LCH in Adults


Symptoms

Langerhans cell histiocytosis (LCH) can involve nearly any part of the body, though some sites are more common than others. A patient may have very limited involvement in one body system or widespread involvement in several different sites and systems. It is also possible to have LCH in a particular location without symptoms.

The following symptoms may indicate disease involvement but are not diagnostic of LCH. This disease varies greatly from patient to patient, and some but not all of these symptoms may be present:

  • Skin (scalp, face, groin, trunk, armpits, arms, legs, ear canals, vulva, anal area, fingernails)
    Rash, ulceration, redness, pain, oozing, hair loss. Lesions may appear as small, solid, reddish elevations on the skin surface; knots under the skin; purplish-red spots; bleeding under the skin; rashes that are scaly and greasy; ulcerations; and small abscesses. Skin lesions may signal that other areas in the body are affected.
  • Bones (skull, bones around eyes, back bones, ribs, pelvis, feet, toes, hands, fingers)
    Pain, swelling, lumps, headaches, spontaneous fracture, ulceration at the site, limp, collapse of disc in the back, inability to walk.
  • Bone Marrow
    Low blood counts (red blood cells, white blood cells, platelets/clotting cells).
  • Lungs
    Chest pain, shortness of breath, collapsed lung, dry cough, weight loss, fatigue, loss of appetite, night sweats, coughing up blood.
  • Teeth/Gums
    Loosening or loss of teeth, swollen or bleeding gums, ulcerations, pain, swelling of face.
  • Ears
    Chronic ear infections, drainage/discharge, balance problems, bleeding, decreased hearing. Ear involvement can be a result of skull lesions expanding to the ear and may cause balance problems. Ear canals may be affected as part of the skin system, causing bleeding/drainage.
  • Endocrine System (pituitary gland/diabetes insipidus, hypothalamus, and thyroid gland)
    Excessive thirst, excessive urination, fatigue, sweats, temperature swings, weight gain, weight loss, nipple tenderness/discharge, menstrual problems in women, erectile dysfunction in men.
  • Female Genital/Reproductive Tract (vulva, vagina, cervix, ovaries)
    Inflammation, rash, and/or ulceration of the vulva, vagina, and/or cervix, but it may also affect the ovaries, causing dysfunction or failure of these organs.
  • Liver/Spleen/Lymph Nodes
    Swelling/tenderness of abdomen; enlargement of lymph nodes, spleen and/or liver; fatigue.
  • Gastrointestinal System (stomach or intestines)
    Diarrhea, nausea, vomiting, and/or weight loss.
  • Central Nervous System (spinal cord, brain lesions, pituitary gland)
    Staggering when walking, seizures, weakness of the arms, legs, or weakness of one side of the body. Since the pituitary gland could be affected, lesions of this system may also cause some of the same endocrine problems mentioned above.
  • Additional Non-specific Symptoms
    Fever, weakness, fatigue, chronic pain, weight loss, night sweats.

Pulmonary LCH (PLCH) can occur as part of multi-system disease, or it can occur alone. Nearly 20% of adults with PLCH have no presenting symptoms. An estimated 10%-15% of patients present with lung collapse as the first symptom, while others may show abnormalities in lung function tests. Dry cough and shortness of breath are the most common complaints, and weight loss, fever, sweats, and loss of appetite occur in approximately 33% of patients.

Many adults experience severe and sometimes overwhelming pain associated with this disease. While pain can be caused by bone lesions or bone defects that do not heal completely with therapy, it has also been observed that some patients have pain even when there is no active disease seen on x-ray. The cause of this pain is not understood; however, it is being currently explored in research. Pain is considered a complication of LCH that should be fully evaluated by a physician and treated with appropriate medications.

 

 

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