Primary macroglobulinemia

Introduction to primary macroglobulinemia Primary macroglottinemia (PM), also known as macroglobulinemia. It is a malignant proliferative disease of plasmacytoid lymphocytes that secretes a large amount of monoclonal IgM (macroglobulin), which often involves bone marrow, lymph nodes and spleen. The main clinical manifestation is hyperviscosity caused by macroglobulin. The cause of this disease is unknown. More than 50 years old, males account for about 2/3. basic knowledge The proportion of illness: 0.0006%-0.0008% Susceptible people: more than 50 years old, males account for about 2/3. Mode of infection: non-infectious Complications: anemia, renal insufficiency, nephrotic syndrome

Cause of primary macroglobulinemia

Cause:

Not clear, may be related to heredity, chronic infections and some cancer diseases.

Pathogenesis

Cytogenetic studies, 89% of patients have clonal changes, but abnormally versatile or complex, involving changes in chromosomes 2, 4 and 5, or monomers 16, 18, 19, 20, 21 and 22, in addition to three bodies 12. It is considered to be a karyotype change of primary macroglobulinemia.

Immunological studies, immunofluorescence analysis of living cells and fixed cells of bone marrow and peripheral blood samples, confirmed that there are a large number of lymphoid cells with surface IgM and showed significant pleomorphism. In bone marrow specimens, the number of fluorescent spots Size and brightness vary widely between cells. Unlike CLL, peripheral blood lymphocytes do not increase, but many lymphocytes have SIgM and SIgD. Monoclonal antibody studies indicate that the composition of this monoclonal malignant cell contains There are B cells at various developmental stages, cell markers from lymphoid progenitor cells, pro-B cells (CD19), pre-B cells (SIg-) and B cells (SIg+, such as CD24), mature B cells (CD20), Even plasma cell (PCA-1) antigens can be expressed on monoclonal B cells, with a variety of different CD45 subtypes, from less differentiated CD45RA to CD45RO (late B cells), in addition to CD5, CD10 antigens. And adhesion cells and adhesion-related molecules such as CD116 and CD9, unlike myeloma, there are a large number of heterogeneous tumor cell populations in the blood. Flow cytometry studies have shown that some morphologically seemingly normal B fine , Monoclonal protein may be expressed, as the disease progresses, abnormal cell morphology visible gradually increased, the number of monoclonal B-cell population are often associated with disease in addition to reduce CD4, CD4 / CD8 ratio decreases or inverted.

Primary macroglobulinemia prevention

There is no effective preventive measure for this disease. Early detection and early diagnosis are the key to the prevention and treatment of this disease.

Primary macroglobulinemia complications Complications anemia renal insufficiency nephrotic syndrome

The most common complication is infection with fever, mostly pulmonary infection, anemia caused by anemia, renal insufficiency, nephrotic syndrome, neurological disease, hepatosplenomegaly and so on.

Symptoms of primary macroglobulinemia Common symptoms Lymph node enlargement Hepatosplenomegaly hemorrhagic tendency Amyloid degeneration Heart failure coma

The course of the disease is slow, no clinical symptoms in the early years, and then anemia, lymph nodes and hepatosplenomegaly, high-viscosity syndrome, and a large increase in monoclonal IgM in the blood causes a significant increase in blood viscosity and leads to high viscosity. Hemorrhagic syndrome, manifested as dizziness, headache, visual impairment, peripheral nerve and / or central nervous system damage, bleeding tendency, severe cases may appear congestive heart failure, coma, Raynaud's phenomenon and amyloidosis found in some patients, osteolytic The lesion is only seen in individual patients.

Examination of primary macroglobulinemia

1. Peripheral blood is anemia, and there may be white blood cells and thrombocytopenia. A small amount of plasma cell-like lymphocytes may appear in peripheral blood smears, and red blood cells are often arranged in a money-like manner.

2. ESR is significantly increased.

3. Bone marrow shows diffuse hyperplasia of plasmacytoid lymphocytes, often accompanied by lymphocytes, plasma cells, and tissue basophils.

4. Serum protein electrophoresis showed the presence of M component in the region. The M component was further identified by immunoelectrophoresis as monoclonal IgM. The immunoglobulin quantification method can determine the monoclonal IgM content.

5. According to the condition, clinical manifestations, symptoms, signs, CT, MRI, chest X-ray, B-ultrasound, electrocardiogram, etc.

