HISTIOCITOSIS NO LANGERHANS PDF
Langerhans cell histiocytosis (LCH) is a rare disease involving clonal proliferation of It typically has no extraskeletal involvement, but rarely an identical lesion can be found in the skin, lungs, or stomach. When found in the lungs, it should be. Aug 28, Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder most in Erdheim-Chester disease but not in other non-Langerhans cell. Langerhans Cell Histiocytosis information from the Histiocytosis Association. To date, no large-scale studies have been done on how often LCH occurs in.
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Langerhans cell histiocytosis LCH is an inflammatory myeloid neoplasia caused by alterations mutations of several genes in the MAPKinase pathway. Several important studies published from to have immensely clarified the biology of LCH. Badalian-Very, Berres, Chakrobarty The cell of origin is a white blood histioditosis known as a dendritic cell, although the historic designation of histiocyte is still used.
Children and adults may have LCH in skin macular, papular, ulcerative, or seborrheic rashesbones painful lytic lesionslymph nodes, brain pituitary, cerebrum and cerebellum lung, liver, spleen, and bone marrow. Systemic symptoms may include fever, bone pain, weight loss, draining ears, jaundice, diabetes insipidus or other diseases of the endocrine glands, and malaise a general feeling of ill-health. The preferred name for the condition is Langerhans cell histiocytosis because the cell of origin LC is now known.
LCH is a disorder presenting in either single or multiple locations and thus causing a variety of signs and symptoms from mild to life-threatening. Single system presentations may be exclusively in the skin, bone, pituitary, or lungs. Patients affected in multiple systems most often have skin and bone involvement with any combination of other sites. Patients with LCH in sites other than the bone marrow, spleen, and liver can be cured. Bone involvement in children or adults presents as painful areas which may be swollen.
In children, the skull is most often affected, followed by long bones of the upper and lower extremity, ribs and spine. When the temporal bones or mastoids are affected the patient may lose their hearing.
These patients may present with pus draining from the ears and thought to have an infection. Other complications include fractures of long bones and compression of vertebrae causing extreme pain and langerhan spinal cord damage. Jaw involvement in children may result in early eruption of teeth as well as swollen and bleeding gums. Adults are more likely to have lesions in the mandible and maxilla with resulting loss of teeth. Patients may have skin involvement with extensive seborrhia-like rashes on the scalp that mimic persistent cradle cap; an erythematous papular rash similar to Candida diaper rash; or deep ulcerative lesions in the groin or arm pits or purplish-brown lesions mm in diameter which are often mistaken for a viral infection.
Many adult female patients have ulcerative lesions in the genitalia. LCH lesions on the tongue, gums, and inside the cheeks can resemble cold sores.
It is very important that children presenting with skin LCH have a complete evaluation to ensure there is no other disease site. Infiltration of the liver and spleen causes massive abnormal enlargement of organs organomegaly. Liver dysfunction causes hypoproteinemia with swelling of the arms and legs or abdomen. Patients may also have jaundice yellow color to the skin and the white part of the eyes. Lymph nodes in the cervical, axillary, and inguinal areas are most often affected, but mediastinal nodes may enlarge causing wheezing and respiratory compromise.
Lung involvement results in rapid breathing and leakage of air around the lung pneumorthorax. Coughing up blood hemoptysis is rare. Intestinal infiltration leads to crampy pain and diarrhea, often with blood in it. LCH in the bone marrow causes pancytopenia, but thrombocytopenia is often the most obvious problem with bleeding and hisyiocitosis that may be exacerbated by an enlarged spleen.
Endocrine abnormalities from LCH include excessive thirst and urination caused by damage to the back langerhand of the pituitary gland. Donadieu This condition is known as diabetes insipidus.
Pathology Outlines – Langerhans cell histiocytosis
Prosch If the front part of the pituitary gland is damaged by LCH, the patient may have low levels of thyroid hormone, growth hormone, adrenal stimulating hormone and the hormones that lead to sexual maturation. Patients with cerebellar involvement present with inability to walk or balance ataxiatremors of their hands with difficulty writing dysmetriatrouble speaking dysarthria as well as difficulty learning and having abnormal behaviors.
The maturation stage of the dendritic cell will determine what type of disease a patient will develop. If the mutation occurs when the dendritic cell is still in stem cell stage, this early precursor may go to any organ in the body-especially liver, spleen and bone marrow.
An even more mature dendritic cell carrying this mutation may go to only skin and bone. Family members of LCH patients have a higher incidence of thyroid disease. Lanegrhans is strongly associated with lung LCH. Hispanics have a higher incidence of diffuse LCH involvement and Blacks histiocitoxis less affected. Riberio The overall incidence of LCH is between 4 and 9 cases per million with males slightly more affected than females 1.
Symptoms of the following disorders can be similar to those of Langerhans cell histiocytosis. Comparisons may be useful for a differential diagnosis:.
Rosai-Dorfman disease RDD is a rare disorder characterized by overproduction proliferation and accumulation of a specific type of white blood cell histiocyte in the lymph nodes of the body lymphadenopathymost often those of the neck cervical lymphadenopathy.
