[Primary plasma cell leukemia. Report of five cases]

Area of Science:

• Hematology
• Oncology
• Clinical Research

Background:

• Primary plasma cell leukemia (pPCL) is a rare and aggressive hematologic malignancy.
• pPCL exhibits distinct biological characteristics compared to multiple myeloma (MM).
• Understanding pPCL features is crucial for improving patient outcomes.

Purpose of the Study:

• To characterize the clinical and biological features of patients diagnosed with pPCL.
• To compare the presentation and outcomes of pPCL with those of MM.

Main Methods:

• Retrospective review of patient data from Hematology Department and laboratory databases.
• Analysis included clinical parameters, laboratory values, bone marrow infiltration, and cytogenetic/FISH findings.
• Treatment regimens and survival data were also assessed.

Main Results:

• Five patients (2.8%) with pPCL were identified among 178 monoclonal gammopathy cases.
• Patients were aged 33-64 years, with significant anemia, leukocytosis (58% plasma cells), and thrombocytopenia.
• High bone marrow infiltration (89%), elevated LDH (2,003 IU/L), hypercalcemia (13 mg/dL), and renal impairment (creatinine 1.5 mg/dL) were observed. Two patients had bone lesions. Genetic analysis revealed complex karyotypes, TP53, and t(11;14) in some cases. Mean survival was 14 months.

Conclusions:

• Patients with pPCL in this cohort were notably younger than typical MM patients.
• pPCL presented with aggressive clinical features and a poor prognosis.
• The findings underscore the aggressive nature and distinct profile of pPCL.