Castleman disease

Introduction to Castleman's disease Castleman's disease (CD) is one of the unexplained reactive lymphadenopathy, clinically rare, characterized by significant swelling of deep or superficial lymph nodes, and some cases may be associated with systemic symptoms and/or multiple systemic damage. In most cases, the surgical treatment of swollen lymph nodes is effective. basic knowledge Sickness ratio: 0.0004% Susceptible people: no specific people Mode of infection: non-infectious Complications: Kaposi's sarcoma thrombotic thrombocytopenic purpura

The cause of Castleman's disease

(1) Causes of the disease

The etiology of CD is unknown, and the plasma cell type is thought to be related to infection and inflammation. Some authors have suggested that immunoregulatory abnormalities are the initiating factors of CD. Clinically, 25% of central cases are confirmed to be associated with HHV-8 infection, and at least part of CD is considered. In the risk of malignant hyperplasia of B cells, a few polycentric types can be converted into malignant lymphoma, but most cases follow the results did not translate into malignant tumors.

(two) pathogenesis

Because there are more than one vascular germinal center in the lymph node follicles, some cases also have angiolipoma components, and the lesions can also occur in the normal absence of lymphoid tissue, so it was considered a hamartoma, angiography The images are similar to other vascular hamartomas. CDs dominated by plasma cell proliferation are thought to be associated with infection (mainly viral infection), inflammation, due to pathologically inflammatory changes such as plasma cells, immunoblasts. And capillary proliferation, while retaining the remaining lymph node structure; clinical signs of inflammatory lesions, such as chronic disease anemia, increased erythrocyte sedimentation rate, hypoalbuminemia and polyclonal immunoglobulin increased, etc. Is the originating factor of CD, such as the typical immunodeficiency disease - AIDS, can occur simultaneously with CD and Kaposi sarcoma, a small number of CD can also be converted into Kaposi sarcoma; clinically some patients with autoimmune blood cells Reduced, antinuclear antibody positive, rheumatoid factor positive or anti-human globulin test positive; some immunological tests showed partial CD patients with antigen reactivity Loss, T inhibits cell deficiency, etc. Some authors believe that CD is a pre-neoplastic lesion, because the plasma cell immunohistochemical staining of CD lesions is monoclonal, individual patients have single immunoglobulin in the blood, a few multi-center Patients can be converted to malignant lymphoma.

It has been reported that IL-6 is involved in the pathogenesis of CD. For example, IL-6 gene is transferred into hematopoietic stem cells of mice, and a pathological model similar to CD can be successfully obtained. It has also been confirmed that B lymphocytes of CD lymph node germinal center can be obtained. A large amount of IL-6 is secreted, and after the lesion is removed, the serum IL-6 level is also decreased as the clinical condition is improved. In addition, it is confirmed in animal experiments that human herpesvirus 8 (HHV-8), ie, card Posey sarcoma herpesvirus (KSHV) is involved in the pathogenesis of CD.

Castleman disease prevention

Prognosis: This disease is a focal lesion with a good prognosis. However, when the multicenter is accompanied by monoclonal hypergammaglobulinemia, the prognosis is poor, prone to malignant transformation, transformation or lymphoma.

Castleman disease complications Complications Kaposi's sarcoma thrombotic thrombocytopenic purpura

1. About one-third of patients can have Kaposi's sarcoma or B-cell lymphoma.

2. Combined with the nervous system, endocrine and renal lesions, may also be combined with Sjogren's syndrome (sjogren's syndrome), thrombotic thrombocytopenic purpura and so on.

Castleman's disease symptoms common symptoms keratitis hepatosplenomegaly low fever high fever lymph node enlargement

CD is clinically divided into focal type and multi-center type.

1. Focal young people are more common, the median age of onset is 20 years old, 90% of the pathology is transparent vascular type, the patient has a single lymph node painless swelling, slow growth, forming a huge mass, ranging from a few centimeters to About 20cm, can occur in any part of the lymphoid tissue, but the most common mediastinal lymph nodes, followed by the neck, hernia and abdominal lymph nodes, occasionally in extranodal tissues, such as the throat, vulva, pericardium, intracranial, subcutaneous, muscle, lung There are cases of eyelids, etc., most of which have no systemic symptoms. The tumor can survive for a long time after resection, which is a benign course. 10% of the pathology is plasma cell type. The abdominal lymph nodes are more common, often accompanied by systemic symptoms, such as long-term low fever or High fever, fatigue, weight loss, anemia, etc., the symptoms can be completely resolved after surgical resection, and no recurrence.

2. Multi-center type is rare compared with focal type, the age of onset is lower, the median age is 57 years old, the patient has multiple lymph nodes, easy to spread to superficial lymph nodes, with systemic symptoms (such as fever) and hepatosplenomegaly, Often multiple systemic manifestations, such as nephrotic syndrome, amyloidosis, myasthenia gravis, peripheral neuropathy, temporal arteritis, Sjogren's syndrome (sjogren's syndrome), thrombotic thrombocytopenic purpura and oral cavity, keratitis Sexual reaction, 20% to 30% of patients can be complicated with Kaposi's sarcoma or B-cell lymphoma in the course of the disease, a small number of patients with multiple neuropathy, organ enlargement (liver, spleen), endocrine lesions, serum individual Immunoglobulins and skin lesions constitute a clinical sign of POEMS syndrome. In addition, multicenter clinical manifestations are often invasive and prone to infection.

