In plasmocytoma (synonyms: Bence Jones plasmacytoma; Hupert’s disease; Kahler-Buzzolo disease; malignant plasmoma; Kahler’s disease; multiple plasma cell tumor; multiple myeloma (MM); multiple plasma cell myeloma; myeloma; myelomatosis; plasma cell myeloma; reticulosis plasmacellularis; medullary plasmacytoma; ICD-10 C90. 0: plasmocytoma [multiple myeloma]) is a malignant (malignant) systemic disease that is one of the non-Hodgkin’s lymphomas of B lymphocytes (B-cell lymphoma). Plasmocytoma (multiple myeloma) is associated with malignant neoplasia (new formation) of plasma cells and the formation of paraproteins (nonfunctional, (un)complete antibodies). Multiple myeloma is also called lymphoplasmocytic lymphoma. Multiple myeloma regularly arises from monoclonal gammopathy of undetermined significance (MGUS). It is the most common malignant neoplasm of bone (approximately one-third of biopsied bone tumors) and the second most common malignant hematologic disease (malignancies of the hematopoietic system) after chronic lymphocytic leukemia (CLL). Symptomatic multiple myeloma (plasmacytoma) is defined by the revised IMWG criteria as “the presence of monoclonal plasma cells in the bone marrow ≥ 10% or biopsy-confirmed plasmacytoma of bone or extramedullary (“outside the bone marrow“) manifestation and one of the following “myeloma-defining events” (see classification below).” A subtype of plasmacytoma is “smoldering multiple myeloma”. It is characterized by a slow progression and the absence of myeloma-typical skeletal changes, anemia and renal insufficiency. Bone marrow infiltration is <10% and paraprotein concentration is constant at 3 g/dL. Symptomatic multiple myeloma requires treatment in the presence of so-called CRAB criteria (“hypercalcaemia”, “renal failure“, “anemia“, “bone lesions”; hypercalcaemia, renal failure, anemia, skeletal changes). Sex ratio: Men are more frequently affected than women. Peak incidence: The maximum incidence of plasmacytoma is usually after the age of 45. The median age at diagnosis is 72 in men and 74 in women. The prevalence (disease incidence) is about twice as high in African Americans compared to white U.S. citizens. The incidence (frequency of new cases) is 6-8 cases per 100,000 population per year (in Germany). Course and prognosis: Typical for this disease is the occurrence of multiple (many) myelomas (tumor foci in the bone marrow). The course of the disease can be very inconsistent, ranging from precancerous (cancerous) to slowly progressive (progressive) to rapidly progressive.At the time of diagnosis, renal involvement is present in 20-50% of cases (renal insufficiency/process leading to a slowly progressive reduction in renal function). Renal insufficiency is mainly due to the toxic effects of monoclonal light chains on the basement membrane of glomeruli and/or renal tubules.Prognosis depends on age at diagnosis, general physical condition, presence of concomitant diseases, and response to therapeutic measures. Adequate therapy can reduce the size of the tumor or slow its growth. Complications can include spontaneous fractures (breaks) of the bones and vertebrae. Often, the immune system of the affected person is weakened.Plasmocytoma often occurs recurrently (recurring). With each recurrence, the likelihood of another recurrence increases. Complete remission (temporary or permanent remission of disease symptoms) is possible with conventional chemotherapy in only about 5 to 10% of cases. The combination of chemotherapy and autologous stem cell transplantation (blood stem cell transplantation) results in complete remission in > 40% of patients.Severe renal failure is associated with an increased risk of early death.Plasmacytoma is usually not curable from today’s perspective. The 10-year survival rate is approximately 20%.
