Introduction
Langerhans cell histiocytosis(Langenhans cell histiocytosis, LCH), formerly known as histiocytosis, is a group of unexplained tissue cell proliferative disorders. Tradition is divided into three clinical types, namely, Leter's syndrome, (Litterer-Siwe disease, referred to as LS disease), Han-Xue-Ke syndrome, (Hand-Schuller-Christian disease, referred to as HSC disease) and bone hobby Eosinphilic granuloma of bone (EGB). The cause is unknown. In recent years, studies have found that it is associated with immune regulation disorders in vivo. The incidence of this disease is estimated to be 1/200,000 to 1/20,000. It mainly occurs in babies and children, but also in adults and even the elderly. Many reports mention that male patients are mostly.
Cause
The etiology is still unclear. Although its genetic characteristics are still unclear, it has a certain familial nature, and the incidence among siblings is much higher than that of ordinary children. It is also considered that the disease has the nature of a tumor.
symptom
Clinical manifestations The onset of this disease varies, the symptoms are diverse, LCH, skin, single or multiple bone damage, with or without diabetes insipidus is limited; liver, spleen, lung, hematopoietic system and other organ damage Or with bone and skin lesions are extensive. This patient involved multiple systems and multiple organs belonging to the extensive LCH.
The lighter is an isolated painless bone lesion, and the severe one is extensive organ infiltration with fever and weight loss.
(1) Skin lesions of the rash are often the primary symptoms of the diagnosis. The rash is a variety of acute infants with initial rash. The rash is mainly distributed in the trunk and scalp, behind the ear, and begins to be a maculopapular rash. It quickly oozes out (similar to eczema). Can be seborrheic dermatitis), may be associated with bleeding, and then crusted, loose, and finally left with pigmentation leukoplakia, white spots are not easy to dissipate for a long time. Each stage of rash can exist at the same time or a batch of retreats can be repeated, and there is often fever in the case of rash. Chronic rash can be scattered throughout the body, initially paleomenopausal papules or sputum nodules, when the depression subsides the central depression, some are dark brown, very similar to scab, and finally the local skin thinning slightly concave Slightly shiny or slightly desquamated. The rash can occur at the same time as other tube damage, or as the only affected manifestation, common in male infants under 1 year old.
(2) Bone lesions Bone lesions are found in almost all patients with LCH. Individual bone lesions have more bone lesions, mainly manifested as osteolytic lesions. Skull lesions are most common, followed by lower extremity bones, ribs, pelvis and spine, and jaw lesions are also quite common. On the X-ray film, the bone disintegration is characterized by irregular edge. The skull damage changes from the worm-like shape to the large defect or the chisel-like change. The shape is irregular, round or elliptical, and the edge is jagged. The boundary of the initial or progressive lesion is blurred, and the common intracranial pressure is increased, the fracture of the bone is broken or the hydrocephalus of the communication may be accompanied by headache. However, during the recovery period, the bone is gradually clear at the edge, the hardening zone appears, the bone density is uneven, the bone defect gradually becomes smaller, and finally the whole repair does not leave any trace. X-ray changes of other flat bones: visible rib swelling, thickening, thin bone or cystic changes, and then bone absorption, atrophy, thinning. Vertebral destruction can become a flat vertebra, but the intervertebral space is not narrow, and angular deformities rarely occur. Spinal arch destruction is prone to spinal nerve compression, and a small number of paravertebral soft tissue swelling. Jaw disease can be expressed as both alveolar and jaw shape.
(3) Lymph node lesions of lymph node LCH can be expressed in three forms. 1 simple lymph node lesions, known as lymph node primary eosinophilic granuloma; 2 is a concomitant lesion of localized or focal LCH, often involving osteolytic lesions or skin lesions; 3 as systemic diffuse LCH portion. Often involved in the isolated lymph nodes in the neck or groin, most patients have no fever, and a few have only pain in the enlarged lymph nodes. Simple lymph node involvement, the prognosis is good.
