Is JXG a cancer?
Juvenile xanthogranuloma, also known as JXG, is a rare, non-Langerhans cell histiocytosis that is usually benign and self-limiting.
How rare is JXG?
In 40–70% of patients, JXG develops in the first year of life [5]. In 5–17% of cases, the skin lesions may appear soon after birth [3, 5]. The highest incidence of JXG in adulthood is observed in patients aged 20 to 30 years, but the disease is generally rare in adults.
How long does it take for JXG to go away?
JXG can sometimes be seen at birth. Other times the bumps can form after birth for up to 1 to 2 years. The bumps normally go away on their own in children over 3 to 6 years.
How common is JXG?
JXG is considered a rare disease in itself, but is the most common type of non-Langerhans histiocytosis. The incidence of juvenile xanthogranuloma is estimated to be 1 per million in children, however it is probably underdiagnosed. Up to 10% of patients with neurofibromatosis type I may develop JXG.
What causes JXG?
The cause of JXG is not clearly understood but it is believed to result from an overproduction of a type of histiocyte cell used in the body’s immune system in response to nonspecific tissue injury. Histiocytes work in the immune system to fight bacteria and dispose of tissue waste products.
What does a histiocyte do?
A histiocyte is a normal immune cell that is found in many parts of the body especially in the bone marrow, the blood stream, the skin, the liver, the lungs, the lymph glands and the spleen. In histiocytosis, the histiocytes move into tissues where they are not normally found and cause damage to those tissues.
How many people have JXG?
JXG is a skin lump caused by an excess of cells known as histiocytes. It is rare and occurs mainly in infants and young children, although about 10% occur in adults. It is not known what causes this condition.
Is Xanthogranuloma malignant?
Juvenile xanthogranuloma (JXG) is a benign, proliferative disorder of histiocytic cells of the dermal dendrocyte phenotype.
Where are histiocytes found in the lymph node?
Sinus histiocytes are the principal cells involved in phagocytosis of foreign particulate matter, and they are primarily located in lymph node sinuses. The pathophysiology of histiocytoses is thought to be uncontrolled immunologic stimulation of normal antigen-processing cells rather than malignant transformation.
Where are histiocytes found in body?
A histiocyte is a normal immune cell that is found in many parts of the body especially in the bone marrow, the blood stream, the skin, the liver, the lungs, the lymph glands and the spleen.
What causes Xanthogranuloma?
What does a histiocyte look like?
Histiocytes (Macrophages) In smears, the typical histiocyte is easily identified by its size, eccentric round or bean-shaped nucleus, and lightly stained lacy cytoplasm (Fig. 19.43). Because histiocytes are phagocytic, their cytoplasm may contain leukocytes, nuclear particles (Fig.
What is juvenile xanthogranuloma (JXG)?
Juvenile xanthogranuloma, also known as JXG, is a rare, non-Langerhans cell histiocytosis that is usually benign and self-limiting. It occurs most often in the skin of the head, neck, and trunk but can also occur in the arms, legs, feet, and buttocks. JXG can affect the eye, most commonly in young children with multiple skin lesions.
Can JXG affect the eye?
JXG can affect the eye, most commonly in young children with multiple skin lesions. Less commonly JXG may involve locations such as the lung, liver, adrenal gland, appendix, bones, bone marrow, pituitary gland, central nervous system, kidney, heart, small and large intestines, and spleen.
What are the signs and symptoms of juvenile myasthenia gravis (JXG)?
Common signs of JMML include anemia, thrombocytopenia, leukocytosis, lymphadenopathy and hepatosplenomegaly. JXG most commonly develops in infants younger than 2 years of age but has been found in older children. In a cohort of 174 JXG patients with cutaneous lesions, the mean age was 3.3 years (median 1 year).
Is JXG associated with neurofibromatosis 1?
JXG has been associated with neurofibromatosis 1, Niemann- pick disease and urticaria pigmentosa. Patents with neurofibromatosis 1 and JXG have a 20- to 32- fold higher risk of juvenile myelomonocytic leukemia (JMML) than patients with NF1 without JXG.