A Rare Presentation of Primary Plasma Cell Leukemia: A Case Report and Review of Literature
Background: The primary plasma cell leukemia (pPCL) is an aggressive plasma cell neoplasm. It is diagnosed by the presence of an absolute plasma cell count of >2 × 109/L or 20% plasma cells in the peripheral blood. pPCL is rare and reported to be <1 in a million. Hence, our case report is a ra...
| Main Authors: | , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
West Asia Organization for Cancer Prevention
2022-11-01
|
| Series: | Asian Pacific Journal of Cancer Biology |
| Subjects: | |
| Online Access: | http://www.waocp.com/journal/index.php/apjcb/article/view/921 |
| _version_ | 1797338788140154880 |
|---|---|
| author | Damdar Gaurav T Prabhakaran Anusree |
| author_facet | Damdar Gaurav T Prabhakaran Anusree |
| author_sort | Damdar Gaurav T |
| collection | DOAJ |
| description | Background: The primary plasma cell leukemia (pPCL) is an aggressive plasma cell neoplasm. It is diagnosed by the presence of an absolute plasma cell count of >2 × 109/L or 20% plasma cells in the peripheral blood. pPCL is rare and reported to be <1 in a million. Hence, our case report is a rare opportunity to describe clinical presentation and management of pPCL.
Case Details: A 72-year-old male patient with benign prostatic hyperplasia, presented to the hospital for cough, breathlessness, and intermittent fever of one month. On arrival, he was tachypnoeic with altered sensorium and rales in his chest. Laboratory examination showed anemia, leucocytosis, and thrombocytopenia. The peripheral blood smears revealed 25-30% circulating atypical plasmacytoid cells. Flow cytometry on the peripheral blood revealed 58.7% lymphoid cells. Out of the total lymphoid cells, 73.6 % cells were characterized by expression of CD38, CD138, CD19, CD49d, CD43, CD27, CD81 (96.9% dim positives) CD 56 with dim kappa light chain restriction suggestive of PCL. Furthermore, serum protein electrophoresis and serum immunofixation showed an M-band (0.53g) of IgG kappa subtype. However, the patient developed lower respiratory tract infections with multi-organ dysfunction and he succumbed to the same.
Conclusion: The prognosis of pPCL is very poor and the high risk of infective complications. Early diagnosis and optimal chemotherapy would be the key to the management. Detailed peripheral blood film examination and characterization of abnormal cells with immune phenotyping are of utmost importance in diagnosing pPCL. |
| first_indexed | 2024-03-08T09:36:21Z |
| format | Article |
| id | doaj.art-f5c63fed92cd4c1d9858785198a02a22 |
| institution | Directory Open Access Journal |
| issn | 2538-4635 |
| language | English |
| last_indexed | 2024-03-08T09:36:21Z |
| publishDate | 2022-11-01 |
| publisher | West Asia Organization for Cancer Prevention |
| record_format | Article |
| series | Asian Pacific Journal of Cancer Biology |
| spelling | doaj.art-f5c63fed92cd4c1d9858785198a02a222024-01-30T09:31:19ZengWest Asia Organization for Cancer PreventionAsian Pacific Journal of Cancer Biology2538-46352022-11-017437738210.31557/apjcb.2022.7.4.377-382921A Rare Presentation of Primary Plasma Cell Leukemia: A Case Report and Review of LiteratureDamdar Gaurav T0Prabhakaran Anusree1Clinical Pharmacologist, Deenanath Mangeshkar Hospital, Pune, India.Clinical Hematologist & Bone Marrow Transplant Specialist at Deenanth Mangeshkar Hospital, Pune, India.Background: The primary plasma cell leukemia (pPCL) is an aggressive plasma cell neoplasm. It is diagnosed by the presence of an absolute plasma cell count of >2 × 109/L or 20% plasma cells in the peripheral blood. pPCL is rare and reported to be <1 in a million. Hence, our case report is a rare opportunity to describe clinical presentation and management of pPCL. Case Details: A 72-year-old male patient with benign prostatic hyperplasia, presented to the hospital for cough, breathlessness, and intermittent fever of one month. On arrival, he was tachypnoeic with altered sensorium and rales in his chest. Laboratory examination showed anemia, leucocytosis, and thrombocytopenia. The peripheral blood smears revealed 25-30% circulating atypical plasmacytoid cells. Flow cytometry on the peripheral blood revealed 58.7% lymphoid cells. Out of the total lymphoid cells, 73.6 % cells were characterized by expression of CD38, CD138, CD19, CD49d, CD43, CD27, CD81 (96.9% dim positives) CD 56 with dim kappa light chain restriction suggestive of PCL. Furthermore, serum protein electrophoresis and serum immunofixation showed an M-band (0.53g) of IgG kappa subtype. However, the patient developed lower respiratory tract infections with multi-organ dysfunction and he succumbed to the same. Conclusion: The prognosis of pPCL is very poor and the high risk of infective complications. Early diagnosis and optimal chemotherapy would be the key to the management. Detailed peripheral blood film examination and characterization of abnormal cells with immune phenotyping are of utmost importance in diagnosing pPCL.http://www.waocp.com/journal/index.php/apjcb/article/view/921primary plasma cell leukemiaatypical plasmacytoid cellsimmunofixationrare caseflow cytometry |
| spellingShingle | Damdar Gaurav T Prabhakaran Anusree A Rare Presentation of Primary Plasma Cell Leukemia: A Case Report and Review of Literature Asian Pacific Journal of Cancer Biology primary plasma cell leukemia atypical plasmacytoid cells immunofixation rare case flow cytometry |
| title | A Rare Presentation of Primary Plasma Cell Leukemia: A Case Report and Review of Literature |
| title_full | A Rare Presentation of Primary Plasma Cell Leukemia: A Case Report and Review of Literature |
| title_fullStr | A Rare Presentation of Primary Plasma Cell Leukemia: A Case Report and Review of Literature |
| title_full_unstemmed | A Rare Presentation of Primary Plasma Cell Leukemia: A Case Report and Review of Literature |
| title_short | A Rare Presentation of Primary Plasma Cell Leukemia: A Case Report and Review of Literature |
| title_sort | rare presentation of primary plasma cell leukemia a case report and review of literature |
| topic | primary plasma cell leukemia atypical plasmacytoid cells immunofixation rare case flow cytometry |
| url | http://www.waocp.com/journal/index.php/apjcb/article/view/921 |
| work_keys_str_mv | AT damdargauravt ararepresentationofprimaryplasmacellleukemiaacasereportandreviewofliterature AT prabhakarananusree ararepresentationofprimaryplasmacellleukemiaacasereportandreviewofliterature AT damdargauravt rarepresentationofprimaryplasmacellleukemiaacasereportandreviewofliterature AT prabhakarananusree rarepresentationofprimaryplasmacellleukemiaacasereportandreviewofliterature |