(4) The inflammation of the outer ear of the ear and mastoid LCH is often the result of proliferation and infiltration of Langerhans cells in the soft tissue of the ear canal or bone tissue. Sometimes it is difficult to distinguish from diffuse bacterial ear infections. The main symptoms are empyema in the external auditory canal, swelling behind the ear and conductive deafness. CT examination can show both bone and soft tissue lesions. Mastoid lesions can include mastoiditis, chronic otitis, cholesteatoma formation, and hearing loss.
(5) Under normal circumstances, there is no LC in the bone marrow, and even LCH invading multiple sites is also difficult to see LC in the bone marrow. Once the LC invades the bone marrow, the patient may have anemia, leukopenia and thrombocytopenia, but the degree of abnormal bone marrow function. It is not proportional to the amount of LC infiltration in the bone marrow. LC alone in the bone marrow is not sufficient as a basis for diagnosis of LCH.
(6) The thymus thymus is one of the organs that LCH often involves.
(7) Pulmonary lesions of lung LCH may be part of systemic lesions, or may exist separately, the so-called primary lung LCH. Pulmonary lesions can occur at any age, but are more common in infants during childhood, manifesting as dyspnea, hypoxia, and lung compliance. In severe cases, pneumothorax and subcutaneous emphysema may occur, and respiratory failure may occur and death may occur. Pulmonary function tests often show restrictive damage.
(8) Liver system diffuse LCH often invades the liver, and the affected parts of the liver are mostly in the liver triangle. The degree of involvement can range from mild gallbladder deposition to severe hepatic hilar infiltration, hepatocyte injury and bile duct involvement, and liver performance. Abnormal function, jaundice, hypoproteinemia, ascites and prolonged prothrombin time can progress to sclerosing cholangitis, liver fibrosis and liver failure.
(9) spleen diffuse LCH often has spleen and elbow swelling, accompanied by peripheral blood one or more blood cell reduction, which may be due to the expansion of the spleen volume, causing blockage of platelets and granulocytes without damage, increased by yin The stagnation of blood cells and peripheral blood cells can still reach a dynamic balance, so bleeding symptoms are not common.
(10) Gastrointestinal lesions are common in systemic diffuse LCH. Symptoms are mostly related to the affected area. The small intestine and ileum are most often involved, showing vomiting, diarrhea and malabsorption, which can cause stagnation in children for a long time.
(11) The involvement of the central nervous system in the central nervous system LCH is not uncommon. The most common site of involvement is the thalamus-pituitary area. Diffuse LCH can have substantial brain lesions. Most patients with neurological symptoms appear in other parts of LCH several years later, often with ataxia, dysarthria, nystagmus, hyperreflexia, rotational dyskinesia, difficulty swallowing, blurred vision and so on. Diabetes insipidus caused by the thalamus and/or pituitary granulomatosis can occur before or after the brain symptoms or with the brain symptoms, or it can be the only manifestation of the CNS.
(12) Letterer-Siwe disease is the most serious type of Langerhans cell histiocytosis, accounting for about 1%. A typical case is a baby less than 2 years old, with a scaly seborrheic eczema-like rash, sometimes Presents a purplish rash that invades the scalp, ear shell, abdomen, and wrinkled areas of the neck and face. Skin damage can become a gateway to microbial invasion, leading to sepsis. Common ear overflow pus, lymphadenopathy, hepatosplenomegaly. In severe cases, hepatic dysfunction can be associated with hypoproteinemia and decreased synthesis of coagulation factors, anorexia, irritability, and weight loss. And have obvious respiratory symptoms (such as cough, shortness of breath, pneumothorax), severe anemia, sometimes happeningNeutrophil reduction. Thrombocytopenia is often a precursor to death. Because of these manifestations, young patients are often misdiagnosed or missed.
diagnosis
The diagnosis method is based on clinical, X-ray and pathological examination results, that is, the pathological examination of the lesions in the lesions can be confirmed by tissue infiltration. The key to the diagnosis of this disease is the pathological examination of the tissue infiltration of Langerhans cells. Therefore, biopsy should be done as much as possible.
