Histiocytosis

Diseases list > Histiocytosis

Histiocytosis is the term used to define a bunch of immune cell histiocyte disorders. During this condition, the macrophages, monocytes, and dendritic cells all become increased - all of which that eventually will lead to health problems.

The condition has three major classifications. The first is Langerhans Cell Histiocytosis (LCH), which is also the most common. Rounding out the trio are Hemophagocytic Syndrome and T-cell Lymphoma - two histiocytes that are quite rare.

Histiocytosis only started becoming a universally recognized medical condition in the year 1998. So since it is a relatively new disease, accurate ways to diagnose it, various medical management, and different statistics still remain a subject of debate.

Histiocytosis is widespread among males more than among females. Though neonates and children are the most affected by it, adults may also suffer from it. Children ages 0-15 are the ones most likely to develop Histiocytosis. In other words, the risk for getting the disease decreases as one grows older. Statistically, an estimated 1 out of every 200,000 babies born are Histiocytosis carriers.

Though it was initially considered to be Cancer-esque, further studies helped conclude that it can be better compared with that of an autoimmune or self-inflicting disease. Several opinionated medical professionals agree that the disease’ main source is through genetics. When there is too much histiocyte production, tumor formation will take place which will affect the bones majority of the time. The pulmonary tract might also be affected at times.

The Mystery Behind Histiocytosis

What exactly causes Histiocytosis is something that has not been clear up to this day. Speculations say that a combination of genetics, cell malfunction, neoplastic mechanisms, immune dysfunction, and viral infection are said to contribute to the occurrence of Histiocytosis. But the jury is still up in the air regarding whether all of these are true.

Histiocytosis symptoms may vary depending on the site being affected. The most well-known classification, LCH, mainly affects the bones. As a result, Histiocytosis sufferers easily fracture a bone or two. One major characteristic of Histiocytosis is the presence of a punched-out tumor in the bones. One of the worst things that could happen is having the skull as the tumor’s location. All the bones in the entire body are assessed since a commonly affected site has not yet been fully determined up to now.

Histiocytosis Disease

Other common things to take note of are lung problems, hormonal imbalances, skin irritation, lymph node changes, anemia, gum disease, and hepatomegaly (enlargement of the liver). The manifestations children experience may entirely differ from adults. But as mentioned repeatedly, this statement still remains inconclusive until further in-depth studies prove otherwise.

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.

Common Histiocytosis Symptoms

Children usually experience the following particular Histiocytosis symptoms:

  • chronic weight loss
  • jaundice
  • fever
  • stunted growth
  • seizures (occasionally)
  • failure to thrive
  • irritability
  • mental retardation
  • limping

On the other hand, here are what adults manifest:

  • dyspnea (trouble breathing)
  • cough
  • weight loss
  • bone pain
  • body weakness
  • fever
  • skin rash
  • increased frequency in urination (dysuria)

Can Histiocytosis Be Detected?

There are still no definitive diagnostic exams to confirm the presence of Histiocytosis. Part of the reason is that several organs can simultaneously get affected by the disease. Furthermore, findings upon thorough physical examination are quite similar to other medical conditions. That means no findings upon PE are definitive or specific to hystiocytosis. Exams that at least come close to giving definitive results are X-rays, Bone Marrow Aspiration Biopsy (BMAB), pulmonary function tests, skin biopsy, blood chemistry tests, and bronchoscopy with biopsy. Because of the disease’ rareness, doctors obviously have had a hard time giving a correct diagnosis.

Like several other diseases, treatment approach for hystiocytosis will depend on various factors. These comprise of age, extent of the disease, and level of affliction. The most conventional way of treating the disease is by chemotherapy since the drugs will help counteract tumor activity.

What Are The Treatments Available?

In treating Histiocytosis, the main objective is to alleviate all signs and symptoms while also minimizing the occurrence of side effects and complications. For children, they may be prescribed with Cladribine, Cyclophosphamide, Etoposide, Methotrexate, and Vinblastine though corticosteroids are typically the ones given regardless of the age. Also, corticosteroids are being administered topically in case the presence of skin lesions is noted. Drug administration to infants revolve around the principle of having as few (or no) side effects as possible.

The purpose of long-term Histiocytosis treatment is to prevent it from reoccurring in the future. This is why the disease is being compared a lot to cancer. Anyone who has had a history of it before needs very close, one on one monitoring to make sure that the recovery is going well.

Negative Results Of Hystiocytiosis

Once someone has suffered from hystiocytiosis, that automatically puts him/her at risk for adversities brought about by simply suffering from the disease. “Minor” problems may include stunted growth and an alteration in sexual maturity. More severe ones include dental problems, skin scarring, hearing disabilities, and problems in the skeletal system.

For psychological management, it is important that individuals suffering from hystiocytosis that they have their own support group. This may include family, close friends, or even others who suffer the same condition to give them empathy. They can also turn to the Histiocytosis Association of America for additional help.

To conclude, Histiocytosis is definitely a rare condition which hasn’t even reached its fifteenth year. Though not many people are confirmed to suffer from it, there are more who do not have a clue of what it is.  This just proves that the research for this disease should go on and on since the exact ways of diagnosing, managing, and preventing it have not yet been 100% proven. In other words, there still is a ton of room for improvement. It would also be good if “survivors” of this disease will share their experiences and give their own insights on how they were able to withstand  the difficulties they underwent.

Medications

Enbrel, Epirubicin, Cytoxan, Cytarabine, Velban, Neosar,Filgrastim, Fulvestrant, Idarubicin, Ondansetron


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