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Published on: June 5, 2015
Plasma cell leukemia producing monoclonal immunoglobulin E
Yuzuru Takemura1,2, Masanobu Ikeda3, Kahori Kobayashi3,4
1Department of Clinical Laboratories, Saku Central Hospital, 197 Usuda, Saku, Nagano, 384-0301, Japan. yuzurutakemura@nifty.com.
Insights
This study reports a rare case of IgE-producing plasma cell leukemia (PCL) in an elderly male. Despite treatment, the patient succumbed to rapid tumor progression, highlighting the aggressive nature of this rare PCL subtype.
Area of Science:
- Hematology
- Oncology
- Immunology
Background:
- Plasma cell leukemia (PCL) is a rare and aggressive plasma cell dyscrasia.
- Monoclonal immunoglobulin E (IgE) production is exceptionally uncommon in PCL.
Observation:
- A 78-year-old male presented with lumbar pain, altered consciousness, hypercalcemia, and renal dysfunction.
- Circulating and bone marrow plasma cells exhibited an immature phenotype (CD19(-)/CD56(-)) and produced significant amounts of IgE/kappa protein.
- Bone imaging revealed a solitary osteolytic lesion, and cytogenetics showed a t(11;14) translocation.
Findings:
- The patient was diagnosed with IgE-producing PCL, characterized by immature plasma cells and a t(11;14) translocation.
- Immunohistochemistry confirmed cyclin D1 overexpression, consistent with the chromosomal abnormality.
- Treatment with dexamethasone and vincristine provided only transient improvement in laboratory parameters.
Implications:
- This case underscores the diagnostic challenges and aggressive clinical course associated with IgE-producing PCL.
- Understanding the immunophenotypic and genetic features of rare PCL subtypes is crucial for potential therapeutic strategies.
- Further research into IgE-producing PCL is warranted to improve patient outcomes.
Abstract:
A 78-year-old male with lumbar pain and dim consciousness presented the clinical pictures of plasma cell leukemia (PCL) producing a large amount of monoclonal immunoglobulin E (IgE)/kappa protein. Laboratory investigation demonstrated an elevated serum calcium level and renal dysfunction. Systemic bone X-ray survey disclosed only a solitary osteolytic lesion. Circulating plasma cells demonstrated CD19(-)/CD56(-) and MPC-1(-)/CD49e(-)/CD45(+/-), the latter indicating the immature phenotype of the tumor cells. Bone marrow was occupied with immature, atypical plasma cells, of which cytoplasms were positive for IgE by direct immunofluorescence analysis. Chromosomes revealed a translocation of (11;14)(q13;q32), which is concordant with cyclinD1-protein overexpression by immunohistochemistry. He was treated with dexamethasone and vincristine, which somewhat improved the laboratory findings. He died of tumor progression after 4-month admission. The clinical and biological characteristics of IgE-producing PCL, a very rare type of plasma cell dyscrasia, are discussed, reviewing the past literature.
