primary cutaneous follicular center cell lymphoma

Introduction

Introduction to primary skin follicular central cell lymphoma The primary cutaneous follicular center cell lymphoma (primary cutaneous follicular center cell lymphoma) is composed of follicular central cells, which include central cells (small and large nucleus follicular center cells) and central blast cells (large follicular center cells) , with obvious nucleoli). Follicular central cell lymphoma (FCCL) includes all follicular and diffuse lymphomas that originate in germinal center cells. basic knowledge The proportion of illness: 0.002% Susceptible people: more common in male seniors Mode of infection: non-infectious Complications: elderly sarcoidosis

Cause

Primary skin follicular central cell lymphoma

The etiology is still unclear. Tumor infiltrating cells are mainly distributed in the superficial part of the reticular reticular layer, even up to the deep and subcutaneous tissues, even in a continuous band-like distribution, with a gap between the epidermis and a cell-free infiltration zone, early along the blood vessels and attachments. The device is surrounded by flaky and agglomerated infiltration, 10% of the lesions are follicular, and most of them are diffuse in the late stage, which is often a mixed type. The composition of the infiltrating cells is different, even if the number of different cellular components is different in different specimens of the same patient. Regardless of the cell type, cells that differentiate into plasma cells are common. In the past, skin FCCL was considered to be secondary to FCCL or other extranodal organs, and the prognosis was poor. It is proven to be primary to the skin. The primary skin FCCL is only partially expanded, and the local treatment effect is also good.

Prevention

Primary skin follicle center cell lymphoma prevention

prevention:

Because the cause of lymphoma patients is not yet clear, the method of prevention is nothing more than:

1 to minimize infection and avoid exposure to radiation and other harmful substances, especially drugs that have an inhibitory effect on immune function;

2 Appropriate exercise, enhance physical fitness and improve your disease resistance.

Mainly for the prevention of various factors that may lead to malignant lymphoma. It is currently believed that the loss of normal immune surveillance function, the tumorigenic effect of immunosuppressants, the activity of latent viruses and the long-term application of certain physical (such as radiation), chemical (such as anti-epileptic drugs, adrenocortical hormone) substances, Lead to the proliferation of lymphatic network, and eventually malignant lymphoma. Therefore, pay attention to personal and environmental hygiene, avoid drug abuse, and pay attention to personal protection when working in a harmful environment.

Complication

Primary skin follicular central cell lymphoma complications Complications, elderly sarcoidosis

The disease can lead to the transfer of surrounding lymphoid tissue, causing difficulty in breathing; late metastasis to other sites, causing corresponding complications, such as metastasis to the gastrointestinal tract, can cause abdominal pain, gastrointestinal ulcers, bleeding, obstruction, etc., invading brain tissue, Cause headache, blurred vision, language barriers, etc., violation of bones caused by fractures, etc., it should be noted to observe the patient's stool characteristics and bone joints have pain, abnormal movements.

Symptom

Primary skin follicle center cell lymphoma symptoms Common symptoms Papular nodule erythema (border clear...

The disease is more common in male elderly, skin nodules, plaques occur in the trunk, especially the back, followed by the scalp, mostly single or scattered multiple, diameter 2.5 ~ 15cm, smooth surface, papillary, rarely off Chips and ruptures, typically surrounded by small papules, mildly infiltrating plaques and/or graphic erythema, mild invasive plaques can occur in rapidly growing skin tumors months or 20 years before, some patients The small papules, nodules and/or mild infiltrating plaques are only maintained for several months.

Lesions are non-scaly single or clustered papules, plaques and/or tumors, surrounded by erythema. In most cases, lesions are confined to a certain site. About 2/3 of the cases occur in the trunk. One-fifth of the cases occurred in the head and neck. The disease is more than twice as many as the female. The number of injuries is increased in a few years without treatment, and the number is increased. However, those affected by the skin are rare, and the head and trunk are well prognosis. The 5-year survival rate after treatment is 100%.

Examine

Examination of primary skin follicular central cell lymphoma

Histopathology: nodular or diffuse infiltration in the dermis, characterized by invasive epidermis, early damage to the central cells, a small number of central mother cells and reactive T cells mixed, as the disease progresses, the tumor B cells increase, The number has increased.

The histological classification of FCCL, according to the new work classification, includes: follicular small split cells, follicular mixed cells and follicular large cells. The infiltrating cells of this tumor are mainly in the superficial part or even deep part of the dermal network layer. And subcutaneous tissue, even or in a continuous band-like distribution, separated from the epidermis by a cell-free infiltration zone, early along the blood vessels and/or appendages, flaky, agglomerate infiltration, 10% lesions showing follicular In the late stage, it is mostly diffuse. The common type is two types (mixed type). The components of infiltrating cells are different. However, regardless of the cell type, some cells that differentiate into plasma cells are common, and are often limited to a single light. The cloning of the chain, in some cases, shows the transition between plasma cells and tumor cells. It is prominent that the aggregation of large lymphoid cells is not seen in the typical neoplastic follicular structure common in the lymph node FCCL. The border is unclear, and the surrounding small lymphocytes are distributed irregularly. According to the new classification of work and the time of damage, the tissue may show small clea red cell (SCC) lymphoma in the early stage, but common Small split cell Large cell mixed (MC) lymphoma; in advanced and even in old tumors, although obvious MC lymphoma can be seen, it often shows large cell (LC) lymphoma, the number of inflammatory cells is different, mainly small lymphoid Cells, plasma cells and macrophages, about 10% of the lesions, can be seen in the fully developed reactive lymphoid follicles and/or structures of the germinal center (immunolabeled B cell polyclonal), immunolabeled tumors In addition to expressing whole B cell antigens (CDl9, C1320 and CD22) and HLA-DR, cells often show immunoglobulin heavy chain turnover, loss of IgD, and IRC or IRA.

Immunopathology: tumor B cell markers CD19, CD20, CD22, CD79 positive, single immunoglobulin products can be found on the cell surface, ie kappa or lambda light chain, while both are positive, tumor immunoglobulin Negative staining, skin follicular central cell lymphoma does not express CD5 or CD10, compared with lymph node follicular central cell lymphoma, primary skin follicle center usually does not express bcl-2 protein, immunoglobulin is seen in most cases Protein gene cloning rearrangements.

Diagnosis

Diagnosis and diagnosis of primary skin follicular central cell lymphoma

diagnosis

According to the clinical manifestations, the characteristics of skin lesions and histopathological features can be diagnosed.

Differential diagnosis

1 primary cutaneous immune cell tumor: tumor mainly occurs in the limb, tumor cells are mainly plasma cell-like lymphocytes, common PAS positive inclusions, immunoglobulin is located in the cytoplasm.

2 Immunization of small lymphocytic lymphoma and mantle cell lymphoma.

3 cutaneous lymphoma: the identification of primary skin FCCL is very difficult, the identification points listed in the previous literature are mostly based on the systemic involvement of the first rash after 5 years as a good, malignant difference, it is known that these are not absolutely reliable Standard, even if the determination of SIg (skin lymphoma showed polyclonal B cell component, FCCL expression of a single light chain or loss of measurable SIg) is helpful for identification, it is sometimes difficult to explain the presence of weakly stained SIg in the skin. Moreover, in the so-called large cell lymphoma, focal single-type SIg staining and deletion of SIg can be seen. The gene rearrangement is more reliable for the identification of the two, but the clonal B-cell component can also be shown in the skin lymphoma. Therefore, it is necessary to combine Clinical and comprehensive differences are identified.

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