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From a 2DE-Gel Spot to Protein Function: Lesson Learned From HS1 in Chronic Lymphocytic Leukemia
Published on: October 19, 2014
A case of primary plasma cell leukemia with hairy-cell morphology and lambda-type Bence-Jones protein.
Fumihiko Tanioka1, Sadahiro Tamashima, Shin-ichi Shimizu
1Division of Pathology and Laboratory Medicine, Iwata City Hospital, Iwata, Shizuoka, Japan.
Insights
This study reports a rare case of primary plasma cell leukemia with hairy cell morphology. Molecular analysis suggests leukemic cells originate from immature germinal center B-cells, differing from multiple myeloma origins.
Area of Science:
- Hematology
- Oncology
- Immunology
Background:
- Primary plasma cell leukemia (PC-LC) is a rare and aggressive plasma cell neoplasm.
- Hairy cell morphology in PC-LC is unusual and presents diagnostic challenges.
- Understanding the cellular origin is crucial for accurate diagnosis and treatment.
Observation:
- A unique case of PC-LC presented with atypical lymphoid cells exhibiting hairy projections in peripheral blood.
- Bone marrow examination revealed plasmablastic and 'tadpole'-like cells.
- Immunohistochemistry showed cytoplasmic lambda light chain and CD38 expression, but lacked typical B-cell markers (CD19, CD20, CD79a) and VLA-5 (CD49e).
Findings:
- Sequence analysis revealed significant somatic hypermutation and intraclonal variations in immunoglobulin light-chain (V(L)) and heavy-chain (V(H)) genes.
- Intraclonal diversity was observed, a phenomenon previously reported in monoclonal gammopathy of undetermined significance (MGUS) but not multiple myeloma.
- These molecular and immunohistochemical features indicated the leukemic cells originated from germinal center B-cells.
Implications:
- This case expands the morphological spectrum of primary plasma cell leukemia.
- The findings suggest a more immature B-cell origin for this type of PC-LC compared to typical multiple myeloma.
- Further research into the B-cell developmental pathways in plasma cell neoplasms is warranted.
Abstract:
A case of primary plasma cell leukemia with hairy-cell morphology and lambda-type Bence-Jones protein is reported. Most of the atypical cells in the peripheral blood of this case were small lymphoid cells or lymphoplasmacytoid lymphocytes with numerous cytoplasmic hairy projections. Plasmablastic cells and 'tadpole'-like cells were also present in the bone marrow. Immunohistochemically, these atypical cells expressed the cytoplasmic lambda light chain and surface CD38 proteins but were negative for B-cell markers such as CD19, CD20 and CD79a. VLA-5 (CD49e), which is supposed to be expressed in mature populations of plasma cells, was negative. A sequence analysis of the variable region gene in the light-chain (V(L)) and heavy-chain (V(H)) loci of immunoglobulin demonstrated significant somatic hypermutation and intraclonal nucleic acid sequence variations. To our knowledge, the intraclonal diversity of these loci has been previously reported in some cases of monoclonal gammopathy of undetermined significance (MGUS), but never in a case of multiple myeloma. The immunohistochemical and molecular characteristics of this case allowed us to delineate the origin of the leukemic cells with hairy cell-morphology as germinal center B-cells, which would be at a more immature stage than the presumable origin of multiple myeloma.
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