CD was first described in the 1820s, and in 1954 Castleman et al. formally reported a tumor-like mass confined to the mediastinum, with histology showing marked hyperplasia of lymphoid follicles and capillaries called vascularfollicularlymphnodehyperplasia. 1969 Flendring and Schillings proposed another morphologic subtype of CD, characterized by plasma cell hyperplasia, often with systemic symptoms. It is also known as giantlymphnodehyperplasia because the enlargement of lymph nodes is often obvious, sometimes reaching more than 10 cm in diameter.
A biopsy of the enlarged lymph nodes shows the specific pathological changes of CD described above. The lesions mainly involve lymphatic tissues anywhere in the body and may occasionally spread to extra-nodal tissues CD pathology is divided into two types as follows.
Hyaline vascular type: 80% to 90% of the cases. The lymph nodes are 3-7 cm in diameter, with the larger ones reaching 25 cm and weighing up to 700 g. Microscopically, many enlarged lymphatic follicle-like structures are seen in the lymph nodes, which are scattered. There were several small vessels penetrating into the follicles, and the endothelium of the vessels was obviously swollen, with thickened walls and glass-like changes at a later stage. There was a variable amount of eosinophilic or hyaline material distributed around the vessels. The follicles were surrounded by multiple layers of lymphocytes arranged in a circular center, forming a special onion skin-like structure or a cap-like band with more capillaries with thickened walls and lymphocytes, plasma cells, and immunoblasts between the follicles, and the lymphatic sinuses disappeared or were fibrotic. In some cases, the hyperplastic lymphoid follicles consist mainly of small lymphocytes, with only a few follicles containing small germinal centers, called the lymphocytic type. This type is most easily confused with follicular lymphoma.
Plasmacytoid type: 10% to 20% of cases. Patients often have systemic symptoms, such as fever, malaise, weight loss, anemia, increased erythrocyte sedimentation rate, increased blood gammaglobulin and hypoalbuminemia. Symptoms may disappear after lymph node dissection. Microscopic examination also shows follicular hyperplasia in the lymph nodes, but the lymphocytic proliferation around the small vessels and follicles is much less pronounced than in the clear-vessel type, and there is usually no typical onion skin-like structure. The main feature of this type is the proliferation of interfollicular plasma cells at all levels in patches, and Russell’s vesicles can be seen, while a few lymphocytes and immunoblasts are still present. This type has been described as the active phase of the hyaline vascular type and may have TCRβ or IgH gene rearrangement. A few patients with plasma cell type have been reported to have Kaposi’s sarcoma, with AIDS with CD being the most common.
A small number of patients have multiple lymph node involvement and extra-nodal multi-organ invasion, and the pathology of both types is called mixed type. There are also a few single lesion patients who have both of the above two types of pathology, which is another sense of mixed type.
Symptoms of CD are clinically divided into focal and polycentric types.
The focal type is more common in young people, with a median age of 20 years. 90% of the patients have a clear vascular pathology. Patients present with painless enlargement of a single lymph node, which grows slowly to form a huge mass with a diameter of several centimeters to about 20 cm and can occur in any part of the lymphatic tissue, but mediastinal lymph nodes are the most common, followed by cervical, axillary and abdominal lymph nodes. Most of them have no systemic symptoms and can survive for a long time after resection, i.e., benign course. 10% of them have plasma cell type pathology and abdominal lymph node involvement is common, often accompanied by systemic symptoms such as prolonged hypothermia or hyperthermia, lethargy and anemia, etc. The symptoms can all subside after surgical resection and do not recur.
2. The multicentric type is less common than the focal type, with a later age of onset and a median age of 57 years. Patients have multi-located lymph node enlargement that easily spreads to superficial lymph nodes. Patients with systemic symptoms (e.g., fever) and hepatosplenomegaly often show multisystem involvement such as nephrotic syndrome, amyloidosis, myasthenia gravis, peripheral neuropathy, temporal arteritis, Schegren’s syndrome (dry syndrome), thrombotic thrombocytopenic purpura, and oral and corneal inflammatory reactions. 20% to 30% of patients may develop Kaposi’s sarcoma or B-cell lymphoma during the course of the disease. In a small number of patients, the presence of polyneuropathy, organ enlargement (liver, spleen), endocrinopathy, serum monoclonal immunoglobulin and skin lesions constitute clinical signs of POEMS syndrome. In addition, the multicentric type often has an aggressive clinical course and is prone to infections.
