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Smouldering myeloma is a disease classified as intermediate in a spectrum of step-wise progressive diseases termed plasma cell dyscrasias.In this spectrum of diseases, a clone of plasma cells secreting monoclonal paraprotein (also termed myeloma protein or M protein) causes the relatively benign disease of monoclonal gammopathy of undetermined significance.
In hematology, plasma cell dyscrasias (also termed plasma cell disorders and plasma cell proliferative diseases) are a spectrum of progressively more severe monoclonal gammopathies in which a clone or multiple clones of pre-malignant or malignant plasma cells (sometimes in association with lymphoplasmacytoid cells or B lymphocytes) over-produce and secrete into the blood stream a myeloma ...
Tyrosine kinase inhibitors like imatinib have improved the prognosis of CML patients to near-normal life expectancy. [14] Recently, a JAK2 inhibitor, namely ruxolitinib, has been approved for use in primary myelofibrosis. [15] Trials of these inhibitors are in progress for the treatment of the other myeloproliferative neoplasms.
Secondary PCL (sPCL) is diagnosed in 1-4% of patients known to have had multiple myeloma for a median time of ~21 months. It is the terminal phase of these patients' myeloma disease. sPCL patients typically are highly symptomatic due to extensive disease with malignant plasma cell infiltrations in, and failures of, not only the bone marrow but also other organs.
The Epstein-Barr virus, which infects >90% of the world population, is also a common cause of these disorders, being responsible for a wide range of non-malignant, pre-malignant, and malignant Epstein-Barr virus-associated lymphoproliferative diseases. [5]
However, patients with these autoimmune disorders who have very small B cell clones either never develop a lymphocyte malignancy or, rarely, do so and only after many years. Consequently, it is now widely recognized that such cases, when associated with very small numbers of monoclonal B-cells, are best diagnosed as CLL/SLL MBL with autoimmune ...
Waldenström macroglobulinemia is characterized by an uncontrolled clonal proliferation of terminally differentiated B lymphocytes. The most commonly associated mutations, based on whole-genome sequencing of 30 patients, are a somatic mutation in MYD88 (90% of patients) and a somatic mutation in CXCR4 (27% of patients). [11]
Multiple myeloma (MM), also known as plasma cell myeloma and simply myeloma, is a cancer of plasma cells, a type of white blood cell that normally produces antibodies. [6] Often, no symptoms are noticed initially. [10] As it progresses, bone pain, anemia, renal insufficiency, and infections may occur. [10]
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