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Histiocytosis - Symptom

Alternative Names

Histiocytosis X; Langerhans cell histiocytosis; Eosinophilic granuloma; Pulmonary histiocytosis X; Nonlipid reticuloendotheliosis; Pulmonary Langerhans cell granulomatosis; Hand-Schuller-Christian disease; Letterer-Siwe disease

Symptoms:

Histiocytosis X often affects the whole body. A disease that affects the whole body is called a systemic disorder.

Symptoms can vary between children and adults, although there can be some overlap. Tumors in weight-bearing bones, such as the legs or spine, may cause the bones to fracture without apparent reason.

Symptoms in children may include:

  • Abdominal pain
  • Bone pain (possibly)
  • Delayed puberty
  • Dizziness
  • Ear drainage that continues long-term
  • Eyes that appear to stick out (protrude) more and more
  • Irritability
  • Failure to thrive
  • Fever
  • Frequent urination
  • Headache
  • Jaundice
  • Limping
  • Mental deterioration
  • Rash (petechiae or purpura)
  • Seborrheic dermatitis of the scalp
  • Seizures
  • Short stature
  • Swollen lymph glands
  • Thirst
  • Vomiting
  • Weight loss

Note: Children over 5 years old often have only bone involvement.

Symptoms in adults may include:

Signs and tests:

The tumors produce a "punched-out" look on a bone x-ray. Specific tests vary depending on the age of the patient.

Tests in children may also include:

Tests in adults may include:

Histiocytosis X is sometimes associated with cancer. CT scans and biopsy should be done to rule out possible cancer.

  • Reviewed last on: 4/8/2008
  • Mark Levin, MD, Hematologist and Oncologist, Newark, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

References

Ladisch S. Histiocytosis Syndromes of Childhood. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap 507.

Raghu G. Interstitial Lung Disease. In: Goldman L, Ausiello D, eds. Cecil Textbook of Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap 92.

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