Complications
About 1/3 of patients may have Kaposi’s sarcoma or B-cell lymphoma.
2. Combination of neurological, endocrine and renal lesions, as well as Schelgren’s syndrome (dry syndrome) and thrombotic thrombocytopenic purpura.
Laboratory tests
1, peripheral blood mild to moderate orthocytic orthochromic anemia, some cases have leukocytosis and/or thrombocytopenia can also be manifested as a typical firepot network of chronic disease anemia.
2, bone marrow image of some patients with elevated plasma cells from 2% to 20%, morphology is basically normal.
3, blood biochemical and immunological examination liver function can be abnormal, manifested as serum aminotransferase and bilirubin levels rise in a few patients with renal involvement serum creatinine levels rise in serum immunoglobulin is polyclonal elevation, more common, a few serum appear M protein, blood sedimentation also increased accordingly. Some patients have positive anti-nuclear antibody rheumatoid factor and anti-human globulin test.
4, urinary routine urine protein is mildly elevated, if accompanied by nephrotic syndrome, there is a large amount of protein.
4. Other examinations are selected according to clinical manifestations, symptoms and signs such as pathological examination, X-ray, CT, ultrasound and electrocardiogram.
Related tests.
>Coombs test > monoclonal gammaglobulin > antinuclear antibody > plasma cells > rheumatoid factor > creatinine anhydride > protein quantification (urine) > platelets > blood sedimentation V. The clinical manifestations of CD are not specific. Any person with obvious enlargement of lymph nodes with or without systemic symptoms should think of the possibility of CD, and lymph node biopsy is only diagnosed when the above-mentioned typical pathological changes of CD are obtained, i.e. the diagnosis of CD must be confirmed by In other words, the diagnosis of CD must be confirmed by pathological evidence, and then a typological diagnosis is made based on clinical manifestations and pathology. Various possible related diseases should be excluded before the diagnosis is made.
Differential diagnosis of CD should be differentiated from malignant lymphoma, reactive hyperplasia of various lymph nodes (mostly due to viral infection), plasmacytoma, AIDS and rheumatic diseases. They have certain similar clinical manifestations and/or pathological changes, and careful pathological examination, including immunohistochemical examination, and the detection of certain primary diseases are the main points of differentiation. The enlarged lymph nodes of this disease must be differentiated from the following diseases.
Lymphoma may have persistent or periodic fever, generalized itching, splenomegaly, and emaciation. The main difference is in the pathology, which is characterized by significant vascular hyperplasia.
2. Angioimmunoblastoma lymphadenopathy is an abnormal non-neoplastic immunoproliferative disease. Clinically, it is mostly seen in women with fever, generalized lymph node enlargement may have rash and pruritus of the skin adjuvant examination leukocytosis, increased blood sedimentation, ineffective antibiotic therapy, hormone can improve the symptoms. Lymph node pathology shows lymph node destruction and capillary wall hyperplasia as immunoblasts. Intervascular endothelial cells are PAS positive with amorphous material deposition and intercellular deposits of eosinophilic structureless material. Biopsy can be distinguished.
3, primary macroglobulinemia this disease is mainly lymphoid plasma cell proliferation secretion of large amounts of monoclonal macroglobulin, and extensive infiltration of bone marrow and extramedullary organs. There is a large amount of monoclonal IgM in the serum, no bone destruction no kidney damage, clinically there is hepatic and splenic lymph node enlargement, about half with hyperviscosity.
4. Multiple myeloma is a common type of plasma cell disease in which proliferating plasma cells (or myeloma cells) in bone marrow infiltrate bones and soft tissues causing a series of organ dysfunction, with clinical manifestations of bone pain, anemia, renal impairment and abnormal immune function, and hypercalcemia. CD lymph node enlargement is obvious and can be identified by lymph node biopsy.
The prognosis of this disease is focal, the prognosis is good, while multicentric with monoclonal hypogammaglobulinemia, the prognosis is poor, prone to malignant transformation or lymphoma, etc.