Castleman's disease check

1. Peripheral blood is mild to moderate positive cell anemia, and in some cases there are white blood cells and/or thrombocytopenia, which can also be manifested as typical chronic anemia.

2. In the bone marrow, some patients have elevated plasma cells, ranging from 2% to 20%, and the morphology is basically normal.

3. Blood biochemistry and immunological examination may be abnormal in liver function, which is characterized by elevated serum transaminase and bilirubin levels, renal function involvement in a small number of patients, elevated serum creatinine levels, and elevated serum immunoglobulins, more common, a few M protein appeared in serum, and erythrocyte sedimentation rate increased accordingly. Some patients were positive for anti-nuclear antibodies, rheumatoid factor and anti-human globulin test.

4. Urine routine urine protein is slightly elevated, such as with nephrotic syndrome, a large amount of urinary protein appears.

5. Histopathologically enlarged lymph node biopsy shows the special pathological changes of the above CD. The lesion mainly affects the lymphoid tissue in any part of the body, and even spreads the extranodal tissue. The pathology of CD is divided into the following two types:

(1) Transparent vascular type: 80% to 90%, many enlarged lymphoid follicular-like structures in the lymph nodes, scattered distribution, several small blood vessels penetrating into the follicles, the vascular endothelium is obviously swollen, and the wall is thickened. In the later stage, there is a glassy change. There are a large number of eosinophilic or transparent substances distributed around the blood vessels. The lymphocytes arranged around the follicles are formed by a plurality of layers of heart-shaped lymphocytes, forming a special onion-like structure or a cap-like band. More capillaries thickened capillaries and lymphocytes, plasma cells, immunoblasts, lymphatic sinus disappeared, or fibrosis, the general specimen see lymph node diameter of 3 ~ 7cm, the largest can reach 25cm, the weight of 700g.

(2) Plasma cell type: 10% to 20%, follicular hyperplasia is also shown in the lymph nodes, but the proliferation of lymphocytes around small blood vessels and follicles is far less than that of transparent blood vessels, and there is no typical onion skin sample. Structure, the main feature of this type is the proliferation of plasma cells between the follicles. The Russell body is small, and there are still a small number of lymphocytes and immunoblasts. Some people call this type a transparent vascular type active period, which can have TCR. Or IgH gene rearrangement, a small number of patients with multiple sites involving lymph nodes, and multiple extra-organ involvement, pathologically have the characteristics of the above two types, known as mixed type, there are also a few single lesions, pathologically have the above two types The characteristics are mixed in another sense. It has been reported that a small number of plasma cell type patients can be complicated with Kaposi's sarcoma, and it is more common in patients with AIDS.

6. According to clinical manifestations, symptoms, signs, X-ray, CT, B-ultrasound and electrocardiogram.

Diagnosis of Castleman's disease

Diagnostic criteria

The clinical manifestations of CD are non-specific. If the lymph nodes are obviously swollen, with or without systemic symptoms, the possibility of CD should be considered. The lymph node biopsy can be diagnosed by the typical CD pathological changes mentioned above, that is, the diagnosis of CD must have pathological evidence. Then according to the clinical manifestations and pathology, the classification diagnosis is made, and various possible related diseases should be excluded before diagnosis.

CD should be differentiated from malignant lymphoma, various lymph node reactive hyperplasia (mostly caused by viral infection), plasma cell tumor, AIDS and rheumatic diseases, they have some similar clinical manifestations and/or pathological changes, carefully The pathological examination, including immunohistochemical examination, and the detection of certain primary diseases are the identification points. The lymphadenopathy of this disease must be differentiated from the following diseases:

Differential diagnosis

1. Although there are lymph nodes in both lymphomas, the performances are different. Lymphomas may have persistent or periodic fever, generalized itching, splenomegaly, weight loss, etc., but the clinical symptoms of this disease are mild, only fatigue or dirty. The most common symptom of the symptoms after compression is the pathological difference. The histological features of this disease are significant vascular proliferation.

2. Angioimmunoblastic lymphadenopathy is an abnormal non-neoplastic immunoproliferative disease, clinically more common in women, manifested as fever, systemic lymphadenopathy, may have rash and itchy skin, auxiliary examination of white blood cells, increased blood sedimentation Fast, antibiotic treatment is ineffective, hormones can improve symptoms, lymph node pathology is lymph node destruction, capillary wall hyperplasia, is immune mother cells, vascular endothelial cells PAS positive, amorphous substance deposition, interstitial eosinophilic structure-free substance deposition , biopsy can be identified.

3. Primary macroglobulinemia is mainly caused by lymphoid plasma cell proliferation and secretion of a large number of monoclonal macroglobulins, and extensive infiltration of bone marrow and extramedullary organs, a large number of individual IgM in serum, no bone destruction, no Kidney damage, clinically liver, spleen, lymph nodes, about half with high viscosity.

4. Multiple myeloma is a common type of plasma cell disease. Plasma cells (or myeloma cells) proliferating in the bone marrow infiltrate bones and soft tissues, causing a series of organ dysfunction. Clinical manifestations include bone pain, anemia, and renal dysfunction. And immune dysfunction, hypercalcemia, myeloma cells infiltrating the liver and spleen, lymph nodes and kidneys, CD lymph nodes are obvious, lymph node biopsy can be identified.

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