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<p>Primary plasma cell leukemia (pPCL) is an aggressive and rare form of plasma cell dyscrasia, requiring early detection and rapid intervention and is characterized by presence of 5% or more circulating plasma cells. Despite advances in diagnosis and management, the prognosis remains dismal. A retrospective analysis from January 2012 to September 2024, diagnosed with primary plasma cell leukemia was carried at our center. Baseline characteristics, treatment and survival outcomes were collected from electronic medical records and analyzed in R meta package software. Among 24 primary plasma cell leukemia patients (median age 63 years; 58% male), fatigue and back pain were the most common clinical presentation. Most of the patients (96%) received Bortezomib based triplet regimen. Response was evaluated in 23 patients; 15 (65%) achieved very good partial response (VGPR), 5 (22%) achieved partial response (PR), and 3 (13%) had progressive disease. With a median follow up of 36.7 months, median progression free survival (PFS) was 22 months (95% CI 14.06 -31.73) and median overall survival (OS) was 31 months (95% CI 18.39 - 44.02). Most common causes of death were disease progression (82%) followed by refractory septic shock (18%). This study showed pPCL is a rare plasma cell neoplasm presents with poor prognosis. Autologous stem cell transplant resulted in improved survival.</p>

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Real World Outcome of Primary Plasma Cell leukemia - a Retrospective Analysis from a Tertiary Care Center from Eastern India

  • 期刊论文

  • 发表日期：

    2026年2月16日

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  • Bikash Shah,
  • Dibakar Podder,
  • Shouriyo Ghosh,
  • Sharthak Ghosh,
  • Jeevan Kumar,
  • Debranjani Chattopadhyay,
  • Saswata Saha,
  • Arijit Nag,
  • Rizwan Javed,
  • Asish Rath,
  • Subhosmito Chakraborty,
  • Mayur Parihar,
  • Reena Nair

摘要

Primary plasma cell leukemia (pPCL) is an aggressive and rare form of plasma cell dyscrasia, requiring early detection and rapid intervention and is characterized by presence of 5% or more circulating plasma cells. Despite advances in diagnosis and management, the prognosis remains dismal. A retrospective analysis from January 2012 to September 2024, diagnosed with primary plasma cell leukemia was carried at our center. Baseline characteristics, treatment and survival outcomes were collected from electronic medical records and analyzed in R meta package software. Among 24 primary plasma cell leukemia patients (median age 63 years; 58% male), fatigue and back pain were the most common clinical presentation. Most of the patients (96%) received Bortezomib based triplet regimen. Response was evaluated in 23 patients; 15 (65%) achieved very good partial response (VGPR), 5 (22%) achieved partial response (PR), and 3 (13%) had progressive disease. With a median follow up of 36.7 months, median progression free survival (PFS) was 22 months (95% CI 14.06 -31.73) and median overall survival (OS) was 31 months (95% CI 18.39 - 44.02). Most common causes of death were disease progression (82%) followed by refractory septic shock (18%). This study showed pPCL is a rare plasma cell neoplasm presents with poor prognosis. Autologous stem cell transplant resulted in improved survival.