Sinus histiocytosis with giant lymphadenopathy
Introduction
Introduction to sinus histiocytosis with giant lymphadenopathy Sinus histiocytosis with massive lymph node disease (SHML), also known as Rosai-Dorfman syndrome, whose cause is unknown, occurs in children and adolescents, is a rare benign and self-limiting disease. basic knowledge Sickness ratio: 0.0001% Susceptible people: good for children and adolescents Mode of infection: non-infectious Complications: anemia
Cause
Sinus histiocytosis with the cause of giant lymphadenopathy
(1) Causes of the disease
The cause is unknown and may be related to Epstein-Barr virus, herpes virus infection and cellular immune damage.
(two) pathogenesis
The pathogenesis of this disease is still unclear. The pathological features are that the lymph node sinus is filled with phagocytic tissue cells, which can phagocytose neutrophils, lymphocytes, plasma cells, red blood cells and nuclear debris. The lymphocytes that are engulfed can sometimes be corolla or The bunch is arranged in a cluster, and the shape is quite special. In addition, a small amount of lymphocytes and plasma cells are infiltrated in the sinus. The longer the course of the disease, the fibrosis of the lymph node envelope and the surrounding of the capsule, immunohistochemical staining can show phagocytic cells. Expression of pan-macrophage antigen, phagocytosis-associated antigen, S-100 protein and monocyte antigen acid phosphatase and non-specific esterase staining positive, suggesting that the pathological cells of this disease are derived from circulating monocytes, which are functionally active. Macrophages.
Prevention
Sinus histiocytosis with prevention of giant lymphadenopathy
There is no effective preventive measure for this disease. Early detection and early diagnosis are the key to the prevention and treatment of this disease.
Complication
Sinus histiocytosis with complications of giant lymphadenopathy Complications anemia
Often accompanied by fever, anemia, about 25% of patients can be associated with skin, upper respiratory tract, bone, genitourinary system, parotid gland, eye, liver, heart, central nervous system lesions.
Symptom
Sinus histiocytosis with symptoms of giant lymphadenopathy Common symptoms Lymph node enlargement Tissue cell proliferation Immunological hemolytic ESR
1. Painless progressive superficial lymphadenopathy: the disease mainly involves cervical lymph nodes, followed by axillary and inguinal lymph nodes. The lymph nodes are characterized by painlessness, progressive enlargement, and often adhere to each other to form a huge mass. The lesion may be localized in a limited manner, or may be multiple superficial lymphadenopathy, or may involve deep lymph nodes, which may be swollen at the same time or in succession. Another prominent feature is that the enlarged lymph nodes are self-limiting and swollen. The lymph nodes can resolve spontaneously after several weeks to several months of progressive enlargement. Most of them disappear completely after 9 to 18 months and leave no trace on the pathology. The lymph nodes of a few patients can continue to be swollen and occasionally lethal. Report.
2. Can be associated with autoimmune hemolytic anemia, glomerulonephritis in children.
The painless giant lymph node mass, which has two characteristics of self-limiting regression, is an important clue for diagnosis, but the final confirmation still depends on the pathological examination results to confirm.
Examine
Sinus histiocytosis with giant lymphadenopathy
1. Peripheral blood: decreased hemoglobin, elevated or normal central granulocytes.
2. ESR increases, and immunoglobulins can also increase.
According to the symptoms, signs, clinical manifestations can choose to do electrocardiogram, B-ultrasound, X-ray, CT, bone wear and other tests.
Diagnosis
Diagnosis and differentiation of sinus histiocytosis with giant lymphadenopathy
Diagnostic criteria
1. Lymph node enlargement: SHML mainly involves cervical lymph nodes, followed by axillary and inguinal lymph nodes. The lymph nodes are painless and progressively enlarged. They often merge into each other and become large blocks. The lesions can exist in isolation or in superficials. Or deep lymph nodes, simultaneous or sequential enlargement, which is very similar to NHL and Castleman's disease and should be noted.
2. Chronic signs of inflammation: clinical manifestations of fever, anemia, elevated neutrophils, increased erythrocyte sedimentation rate and polyclonal hyperglobulinemia and other signs of inflammation.
3. Extranodal lesions: About 1/4 of patients may be associated with extranodal lesions, usually skin, upper respiratory tract, bone and genitourinary system, but also involving the parotid gland, eye, liver, heart and central nervous system, etc. Rare.
4. Diagnostic steps: The diagnostic procedure is similar to lymphoma. When clinical signs of chronic inflammation such as fever, anemia, etc., accompanied by painless cervical lymph nodes, the disease should be considered, but due to clinical manifestations. It is very similar to malignant lymphoma, Castleman's disease and AILD. The final diagnosis depends on histopathological examination. It is the only basis for diagnosis and the main basis for identification.
Differential diagnosis
The diseases to be differentiated from SHML are: malignant lymphoma, Castleman's disease, vascular immunoblastic lymphadenopathy (AILD) and various histiocytosis, including malignant histiocytosis, hemophagocytic syndrome, etc., previously described, identification Diagnosis depends on pathological biopsy, clinically similar, but the course of the disease is self-limiting, usually without treatment, corticosteroids, chemotherapy and radiotherapy are ineffective, unlike other neoplastic diseases, children with abnormal immune disorders, such as Wiskott-Aldrich Syndrome, autoimmune hemolytic anemia, glomerulonephritis may be associated with SHML.
