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Primary Plasma Cell Leukemia Revealed by a Mandibular Lesion: A Case Report
Houda Youssefi1,2, Maryame Ahnach1,2, Mounia Bendari1,2
1Department of Hematology, Cheikh Khalifa International University Hospital, Casablanca, MAR.
Cureus
|January 10, 2022
Summary
Primary plasma cell leukemia (PCL) is a rare, aggressive blood cancer. This case highlights a patient with PCL presenting with oral lesions, achieving remission with VTD chemotherapy and stem cell transplant, but later relapsing.
Area of Science:
- Hematology
- Oncology
Background:
- Primary plasma cell leukemia (PCL) is a rare and aggressive hematological malignancy.
- PCL is characterized by a high percentage of plasma cells in the peripheral blood or bone marrow.
Observation:
- A 37-year-old woman presented with a two-month history of an oral mass and weakness.
- Physical examination revealed a maxillary gingival mass; CT confirmed a solid tissue mass.
- Biopsy showed plasma cell infiltration, with laboratory findings of anemia, renal impairment, and a monoclonal IgG lambda peak.
Findings:
- Peripheral blood smear showed 25% plasma cells, and bone marrow aspiration revealed 50% plasma cell infiltration, confirming PCL.
- The patient achieved complete remission after VTD chemotherapy (bortezomib, thalidomide, dexamethasone) and autologous stem cell transplant (ASCT).
- Relapse occurred at six months with extramedullary lesions despite rescue therapy, indicating a short-lived response.
Implications:
- This case underscores the aggressive nature of PCL and the need for heightened clinical awareness.
- Early recognition of associated clinical features is crucial for timely diagnosis and management.
- Despite initial treatment success, the prognosis remains poor with a median survival below one year.
Keywords:
extramedullary lesionsnovel immunomodulatory agentsplasmacytomaprimary plasma cell leukemiarelapse