6. Pathological examination of bone marrow pathology showed lymphoid cell infiltration, diffuse or nodular type, lymph node pathology showed polymorphic cell infiltration, lymph nodes and peripheral fat showed atypical lymphocytes, monocytes, plasma cells and lymphoplasmacytic cells Etc., there are PAS positive substances in these cytoplasm.

Diagnosis and differentiation of primary macroglobulinemia

diagnosis

1. Diagnostic criteria

(1) elderly patients have anemia, bleeding tendency, hepatosplenomegaly, and high-viscosity syndrome.

(2) The monoclonal IgM in the serum is >10 g/L.

(3) Bone marrow, lymphoid plasma cell infiltration in the liver and spleen lymph nodes. Monoclonal IgM>10g/L in serum and lymphoid plasma cell infiltration in bone marrow are necessary for the diagnosis of this disease.

2. Diagnostic evaluation

(1) The disease is an senile disease: age is one of the conditions that must be considered when diagnosing this disease. The diagnosis of this disease should be carefully considered for patients under 40 years of age.

(2) The disease is clonal plasma cell disease: synthesized by clonal plasma cells, the secreted IgM must be monoclonal, so the large amount of IgM in the serum of this disease must be monoclonal IgM, the disease is a malignant plasma cell In the disease category, although the course of disease is a chronic process, but it has continuous progressive characteristics, its IgM not only has a monoclonal characteristic, but also shows an increasing trend, while normal immunoglobulin (polyclonal IgM, IgG, IgA, etc.) Synthesis and secretion are inhibited and show a downward trend.

(3) The primary site of the disease is in the bone marrow: lymphoid plasma cells are lesional plasma cells, so lymphoid plasma cell infiltration in the bone marrow is one of the necessary basis for the diagnosis of this disease.

(4) The course of the disease progresses rapidly, and the severity is very different among different patients: the clinical manifestations are also different in different patients, therefore, anemia, bleeding tendency, high-viscosity syndrome, amyloidosis, Raynaud's phenomenon, hepatosplenomegaly, and osteolytic lesions may or may not be present in different patients. It may be light or heavy in different stages of disease, so it cannot be used as a basis for diagnosing this disease. It can only be used to judge the severity of the disease, and the disease is sooner or later. Based on.

Differential diagnosis

1. Secondary macroglobulinemia Secondary macroglobulinemia is increased by polyclonal IgM and the level of increase is limited. If immunoelectrophoresis confirms that the increased IgM is polyclonal by protein electrophoresis, it can be diagnosed as Primary macroglobulinemia, however, a small number of secondary macroglobulinemias are monoclonal, and the main points for identifying such secondary macroglobulinemia and Waldenström macroglobulinemia are:

1 Secondary macroglobulinemia has obvious clinical manifestations of its primary disease (chronic lymphocytic leukemia, lymphoma, rheumatoid arthritis, etc.).

2 secondary macroglobulinemia without the characteristics of lymphoid plasma cell infiltration in the bone marrow.

3 The level of monoclonal IgM in secondary macroglobulinemia is limited, and often does not have the characteristics of continuous increase.

4 Normal polyclonal immunoglobulin levels generally remain normal in secondary macroglobulinemia.

2. Identification of the disease and multiple myeloma IgM type

1 In the bone marrow of multiple myeloma, myeloma cells (primary or immature plasma cells) are infiltrated, and the bone marrow of this disease is lymphoid plasma cell infiltration.

2 osteolytic lesions are common in multiple myeloma, but rare in this disease (only about 2%).

3 Renal dysfunction is common in multiple myeloma, but it is rare in this disease. The first point in the above 3 points is the most critical.

3. The main points of this disease and the significance of the identification of monoclonal IgMemia

1 Unknown meaning Monoclonal IgMemia (MGUS) has no clinical symptoms, and the disease has anemia, hemorrhage, hepatosplenomegaly, hypertrophic blood syndrome and other clinical manifestations.

2MGUS has a limited increase in monoclonal IgM (generally <15g/L), and it has remained unchanged for many years, and the monoclonal IgM of this disease is continuously increasing.

In the bone marrow of 3MGUS, the normal form of plasma cells is increased and the number is increased. In the bone marrow of this disease, lymphoid plasma cells are infiltrated and progressing. It should be noted that some IgM type MGUS can be transformed into Waldenström after many years of development. Macroglobulinemia.

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