IgM plasma cell myeloma in the era of novel therapy: a clinicopathological study of 17 cases
Autor: | Shaoying Li, Carlos E. Bueso-Ramos, Evgeniya Angelova, Guilin Tang, Wei Wang, L. Jeffrey Medeiros, Pei Lin |
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Rok vydání: | 2019 |
Předmět: |
Adult
Male 0301 basic medicine medicine.medical_specialty Anemia Gastroenterology Pathology and Forensic Medicine 03 medical and health sciences 0302 clinical medicine Immunophenotyping hemic and lymphatic diseases Internal medicine Plasma Cell Myeloma medicine Humans Aged CD20 Plasma cell leukemia biology CD117 business.industry Middle Aged medicine.disease Treatment Outcome 030104 developmental biology medicine.anatomical_structure Immunoglobulin M 030220 oncology & carcinogenesis biology.protein Proteasome inhibitor Female Bone marrow Multiple Myeloma business medicine.drug |
Zdroj: | Human Pathology. 84:321-334 |
ISSN: | 0046-8177 |
Popis: | IgM plasma cell myeloma (PCM) is a rare subtype of myeloma, and its response to novel therapies has not been fully characterized. We describe clinicopathological features and outcome of 17 patients with IgM PCM (11 men and 6 women) with a median age of 63 years. Patients presented with serum hyperviscosity (77%), bone lesions (71%), anemia (65%), renal dysfunction (53%), and hypercalcemia (35%). Median serum IgM level was 6.4 g/dL (0.7-12.1 g/dL). Bone marrow plasma cells (median, 80%; range, 20%-90%) were frequently of lymphoplasmacytic type (8/17; 47%). Immunophenotypically, the myeloma cells were positive for CD38, CD138, CD20 (5/16; 31%), CD56 (4/16; 25%), and CD117 (2/12; 17%); negative for CD19; and decreased or absent CD27 and CD81 in all cases. Seven (41%) patients had a complex karyotype, and fluorescence in situ hybridization showed CCND1-IGH (13/16; 81%), and deletions of 17p13/TP53 (29%) and 13q14/RB1 (38%). No MYD88 L265P mutation was detected. Most patients (94%) received proteasome inhibitor with or without immunomodulatory drug, 62% of patients required multiple regimens because of refractory disease, and 11 (65%) of 17 patients underwent autologous stem cell transplant (ASCT). The median OS was 67 months. After a median follow-up of 38 months (range, 3-106 months), only 5 patients achieved complete remission, 5 had persistent disease, and 7 died (2 progressed to plasma cell leukemia and 1 to blastic variant). In summary, IgM PCM is highly associated with t(11;14) and lymphoplasmacytic morphology. Patients are refractory to novel therapy and progression to high-risk myeloma is common, suggesting a need for alternative novel therapies. |
Databáze: | OpenAIRE |
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