orbital non-Hodgkin lymphoma

Introduction

Introduction to non-Hodgkin's malignant lymphoma of the eyelid Non-Hodgkinsmalignant lymphoma is a group of lymphomas with varying degrees of malignancy. It can be divided into more than 10 types according to cell morphology, differentiation and distribution. The clinical manifestations are mostly similar. Non-Hodgkin's malignant lymphoma It occurs mostly in the lymph nodes of the whole body and in the liver and spleen, but it can also occur in lymph node network or non-lymphoid network. basic knowledge The proportion of the disease: the incidence rate is about 0.001% -0.002% Susceptible people: no special people Mode of infection: non-infectious Complications: eye movement disorders

Cause

Eyelid non-Hodgkin's malignant lymphoma

(1) Causes of the disease

The cause of non-Hodgkin's lymphoma involves viruses, bacteria, radiation, certain chemicals, and herbicides. It is known that EB virus and high-incidence areas Burkitt lymphoma and extranodal T/NK cell lymphoma, nasal type Related, adult T-cell lymphoma/blood disease is closely related to human pro-T-cell virus type I (HTLV1) infection, and gastric mucosa-associated lymphoid tissue lymphoma is a malignant change caused by the initiation of reactive lesions infected by H. pylori. Survivors of radiation exposure such as nuclear explosions and nuclear reactor accidents, cancer patients receiving radiotherapy and chemotherapy have an increased risk of developing non-Hodgkin's lymphoma, AIDS, certain hereditary, acquired immunodeficiency diseases or autoimmune diseases such as ataxia - telangiectasia, combined immunodeficiency syndrome, rheumatoid arthritis, systemic lupus erythematosus, Sjögren's syndrome, hypogammaglobulinemia, and long-term immunosuppressive drug therapy (eg organ transplantation) Immune dysfunction caused by other diseases is a high risk factor for the development of non-Hodgkin's lymphoma.

(two) pathogenesis

Due to the different stages of lymphocyte differentiation, different stages of tumor cells may occur in the invaded lymph nodes or lymphoid tissues. In the same lesion, there may be poorly differentiated tumor cells or cells with more mature differentiation. The progression of the lesion, the histological type of malignant lymphoma may be transformed, such as nodular type can be converted into diffuse type.

The proliferating tumor tissue may be a single cell component, but since the original pluripotent stem cells may differentiate in different directions, sometimes the cellular components may be more than two or more.

In recent years, due to the widespread use of monoclonal antibodies and immunohistochemistry, it has been possible to distinguish T, B lymphocytes in different stages of differentiation.

Tumors that occur in subcapsular cortical thymocytes are usually T-cell acute lymphoblastic leukemia and lymphoblastic lymphoma. All other T-cell lymphomas are derived from more mature T cells, CD4-positive, including adult T-cell lymphoma. (ATL), mycosis fungoides, Sezary syndrome, most so-called peripheral T-cell lymphoma and more than half of T-cell chronic lymphocytic leukemia, some peripheral T-cell lymphoma, nearly half of T-cell chronic lymphocytic leukemia And some T lymphoproliferative disorders, CD8 positive.

B-cell lymphoma has fewer specific antibodies, but has surface immunoglobulin expression. The earliest B cells have CD10 and CD19 on the surface, and there are terminal transferases in the cell and recombination of heavy-bond genes. , the production of heavy bonds in the cytoplasm, the reorganization of the K light bond gene, the recombination of the light bond gene and the loss of the terminal transferase, which represent the development of the pre-B cell stage, and the expression of CD10 after cell loss becomes immature. B cells have IgM expression on the surface, and IgD and IgM are produced on the cell surface expressing CD21 receptor (C3d) membrane. The developmental stages of all B cells occur under antigenic stimulation, and the immunoglobulin gene is stimulated by antigen. It is activated and secreted. After that, the cells lose CD21, CD20 and surface immunoglobulin, and the plasma cells are labeled with PC-1 and PC-2 to secrete immunoglobulin, which is the development process of cell follicle center B cells, which is malignant. After the change, it becomes lymphocytic lymphoma.

