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Home > West Medicine > Internal Medicine > Hematology
C88.000x001

Macroglobulinemia

血液科

Common symptoms

血液粘滞度增高、出血倾向、淋巴结肿大、慢性肾功能不全、视力障碍、血小板减少、乏力、消瘦

The pathology of disease

Abnormal increase of IgM in blood = macroglobulinemia; divided into primary and secondary. Primary (Waldenstrom macroglobulinemia) = unexplained monoclonal IgM elevation. Secondary = monoclonal/polyclonal IgM elevation secondary to other diseases. Clinical features: elderly onset, anemia, bleeding tendency, hyperviscosity syndrome. Diagnosis: large monoclonal IgM + lymphoplasmacytic bone-marrow infiltrate. Chronic course; asymptomatic cases need no chemo; progressive disease = chemo.

Common Symptoms

Elevated blood viscosity; bleeding tendency; lymph-node enlargement; chronic renal insufficiency; visual disturbance; thrombocytopenia; fatigue; weight loss

Causes and Pathogenesis

~2% of hematologic malignancies; more common in Caucasians; only 5% in African ancestry. Familial clustering; ~20% first-degree relatives have B-cell disorders (hypo/hypergammaglobulinemia, autoantibodies). Environmental/viral causes uncertain; HCV/HHV-8 association debated. Cytogenetics: numeric abnormalities (loss of 17/18/19/20/21/22/X/Y; gain of 3/4/12); 6q21-22 deletion in 40-90% (tumor-suppressor region, BLIMP-1). No IgH switch recombination (distinguishes from IgM myeloma). Bone-marrow clonal B cells: small lymphocytes → lymphoplasmacytic → plasma cells.

Treatment

Drug, supportive therapy

Preventive Measures

Asymptomatic: observation. Symptomatic: early treatment to reduce mortality/extend survival.

Department to Visit

It is recommended to visit the Hematology department; an appointment can be booked on this platform before the visit.

Warm reminder: The above information is for health science reference only and cannot replace professional medical diagnosis. If you have related symptoms, please seek medical attention at a regular hospital promptly.