Type I immunoblastic lymphadenopathy
Introduction
Introduction to type I immunoblastic lymphadenopathy Immunoblastic lymphodenopathy (immunoblastic lymphodenopathy) is a hyperimmune disease caused by hyperproliferation of lymphoblasts associated with autoimmunity. It is also called vascular immunoblastic lymphadenopathy, vascular immunoblastic lymph node. The disease is accompanied by abnormal proteinemia and diffuse plasma cell sarcoma. basic knowledge The proportion of illness: 0.005%-0.008% Susceptible people: no specific population Mode of infection: non-infectious Complications: nodular erythema, angioedema, hepatomegaly, myasthenia gravis, amyloidosis
Cause
The cause of type I immunoblastic lymphadenopathy
(1) Causes of the disease
The cause of this disease is unknown. Some people think that this disease is related to viral infection. Some patients have detected the DNA of Epstein-Barr virus in the lymph nodes (PCR method). Some people think that due to the infection of the subtype of artificial cell lymphoma virus, the immune function of the body is abnormal. Lead to this disease, because the patient's lymph node biopsy and AIDS histology are similar.
(two) pathogenesis
The pathogenesis of this disease is still unclear. The T cell subset analysis of the patient found that the disease is T cell lesions. In some cases, the number of inhibitory T cells (TS) increased, and some of them mainly increased T cells (TH). There are also two, these T cells may produce B cell stimulating factors, promote B cell proliferation, and secrete immunoglobulin.
1. Abnormal increase in immunoglobulin production in the body, the age of the patient is large, prone to infection, often accompanied by autoimmune hemolytic anemia, indicating T cell and B cell dysfunction, Knocht study found that 75 cases of immunoblastic lymph nodes Among the patients, 44 had T cell receptor B monoclonal rearrangement, 19 had T cell receptor B and immunoglobulin gene germline characteristics, and 11 T cell receptor B and immunoglobulin gene rearrangements. One case was an immunoglobulin gene rearrangement.
Cellular immunity is the main mechanism causing damage to autoimmune tissues. Many organ systems are involved in this disease, but lymph nodes are dominant and have characteristic lesions. The distribution of this lesion may be due to cytotoxic T cells (TC cells). Systemic antigens or expression of immune challenge against antigens present in lymphoid organs or other tissues.
2. Pathology: All or most of the lymph node structure is destroyed, the germinal center disappears, but some remains visible, and all or part of the envelope and extracapsular tissue are infiltrated by plasmacytoid cells.
(1) vascular hyperplasia: the para-cortical area of the lymph nodes, the medullary area has obvious post-capillary venous hyperplasia, part of which is branched, with PAS-positive amorphous substance deposition in the blood vessel wall, endothelial cells proliferating and swelling, and some blood vessels The cavity seems to be occluded.
(2) Immunoreactive cell infiltration and proliferation: These cells are immunoblasts, plasmacytoid immunoblasts, plasma cells and epithelioid cells. The immunoblasts are large in size, 15 to 25 m, polygonal, and cytoplasmic. More, HE staining is double staining or basophilic, methyl green piperazine staining is positive (red), Giemsa staining is basophilic, sky blue, large, round or oval, slightly biased The nuclear membrane is thick and the chromatin is finely meshed and loose. There are 1 to 3 basophilic round nucleoli, arranged in the nucleus or close to the nuclear membrane. Occasionally, the binuclear nucleus can be seen.
(3) PAS and acid mucopolysaccharide staining were positive in the deposition of amorphous substances between cells.
Liver, spleen, bone marrow and skin often have similar changes, but not as typical as lymph nodes, the appearance of lung tissue is diffuse nodular, microscopic alveolar septum and interstitial diffuse lymphocytes, plasma cells and immunoblasts infiltration With a small amount of fibrosis, alveolar shrinkage, hyperplastic epithelial lining, increased number of macrophages, bronchial stenosis due to intimal infiltration, immunofluorescence staining showed that the entire alveolar wall is specific for intracellular IgG and IgM deposition .