Most acute lymphocytic leukemias are derived from pre-B cells, Burkitt lymphomas and leukemias are derived from surface IgM-positive immature B cells, and most follicular and diffuse B-cell lymphomas are derived from mature or activated B cells, giant balls. Proteinemia (Waldenström syndrome) and multiple myeloma are derived from the terminal stage of differentiation. Chronic lymphocytic leukemia expresses CD5, and diffuse moderately differentiated lymphoma expresses CD5 and CD10, suggesting that these are from the mantle cell area rather than the filter. B cells in the center of the bubble.

Prevention

Eyelid non-Hodgkin's malignant lymphoma prevention

Pay attention to the usual habits and find timely treatment in time.

Complication

Eyelid non-Hodgkin's malignant lymphoma complications Complications, eye movement disorders

Eye movement and movement disorders.

Symptom

Eyelid non-Hodgkin's malignant lymphoma symptoms Common symptoms Eyeball can not be free to move the eye to highlight the eyelid edema swollen lymph nodes swollen eyelids drooping

Although the classification of non-Hodgkin's malignant lymphoid tumors is complicated, the clinical manifestations are more consistent and more common in the lacrimal gland. This is due to the presence of lymphoid tissue in the normal lacrimal gland. One or both of the eyelids are swollen, drooping, and Painless hard mass, prominent eyeball, and shift to one side, conjunctival edema, due to invasive hyperplasia of the lesion, affecting the optic nerve and extraocular muscles, vision loss often occurs, eye movement is limited, and even the eyeball is fixed, conjunctiva Invasive, pink fish-like mass can be seen through the conjunctiva. The tumor with higher malignancy develops faster, the eyelids infiltrate and harden, cover the eyeball, and connect with the tumor in the sac, the age and clinical manifestation of the tumor. It is quite similar to lymphatic infiltration inflammatory pseudotumor, especially in the differential diagnosis of lacrimal gland type pseudotumor. Intraorbital malignant lymphoma is sometimes accompanied by lymphadenopathy in other parts, requiring detailed examination.

Examine

Examination of non-Hodgkin's malignant lymphoma of the eyelid

1. The peripheral blood picture of patients with early blood is normal, secondary to autoimmune hemolysis or tumor involving bone marrow can cause anemia, thrombocytopenia and hemorrhage, 9% to 16% of patients may have leukemia transformation, common in diffuse small lymphocytic lymph Tumor, follicular lymphoma, lymphoblastic lymphoma and diffuse large cell lymphoma.

2. Biochemical examination may have erythrocyte sedimentation rate, serum lactate dehydrogenase, 2-microglobulin and alkaline phosphatase increased, monoclonal or polyclonal immunoglobulin increased, the above changes can often be used as indicators of tumor burden and disease detection.

3. The erythrocyte sedimentation rate increases during the active period and the remission period is normal. It is a simple method for determining the remission period and the active period.

4. Immunological phenotype detection monoclonal antibody immunophenotypic examination can identify the cell lineage and differentiation level of lymphoma cells, and the commonly used monoclonal antibody markers including diagnosis and typing include CD45 (white blood cell common antigen) for identification. Leukocyte origin; CDl9, CD20, CD22, CD45RA, CD5, CD10, CD23, immunoglobulin light chain and are used to identify B lymphocyte phenotype; CD2, CD3, CD5, CD7, CD45RO, CD4, CD8, etc. T lymphocyte phenotype; CD30 and CD56 are used to identify anaplastic large cell lymphoma and NK cell lymphoma, respectively, CD34 and TdT are common in lymphoblastic lymphoma phenotype.

5. Genetics 90% of non-Hodgkin's lymphomas have non-random karyotype abnormalities, usually chromosomal translocation, partial deletion and amplification, etc. Different types of non-Hodgkin's lymphomas have their own Cytogenetic features, non-Hodgkin's lymphoma is a monoclonal malignant proliferation that occurs in a single parental cell. The gene rearrangement of tumor cells is highly consistent. IgH gene rearrangement is often used as a genetic marker for B cell lymphoma, TCR gamma or Beta gene rearrangement is often used as a genetic marker for T-cell lymphoma, and the positive rate can reach 70%-80%. Cytogenetics and gene markers can be used for the diagnosis, classification and detection of small tumors of non-Hodgkin's lymphoma. .

