A group of rare disorders in which too many histiocytes (a type of white blood cell) build up in certain tissues and organs, including the skin, bones, spleen, liver, lungs, and lymph nodes. This can cause damage to tissue or tumors to form in one or more parts of the body. Is Langerhans a cancer?
Langerhans cell histiocytosis (LCH) LCH is classified as a cancer and sometimes requires treatment with chemotherapy.

What does Histiocytic mean in medical terms?

Medical Definition of histiocytosis : abnormal multiplication of macrophages Langerhans cell histiocytosis broadly : a condition characterized by such multiplication. Is histiocytosis an autoimmune disease?
Langerhans cell histiocytosis historically was thought of as a cancer-like condition, but more recently researchers have begun to consider it an autoimmune phenomenon in which immune cells begin to overproduce and attack the body instead of fighting infection.

How is histiocytosis diagnosed?

Radiology Scans CT scans can identify areas of histiocytosis. They can be used to look at any part of the body, but CT is most helpful for detecting histiocytosis in the lungs, heart, kidneys, lymph nodes, and other organs. MRI scans can get a very detailed picture of certain organs. What causes Langerhans?

The cause of this disease is unknown, although most data suggest that it is characterized by a growth of immature Langerhans cells that appear to have mutations of the BRAF gene in about half the cases. LCH is not caused by a known infection, is not contagious, nor is it believed to be inherited .

Frequently Asked Questions(FAQ)

What are the symptoms of Langerhans?

What are the symptoms of Langerhans cell histiocytosis in a child?

Is Langerhans Cell Histiocytosis a type of lymphoma?

In a small subset of patients, LCH has been identified as an incidental finding in biopsy specimens involved by lymphoma. Classical Hodgkin lymphoma is the most common associated lymphoma,23 26 whereas only sporadic cases of other types of non-Hodgkin lymphoma associated with LCH are reported in the literature.

Is histiocytosis curable?

Usually a cure is linked to being in remission for a certain period of time. There is no established period of “non-active” disease before LCH is considered cured, but the chance for recurrence is low after five years from end of treatment.

Is there a cure for histiocytosis?

Radiation therapy can be extremely effective for certain types of histiocytosis, especially Langerhans cell histiocytosis. Radiation is often recommended to treat people whose histiocytosis is affecting only one area of the body.

Is LCH a leukemia?

What is histiocytosis in adults?

Langerhans cell histiocytosis (LCH) in adults is a rare disorder that occurs when the body produces too many Langerhans cells (histiocytes), which are a type of white blood cell that helps fight infection.

What is a Xanthogranuloma?

Xanthogranuloma is a rare benign tumour, part of the non-Langerhans cell histiocytosis group, uncommon in adults and even less common in the paranasal sinuses. Despite its benign nature, it mimics neoplasm due to its local effects which can have serious functional consequences depending on the anatomical location.

What is histiocytic infiltration?

Histiocytic disorders (HDs) are a diverse group of diseases characterized by pathologic infiltration of normal tissues by cells of the mononuclear phagocyte system. The spectrum of these diseases ranges from treatable infectious diseases to rapidly progressive, life-threatening conditions.

What is malignant histiocytosis?

Malignant histiocytosis is a rare invasive proliferation of neoplastic histiocytes. Cases previously reported as malignant histiocytosis were shown to be lymphomas of T or B lineage, especially anaplastic large‐cell lymphomas.

What is Langerhans?

Langerhans cells (LC) are a unique population of tissue-resident macrophages that form a network of cells across the epidermis of the skin, but which have the ability to migrate from the epidermis to draining lymph nodes (LN). Their location at the skin barrier suggests a key role as immune sentinels.

Do Histiocytomas go away?

Histiocytomas are considered highly treatable skin masses. Though they will typically regress spontaneously within a couple of months, they don’t always do so quickly or completely enough for a veterinarian’s (or owner’s) comfort.

Is LCH life threatening?

The disease is rarely life-threatening. However, some LCH survivors experience long-term effects, such as orthopedic disabilities, hearing impairment, diabetes insipidus, and skin scarring.

What is PLCH?

Pulmonary Langerhans cell histiocytosis (PLCH), previously called eosinophilic granuloma of the lung, pulmonary Langerhans cell granulomatosis, and pulmonary histiocytosis X, is an uncommon cystic interstitial lung disease that primarily affects young adults [1-5]. It is caused by a disorder of myeloid dendritic cells.

How is Langerhans histiocytosis treated?

Steroid therapy, such as prednisone, is used to treat LCH lesions. Bisphosphonate therapy (such as pamidronate, zoledronate, or alendronate) is used to treat LCH lesions of the bone and to lessen bone pain.

How do Langerhans cells protect the body?

Langerhans cells send out special agents — immune cells such as T cells and B cells — immediately after sensing any kind of danger in the skin. The immune cells capture trespassers such as bacteria and viruses and fight off injuries like cuts and scrapes.

Where are Langerhans cells commonly found?

Langerhans cells are bone marrow-derived, antigen-processing and -presenting cells found mainly in the suprabasal epidermal layers. They are, however, not unique to the epidermis and are found in other squamous epithelia and in the normal dermis.

Is Langerhans Cell Histiocytosis a solid tumor?

Langerhans cell histiocytosis (LCH) is a rare disorder of histiocyte proliferation. Previous case studies suggest a higher prevalence of hematologic and solid malignancies among LCH patients, possibly due to treatment with tumorigenic agents such as etoposide.

Can Langerhans Cell Histiocytosis go away on its own?

In many people with Langerhans cell histiocytosis, the disorder eventually goes away with appropriate treatment. It may even disappear on its own, especially if the disease occurs only in the skin.

What is Langerhans Cell Histiocytosis of the lungs?

Pulmonary Langerhans cell histiocytosis (PLCH) is a lung disease that creates air-filled spaces (called cysts) in the lungs. It occurs most often in young adults if they smoke and may improve if they stop.

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