pertains to a group of disorders concerning the immune cell histiocyte. It involves an increase in histiocyte cells including dendritic cells, monocytes, and macrophages. This condition could lead to different health problems.
Histiocytosis is classified into three major conditions. The most common of which is Langerhans Cell Histiocytosis or LCH. The other two, less common Histiocytosis conditions are known as hemophagocytic syndrome and T-cell lymphoma
Histiocytosis is found more common in male than in female. It can be congenital but may also occur in adults. Most Histiocytosis patients are children. Previous diagnoses shown 0-15 children are most susceptible to suffer from this kind of condition.
Histiocytosis is fairly new to the medical world. The group of syndromes that form part of Histiocytosis has been recognized only in 1998. As such, medical treatment procedures remain unspecified.
About one in 200,000 births in America carry Histiocytosis. All the figures, however, are suspected to be underestimated since not every physician in the world is familiar with this condition.
The excessive production of histiocyte tissues forms tumors affecting the bones
eighty percent of the time. It also sometimes thrives in the pulmonary tract. Histiocytosis was first thought of as similar to Cancer
but after series of clinical tests, Histiocytosis has been found to be more of an autoimmune disorder. The main source of Histiocytosis known to the members of the medical community is that which stems in genetics.
The causes of Histiocytosis are yet unknown but experts speculate that viral infection, cell malfunction, immune dysfunction, neoplastic mechanisms, and as mentioned earlier, a host of genetic factors.
The symptoms of the disease depend on its site of action. LCH, which is the most popular syndrome, is found to affect the bones primarily. Patients with Histiocytosis are susceptible to bone fractures
for no apparent reason. Skin rashes, lung
problems, gum disease, swollen lymph
gland, hormonal disorders
enlargement, and anemia
are also common. Histiocytosis may affect not just one organ but others as well. In this sense, it can easily cause death.
Histiocytosis in children five years and above is commonly just about the bones. Systemic involvement, meanwhile, is found mostly in infants, which triggers the fatal outcome.
The symptoms of Histiocytosis in children may be different from what adults may experience. However, since the study regarding this rare disease is still ongoing, that cannot be claimed as absolute truth.
Common Histiocytosis symptoms in children include failure to thrive, chronic weight loss
, irritability, fever
, abdominal pain, jaundice, limping, vomiting
, delayed growth, mental deterioration, and seizures among others. In case of adults, the tall tale signs of Histiocytosis include cough
, breathing difficulty, chest pain
, weight loss, fever, malaise, increased urine, bone pain, and skin rash.
Diagnosis of Histiocytosis is still quite nonspecific because there could be many organs involved at one point. No amount of physical examinations can possibly trace the occurrence of this disease because all the symptoms can be associated with many other health conditions. Histiocytosis can only be singled out through x-ray
of the bone, the entire skeleton, and the pulmonary tract, Bronchoscopy with biopsy
, bone marrow and skin biopsy
, complete blood count
, and Pulmonary function tests.
Histiocytosis is mostly characterized by a punched-out tumor in the bones. No particular site has been singled out that is why a survey of the whole skeletal system
is actually needed to provide a full view of the condition. Sometimes the tumor even occurs in the skull
Proper diagnosis is very significant in such a rare disease as Histiocytosis. Medical updates occurring alongside the disease is needed for the research documents to deliver absolute answers to the physicians’ questions.
At present, Histiocytosis is treated depending on the patient’s age, level of affliction, and the extent of which his body is affected. Chemotherapy is the most common form of treatment that is out to battle the activity of the tumor. Corticosteroids are often used to suppress the immune function but children may also be given Methotrexate
, Vinblastine, Cyclophosphamide
, and Etoposide
Radiation therapy is also found effective in preventing the tumors from metastasizing. Spread of excessive histiocyte in critical organs or parts of the body may cause permanent or uncontrollable damage.
Skin lesions that occur with Histiocytosis are often prescribed with topical medications of corticosteroids. However, if it involves infants and very young children, treatment is handled with utmost care ensuring that it provides rapid response with very minimal side effects.
The major objective of medical treatment in Histiocytosis is to relieve the symptoms and avoid the progression of the condition and cause any more complications.
Long-term medical care is needed to ensure that a Histiocytosis survivor is completely safe of recurrence. Since patients who have championed the disease have a pretty huge chance of suffering from relapse, close monitoring by a specialist knowledgeable about Histiocytosis is important.
A patient with Histiocytosis relapse has odds of developing adverse effects that come out at a later stage. If this is not seen because of the absence of follow-up care, Histiocytosis may cause stunted growth and a disturbed sexual maturity. Orthopedic problems, dental disorders, hearing loss, and skin scarring may also occur.
For the moment, patients of Histiocytosis may find shelter and support from some groups specializing in this particular condition. The Histiocytosis Association of America is especially supportive of any clinical study that will be done about the disease. It is also very open about providing support and sharing treatment protocols that are in its research stable.
Histiocytosis is not a very common disease. Its recognition close to a decade ago is a proof that not too many individuals suffer from its disabling symptoms. That is not a reason, however, for the medical industry to stop on learning new and absolute ways that will put off Histiocytosis completely. Patients suffering from this disease can do so much by sharing their experiences and being open to be part of the research process. Clinical research teams need to learn as much as they can about Histiocytosis to provide an antidote that will be able to tackle its occurrence.