Histiocytosis
Introduction
Introduction to histiocytosis Histiocytosis (reticulocyte proliferation or reticuloendotheliosis) refers to a group of diseases in which histiocytic or pseudotumoral hyperplasia of histiocytes can not be classified as malignant lymphoma, generally including bone eosinophilic granuloma, Xanthomatosis and infant malignant reticulocyte proliferation. The three should be considered as a chronic and acute process of the same lesion, with transitional and intermediate types in between. Due to different stages of development, the lesion may be single or multiple, and may be located within or outside the bone. Tissue cell proliferative disorders, including all variants thereof, are a systemic disorder. These kinds of performances are not so much a disease, but rather a syndrome. Although they are different from each other, the boundaries are not very clear. Many authors at home and abroad have reported that eosinophilic granuloma has turned into a yellow tumor. Histiocytosis can be divided into single and multiple. The former is bone eosinophilic granuloma, and the latter is divided into a chronic type (with or without yellow lipoma), and an acute type (children with malignant reticulocyte proliferation) with an intermediate type (transition type). Some authors divide tissue proliferative disorders into localized and diffuse types, the former occurring in older children and adults, and the latter more common in infants. Eosinophilic granuloma is more common in male patients. The common age of onset is 1 to 15 years old, especially between 5 and 10 years old. However, it can also occur in adulthood. Eosinophilic granuloma usually occurs in the ribs and mandibles in those over 20 years old, and occurs in the skull at the age of 20 years. Occurring in the spine is rare. basic knowledge The proportion of illness: 0.002% Susceptible people: no specific population Mode of infection: non-infectious Complications: obstructive emphysema
Cause
Causes of histiocytosis
Hyperplasia of reticular cells (40%):
The main pathological changes of the eosinophilic granuloma of the vertebrae are the proliferation of extensive reticular tissue cells, with varying eosinophils, various inflammatory cells, and multinucleated giant cells, some of which can be transformed into foam cells. And hemocytic cells containing hemosiderin or cell residual, small blood vessels may have cellulose-like necrosis, advanced fibrosis, tissue nuclei large, oval, gear-shaped or kidney-shaped, often aggregated into a pile or strip.
Histiocytosis (30%):
The main lesion of xanthomatosis is histiocytosis, which can be seen in reticulocytes, foam cells and Touton-type giant cells. Foam cells are also called yellow cells, have acicular cholesterol crystals, eosinophils are not obvious, and some parts have granulation tissue. Poor differentiation, there may be a small number of nuclear division, tissue cells are replaced by fibroblasts, forming connective tissue, some between foam cells, instead of granulation tissue.
In the same patient, the same lesion at different times, the pathology can be different, such as the beginning of the eosinophilic tissue cell granuloma, but after a few years can be transformed into a foam cell-based lipid-containing granuloma Therefore, it is not possible to confirm the diagnosis of xanthomatosis based solely on the presence or absence of foam cells. Even in infant reticulocyte proliferation, foam cells can be seen occasionally, but it is rare because the lesions develop less than soon.
Tissue cell proliferation (20%):
The lung begins to be interstitial granuloma, and then the tissue cells invade the small arteries and the distal end of the bronchioles, obstructive emphysema occurs, cysts and vesicles are formed and rupture, and there are many eosinophils in the interstitial granuloma. , neutrophils and lymphocytes infiltrate.
Prevention
Tissue cell proliferative prevention
There are no special preventive measures for this disease. Early detection and early treatment are the key to prevent this disease.
Complication
Histiocytosis complications Complications obstructive emphysema
Accompanied by skull, rib lesions, local lumps, tenderness, obstructive emphysema, pathological fractures and deformities; with femoral lesions, many claudication, primary eosinophilic granuloma of the vertebrae can oppress the spinal cord Causes embarrassment.
