HISTIOCYTOSIS X

Histiocytosis X is rare and affects children. This disease causes unusually high histiocyte counts. Histiocytes help the immune system fight diseases and foreign bodies. This illness may cause immune cells to form tumors that damage bones and spread to other parts of the body.

Moreover, Histiocytosis X is an autoimmune disease in which immune cells overproduce and attack the body rather than battling infection. Researchers used to think of it as a cancer-like illness. The reason is usually unknown but may often be inherited.

TYPES

The Five types of histiocytosis X  are:

  • L group — includes Langerhans cell histiocytosis and Erdheim-Chester disease
  • C group — includes non-Langerhans cell histiocytosis that involves the skin
  • M group — includes malignant histiocytosis
  • R group — includes Rosai-Dorfman disease
  • H Group — includes hemophagocytic lymphohistiocytosis

SYMPTOMS

Children and adults may have different symptoms.

Symptoms for children might include:

  • Abdominal pain
  • Bone pain
  • Delayed puberty
  • Dizziness
  • Ear drainage that continues long-term
  • Eyes that appear to stick out more and more
  • Irritability
  • Failure to thrive
  • Fever
  • Frequent urination
  • Headache
  • Jaundice
  • Limping
  • Mental decline
  • Rash
  • Seborrheic dermatitis of the scalp
  • Seizures
  • Short stature
  • Swollen lymph glands
  • Thirst and increased drinking of fluids
  • Vomiting
  • Weight loss

Meanwhile, adult symptoms might consist of the following:

  • Bone pain
  • Chest pain
  • Cough
  • Fever
  • General discomfort, uneasiness, or ill feeling
  • Increased amount of urine
  • Rash
  • Shortness of breath
  • Thirst and increased drinking of fluids
  • Weight loss

DIAGNOSIS

A biopsy examines a small skin or bone sample under a microscope for abnormal cells. Blood tests and standard and specialized X-rays, along with scans of the bones, head, and lungs, are further diagnostic techniques. These tests will help the doctor determine whether the sickness affects many systems.

An X-ray of the whole skeletal system may determine the severity and if systemic involvement is needed.

TREATMENT

Local surgery may treat histiocytosis X in one area, such as the skin or bone. They must be examined routinely to detect sickness transmission.

Histiocytosis X is rare, and the best treatment is unknown. Clinical trials seeking innovative treatments may want these conditions.

Treatments may includes:

  • Interferon alpha
  • Cyclophosphamide or vinblastine
  • Etoposide
  • Methotrexate
  • Vemurafenib, if the BRAF V600E mutation is found
  • Stem cell transplantation

Other treatments might be:

  • Hormone replacement therapy
  • Physical therapy
  • Scalp shampoos
  • Antibiotics to fight infections
  • Breathing support 
  • Supportive or comfort care treats symptoms.

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