Identification
1. Seborrheic dermatitis: The lesions of Langerhans cell hyperplasia sometimes appear as seborrheic dermatitis, but seborrheic dermatitis in infancy does not have systemic symptoms and hepatosplenomegaly.
2. Xanthomas: Langerhans cell hyperplasia with yellow tumors should be differentiated from other diseases that may cause yellow tumor damage, the latter may have hyperlipoproteinemia and other underlying diseases, generally no obvious systemic symptoms and bone Loss, if necessary, should be examined for bone marrow, histopathology, etc.
complication
Chronic otitis media and otitis externa: caused by involvement of the humeral mastoid and rocky parts;
A lump in the eyelid can cause a prominent eye, and the optic nerve or the eye muscle is invaded to cause vision loss or strabismus;
The most common sites of bone invasion are the flat bones (such as the skull, ribs, pelvis and scapula). Long bones and lumbar vertebrae, the tibia is less affected. The lesion on the long bone resembles Ew-ing sarcoma, osteosarcoma and osteomyelitis. Wrist, head, knee, foot or cervical vertebrae are rare. Parents often report the early maturity of the child, which is actually due to the gingival recession and the exposure of immature dentin.
treatment
Western medicine treatment
The low-risk group was >2 years old, and the hematopoietic system, liver, lung or spleen was not violated. The risk group is <2 years old, or with the aforementioned organs being violated. Due to the persistence of the disease, patients often cannot follow the strict design of the program, combined with treatment, so there may be multiple organ involvement symptoms (Table 137-2). Patients in the 0-II group, especially those with a single systemic disease, require almost no systemic treatment and no morbidity and no death. Some group II and most group III (ie, with multi-system disease) require systemic therapy, but are generally effective. Group IV young and multi-system patients have morbidity and mortality rates as high as 20%. Although recurrence is common, almost all patients with good outcomes can eventually discontinue treatment. In adult patients, the process of chronic disease can also be presented.
Severe patients should be hospitalized and given maximum doses of antibiotics to maintain airway patency, nutritional support (including high-energy nutrition), blood products, skin care, physiotherapy and necessary medical care. Strict hygiene measures can effectively reduce hearing, skin and gum damage. Debridement can even remove severely damaged gingival tissue to limit oral lesions. A selenium-containing shampoo can be used for seborrheic dermatitis (2 times a week). If the shampoo is not effective, a small amount of corticosteroid can be used locally to control small lesions in a short period of time. Most patients with diabetes insipidus or other symptoms of hypopituitarism need to be supplemented with hormone therapy.
Topical treatment (surgical and radiotherapy)
After a complete assessment, topical therapy can be used in patients with individual bone violations and in patients with multiple sites in some cases. The patient's lesions are superficial and can be surgically scraped off in accessible and non-hazardous areas. However, excessive orthopedic and plastic surgery and injury function should be avoided during surgery. Local radiotherapy (using high-voltage devices) often gives skeletal malformations, vision loss due to eyeball protrusion, pathological fractures, spinal compression and spinal cord injury or severe pain, or generalized lymphadenopathy.
Chemotherapy
Low-risk children > 2 years old, with a single system of disease, or a bone or multiple site bone damage, often given local treatment, no systemic treatment. However, ineffective treatment or elevated ESR may indicate the presence of severe systemic disease. Low-risk patients > 2 years old with multiple system diseases, but did not invade the blood system, liver, lung or spleen, can be given chemotherapy, often have a sustained effect, but most <2 years old, the aforementioned organ system is violated, especially dysfunctional The child died despite chemotherapy.
The above content is for reference only, please consult the relevant physician or relevant medical institution if necessary.
prevention
In patients with systemic Langerhans cell histiocytosis, chronic disability, such as cosmetic or functional orthopedic and cutaneous lesions and neurotoxicity, and mood swings that may be caused by disease and treatment should be monitored. .
简体中文