Prevention
Type I immunoblastic lymphadenopathy prevention
Every effort to avoid provoking the body's immune response is the key to preventing autoimmune diseases.
1. Eliminate and reduce or avoid the disease factors, improve the living environment, develop good living habits, prevent infection, pay attention to food hygiene, and rational diet.
2. Insist on exercising, increase the body's ability to resist disease, do not over-fatigue, excessive consumption, quit smoking and alcohol.
3. Early detection and early diagnosis and treatment, establish confidence in the fight against disease, adhere to treatment, and maintain optimism.
4. Prevention of viral infection and prevention of streptococcal infection are important links in autoimmune rheumatic diseases and co-morbidity.
Complication
Type I immunoblastic lymphadenopathy complications Complications nodular erythema angioedema hepatomegaly myasthenia gravis amyloidosis
1. Can be complicated by painless nodular erythema, eyelid edema, angioedema, penile and scrotal edema.
2. Can also be complicated by hepatomegaly; exudative pleurisy; multiple radiculitis, muscle weakness and ENT lesions, autoimmune thyroiditis, cutaneous vasculitis, amyloidosis, mixed cryoglobulinemia.
Symptom
Type I immunoblastic lymphadenopathy Symptoms Common symptoms Irregular heat and high heat relaxation Heat spotted papules Excessive heat breathing difficulty Chest pain Itching amyloidosis Penis and scrotal edema
1. Fever: 1/3 of patients may have different degrees of fever, mostly moderate or high fever, heat type is mostly relaxation heat, but also for heat retention, irregular heat, intermittent or continuous, after fever More accompanied by a lot of sweating.
2. Lymph node enlargement: Found in all patients, beginning with localized lymphadenopathy, the most common site is the neck or clavicle, followed by the submandibular or axillary fossa, which can later develop into a systemic lymphadenopathy, superficial lymph nodes and deep Lymph nodes can be involved, high fever can occur before or during swollen lymph nodes. After the lymph nodes enlarge, the body temperature returns to normal. The swollen lymph nodes are soft and movable, and the diameter generally does not exceed 3 to 4 cm.
3. Skin lesions: About half of the patients have skin lesions, which may occur before or at the same time as other symptoms and signs. Patients with skin lesions have a history of useful medications, with penicillin as the most, followed by sulfa drugs or other Drugs such as aspirin, skin lesions mostly manifest as pruritic rash or measles-like rash, a small number of patients can appear small painless nodular erythema, mainly distributed in the face and trunk, other skin lesions have facial and eyelid edema, blood vessels Neuropathic edema, penis and scrotal edema.
4. Hepatosplenomegaly: 3/4 patients have large liver, usually 3 to 4 cm under the ribs, no tenderness, smooth surface, about half of the patients have mild to moderate splenomegaly, and more with hepatomegaly.
5. Lung and pleura: manifested as varying degrees of cough, cough, chest pain and difficulty breathing, respiratory symptoms can be caused by the primary disease, can also be caused by secondary infection, pleural involvement manifested as chest pain and exudative pleurisy.
6. Others: A small number of patients may have multiple radiculitis, muscle weakness and ENT lesions. Because this disease is an autoimmune disease, it can overlap with many other autoimmune diseases, the most common of which is condensin. Hemolytic anemia, Coomb test positive hemolytic anemia; followed by autoimmune thyroiditis, which can cause mucinous edema; also can be combined with cutaneous vasculitis, amyloidosis, mixed cryoglobulinemia.
Examine
Examination of type I immunoblastic lymphadenopathy
1. Blood routine and erythrocyte sedimentation rate: Most patients have moderate or severe anemia, most of them are hemolytic anemia, white blood cells are often increased, accompanied by eosinophilia, and about one-third of patients have absolute lymphocyte counts less than 1 × 109 / L Sometimes platelets are reduced and erythrocyte sedimentation rate is increased.
