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Plasma cell leukemia mimicking hairy cell leukemia
images and diagnosis Plasma cell leukemia mimicking hairy cell leukemia Amr Hanbali
a,*
, Abdulaziz Alrajeh b, Walid Rasheed
a
a Hematology Department, King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia, King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia
b
Hematopathology Department,
* Corresponding author. Tel.: +966 500527245; fax: +966 114423941 Æ [email protected] Æ Received for publication 22 April 2015 Æ Accepted for publication 7 May 2015 Hematol Oncol Stem Cell Ther 2015; 8(2): 91–92 ª 2015 King Faisal Specialist Hospital & Research Centre. Published by Elsevier Ltd. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). DOI: http://dx.doi.org/10.1016/j.hemonc.2015.05.001
A
42-year-old female presented with fever and shortness of breath, and was diagnosed with community acquired pneumonia. On presentation she was found to have a white cell count of 13 · 109/L, hemoglobin of 7.1 g/dL and platelets of 51 · 109/L. Peripheral blood smear showed left shift and many cells with hairy projections (56%) (See Fig. 1) that were positive for CD38 and CD138 by peripheral blood flow cytometry. Other laboratory findings included B-2 microglobulin of 15.3 mg/L with normal creatinine and calcium levels. Serum protein electrophoresis evaluation revealed monoclonal gammopathy (IgG-lambda) of 45.87 g/L. Urine protein electrophoresis showed the presence of Ig-G lambda as well as free-lambda light chain monoclonal bands.
Bone marrow biopsy showed increased cellularity (95%) with increased number of plasma cells (45%). Flow cytometry showed 44% of the cells to have clonal plasma cell phenotype (CD38+ve, CD138+ve, CD20 ve, CD22 ve, and cytoplasmic lambda light chin restriction). A diagnosis of plasma cell leukemia was made and treatment was started with bortezomib, cyclophosphamide, and dexamethasone. The patient achieved partial remission. Treatment was followed by autologous bone marrow transplantation and then two cycles of lenalidomide and dexamethasone consolidation. The patient subsequently achieved complete remission. She has been on maintenance lenalidomide over the past six months and continues to be in complete remission.
Figure 1. Peripheral blood smear showing plasma cells with hairy projections.
Hematol Oncol Stem Cell Ther 8(2)
Second Quarter 2015
91
images and diagnosis This case highlights the rare presentation of an uncommon disease. Plasma cell leukemia is a rare and aggressive neoplasm with short survival. Only one case is reported in the literature describing a similar presentation to our case with hairy cell morphology. The clinical significance of such a presentation and its effect on prognosis is unknown.
AUTHOR CONTRIBUTIONS Amr Hanbali: information collection, writing of manuscript.
92
PLASMA CELL LEUKEMIA WITH HAIRY-CELL MORPHOLOGY
Abdulaziz Alrajeh: preparation of pathological image. Walid Rasheed: manuscript editing.
CONFLICT OF INTEREST DECLARATION This statement is to certify that all authors have seen and approved the manuscript being submitted. All authors have no financial or personal relationships with other people or organizations that could inappropriately influence their work.
Hematol Oncol Stem Cell Ther 8(2)
Second Quarter 2015
a,*
, Abdulaziz Alrajeh b, Walid Rasheed
a
a Hematology Department, King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia, King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia
b
Hematopathology Department,
* Corresponding author. Tel.: +966 500527245; fax: +966 114423941 Æ [email protected] Æ Received for publication 22 April 2015 Æ Accepted for publication 7 May 2015 Hematol Oncol Stem Cell Ther 2015; 8(2): 91–92 ª 2015 King Faisal Specialist Hospital & Research Centre. Published by Elsevier Ltd. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). DOI: http://dx.doi.org/10.1016/j.hemonc.2015.05.001
A
42-year-old female presented with fever and shortness of breath, and was diagnosed with community acquired pneumonia. On presentation she was found to have a white cell count of 13 · 109/L, hemoglobin of 7.1 g/dL and platelets of 51 · 109/L. Peripheral blood smear showed left shift and many cells with hairy projections (56%) (See Fig. 1) that were positive for CD38 and CD138 by peripheral blood flow cytometry. Other laboratory findings included B-2 microglobulin of 15.3 mg/L with normal creatinine and calcium levels. Serum protein electrophoresis evaluation revealed monoclonal gammopathy (IgG-lambda) of 45.87 g/L. Urine protein electrophoresis showed the presence of Ig-G lambda as well as free-lambda light chain monoclonal bands.
Bone marrow biopsy showed increased cellularity (95%) with increased number of plasma cells (45%). Flow cytometry showed 44% of the cells to have clonal plasma cell phenotype (CD38+ve, CD138+ve, CD20 ve, CD22 ve, and cytoplasmic lambda light chin restriction). A diagnosis of plasma cell leukemia was made and treatment was started with bortezomib, cyclophosphamide, and dexamethasone. The patient achieved partial remission. Treatment was followed by autologous bone marrow transplantation and then two cycles of lenalidomide and dexamethasone consolidation. The patient subsequently achieved complete remission. She has been on maintenance lenalidomide over the past six months and continues to be in complete remission.
Figure 1. Peripheral blood smear showing plasma cells with hairy projections.
Hematol Oncol Stem Cell Ther 8(2)
Second Quarter 2015
91
images and diagnosis This case highlights the rare presentation of an uncommon disease. Plasma cell leukemia is a rare and aggressive neoplasm with short survival. Only one case is reported in the literature describing a similar presentation to our case with hairy cell morphology. The clinical significance of such a presentation and its effect on prognosis is unknown.
AUTHOR CONTRIBUTIONS Amr Hanbali: information collection, writing of manuscript.
92
PLASMA CELL LEUKEMIA WITH HAIRY-CELL MORPHOLOGY
Abdulaziz Alrajeh: preparation of pathological image. Walid Rasheed: manuscript editing.
CONFLICT OF INTEREST DECLARATION This statement is to certify that all authors have seen and approved the manuscript being submitted. All authors have no financial or personal relationships with other people or organizations that could inappropriately influence their work.
Hematol Oncol Stem Cell Ther 8(2)
Second Quarter 2015