Deeksha Grover,
Rao Moravineni,
Oluchi Idenyi,
Daniel Assaf,
Vishwanath Anil,
Madhu Bhargavi Chandra,
- MD, Department of Internal Medicine, Wellstar Spalding Regional Hospital, Griffin, GA 30224, United States
- MD, Department of Internal Medicine, Wellstar Spalding Regional Hospital, Griffin, GA 30224, United States
- MD, Department of Internal Medicine, Wellstar Spalding Regional Hospital, Griffin, GA 30224, United States
- MD, Department of Internal Medicine, Wellstar Spalding Regional Hospital, Griffin, GA 30224, United States
- MD, Department of Internal Medicine, Wellstar Spalding Regional Hospital, Griffin, GA 30224, United States
- MD, Department of Internal Medicine, Wellstar Spalding Regional Hospital, Griffin, GA 30224, United States
Abstract
Introduction: Primary plasma cell leukemia (pPCL) is a rare and aggressive plasma cell dyscrasia, defined by ≥5% circulating plasma cells, with poor prognosis and rapid progression. Case Presentation: A 66-year-old African–American female with no prior medical history presented with one week of fatigue, dehydration, and polydipsia. Laboratory evaluation revealed leukocytosis, severe thrombocytopenia, acute kidney injury (creatinine 4.03 mg/dL), and hypercalcemia (13.0 mg/dL) with suppressed PTH. Imaging showed intra-abdominal lymphadenopathy without hepatosplenomegaly. Myeloma workup demonstrated markedly elevated kappa free light chains (>3000 mg/L), abnormal kappa/lambda ratio (>197), and IgG kappa monoclonal protein. Peripheral smear showed 18% circulating plasma cells. Bone marrow biopsy revealed 45% clonal plasma cells with kappa restriction. Pleural fluid cytology confirmed malignant plasma cells, indicating extramedullary involvement. Cytogenetics showed chromosome 1p abnormality. A diagnosis of pPCL was made. The patient received carfilzomib-based quadruplet therapy and was referred for stem cell transplantation. Her course was complicated by tumor-lysis syndrome and renal failure. Discussion: pPCL is characterized by aggressive disease biology, high tumor burden, and frequent extramedullary involvement. Compared to multiple myeloma, patients often present with cytopenias and end-organ damage, though atypical presentations may occur, as in this case with isolated fatigue. Treatment strategies are evolving and include proteasome inhibitor-based induction regimens, anti-CD38 monoclonal antibodies, and early consideration for stem cell transplantation. Despite advances, outcomes remain poor, and there is no standardized treatment approach due to the rarity of the disease and exclusion from most clinical trials. Conclusion: pPCL can present subtly despite aggressive disease. Early recognition and prompt treatment are essential, and further research is needed to guide management.
Keywords: Plasma cell leukemia, multiple myeloma, hypercalcemia, monoclonal gammopathy, proteasome inhibitors
[This article belongs to Research and Reviews: Journal of Oncology and Hematology ]
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Research and Reviews: Journal of Oncology and Hematology
| Volume | 15 | |
| Issue | 02 | |
| Received | 20/03/2026 | |
| Accepted | 11/06/2026 | |
| Published | 31/08/2026 | |
| Publication Time | 164 Days |