Pediatric angioimmunoblastic lymphadenopathy
Introduction
Introduction to pediatric vascular immunoblastic lymphadenopathy Angioimmunoblast lymphadenopathy (angioimmunoblastlymphadenopathy), also known as angioimmunoblastic lymphopathy with abnormal protein syndrome, is an autoimmune disease. There are many kinds of tissue and organ damage in this disease. basic knowledge The proportion of illness: 0.005% Susceptible people: children Mode of infection: non-infectious Complications: anemia, lymphoma
Cause
Etiology of pediatric vascular immunoblastic lymphadenopathy
(1) Causes of the disease
This disease is generally considered to be an autoimmune disease.
(two) pathogenesis
This disease is due to defects in T cell regulatory function, which can be transformed into lymphoma, such as lymph nodes, liver, spleen, bone marrow, skin and lungs, so that B cells are non-neoplastic abnormal proliferation and converted into immune mother cells, resulting in excessive immunity. Globulin, the lesion is between benign immunoblasts and immunoblastic lymphoma, and some patients may be converted to lymphoma.
Prevention
Pediatric vascular immunoblastic lymphadenopathy prevention
The cause is not clear and there are no systemic precautions.
Complication
Pediatric vascular immunoblastic lymphadenopathy complications Complications anemia lymphoma
Can cause anemia, and some patients develop lymphoma.
Symptom
Pediatric Angioimmunoblastic Lymph Node Disease Symptoms Common Symptoms Lymph node enlargement, high fever, lack of appetite, loss of weight, hepatosplenomegaly, large papules, joint pain
1. The general performance of more than 70% has irregular or persistent fever, with high fever, accompanied by excessive sweating, anemia, joint pain, weight loss, fatigue, loss of appetite.
2. Lymph node enlargement Systemic or localized lymphadenopathy is another feature that can involve the hilar and mediastinal lymph nodes and/or abdominal lymph nodes, occasionally tenderness.
3. Rash rash can be the first symptom, which can be red maculopapular rash, urticaria, purpura.
4. More than 60% of hepatosplenomegaly has mild to moderate hepatosplenomegaly.
Examine
Examination of pediatric vascular immunoblastic lymphadenopathy
1. Blood examination has different degrees of anemia, white blood cells increase, sometimes leukemia-like reactions, neutrophils and eosinophils increase, platelets can be reduced, and 1/4 of cases peripheral blood can be seen in immune cells.
2. Bone marrow examination is markedly active, and the immune mother cells can account for 3% to 46%. There are often plasma cells and reticular cells, and eosinophils are high.
Immune mother cells: about 15 ~ 25m in size, oval or polygonal, cytoplasmic amount, nucleus is oval, slightly eccentric, thick nuclear membrane, chromatin is fine mesh, there is a large Or 2 to 3 small nucleoli near nuclear membranes, sometimes dinuclear or multinuclear, Sudan black, non-specific esterase staining negative, PAS partial positive, IgG Fc receptor and complement receptor on the surface of the cell membrane.
3. Lymph node pathological examination must be done multiple times of lymph node biopsy in different parts, as a basis for diagnosis, the characteristic changes are:
(1) The immune-active cells mainly composed of immunoblasts are infiltrated, and the normal lymph node structure is destroyed or disappeared.
(2) Significant hyperplasia of small blood vessels, most prominent in the paracortical area.
(3) The eosinophilic amorphous substance is deposited between the infiltrating cells.
4. Most of the immunological examinations showed an increase in clone Ig, and some were increased in individual plants (IgG or IgM). There were and light chains in the serum, positive for anti-human globulin test, lower CH50, and a small decrease in gamma globulin.
5. Blood biochemical examination showed rapid increase in erythrocyte sedimentation rate and increased alkaline phosphatase and LDH activity.
1. X-ray examination may have lung infiltration and pleural effusion.
2. B superabdominal B super visible liver, splenomegaly.
Diagnosis
Diagnosis and differential diagnosis of pediatric angioimmunoblastic lymphadenopathy
Fever, systemic lymph nodes, hepatosplenomegaly, increased polyclonal Ig, lymph node biopsy can be diagnosed, but the extranodal tissue lesions are not typical when the disease can not be ruled out.
Need to be differentiated from lymphoma, infectious mononucleosis, leukemia, systemic lupus erythematosus.