6. The bone marrow is normal in the early stage, and the bone marrow can change when the bone marrow is infiltrated in the late stage. If the lymphoma cells are found, it can be called lymphoma leukemia.

7. Pathological examination The visual appearance of lymphoid tumors is a homogeneous yellow or pink mass. Although the internal lobular shape is obvious, the boundary contours are often distinct. Under the microscope, it may be difficult to distinguish these tumors into benign and malignant types because They represent a continuum of changes in cell morphology, although some tumors can be clearly diagnosed as benign reactive lymphocytosis, others are diagnosed as malignant lymphoma, but some are in transitional cell morphology called atypical lymphocyte proliferation, In order to solve the diagnosis problem, diagnostic criteria for benign reactivity, atypical lymphoid hyperplasia and malignant lymphoma have been proposed.

Reactive lymphocytosis: the lesion consists of diffusely proliferating lymphocytes. Compared with inflammatory pseudotumor, lymphoid hyperplasia is more pronounced. Lymphoid follicles are more common. The lesions are mainly small and round mature lymphocytes with pulp. The pleomorphic arrangement of cells is characterized by an active mitotic germinal center, a primordial fibrous matrix with several eosinophils and endothelial cell proliferation.

Atypical lymphocytosis: represents an intermediate transitional disease between reactive lymphocytosis and malignant lymphoma. It is characterized by lymphocyte composition. Lymphocytes are diffusely hyperplasia, lymphoid follicles are less, and lesions are mainly mature. The composition of cells, which differs from reactive lymphoid hyperplasia, is that the number of immature lymphocytes is large, and there is a nuclear fission image outside the germinal center.

Malignant lymphoma: consists of morphologically simple, immature lymphocytes or distinctly shaped lymphocytes, with more, larger mitotic figures of degenerative developmental cells, more polymorphonuclear and often coexisting nucleus Ren characteristics, lymphoid follicles are absent or inconspicuous, and endothelial cell proliferation is not obvious.

Histopathologically benign reactive lymphoid hyperplasia shows lymphoid follicles with reactive germinal centers and a variety of cellular components including lymphocytes, histiocytes and plasma cells; malignant lymphomas with lymphoid invasive lesions Cytologically, it is characterized by atypical, atypical cell composition. According to pathological features, it is divided into inflammatory pseudotumor, reactive lymphoid hyperplasia, atypical lymphocyte hyperplasia and malignant lymphoma. Through the organic combination of histopathological classification and immunological classification, it can provide a more reliable basis for clinical diagnosis and treatment. Pathological examination is the main basis for diagnosis of MHL and pathological types.

1. Ultrasound exploration Because lymphoma is composed of a large number of lymphocytes, the fibrous tissue is sparsely spaced, A-superior shows low reflex within the lesion, the sound attenuation is not obvious, the boundary is clear, and the B-ultrasound shows that the lesion is irregular, flat or elliptical. Shape, clear boundary, less internal echo, light attenuation, generally CDI often found rich blood flow in the lesion.

2. CT scan Most tumors are located in the front of the eyelid and involve the eyeball, extraocular muscles or optic nerve. The boundary is unclear, the shape is irregular, the enhancement is obvious, and bone destruction rarely occurs, but it can fill the eyelids.

3. MRI lymphoma is mostly located in the lacrimal gland, orbit, and can also diffuse into the soft tissue of the iliac. In MRI, TlWI is mostly medium signal, T2WI is high signal, or medium and high heterogeneous signal, and the enhancement is obvious. Because the lesion is invasive hyperplasia, It can show the normal structure of the eyelids, even full of eyelids.

Diagnosis

Diagnosis and differentiation of non-Hodgkin's malignant lymphoma of the eyelid

According to the history and clinical manifestations, combined with imaging examination, the diagnosis is not difficult, and the final diagnosis depends on the biopsy.

Mainly differentiated from lacrimal gland epithelial tumors, the latter is multi-echo or moderate-high reflex, but it is difficult to distinguish from lacrimal gland pseudotumor. If necessary, biopsy confirms the diagnosis. The most easily confused with lymphocytic inflammatory pseudotumor, two The clinical and imaging findings are similar, only the age of the lymphoma is too large, and the final identification requires pathological confirmation.

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