subcutaneous panniculitis inflammatory T-cell lymphoma
Introduction
Introduction to subcutaneous panniculitis T-cell lymphoma Subcutaneous panniculitic T-cell lymphoma (SPTCL) is a rare primary cutaneous lymphoma that has been recognized in recent years. The clinical features are deep subcutaneous nodules or plaques, hard, tan to red, almost all of them, the largest diameter can reach more than 10cm, occur in the limbs, followed by the trunk, can also affect the skin of the face and neck, there are Some patients can spontaneously resolve, and some patients have repeated subcutaneous nodules. The history can be several years to more than 10 years before diagnosis. Most patients have systemic symptoms such as fever, chills, fatigue, weight loss and myalgia. When the tumor recurs, it is often confined to the skin. Organization, rarely spread. basic knowledge The proportion of illness: 0.0006% Susceptible people: no special people Mode of infection: non-infectious Complications: subcutaneous T-cell lymphoma
Cause
Subcutaneous panniculitis T-cell lymphoma etiology
Pathological features
Subcutaneous fat is infiltrated by diffuse and dense lymphoid tumor cells, and the fat septum and fat lobules are invaded. The tumor cells are arranged in a lace-like manner around a single fat cell. This is a characteristic infiltration method of the disease. The tumor cells vary in size and small cells. It is characterized by marked irregularity of the nucleus, deep staining of chromatin, insignificant nucleoli, sparse cytoplasm and cytoplasm. The morphological characteristics of large cells are large and round nuclei, scattered chromatin, obvious nucleoli, abundant cytoplasm and transparent to Double astigmatism, a large number of tissue cells in tumor tissue, scattered around the tumor cells, a large number of nuclear fragmentation bodies are visible inside and outside the cell, 60% of SPTCL tumor tissue has large pieces of fat necrosis and thus secondary The granuloma reaction, in most cases, has tumor cell infiltration of blood vessels.
2. Nuclear fragmentation and apoptosis
A large number of nuclear fragmentation bodies inside and outside the tissue suggest a general apoptotic phenomenon in the tumor tissue. It is unclear what kind of cells the apoptotic body is derived from, and it is speculated that it may be derived from cytotoxic T lymphocyte (CTL). Target cells such as adipocytes or other non-neoplastic cells, but may also be derived from tumor cells themselves, it is now clear that CTL can induce apoptosis of target cells through a variety of pathways, when the ratio of effector cells/target cells is high, A cloned CTL cell can attack other CTL cells to cause apoptosis.
3. Immunological phenotype
Expressing characteristic markers of one or more T cells, such as CD3, CD2, CD43 or CD45RO, recent studies have shown that most tumor cells are CD8-positive, and intracellular cytotoxic granule protein T-cell antigen-1 (T-cell) Intracellular antigen-1, TIA-1) and perforin are diffusely strongly positive. These two points strongly suggest that the tumor originates from CTL, and both CD4 and CD8 are expressed simultaneously, and some CD4 and CD8 are negative, and a few CD30 are positive. CD56 Positive, B cell-specific markers, CD69 and lysosomal negative.
Genotype analysis revealed that most of them had clonal rearrangements of TCR.
Prevention
Subcutaneous pannic inflammatory T-cell lymphoma prevention
Strengthen physical exercise, enhance physical fitness, and exercise more in the sun. Excessive sweating can excrete acidic substances in the body with sweat, avoiding the formation of acidic physique, and thus leaving no hidden dangers to the human body.
Complication
Subcutaneous panniculitis T-cell lymphoma complications Complications Subcutaneous T-cell lymphoma
The main complications are infection and bleeding, which is also the most common cause of death in this disease.
Symptom
Subcutaneous panniculitis T-cell lymphoma symptoms Common symptoms Weight loss, generalized red plaque nodules, liposuitis, muscle pain, chills
The clinical features are deep subcutaneous nodules or plaques, hard, tan to red, almost all of them, the largest diameter can reach more than 10cm, occur in the limbs, followed by the trunk, can also affect the skin of the face and neck, there are Some patients can spontaneously resolve, and some patients have repeated subcutaneous nodules. The history can be several years to more than 10 years before diagnosis. Most patients have systemic symptoms such as fever, chills, fatigue, weight loss and myalgia. When the tumor recurs, it is often confined to the skin. Organization, rarely spread.
Examine
Examination of subcutaneous panniculitis T-cell lymphoma
Early biopsy pathology is often diagnosed as non-specific lipid membrane inflammation, and some patients have abnormal liver function.
Peripheral blood
Anemia, granulocyte and thrombocytopenia, white blood cells can be reduced to 1 × 10 9 /L, platelets as low as 20 × 10 9 /L.
2. Tissue biopsy
Early manifestations of non-specific panniculitis followed by lymphoid tumor cell infiltration.
According to clinical manifestations, symptoms and signs can be checked.
Diagnosis
Diagnosis and diagnosis of subcutaneous panniculitis T-cell lymphoma
diagnosis
According to clinical manifestations and pathological examination confirmed.
Differential diagnosis
Benign subcutaneous lipid membrane inflammation
The bed is also characterized by similar deep subcutaneous nodules. The histological subepithelial tissue is also infiltrated by dense lymphocytes, which may be accompanied by fat necrosis, but the clinical progress is slow, there is a tendency to spontaneous regression, and generally does not develop lymphoma and systemic invasion. Histologically, infiltrating cells have typical manifestations and are arranged around a single fat cell lace-like pattern. These two points can distinguish most cases from this disease, but in the early stage of lymphoma, the atypicality of tumor cells is not obvious. At this time, the immune phenotype can be used to distinguish the two diseases. The cell population of benign subcutaneous lipid membrane inflammation is mainly B cells and non-single-form plasma cells, in which T cells are scattered, often with CD4 cells or CD4 and CD8. The proportion of the two cells is similar, only a few cells have positive cytotoxic granule protein staining, while subcutaneous pannic inflammatory lymphoma is mainly CD8 cells, cytotoxic granule protein TIA-1 and perforin are generally strongly positive, benign subcutaneous lipid membrane inflammation The clonal rearrangement of the TCR-free gene is also helpful in identifying the two.
2. Other T cell lymphoma
Such as large cell anaplastic lymphoma (ALCL), nasal T/NK cell lymphoma, non-specific peripheral T-cell lymphoma, these lymphomas can be secondary to subcutaneous tissue, easy to be confused with this disease However, the infiltration of these tumors is different from that of the disease, and often merges into a piece, and there is no phenomenon that the fat cells are arranged in a lace-like manner, and the nuclear fragmentation body is rare.
3. Non-specific peripheral T-cell lymphoma
Often CD4 positive, intracellular cytotoxic granule protein, nasal NK/T cell lymphoma, vascular central lymphoma, can also be expressed as deep subcutaneous nodules and subcutaneous lipid membrane necrosis, a tendency to invade blood vessels, part Patients also have phagocyte syndrome, but many skin organs such as the nasopharynx and lungs, tumor cells are often CD56-positive, no TCR gene rearrangement, often associated with EB virus infection.
4. Large cell anaplastic lymphoma
Perforin and TIA-1 can also be expressed simultaneously. Although a small number of SCPTCL can also express CD30, CD30 of ALCL is generally strongly positively expressed, with characteristic features of membrane and Golgi staining, and then, t(2;5) Or the detection of p80 NPM/ALK fusion protein contributes to the diagnosis of ALCL and SPCTL.
