Langerhans cell histiocytosis
Introduction
Introduction to Langerhans cell histiocytosis Langerhans 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 Acid granuloma (eosinphilic granulomaofbone, EGB), the cause is unknown, in recent years, studies have been found to be associated with immune regulation disorders in vivo. basic knowledge The proportion of illness: 0.0003% Susceptible people: children Mode of infection: non-infectious complication:
Cause
Langerhans cell histiocytosis
The etiology is still unclear. Although its genetic characteristics are still unclear, it has a certain familial nature. The incidence among siblings is much higher than that of ordinary children. It is also considered to be a tumor.
Prevention
Langerhans cell histiocytosis 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. .
Complication
Langerhans cell histiocytosis complications 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 bulge, and the optic nerve or the eye muscle is violated, resulting in decreased vision or strabismus.
The most common sites of bone invasion are flat bones (such as cranium, ribs, pelvis and scapula). Long bones and lumbar vertebrae, the humerus is less affected. The lesions on the long bone resemble Ew-ing sarcoma, osteosarcoma and osteomyelitis. Head, knee, foot or cervical vertebrae are rare. Parents often state that the child has premature teething, which is actually due to gingival recession and immature dentin exposure.
Gastrointestinal symptoms, diabetes insipidus and enlarged thyroid gland.
Symptom
Langerhans cell histiocytosis symptoms common symptoms hepatosplenomegaly papules liver function abnormalities dysphagia ascites urinary diarrhea diarrhea soft tissue swelling intracranial pressure increased liver fibrosis
The onset of this disease is different, 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 Patients with bone and skin lesions are extensive. In this case, patients have multiple systems, and multiple organs are 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, and the ear begins to be a maculopapular rash, which quickly oozes (similar to eczema). Can be seborrheic dermatitis), may be associated with bleeding, and then scarring, de-smooth, and finally left with pigmentation leukoplakia, leukoplakia is not easy to dissipate for a long time, rash can exist at the same time or a batch of retreats, and often in the case of rash There is fever, chronic rash can be scattered throughout the body, initially paleomenopausal pigmented papules or sputum nodules, when the depression subsides the central depression, some are dark brown, very similar to scab, and finally local thinning Slightly concave, slightly lustrous or a little desquamation, 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 almost common in all LCH patients, single bone lesions more bone lesions, mainly manifested as osteolytic lesions, the most common skull disease, lower limb bone, ribs, pelvis and spine times The jaw lesions are also quite common. On the X-ray films, the bones are irregularly deformed. The skull damage changes from the worm-like lesion to the huge defect or the chisel-like change. The shape is irregular and round or oval. Shape defect, marginal jagged, blurred border of initial or progressive lesions, and common intracranial pressure, bone fracture or traffic hydrocephalus, may be associated with headache, but in the recovery period, the bone is gradually clear and appears Hardened zone, uneven bone density, bone defect gradually smaller, and finally completely repaired without leaving traces, X-ray changes of other flat bones: visible rib swelling, thickening, bone thinning or cystic changes, and then bone absorption Atrophy, thinning, vertebral destruction can become flat vertebrae, but the intervertebral space is not narrow, rarely angular deformity, vertebral arch destruction is prone to spinal nerve compression, a few have paravertebral soft tissue swelling, jaw lesions can be Now the mandibular alveolar type and shape two kinds.
(3) Lymph node LCH lymph node lesions 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 involved Osteolytic damage or skin lesions; 3 as part of systemic diffuse LCH, often involving the isolated lymph nodes in the neck or groin, most patients have no fever, a few only swollen lymph node pain, simple lymph node involvement, 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 it from diffuse bacterial ear infection. 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) In normal bone marrow, there is generally 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) Lung lung lesions may be part of systemic lesions, or may exist alone, the so-called primary lung LCH, lung lesions may occur at any age, but more common in infants in childhood, the performance is not heavy Such as breathing difficulties, hypoxia and lung compliance changes, severe cases may appear pneumothorax, subcutaneous emphysema, prone to respiratory failure and death, lung 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, and thus can develop into 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 more related to the site of invasion. The most common intestines in the small intestine and ileum are vomiting, diarrhea and malabsorption, which can cause stagnation in children for a long time.
