Primary plasma cell leukaemia in a 39-year-old man

Area of Science:

• Hematology
• Oncology

Background:

• Plasma cell leukaemia (PCL) is a rare and aggressive hematological malignancy.
• PCL is categorized into primary (pPCL) and secondary forms.
• Early diagnosis and intervention are critical due to the typically poor prognosis.

Observation:

• A 39-year-old male presented with fever and lethargy, exhibiting pallor and febrile symptoms.
• Blood tests revealed bicytopenia with leucocytosis, and peripheral blood film showed rouleaux formation and 45% circulating plasma cells.
• Diagnostic workup, including serum protein electrophoresis, immunofixation, bone marrow aspirate, flow cytometry, and trephine biopsy, confirmed IgG lambda primary plasma cell leukaemia.

Findings:

• The patient received six cycles of bortezomib, thalidomide, and dexamethasone chemotherapy.
• High-dose melphalan conditioning followed by autologous stem cell transplant was performed.
• The patient achieved complete remission lasting 18 months and is currently on lenalidomide maintenance therapy.

Implications:

• Aggressive treatment regimens, including novel agents and stem cell transplantation, can induce remission in pPCL.
• Prompt initiation of therapy is crucial for improving survival outcomes in primary plasma cell leukaemia.
• This case highlights the potential for successful management of a dismal prognosis malignancy with modern therapeutic approaches.