primary monoclonal immunoglobulin disease
Introduction
Introduction to primary monoclonal immunoglobulin disease The primary monoclonal immunoglobulin disease is called "unexplained monoclonal immunoglobulin hyperplasia" and is also known as "benignmonoclonal immunoglobulinopathy" or "unknown monoclonal immunoglobulin" "monoclonal immunoglobulinopathy of unknown significance" (US). It is characterized by no malignant plasma cell disease or a disease that causes an increase in immunoglobulin, and the level of monoclonal immunoglobulin is limited and does not cause any clinical symptoms. basic knowledge The proportion of illness: this disease is rare, the incidence rate is about 0.0005% - 0.0009% Susceptible people: no special people Mode of infection: non-infectious Complications: lymphoma
Cause
Primary monoclonal immunoglobulin disease etiology
Causes:
The etiology is still unclear, and viruses, bacteria, tumors, autoantigens, etc. are stimulated as antigens, leading to excessive proliferation of monoclonal B cell-plasma cells, and the theory of secreting monoclonal immunoglobulins has not been confirmed.
Pathogenesis
The monoclonal B cell-plasma cell proliferation of this disease is limited, generally kept below the number of 1×1011 cells, and does not inhibit the proliferation of normal hematopoietic cells, does not inhibit the normal differentiation of B cell-plasma cells and the secretion of immunoglobulin, nor causes Osteolytic lesions, cell morphology and normal mature plasma cells are no different, therefore, the monoclonal B cell-plasma cell proliferation of this disease and malignant plasma cell disease (such as multiple myeloma) monoclonal B cell-plasma malignant proliferation Both quantity and quality are different.
Prevention
Primary monoclonal immunoglobulin disease prevention
Prevention: There is no effective preventive measure for this disease. Avoiding the irritating and accidental damage of adverse factors can play a certain preventive role. Early detection, early diagnosis and early treatment are the key to prevention and treatment of this disease. Once the disease occurs, it should be actively treated to prevent the occurrence of complications.
Complication
Primary monoclonal immunoglobulin disease complications Complications lymphoma
At present, there are no reports of complications of this disease! In case of onset, active treatment should be actively carried out to prevent secondary damage.
Symptom
Symptoms of primary monoclonal immunoglobulin disease Common symptoms Bone marrow plasma cell increase serum immunoglobulin can increase erythrocyte sedimentation rate
Primary monoclonal immunoglobulin disease, characterized by no malignant plasma cell disease or a disease that causes an increase in immunoglobulin, has a limited increase in monoclonal immunoglobulin levels and does not cause any clinical symptoms.
Examine
Examination of primary monoclonal immunoglobulin disease
1. Peripheral blood, blood routine is associated with associated diseases, red blood cells, hemoglobin, white blood cells, platelets normal or slightly lower.
2. Serum protein examination Serum protein electrophoresis is shown in the gamma region or fast gamma region, the region has M protein, and the M protein concentration is generally lower than 3 g/L (IgG<2g/L, IgA, IgM).
3. Urine-week protein is mostly negative or small.
4. ESR can increase.
5. Bone marrow examination Bone marrow smear examination is about the normal range, plasma cells can be slightly increased, but the morphology is close to normal, the number is <10%.
According to the condition, clinical manifestations, symptoms, signs, choose to do ECG, B-ultrasound, X-ray, CT, biochemical blood glucose and other tests.
Diagnosis
Diagnosis and identification of primary monoclonal immunoglobulin disease
diagnosis
Diagnosis of this disease should meet the following conditions: 1 no clinical symptoms or signs; 2 no anemia without bone destruction, no reduction of normal immunoglobulin; 3 serum monoclonal IgG <30g / L or monoclonal IgA <15g / L, IgM <15 g/L light chain urinary-peripherin.
Differential diagnosis
The disease must be differentiated from secondary monoclonal immunoglobulinemia, malignant plasma cell disease Waldenstrom macroglobulinemia, especially with multiple myeloma.
It should be pointed out that this disease is more difficult to distinguish from "pre-myeloma". Pre-myeloma refers to the occurrence of monoclonal immunoglobulin in the blood of some patients with multiple myeloma for several years or even more than ten years. The clinical manifestations of multiple myeloma, the clinical manifestations of multiple myeloma after the pre-myeloma period, due to the clinical manifestations of myeloma in the early stage of myeloma, the items listed in the table can not be used as identification indicators, recent studies pointed out Several tests are helpful to identify the disease and pre-myeloma:
1 Determination of plasma cell DNA synthesis rate, the plasma cell labeling index (PCLI), the bone marrow was incubated with 3H-labeled thymidine, counting 500 plasma cells, calculating the percentage of the total number of plasma cells in the labeled plasma cells, the disease PCLI was normal (0-0.4%), and the pre-myeloma was often 0.4%.
2 In the early stage of myeloma, more than 60% of the plasma cells contained the "J" chain, and the plasma cells containing the "J" chain in this disease were less than 35%.
3 serum 2-microglobulin is normal in this disease, and often higher than normal in the early stage of myeloma.
4 The level of monoclonal immunoglobulin can remain unchanged for many years in this disease, but it can increase slowly in the early stage of myeloma. In clinical practice, it emphasizes the long-term follow-up of patients with this disease, and regularly checks the above indicators, especially It is simple and easy to use 3 and 4 items, which will distinguish the disease from the pre-myeloma.
