Overview of Waldenstroms macroglobulinemia
Renal lesion of Waldenstroms macroglobulinemia (WMG) refers to a renal lesion caused by a malignant disease of B lymphocytes characterized by the presence of large amounts of monoclonal gammaglobulin (IgM) and plasma cells in the blood, and a lymphomatous bone marrow infiltration. Fahrenheit’s macroglobulinemia, also known as primary macroglobulinemia, is a malignant proliferative B-lymphocyte disorder characterized by the presence of large amounts of monoclonal macroglobulin (IgM) in the blood. The mainstay of treatment for renal damage in Fahrenheit’s macroglobulinemia is symptomatic treatment, with glucocorticoid therapy, combined with alkylating agents where appropriate, if nephrotic syndrome or acute renal failure manifests.
Etiology
Renal damage due to Wahl’s macroglobulinemia is mainly due to renal lymphoid cell infiltration; hyperviscosity; amyloidosis; and immune-mediated glomerulonephritis, mostly IgM deposition in renal tissue.
Symptoms.
The clinical features of Wahl’s macroglobulinemia are enlarged lymph nodes, hepatosplenomegaly, proliferation of lymphocytes and plasma cell-like lymphocytes in the bone marrow and lymph nodes, and increased monoclonal IgM in the blood. Renal damage in Wahl’s macroglobulinemia is characterized by proteinuria, nephrotic syndrome and renal failure, hypertension and hematuria are rare, and both kidneys tend to increase in size. Proteinuria is mostly mild-moderate, occasionally progressing to nephrotic syndrome range of proteinuria, with reduced glomerular filtration rate, azotemia, and tubular concentrating dysfunction.
Examination
1. Blood test
There is orthochromatic normocytic anemia, with decreased erythrocyte deformability index, stringing of erythrocytes in the form of coils of money, and there may be a decrease in whole blood cells, and a small number (< 5%) of atypical naïve plasma cells, i.e., small lymphocytes or plasma cell-like lymphocytes, may be present in the peripheral blood; the blood sedimentation rate is increased; the M protein can be seen in the electrophoresis of the serum proteins, and the rate of the appearance of the M protein is higher with the increase in the patient’s age, and in patients greater than 50 years of age, the M protein The rate of elevation of M protein is 3.5% in patients older than 50 years and 11% in those aged 80 to 90 years. immunoprotein fixation electrophoresis reveals a marked increase in monoclonal IgM, which is elevated in all patients and is considered a circulating tumor marker. the Sia test is a rapid screening test for macroglobulinemia, in which a drop of patient serum is placed in a tube of distilled water and precipitation occurs immediately. in addition, more than 10% of macroglobulins have cold-precipitating properties and are jelly-like when cold. In addition, more than 10% of the macroglobulins have cold precipitation properties and appear jelly-like when cold.
2. Bone marrow examination
Abnormal plasma cell-like lymphocyte proliferation can be seen, and these lymphocytes have the characteristics of secretory cells, such as abundant rough endoplasmic reticulum for synthesizing and secreting immunoglobulin and well-developed Golgi apparatus.
3. Karyotype examination
Primary macroglobulinemia may be a variant of B-cell lymphoma.
4. Urinalysis
Proteinuria and hematuria can be seen when there is renal damage, proteinuria is mostly mild-moderate, and occasionally it can develop into proteinuria in the range of nephrotic syndrome, glomerular filtration rate decreases, azotemia occurs, tubular concentrating dysfunction occurs, and when there is a large number of lymphocyte infiltration, immunofluorescence microscope can find that IgM and plasma cell-like cells often appear in the urine, and the positivity rate of urinary immunoprotein fixation electrophoresis can be as high as 90%, and urinary light chain protein positivity can be up to 90%. The positive rate of urine immunoprotein fixed electrophoresis can be as high as 90%, and the positive rate of urine light chain protein is 30%~50%.
5. Pathologic examination
Thrombosis in glomerular capillaries, the thrombus contains a lot of IgM and less fibrin, so it is also called “pseudo-thrombosis”, there are IgM deposits on the inner side of the glomerular basement membrane, which are highly eosinophilic under the light microscope, and the PAS staining is dark purple, and the Tichome staining is red or green, in addition, there are also positive deposits of PAS staining on glomerular tunica, and there are positive deposits of tunica. In addition, there are PAS-positive deposits in the glomerular mesangial area, and the mesangium is nodular, which is not easy to distinguish from diabetic glomerulosclerosis under the light microscope.
6. Imaging
If secondary amyloidosis is present, both kidneys are markedly enlarged, and bone imaging is usually free of osteolytic lesions.
Diagnosis
1. Diagnostic basis of Huperzine macroglobulinemia
(1) Typical signs and symptoms: patients have unexplained anemia and bleeding tendency, central and/or peripheral nervous system symptoms, visual impairment, Raynaud’s phenomenon, liver and spleen lymph node enlargement.
(2) Serum IgM monoclonal gammaglobulin concentration > 30 g/L.
2. Diagnostic basis of renal damage caused by macroglobulinemia
On the basis of Fahrenheit’s macroglobulinemia, the presence of proteinuria, hematuria and renal function abnormalities can be diagnosed.
Treatment
The main points of treatment for renal damage in Fahrenheit’s macroglobulinemia are:
1. Treatment of Macroglobulinemia
If the condition is stable and the symptoms are mild in the early stage, intensive treatment should not be given only on the basis of abnormalities in blood and bone marrow tests because the disease is slow in onset and can remain stable for a long period of time. Symptomatic treatment, chemotherapy, and bone marrow transplantation should be given to patients with anemia, bleeding, and hyperviscosity syndrome.
2. Treatment of renal damage
If there is no indication for the treatment of macroglobulinemia, but the patient has nephrotic syndrome or acute renal failure, glucocorticoids may be given, and alkylating agents may be used as appropriate.