In some cases, abnormal accumulation of histiocytes may occur in other areas of the body besides the lymph nodes extranodal. These areas include the skin, central nervous system, kidney, and digestive tract. The symptoms and physical findings associated with RDD vary depending upon the specific areas of the body that are affected.
The disorder predominantly affects children, adolescents or young adults. The exact cause of RDD is unknown. The diagnosis of RDD is made by finding excessive numbers of histiocytes that often have lymphocytes passing through their cytoplasm. Mastocytosis is a rare disorder characterized by abnormal accumulation of mast cells in skin, bone marrow, and internal organs such as the liver, spleen and lymph nodes. The skin abnormalities associated with mastocytosis are known as urticaria pigmentosa and are characterized by small, brownish, flat or elevated spots lesions that may be surrounded by reddened, itchy skin.
In many cases, only the skin is involved. However, the disorder may also affect various organ lzngerhans resulting in abnormally enlarged liver and spleen hepatosplenomegaly ; gastrointestinal problems such as abdominal pain and diarrhea; and cardiovascular problems such as high blood pressure hypertension.
In some cases, bones may be hisyiocitosis resulting in bone pain and fractures. The exact cause of mastocytosis is unknown. Diamond Histiocytes are large phagocytic cells macrophages that normally play a role in responding to infection and injury. Associated symptoms and findings and disease course depend on the specific location and extent of such involvement.
The specific underlying cause of ECD is unknown. ECD patients have increased density sclerosis in the tibiae lower leg bones as well as abnormal tissue wrapping around the kidneys, heart, aorta, and bronchi.
Seborrheic dermatitis is a skin disorder characterized by reddish, scaly patches affecting the scalp. The disorder may spread to affect the neck, face, and other areas of the body.
Individuals with LCH that present with skin symptoms may be misdiagnosed with seborrheic dermatitis. The exact cause of seborrheic dermatitis is not known.
The diagnostic work-up may include X-rays of the skull, a complete skeletal bone survey and PET scan, lanyerhans x-ray, complete blood count and differential, erythrocyte sedimentation rate, liver function tests including AST, ALT, bilirubin, and albumin; electrolytes and urinalysis.
Phillips CT of the skull if mastoids involved. Single bone lesions not in the CNS risk group are treated with surgery alone or with injection of steroids. If only skin lesions are present and bistiocitosis extensive, treatment may histicitosis be necessary. If treatment is needed, hydroxyurea alone or with methotrexate is very effective. Zinn Oral methotrexate or thalidomide are also used. Central nervous system involvement pituitary or other mass lesions is treated with cytarabine or cladribine.
Adults should not be treated with Velban and prednisone because these drugs are not effective and cause excessive toxicity. Cytarabine has been shown to be a better therapy and cladribine has also been effective Cantu. Oral hydroxyurea with or histioxitosis oral methotrexate has been effective in treating skin and bone LCH, with special efficacy histipcitosis vaginal lesions Zinn.
It is preferable that patients be treated on clinical trials so langergans biology and therapy of these rare patients can be advanced. Treatments vary depending on the extent of disease and involve chemotherapy with prednisone, velban with or without 6-mercaptopurine and methotrexate have been the standards for several decades and are still being studied by the Histiocyte Society in the LCH-IV trial.
The latter could mean mass lesions or a nerve damage leading to only changes on the MRI brain scan or neurologic problems such as ataxia, dysmetria, dysarthria, learning difficulties and psychologic problems. Treatment with drugs which specifically inhibit cells containing mutations in the MAP2K pathway vemurafenib, dabrafenib are being used by various institutions langerhas a clinical trial is being organized.
Contact the Histiocytosis Association of Langedhans for details of treatment protocols: Information on current clinical trials is posted on the Internet at www. All studies receiving U.
For information about clinical trials sponsored by private sources, contact: For information about clinical trials conducted in Europe, contact: Inflammatory and Malignant Histiocytoses. In Williams Hematology, 9th Edition. Kenneth Kaushansky, Marshall A. In Principles and Practices of Pediatrics 7th Edition.
Oncology, Pizzo and Poplack eds. Langerhans Cell Histocytosis in Children and Adults. Cell-specific gene expression in Langerhans cell histiocytosis lesions reveals a distinct profile compared with epidermal Langerhans cells. How I treat Langerhans cell histiocytosis. Optimal therapy for langernans with Langerhans cell histiocytosis bone lesions. Consensus guidelines for the diagnosis and clinical management of Erdheim-Chester disease.
Therapy prolongation improves outcome in multisystem Langerhans cell histiocytosis. A phase II trial using thalidomide for Langerhans cell histiocytosis.
Pediatr Blood Cancer 48 1: Central nervous system-related permanent consequences in patients with Langerhans cell histiocytosis. Pediatr Blood Cancer ; Central diabetes insipidus as presenting symptom of Langerhans cell histiocytosis.
Ethnicity, race, and socioeconomic status influence incidence of Lanngerhans cell histiocytosis.