Symptom
Symptoms of histiocytosis common symptoms, loss of appetite, granuloma, urinary disintegration, lymphadenopathy, bacterial infection, hepatosplenomegaly
Single or multiple eosinophilic granuloma with fewer systemic symptoms, the initial symptoms are pain, accompanied by skull, rib lesions, local lumps, tenderness, pathological fractures and deformities; with femoral lesions, many limps Multiple lesions are generally found in succession. Patients may have systemic symptoms such as fever, loss of appetite, weight loss, and may also be associated with hilar lesions. Primary eosinophilic granuloma of the vertebra may cause compression of the spinal cord, and xanthomatosis usually has Three typical signs, namely skull defect, eyeball protrusion and diabetes insipidus, are mainly multiple skull lesions. The forehead and parietal bone are the most common, and the occipital and occipital bones are the second. They usually have only one, and rarely have three typical signs at the same time. In addition to the above clinical changes, there may also be developmental disorders, liver, splenomegaly, rash, pigmentation and gum ulcers. The gums are gradually surrounded by granulation tissue, and the cysts are surrounded by single cystic or polycystic destruction. Chronic gingival ulcers that have loosened teeth and clinically unhealed may be one of the early signs of inflammation.
The incidence of malignant reticulocyte proliferation in infants is under 3 years old, clinical manifestations of fever, otitis media, recurrent bacterial infection, anemia and hemorrhage, in addition, hepatosplenomegaly, extensive painless peripheral lymphadenopathy, dissolution Bone changes and seborrheic eczema.
Examine
Examination of histiocytosis
Eosinophilic granuloma is located in the skull. It is usually characterized by osteolytic destruction of the inner and outer plates. The bones are excavated and there is no hardening or periosteal reaction around. It is called a map skull.
Due to the pathological fracture of the spine, the vertebral body is partially compressed into a wedge-shaped vertebrae. After compression, only the upper and lower marginal cortex is left. The height of the vertebral body is reduced, but it does not spread in the horizontal direction. The upper and lower intervertebral spaces remain unchanged. Spontaneous fusion occurs, occasionally a soft tissue fusiform mass can appear in the paravertebral, the density of the affected vertebra is increased, and it is disc-shaped. Therefore, the typical X-ray manifestation of the eosinophilic granuloma of the vertebra is generally described as a flat vertebra, a cranial vertebral cartilage disease.
For X-ray films showing flat vertebrae, caution should be taken when diagnosing bone eosinophilic granuloma, which is suitable for the following criteria:
(1) Only one vertebral body is involved.
(2) The upper and lower vertebrae of the affected vertebra are normal.
(3) The density of the compressed vertebral body is consistent, the X-ray of the lung appears to be bronchitis or bronchiectasis, the diffuse reticular nodules infiltrate in the lung, and the densely strip-shaped radiation from the hilum to the periphery, and there are diffuse claws. In severe cases, it is honeycomb-shaped, and the hilar is enlarged and dense.
Diagnosis
Diagnosis and differentiation of histiocytosis
Diagnosis can be based on medical history, clinical symptoms, and laboratory tests.
Need to be differentiated from malignant lymphoma:
1. Clinically, typical patients have painless progressive superficial lymphadenopathy; atypical patients are mainly with deep lymphadenopathy, fever is irregular, persistent or periodic fever; often accompanied by lymph nodes Other parts, such as tonsils, nasopharynx, gastrointestinal tract, bones, skin, kidneys or nerves, and other symptoms, most patients have varying degrees of systemic symptoms, such as fatigue, body weight loss, night sweats, spontaneous sweating, Itchy skin, weight loss, progressive anemia, etc., swollen lymph nodes, quality such as hard rubber, early non-adhesive, late adhesions into blocks, mostly occur in both sides of the neck, followed by underarm and groin, deep lymph nodes can also be swollen Large, such as mediastinal and thoracic lymphoid tissue involved, can cause mediastinal tumor syndrome, pulmonary infiltration, atelectasis, secondary lung infection and pleural effusion, etc., some patients with hepatosplenomegaly as the first symptom, and some are Abdominal pain, abdominal mass is the main symptom. With the primary and invasive parts of malignant lymphoma, there are corresponding symptoms and signs.
2. Pathological examination and early swollen lymph node biopsy, pathological biopsy can generally be diagnosed by typing.
3. Bone marrow examination is conducive to diagnosis and staging.
4. X-ray, CT, M-lay, ultrasound and 67Ga scan, lower limb lymphography, radionuclide bone scan, liver biopsy, etc., contribute to clinical staging.
