Clinical features and outcomes of plasma cell leukemia: A single-institution experience in the era of novel agents

Giampaolo Talamo, Nathan G. Dolloff, Kamal Sharma, Junjia Zhu, Jozef Malysz

Research output: Contribution to journalArticle

11 Citations (Scopus)

Abstract

Plasma cell leukemia (PCL) is a rare hematologic malignancy with aggressive clinical and biologic features. Data regarding its prognosis with the use of the novel agents, i.e., the immunomodulatory drugs thalidomide and lenalidomide, and the proteasome inhibitor bortezomib, are limited. We retrospectively reviewed clinical outcomes, response to therapy, and survival of 17 patients seen at the Penn State Hershey Cancer Institute since the availability of novel agents (2006-2011). Twelve patients had primary PCL (pPCL), and 5 secondary PCL (sPCL). PCL was associated with aggressive clinicobiological features, such as high-risk cytogenetics, elevated serum beta-2-microglobulin and lactate dehydrogenase, International Staging System stage III, and rapid relapse after therapy. With the use of thalidomide, lenalidomide, and bortezomib in 53%, 53%, and 88% patients, respectively, median overall survival (OS) was 18 months in the whole group (95% confidence interval, 11-21 months), and 21 and 4 months in pPCL and sPCL, respectively (P=0.015). OS was inferior to that of 313 consecutive patients with multiple myeloma (MM) treated in the same period, even when compared with a subset of 47 MM with high-risk cytogenetics. Although our data are limited by the small sample size, we conclude that novel agents may modestly improve survival in patients with PCL, when compared to historical controls. Novel therapies do not seem to overcome the negative prognosis of PCL as compared with MM.

Original languageEnglish (US)
Pages (from-to)123-126
Number of pages4
JournalRare Tumors
Volume4
Issue number3
DOIs
StatePublished - Sep 26 2012

Fingerprint

Plasma Cell Leukemia
Multiple Myeloma
Thalidomide
Survival
Cytogenetics
beta 2-Microglobulin
Proteasome Inhibitors
Hematologic Neoplasms
L-Lactate Dehydrogenase
Sample Size
Therapeutics
Confidence Intervals
Recurrence
Serum
Pharmaceutical Preparations
Neoplasms

All Science Journal Classification (ASJC) codes

  • Histology
  • Oncology

Cite this

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title = "Clinical features and outcomes of plasma cell leukemia: A single-institution experience in the era of novel agents",
abstract = "Plasma cell leukemia (PCL) is a rare hematologic malignancy with aggressive clinical and biologic features. Data regarding its prognosis with the use of the novel agents, i.e., the immunomodulatory drugs thalidomide and lenalidomide, and the proteasome inhibitor bortezomib, are limited. We retrospectively reviewed clinical outcomes, response to therapy, and survival of 17 patients seen at the Penn State Hershey Cancer Institute since the availability of novel agents (2006-2011). Twelve patients had primary PCL (pPCL), and 5 secondary PCL (sPCL). PCL was associated with aggressive clinicobiological features, such as high-risk cytogenetics, elevated serum beta-2-microglobulin and lactate dehydrogenase, International Staging System stage III, and rapid relapse after therapy. With the use of thalidomide, lenalidomide, and bortezomib in 53{\%}, 53{\%}, and 88{\%} patients, respectively, median overall survival (OS) was 18 months in the whole group (95{\%} confidence interval, 11-21 months), and 21 and 4 months in pPCL and sPCL, respectively (P=0.015). OS was inferior to that of 313 consecutive patients with multiple myeloma (MM) treated in the same period, even when compared with a subset of 47 MM with high-risk cytogenetics. Although our data are limited by the small sample size, we conclude that novel agents may modestly improve survival in patients with PCL, when compared to historical controls. Novel therapies do not seem to overcome the negative prognosis of PCL as compared with MM.",
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Clinical features and outcomes of plasma cell leukemia : A single-institution experience in the era of novel agents. / Talamo, Giampaolo; Dolloff, Nathan G.; Sharma, Kamal; Zhu, Junjia; Malysz, Jozef.

In: Rare Tumors, Vol. 4, No. 3, 26.09.2012, p. 123-126.

Research output: Contribution to journalArticle

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