Langerhans cell histiocytosis (LCH) was named in 1868 after Paul Langerhans, who discovered abnormally high levels of white blood cells in bone marrow.
It is also known as Langerhans Cell Granulomatosis, Interstitial Lung Disease, and Pulmonary Langerhans Cell Histiocytosis. LCH is typically found in bones and the head and neck. LCH belongs to a group called dendritic cell histiocytosis.
It is most common in children under 10. Its causes are unknown, but researchers speculate that viral infections and environmental circumstances might cause it.
Risk Factors
LCH is a rare disease; there are only about 1,200 new cases reported in the US each year. Further, LCH appears in 1 in 50,000 patients, mostly infants. LCH seems to affect children between 1-15 years of age; however, adult have also been diagnosed.
However, more than half of patients under two have died from the disease. LCH seems to strike Caucasians most frequently. It is more common in males than in females. LCH is not a genetic condition.
Prognosis
Patients with single-system disease have a good prognosis. Even those with skin, lymph node, or bone disease are likely to fair well. A vast majority of patients survive, although some will have life-long chronic health problems. However, if organs have been impacted by LCH, or if the patient is very young, the prognosis is poorer. The survival rate for multi-organ disease is 80%. Recurrence is common.
Prevention
Cigarette smoking may play a role in LCH.
Signs & Symptoms
Signs and symptoms of LCH are widespread and will depend on the extent and location of the disease. Many symptoms seem like other ailments, but multiple symptoms should cause alarm.
Common Signs & Symptoms
- Fever/night sweats
- Swollen lymph nodes, spleen, liver, or eye lids
- Lethargy/Weight loss
- Skin lesions or rashes
- Solitary bone lesions (often in vertebra, rib, jaw, thigh bone, shoulder blade, pelvis)
- Broken or fractured bones
- Tenderness or pain in bones
- Loose teeth/swollen gums
- Ear infections
- Excessive thirst/urination
Other Signs & Symptoms
- Skin lesions at birth or in early postnatal period
- Spinal cord compression
- Anemia
- Hepatosplenomegaly
- Pituitary and Pulmonary dysfunction
- Orthopedic difficulties
- Hearing impairment
- Diabetes insipidus
- Liver cirrhosis
- Growth retardation
- Hyperprolactinemia and hypogonadism
Associated Disorders
The following are manifestations of LCH:
- Solitary or multifocal eosinophilic granuloma: lesions typically impact bone.
- Hand-Schueller-Christian disease: typically impacts flat bones
- Letterer-Siwe disease: the most severe form of LCH.
Diagnosis & Staging
A diagnosis is based on the patientís medical health and a physical examination and typically a biopsy, which would indicate Langerhansí cells in most cases. A severe purple rash, bone lesions, skin abnormalities, and pulmonary problems all necessitate further testing to confirm LCH.
Tests
- Blood test
- Liver biopsy
- Urine test
- X-rays of chest and skeleton
- CT scan or MRI of hypothalamus and pituitary glands
- Bowel test
- Hormonal study
- Visual and neurological test
- Skin/bone marrow/liver biopsy
Treatment
Treatment of LCH will depend on whether it is single-system or multi-system; the severity of the disease; whether organs have been impacted; the condition of the patient; and the patient’s tolerance for various treatment options. In some cases, LCH will subside without treatment.
Patients are well advised to seek counsel from an institution with experience in treating LCH. For all cases, patients should consult with an orthopedic surgeon, an otolaryngologist, an ophthalmologist, a dentist, and an endocrinologist.
Common Treatment Options
For single-system disease: bone lesions are removed and treated with steroids; skin disease is treated with steroids; affected lymph nodes are removed or treated with steroids. Chemotherapy is the secondary choice.
For multi-system disease: system-wide chemotherapy is the primary treatment. Cytotoxic drugs and steroids are also effective.
Surgery to remove the LCH cells is also an option, although more typical for single-system disease. Many patients also require hormone replacement if the hypothalamus is impacted.
Other Treatment Options
Clinical trials are experimenting with chemotherapeutic agents including: vinblastine, etoposide, prednisolone, mercaptopurine, and methotrexate. And, other options include: bone marrow transplants, experimental chemotherapy, or immunosuppressive therapy.
