A case of Castleman’s disease was seen in the clinic yesterday, and because it was solitary, the patient was told that surgery should be the main focus and postoperative observation should be separated from the HHV8 (human herpesvirus)-associated multicentricity of castleman’s disease, which is classified as a subclass of DLBCL.
Castleman’s disease (CD) is one of the reactive lymphadenopathies of unknown origin and is clinically rare. Its pathology is characterized by distinct lymphatic follicles, blood vessels and plasma cells in varying degrees of hyperplasia, and is characterized clinically by significant enlargement of deep or superficial lymph nodes.
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 lymphatic follicles and capillaries called vascular follicular lymphnode hyperplasia (VFLH). 1969 Flendring and Schillings proposed another morphologic subtype of CD, characterized by plasma cell hyperplasia, often with systemic symptoms. It is also known as giant lymph node hyperplasia because the enlargement of lymph nodes is often very pronounced, sometimes reaching 10 cm or more in diameter.
Etiology
The etiology of CD health search is unknown. Some authors have suggested that abnormal immune regulation is the initiating factor of CD. 25% of central cases are clinically confirmed to be associated with HHV-8 infection, and it is also believed that at least some CD is at risk of B-cell malignant proliferation, and a few multicentric types can be transformed into malignant lymphoma. However, most cases are not followed up to malignancy.
Symptoms
CD is clinically classified into focal and multicentric types.
Focal type is more common in young people, with a median age of 20 years. 90% of the pathologies are hyaline vascular. 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, wasting and anemia, etc. The symptoms can all subside after surgical resection and do not recur.
2.Multicentric type is less common than focal type, with a later age of onset and a median age of 57 years. Patients have multi-located lymph node enlargement easily involving superficial lymph nodes. With systemic symptoms (such as fever) and hepatosplenomegaly, there are often manifestations of multisystem involvement such as nephrotic syndrome, amyloidosis, myasthenia gravis, peripheral neuropathy, temporal arteritis, Schegren’s syndrome (dry syndrome), thrombotic thrombocytopenic purpura and inflammatory reactions of the oral cavity and cornea in 20% to 30% of patients with Kaposi’s sarcoma or B-cell lymphoma. 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 form often has an aggressive clinical course and is susceptible to concomitant infections.
Complications.
1.About 1/3 of patients may have Kaposi’s sarcoma or B-cell lymphoma.
2. Combination of neurological, endocrine and renal pathologies, as well as Schegren’s syndrome (dry syndrome), thrombotic thrombocytopenic purpura, etc.
Examination
Laboratory tests.
1, peripheral blood Mild to moderate orthocytic orthochromic anemia health search, some cases with leukocytosis and (or) thrombocytopenia can also be manifested as a typical firecan.com chronic disease anemia.
2, bone marrow image Some patients have elevated plasma cells ranging from 2% to 20%, and the morphology is basically normal.
3, blood biochemical and immunological examination liver function can be abnormal, manifested as serum aminotransferase and bilirubin levels rise a few patients with renal involvement serum creatinine levels rise 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.
Other ancillary tests.
1, Histopathology Biopsy of enlarged lymph nodes shows specific pathological changes of CD as described above. The lesions mainly involve lymphoid tissue anywhere in the body and may occasionally spread to extra-nodal tissues CD pathology is divided into two types as follows.
(1) hyaline vascular type: 80% to 90% of healthy search lymph nodes show many enlarged lymphatic follicle-like structures in a scattered distribution. There are several small vessels penetrating into the follicles, with marked swelling of the endothelium, thickening of the vessel wall, 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 thickened capillaries and lymphocytes, plasma cells, and immunoblasts between the follicles, and the lymphatic sinuses disappeared or were fibrotic. Large specimens were seen with lymph nodes of 3-7 cm in diameter, with larger ones up to 25 cm and weighing up to 700 g.
(2) Plasma cell type: 10% to 20% of the cases. The lymph nodes also show follicular hyperplasia but the lymphocytic hyperplasia around the small vessels and follicles is much less obvious than that of the hyaline vascular type, and there is generally 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. It has been described as the active phase of the hyaline vascular type and may have TCRβ or IgH gene rearrangement. In a small number of patients, the lesion involves multiple lymph nodes with extra-nodal multi-organ invasion, and the pathology is characterized by both of these types. A small number of patients with a single lesion that has both of these pathological features are also known as mixed type in another sense. It has been reported that a few patients with plasma cell type can have Kaposi’s sarcoma, and it is more common to have AIDS with CD.
2. X-rays, CT, ultrasound and electrocardiogram are selected according to clinical manifestations, symptoms and signs.
Related tests.
> Coombs test
> Monoclonal gammaglobulin
> anti-nuclear antibody
> Plasma cells
> rheumatoid factor
> creatinine anhydride
> protein quantification (urine)
> Platelets
> Blood sedimentation
Treatment
All focal CD should be surgically removed, and the majority of patients can survive for a long time with few recurrences. Focal CD that is pathologically plasmacytic in nature may disappear rapidly after removal of the diseased lymph nodes if systemic symptoms are present.
For multicentric CD, if the lesion only affects a few sites, it can be removed surgically, and chemotherapy or radiotherapy can be added after surgery. Chemotherapy is usually chosen as a combination chemotherapy regimen for malignant lymphoma. Autologous hematopoietic stem cell transplantation is also a treatment option.
Prognosis
The prognosis is good in focal lesions, but poor in multicentric with monoclonal hypogammaglobulinemia, which predisposes to malignant transformation or lymphoma.