Abstract | March 24, 2024
Rare Case of Lymphoma
Learning Objectives
- Lymphoplasmacytic malignant lymphoma is an extremely rare neoplasm. in rare cases, it can become an aggressive immunoblastic variant or other high grade lymphoma.
- It is commonly associated with Waldenstron macroglobulinemia. It presents with symptoms related to bone marrow infiltration and IgM monoclonal gammopathy.
- It is diagnosed by identifying small malignant lymphocytes in the bone marrow that are usually admixed with plasma cells, plasmacytoid lymphocytes, and/or mast cells.
- Overall, lymphoplasmacytic lymphoma evaluation in the bone marrow and lymph node is a diagnostic challenge due to the variability of presentation and the wide differential diagnosis related to plasmacytoid differentiation.
- Lymphoplasmacytic lymphoma usually shows immunoglobulin gene rearrangement and somatic hypermutation.
- Although no specific chromosomal abnormality has been reported in lymphoplasmacytic lymphoma, the presence of an MYD88 L265P mutation can favor the diagnosis.
Case Presentation: 69 year old female presented with increased weakness, fatigue and overall generalized malaise with severe anemia, thrombocytopenia. On presentation hemoglobin 4.1 received at least 5 units of packed red cells. She has had anemia and thrombocytopenia for at least 2 years. She also had elevated indirect bilirubin. She was worked up for anemia and found to have autoimmune hemolytic anemia, warm IgG. Coombs test Direct as strongly positive. She was also found to have a non clinically significant cold antibody. Labs were significant for LDH 620, Haptoglobin < 10, Retic ct 10.65%, ARC 250,000, Antibody screen POSITIVE. Lab reported Cold agglutinins to be present in the blood specimen. Peripheral smear had a lot of polychromasia. Schistocytes were NOT present in the blood. Zn , Cu, B12, HIV, ANA, RF all were negative or Normal. In the serum we found her to have MGUS with IgM Kappa. Ig M was 732. She also had a mild thrombocytopenia. She was initially treated with 5 days of Solumedrol followed by Prednisone 80mg po every day. She had a bone marrow biopsy as recommended.
Final /working diagnosis: A bone marrow was ordered study showed a MYD88-lymphoplasmacytic lymphoma. MYD88-mutated lymphoplasmacytic neoplasm most consistent with lymphoplasmacytic lymphoma (10-15% involvement) involving a slightly hypercellular bone marrow for age (50%) with trilineage maturation. Flow cytometric immunophenotyping performed at Molecular Pathology Laboratory detected two abnormal populations, one lymphoid and one plasma cell. The abnormal B-cell population (13% of sample) expressed CD45, CD19, CD20, and surface kappa light chain. It was negative for CD5 and CD10. The aberrant plasma cell population (0.1%) expressed CD45, CD19, CD56-/+, and cytoplasmic kappa. The granulocytes demonstrated an increased proportion of mature forms indicative of hemodilution.
Management/Outcome: She was treated with CyBorD C1D1. She is currently treated with Velcade, Cytoxan D1,8 and 15 every 28 days with combination of Rituxan. Currently following up with Hem-Oncology closely.
References and Resources
- https://www.uptodate.com/contents/classification-of-hematopoietic-neoplasms?search=lymphoplasmacytic%20lymphoma&source=search_result&selected
Title=5~32&usage_type=default&display_rank=5 - https://journalgrid.com/view/article/rjms/12433469
