacement therapy
  • Antibiotics to combat infections
  • Surgical removal of LCH cell growth
  • Breathing support using a breathing machine

Prognosis:

  • Most children having LCH survive the disease.
  • Each child responds differently to the treatments for LCH.
  • If the patient is an infant, the chances of LCH being severe in form are much greater.
  • Diseases affecting the liver, lungs, spleen and bone marrow due to LCH are difficult to treat.
  • Although not fatal, LCH may leave a child with chronic problems like breathing problems, deafness, chronic ear discharge, protuberant eyes, unsightly scalp lumps or diabetes insipidus.
  • Single system disease often cures spontaneously.
  • Multi-system LCH may remit spontaneously but usually requires treatment.
  • In some cases, LCH might eventually lead to death.

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