2. Urine routine: Proteinuria, hematuria and tubular urine may occur in patients with mixed cryoglobulinemia or amyloidosis.
3. Biochemical examination: 1/4 of patients have elevated serum LDH, some patients have elevated lysozyme, albumin is reduced, and globulin is elevated.
4. Immunological examination: During the active period, gamma globulin increased, and half of them were multi-strain high gamma globulinemia, mainly IgG, IgM increased, Coomb test, cold agglutinin, rheumatoid factor positive, E- In the rosette test, the lymphocyte transformation test and the DNCB test were mostly reduced.
5. X-ray examination: The chest radiograph shows a moderate enlargement of the mediastinum and hilar lymph nodes. It can also show the infiltration of the lung parenchyma and the diffuse, reticular or nodular section of the lungs. The lesions can be flaky. Or patchy shadows, a small number of patients may have pleural effusion (unilateral or bilateral).
6. Lymphatic angiography: more showing visceral lymph nodes, accompanied by a foamy appearance.
Diagnosis
Diagnosis and differentiation of type I immunoblastic lymphadenopathy
Diagnostic criteria
In clinically, middle-aged and elderly patients have acute onset, with systemic lymphadenopathy, hepatosplenomegaly, sometimes accompanied by fever and rash, and with erythrocyte sedimentation rate, gamma-globulin is significantly elevated, that is, the disease should be highly suspected. Lymph node biopsy should be performed. The pathological criteria proposed by Luks are as follows:
1 small blood vessels showed branching hyperplasia, thickened wall, positive PAS response.
2 immunoblasts and plasma cells proliferate.
3 lymphocyte reduction and intercellular eosinophilic deposition, the above changes are found in the lymph nodes of the whole lesion, specific to the disease, the above three typical and obvious lesions, can be diagnosed as immunoblastic lymphadenopathy.
If the lesion is not typical, Luks has proposed the following two additional conditions:
1 lesions affect the entire lymph node, and scar formation and lymphatic follicles disappear.
2 As far as the whole lesion is concerned, it has the characteristic of lymphopenia.
Differential diagnosis:
1. Malignant lymphoma: Especially Hodgkin's disease, similar to the clinical manifestations of this disease, lymph node biopsy with Sternberg-Reed cells, no immune cell proliferation, and immunolymphocytic disease with PAS staining positive, It is the main point of identification for the proliferation of branched small blood vessels with thickened wall and the interstitial PAS-positive eosinophilic amorphous sedimentation triad.
2. Drug reaction: The drug reaction and the clinical symptoms and signs of the disease can be exactly the same, but the drug reaction is generally not significant high gamma globulinemia, anemia is also rare, lymph node biopsy also has no vascular proliferation and a variety of immune response cells Hyperplasia.
3. Giant lymph node hyperplasia: also known as lymph node hamartoma, is a special type of lymph node inflammatory reactive hyperplasia, clinical features similar to this disease, but the age of onset is light, the mass is common in the chest, especially in the mediastinum See, the diameter can reach more than 10cm, the pathological mainly is plasma cell proliferation, the lymph node structure is normal or only partially destroyed, the proliferating blood vessels are concentrated in the follicular germinal center, and the blood vessels are transparently denatured.
4. Idiopathic hypergamma-globulinemia: mainly seen in young men, the clinical symptoms are very similar to the disease, there is no M protein in the serum, the pathological changes of lymph node biopsy are obvious proliferation of plasma cells, and there may be mild hyperplasia of hair growth. But the lymph node structure is basically intact.
5. Oehlert chronic pluripotent immune hyperplasia syndrome: its clinical and pathological similarities with this disease, in the pathology, there are necrotizing vasculitis around the capsule and capsule, small movement of lymph nodes, intravenous Inflammatory changes can sometimes affect the medulla and paracortical areas, and the middle and small arteries outside the lymph nodes are diffuse embolism and segmental necrosis.
