primary cutaneous immunocytoma
Introduction
Introduction to primary cutaneous immune cell tumor Primary cutaneous immunocytoma is a rare lymphoma, synonymous, lymphoplasmacytoidtymphomaofskin. The prognosis is good (100% survival in 5 years), characterized by small lymphocytes expressing lymphoid cytoplasmic immunoglobulin, lymphoplasmacytic cells and plasma cell proliferation. basic knowledge The proportion of illness: 0.0025% Susceptible people: mainly in middle and old Mode of infection: non-infectious Complications: Sjogren's syndrome Leukemia
Cause
Primary cutaneous immune cell tumor etiology
(1) Causes of the disease
The cause is not yet clear. It depends on the actual condition of the patient.
(two) pathogenesis
The pathogenesis is still unclear.
Prevention
Primary cutaneous immune cell tumor prevention Stay optimistic and happy. Long-term mental stress, anxiety, irritability, pessimism and other emotions will make the balance of the cerebral cortex excitatory and inhibition process imbalance, so you need to maintain a happy mood.
Complication
Primary cutaneous immune cell tumor complications Complications Sjogren's syndrome
A small number of concurrent autoimmune diseases, such as Sjogren's syndrome, acquired bullous epidermolysis, unexplained thrombocytopenia, advanced leukemia in the late stage, and dedifferentiation to develop highly malignant immunoblastic lymphocytes Tumor or Waldenström disease.
Symptom
Primary cutaneous immune cell tumor symptoms common symptoms thrombocytopenia splenomegaly
Skin immune cell tumors are more common in Western European countries, accounting for 15% to 20% of CML. Although it has not been reported in China, it is rare, and it is still worth noting in the future. It mainly occurs in middle and old people, without gender differences. Hairy or multiple (subcutaneous) skin nodules, occur in the limbs, skin nodules occur quickly, red, purple to brown red, or merge into invasive plaque, higher than the skin, very little scales, very few Occlusion, even or self-resolving, the nodules of individual patients can be developed from the original chronic atrophic dermatitis, the primary cutaneous immune cell tumor has a good course of disease, good local treatment, secondary skin immunity About 20% of patients with cell tumors show a single gamma globulin, which can develop lymph nodes and splenomegaly, and a few can be complicated by autoimmune diseases such as Sjogren's syndrome, acquired bullous epidermolysis, and unexplained thrombocytopenia. Late stage can be secondary to leukemia, and can be dedifferentiated to develop highly malignant immunoblastic lymphoma or Waldenström disease.
Examine
Examination of primary cutaneous immune cell tumor
Histopathology: Infiltration of tumor cells in the dermis and subcutaneous tissue, mostly in the form of lamella or clumps in primary cutaneous immune cell tumors; often diffuse in secondary cutaneous immune cell tumors, except for a few tissue cells and Acid granulocytes, as well as many mast cells, appear to appear in the background of small lymphocytes:
1. More plasma cell-like lymphocytes, substances (immunoglobulin) that are positive for MGP and PAS in the nucleus or cytoplasm.
2. More or even a lot of scattered plasma cells, the percentage of plasma cell-like lymphocytes and (or) plasma cells accounted for 20% of primary cutaneous immune cell tumors, mostly located around the infiltrating foci, in secondary skin immune cells 40% of the tumors.
3. There are still some mixed infiltrations of cells at different stages of differentiation into plasma cells (central cells with irregular nucleus, central mother cells, immunoblasts, special plasma cells and plasmablasts). Obviously, mitotic figures are more common. Immunolabeling shows plasma-like lymphocytes and plasma cells synthesize monoclonal C3 receptors and IgG (IgA or IgM in a few cases), and immunoglobulins (mostly IgMK) in tumor tissues increase. .
4. Immunohistochemistry: characterized by tumor cells expressing unicellular cytoplasmic immunoglobulin heavy and light chain molecules, immune cell tumors, tumor cells expressing CD70 but lacking CD50 infiltrating endoplasmic cells CD20-, immunoglobulin can be found in most cases Protein gene rearrangement.
Diagnosis
Diagnosis and diagnosis of primary cutaneous immune cell tumor
diagnosis
According to the clinical manifestations, the characteristics of skin lesions, histopathology, and immunohistochemistry can be diagnosed.
Differential diagnosis
1. Plasma cell tumor: Most of the skin plasma cell tumors are multiple myeloma, which is very rare in the primary.
2. Primary skin follicle center cell lymphoma: more common in the trunk and face, tumor cells belong to the central cell / central mother cell, no PAS positive inclusions, immunoglobulin staining on the surface of the cell membrane or absent.
3. The difference between primary and secondary cutaneous immune cell tumors: the former skin lesions are mostly located in the limbs, no autoimmune diseases, single-type cells account for 20%; the latter skin lesions are distributed throughout the body, 50% have single-propane genus Protein, half with autoimmune diseases, single type cells accounted for 40%.
4. B-cell pseudocutaneous lymphoma: Single-type plasma cells and/or plasmacytoid lymphocytes were found to contribute to the difference between cutaneous immune cell tumors and B-cell pseudocutaneous lymphomas.
5. Waldenström macroglobulinemia: IgM-producing tumor cells secrete macroglobulin into the blood.
