Histiocytosis

Introduction Langerhans cell histiocytosis or Langerhans' cell disease, also known as Histicytosis X, is a rare group of Langerhans cells. Hyperplastic disease. The etiology and pathogenesis of this disease are still unclear. Some people think it is a reactive disease, not a true tumor. Some people think that this disease is caused by abnormal immune system, but more positive cells can be seen by PCNA immunohistochemistry, and the common mitotic figures in lesions are considered to be proliferative diseases, which may be neoplastic hyperplasia.

Cause

The disease is mainly composed of proliferating Langerhans cells, eosinophils and other inflammatory cells. Multinucleated giant cells and necrotic tissues are also seen. There is often a layer of connective tissue that is not invaded under the epithelium.

Langerhans cells are mostly follicular and flaky, with large cell volume, abundant cytoplasm, weak eosinophilic, nucleus round, elliptical or lobulated, with obvious nucleoli and nuclear nucleus often appearing in the nucleus.

In Han Xueke's disease, a large number of tissue cells that phagocytose lipids are called foam cells, which are more common around the necrotic area, and less eosinophils.

In Lexue disease, Langerhans cells proliferated in a large amount, and there were many heteromorphic nuclei and mitotic phases, but no foam cells.

OKT6, S-100 protein (fig. 6) can be used as an immunohistochemical marker for Langerhans cells. Both cytoplasm and nucleus are strongly positive. Under electron microscopy, Langerhans cells have low cytoplasmic electron density, also known as bright cells. The nuclear membrane of the nucleus forms a defect, and the characteristic Languehans particles, also known as Birbeck particles, are rod-shaped. , bounded membrane, particle length 0.2-1um, width 40nm. The surface has regularly spaced horizontal stripes, or one end is swollen into a tennis racket shape.

an examination

Related inspection

Histochemical staining

This group of diseases include eosinophilic granuloma, Han Xueke disease and Lexue disease. Due to different ages of onset, lesions and Langerhans cell proliferation, different symptoms, course and prognosis occur.

Eosinophilic granuloma

Chronic limitations, occur in children and adolescents, adults can also occur, more common in men, the disease occurs in the bone, the lesions can be isolated or multiple. The skull, mandible, and ribs are the most frequently invaded sites. Individual cases can involve the lungs, usually with single bone damage. Oral lesions often invade the jaw and gums, the most common in the lower jaw, patients often see the diagnosis of gum swelling, ulcers, jaw swelling, pain and loose teeth. Check the gums are slightly yellow swelling but no pus, the texture is soft, the contact is easy to hemorrhage, the gingival margin can be worm-like destruction, the nipple erosion disappears, the X-ray shows osteolytic destruction or piercing damage, and the jaw center is destroyed. The main or the alveolar bone destruction is the main damage, and extensive damage can also occur. Clinically misdiagnosed as malignant mass, necrotizing gingivitis, periodontal disease, osteomyelitis, jaw tumor or cyst. Single bone lesions generally have a good prognosis. Multiple lesions are prone to recurrence after treatment.

Hand-Schuller-Christian disease

For the chronic disseminated type, it is easy to occur in children over 3 years old, males are more common, generally slow onset, longer course, often multi-bone lesions and extra-osseous lesions. The disease can have three major characteristics: skull disease, exophthalmos and diabetes insipidus. Invasion of the tibia can cause the eyeball to protrude. When the lesion is located in the sella, it can invade the pituitary gland and cause diabetes insipidus. Often invade other organs such as the lungs. The patient may have a fever. The X-ray shows that the skull has irregular penetrating damage, and the jaw has a transmissive area of bone destruction. The patient can be cured, but often sequelae such as diabetes insipidus or developmental delay. It is generally believed that the earlier the onset age, the worse the prognosis.

Letterer-Siwe disease is an acute disseminated type, and the incidence is mostly infants and young children under 3 years old. The course of illness is acute or subacute, which is the most serious type. There can be a wide range of internal organs involved. Clinically, there may be systemic symptoms such as repeated or persistent high fever, rash, anemia, hepatosplenomegaly, and diarrhea. The deciduous teeth are loose, the tongue is invaded to form a giant tongue, and the cervical lymph nodes are often swollen. X-ray shows that the skull and long bone have obvious bone destruction, and the jaw bone can be a clearly defined osteolytic change. This type of disease is heavier and the prognosis is poor.

Differential diagnosis

Increased eosinophils: Eosinophilia is called eosinophilia when the absolute value of eosinophils in the surrounding blood is greater than 400-450/mm3. It is often associated with a variety of diseases in the clinic, especially parasitic infections and allergic diseases. The normal value of eosinophil count is 50-250/mm3, which is often reduced in the morning when the level of endogenous glucocorticoids is elevated.

Hepatic stellate cell hyperplasia: There are two kinds of physiological hyperplasia and pathological hyperplasia. It occurs because it meets physiological needs and its degree does not exceed the normal limit. It is called physiological hyperplasia. After some tissue damage in the human body, the compensatory hyperplasia of the rest is also physiological hyperplasia. Proliferation beyond the normal range caused by pathological causes is called pathological hyperplasia. Physiological hyperplasia is divided into compensatory hyperplasia and endocrine hyperplasia.

The mechanism of physiological hyperplasia is not fully understood. Pathological hyperplasia is mostly related to hormone stimulation; tumor hyperplasia caused by tumor cell enlargement also belongs to the pathological hyperplasia range. However, it is customary for narrowly defined hyperplasia to refer to benign non-neoplastic lesions. Although hyperplasia and hypertrophy are two different concepts, in fact, hyperplasia often has hypertrophy. Compensatory hyperplasia after hyperplasia or injury due to adaptation to physiological needs can enhance or compensate for local metabolic and functional changes, which is beneficial to the body. But pathological hyperplasia is often harmful to the body. Benign prostatic hyperplasia (BPH) is the most common disease in older men and the most common benign tumor in men. BPH is often associated with a range of irritating symptoms and lower urinary tract symptoms (LUTS), which have a negative impact on the patient's quality of life. Untreated BPH can cause some complications, the most common of which are acute urinary retention (AUR), gross hematuria, recurrent urinary tract infections, urinary tract obstruction, bladder stones, and rare renal failure.

Increased cerebrospinal fluid leukocytes: refers to the number of cells in the cerebrospinal fluid counted to increase the number of white blood cells. Normal cerebrospinal fluid does not contain red blood cells, no white blood cells or very small amounts of white blood cells. Many neurological diseases can cause changes in the cellular components of the blood and cerebrospinal fluid, especially the abnormalities of the cerebrospinal fluid cell components, but often lack specificity, so the comprehensive analysis of the test results should be carried out.

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