hematology and oncology
Langerhans Cell Histiocytosis
Symptoms and Signs of Langerhans Cell Histiocytosis
Symptoms and signs of Langerhans cell histiocytosis vary considerably depending on which organs are infiltrated. Patients are divided into 2 groups based on organ involvement: Single system Multisystem Single system diseaseis unifocal or multifocal involvement of one of the following organs: bone, skin, lymph nodes, lungs, central nervous system, or other, rare locations (eg, thyroid, thymus). An example of single system disease is eosinophilic granuloma. Multisystem diseaseis disease in two or more organ systems. Risk organs (organs in which disease involvement portends a worse prognosis), which include the liver, spleen, and organs of the hematopoietic system, may or not be affected. An example of multisystem disease without risk organ involvement is Hand-Schüller-Christian disease. An example of multisystem disease with risk organ involvement is Letterer-Siwe disease. Here, the syndromes are described by their historical designations, but few patients present with classic manifestations, and other than eosinophilic granuloma, these designations are no longer used. Unifocal or multifocal single-system lesions (60 to 80% of LCH cases) occurs predominantly in older children and young adults, usually by age 30; incidence peaks between ages 5 and 10 years. Lesions most frequently involve bones, often with pain, the inability to bear weight, or both and with overlying tender (sometimes warm) swelling. Congenital self-healing reticulohistiocytosis (previously called Hashimoto-Pritzker disease) is a single-system disease with isolated skin lesions that occurs in neonates. Lesions generally resolve on their own or respond to topical treatment. Patients should be evaluated to exclude systemic disease. This syndrome (15 to 40% of LCH cases) occurs in children aged 2 to 5 years and in some older children and adults. Classic findings in this systemic disorder include involvement of the flat bones of the skull, ribs, pelvis, scapula, or a combination. Long bones and lumbosacral vertebrae are less frequently involved; the wrists, hands, knees, feet, and cervical vertebrae are rarely involved. In classic cases, patients have Proptosis caused by orbital tumor mass. Rarely, vision loss or Otitis Media (Chronic Suppurative) and 2 years presents with a scaly seborrheic, eczematoid, sometimes purpuric rash involving the scalp, ear canals, abdomen, and intertriginous areas of the neck and face. Denuded skin may facilitate microbial invasion, leading to Sepsis and Septic Shock . Frequently, there is ear drainage, lymphadenopathy, hepatosplenomegaly, and, in severe cases, hepatic dysfunction with hypoproteinemia and diminished synthesis of clotting factors. Anorexia, irritability, failure to thrive, and pulmonary manifestations (eg, cough, tachypnea, pneumothorax) may also occur. Significant anemia and sometimes neutropenia occur; thrombocytopenia is of grave prognostic significance. Parents frequently report precocious eruption of teeth, when in fact the gums are receding to expose immature dentition. Patients may appear abused or neglected. can be used to treat skin lesions. . Systemic lesions are treated with chemotherapy similar to what is used in Treatment . Mitogen-activated protein kinase (MAPK) pathway mutations have been identified, so targeted therapy can also be used. Juvenile xanthogranuloma is rarely associated with neurofibromatosis and/or juvenile myelomonocytic leukemia. 