(11) Central nervous system LCH with central nervous system involvement is not uncommon, the most common site of involvement is the thalamus-pituitary posterior lobe, diffuse LCH may have brain substantial lesions, most patients with neurological symptoms appear in other parts of LCH After a few years, there are common ataxia, dysarthria, nystagmus, hyperreflexia, rotational dyskinesia, difficulty swallowing, blurred vision, etc. Diabetes insipidus caused by thalamus and/or pituitary granulomatosis may precede Brain symptoms or occurring simultaneously with or after brain symptoms can also 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 Presenting purplish rash, invading the scalp, ear shell, abdomen and wrinkles of the neck and face. Skin damage can become a gateway to microbial invasion, leading to sepsis. Common ear pus, lymphadenopathy, hepatosplenomegaly. Severe Hepatic dysfunction with hypoproteinemia and decreased synthesis of coagulation factors, anorexia, irritability, weight loss, and obvious respiratory symptoms (such as cough, shortness of breath, pneumothorax), severe anemia, and sometimes neutrophils Cell reduction. Thrombocytopenia is often a precursor to death. Because of these manifestations, young patients are often misdiagnosed or missed.
Examine
Langerhans cell histiocytosis examination
(1) The blood-like system of diffuse LCH often has moderate to severe anemia, reticulocytes and white blood cells can be slightly elevated, platelets are reduced, and in a few cases, white blood cells can be reduced.
(2) Bone marrow examination Most patients with LCH have normal bone marrow hyperplasia, a few may be active or reduced hyperplasia, and a few LCH have bone marrow invasion, showing anemia and thrombocytopenia, so this test is only done when abnormal peripheral blood is found.
(3) ESR in some cases of ESR can be seen.
(4) Some cases of liver and kidney function have abnormal liver function and suggest poor prognosis, including SGOT, SGPT, alkaline phosphatase and bilirubin increase, plasma protein decrease, prothrombin time prolongation, fibrinogen content and partial coagulation Live enzyme production test is reduced, renal function includes urine osmotic pressure, and those with diabetes insipidus should measure urine specific gravity and do water restriction test.
(5) X-ray examination X-ray examination of the lungs is mostly net-like or reticular shadow, the edges of the particles are blurred, not arranged according to the tracheal branches, and some lung fields are frosted glass, but the lung transmittance increases in most cases. Common small cystic emphysema, severe pulmonary veins, may be associated with interstitial emphysema, mediastinal emphysema, subcutaneous emphysema or pneumothorax, many patients may be associated with pneumonia, at this time more likely to occur cystic changes, pneumonia subsided After the cystic change can disappear, but the granule change is more obvious, the chronic disease can occur pulmonary fibrosis, bone X-ray changes see above.
(6) Blood gas analysis If there is obvious hypoxemia, it indicates impaired lung function.
(7) Pulmonary function test Patients with severe lung disease may have different degrees of pulmonary dysfunction, which may indicate poor prognosis.
(8) Immunological examination In view of the fact that this syndrome often involves immune regulation dysfunction, such as the abnormal number of T lymphocyte subsets and the ratio of T-assisted to T-suppressor cells, the conditioned unit should perform phenotypic analysis of T subgroups. , lymphoblastic transformation test and serum immunoglobulin quantification.
(9) Those with new rash should be rashed. If they can do skin biopsy on the rash site, it is more reliable. If there is swollen lymph node, lymph node biopsy can be done. If there is bone destruction, it can be swollen. In addition, at the same time, the scraping material is sent to the pathology, or a thick needle is used for puncture drainage at the bone destruction site, and the smear is sent for examination.
(10) Immunohistochemical staining As mentioned above, Langerhans cells have been found to have an immunophenotype of CDla in recent years, and anti-CDla monoclonal antibody is specifically positive for immunohistochemical staining, and the following four enzymes may also be present. Positive reactions, namely S-100 neuroprotein, -D-mannosidase, ATPase and peanut agglutinin.
Diagnosis
Diagnosis and differentiation of Langerhans cell histiocytosis
diagnosis
The diagnosis method is based on clinical, X-ray and pathological examination results, that is, the pathological examination of the lesions in the hair follicle can be confirmed by tissue infiltration, the key to the diagnosis of this disease is the pathological examination of the Langerhans cell tissue infiltration, Therefore, biopsy should be done as much as possible.
Differential diagnosis
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 do bone marrow, histopathology and other tests.
