Successful treatment of synchronous hairy cell leukaemia and diffuse large B cell lymphoma in a patient with severe hypercalcemia and extensive osteolytic lesions
Introduction. Although secondary malignancies usually occur at different times after hairy cell leukemia (HCL) treatment, the occurrence of HCL and other malignancies at the same time is very rare. Synchronous HCL and diffuse large B-cell lymphoma (DLBCL) have not been described so far. Case report....
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Military Health Department, Ministry of Defance, Serbia
2022-01-01
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| Series: | Vojnosanitetski Pregled |
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| Online Access: | http://www.doiserbia.nb.rs/img/doi/0042-8450/2022/0042-84502100073M.pdf |
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| author | Marković Olivera Gotić Mirjana Čemerikić Vesna Divac Anica Marisavljević Dragomir |
| author_facet | Marković Olivera Gotić Mirjana Čemerikić Vesna Divac Anica Marisavljević Dragomir |
| author_sort | Marković Olivera |
| collection | DOAJ |
| description | Introduction. Although secondary malignancies usually occur at different times after hairy cell leukemia (HCL) treatment, the occurrence of HCL and other malignancies at the same time is very rare. Synchronous HCL and diffuse large B-cell lymphoma (DLBCL) have not been described so far. Case report. The report presents a 62-year-old female patient with intense constitutional symptoms, hypercalcemia, pancytopenia, and osteolytic destruction of the left shoulder joint. Immunohistochemical analysis of the bone marrow revealed the presence of two cell populations: a population of HCL cells and a population of DLBCL cells with the expression of CMYC and BCL-2 proteins ("double expressor" DLBCL) and high proliferative activity (Ki-67+cells > 90%). Fluorescence in situ hybridization (FISH) analysis showed amplification of the BCL-2 gene. In addition, BRAF gene V600E mutation was detected. After intensive treatment with immunochemotherapy, radiotherapy, and bisphosphonates, the patient achieved complete remission, lasting for more than two years. Conclusion. As the association of HCL and lymphoma is very rare, diagnosis of synchronous occurrence of two lymphoproliferative diseases is a diagnostic and therapeutic challenge. It remains unclear whether DLBC and HCL originated from two different malignant clones or DLBCL developed by the transformation of HCL as the result of clonal evolution of the B-cell clone. |
| first_indexed | 2024-04-13T07:01:10Z |
| format | Article |
| id | doaj.art-8f9efe09a7b64b5da610e6edec6fb38a |
| institution | Directory Open Access Journal |
| issn | 0042-8450 2406-0720 |
| language | English |
| last_indexed | 2024-04-13T07:01:10Z |
| publishDate | 2022-01-01 |
| publisher | Military Health Department, Ministry of Defance, Serbia |
| record_format | Article |
| series | Vojnosanitetski Pregled |
| spelling | doaj.art-8f9efe09a7b64b5da610e6edec6fb38a2022-12-22T02:57:07ZengMilitary Health Department, Ministry of Defance, SerbiaVojnosanitetski Pregled0042-84502406-07202022-01-0179101044104710.2298/VSP210118073M0042-84502100073MSuccessful treatment of synchronous hairy cell leukaemia and diffuse large B cell lymphoma in a patient with severe hypercalcemia and extensive osteolytic lesionsMarković Olivera0https://orcid.org/0000-0002-5957-0716Gotić Mirjana1https://orcid.org/0000-0002-8590-0328Čemerikić Vesna2Divac Anica3Marisavljević Dragomir4University Hospital Medical Center “Bežanijska Kosa”, Belgrade, Serbia + University of Belgrade, Faculty of Medicine, Belgrade, SerbiaUniversity of Belgrade, Faculty of Medicine, Belgrade, Serbia + University Clinical Center of Serbia, Clinic for Hematology, Belgrade, SerbiaBeolab, Belgrade, SerbiaUniversity Hospital Medical Center “Bežanijska Kosa”, Belgrade, Serbia University Hospital Medical Center “Bežanijska Kosa”, Belgrade, Serbia Introduction. Although secondary malignancies usually occur at different times after hairy cell leukemia (HCL) treatment, the occurrence of HCL and other malignancies at the same time is very rare. Synchronous HCL and diffuse large B-cell lymphoma (DLBCL) have not been described so far. Case report. The report presents a 62-year-old female patient with intense constitutional symptoms, hypercalcemia, pancytopenia, and osteolytic destruction of the left shoulder joint. Immunohistochemical analysis of the bone marrow revealed the presence of two cell populations: a population of HCL cells and a population of DLBCL cells with the expression of CMYC and BCL-2 proteins ("double expressor" DLBCL) and high proliferative activity (Ki-67+cells > 90%). Fluorescence in situ hybridization (FISH) analysis showed amplification of the BCL-2 gene. In addition, BRAF gene V600E mutation was detected. After intensive treatment with immunochemotherapy, radiotherapy, and bisphosphonates, the patient achieved complete remission, lasting for more than two years. Conclusion. As the association of HCL and lymphoma is very rare, diagnosis of synchronous occurrence of two lymphoproliferative diseases is a diagnostic and therapeutic challenge. It remains unclear whether DLBC and HCL originated from two different malignant clones or DLBCL developed by the transformation of HCL as the result of clonal evolution of the B-cell clone.http://www.doiserbia.nb.rs/img/doi/0042-8450/2022/0042-84502100073M.pdflymphoma, large b-cell, diffusedrug therapygene expressionleukemia, hairy cellhypercalcemiaimmunotherapymagnetic resonance imagingmutationtomography, emission-computed |
| spellingShingle | Marković Olivera Gotić Mirjana Čemerikić Vesna Divac Anica Marisavljević Dragomir Successful treatment of synchronous hairy cell leukaemia and diffuse large B cell lymphoma in a patient with severe hypercalcemia and extensive osteolytic lesions Vojnosanitetski Pregled lymphoma, large b-cell, diffuse drug therapy gene expression leukemia, hairy cell hypercalcemia immunotherapy magnetic resonance imaging mutation tomography, emission-computed |
| title | Successful treatment of synchronous hairy cell leukaemia and diffuse large B cell lymphoma in a patient with severe hypercalcemia and extensive osteolytic lesions |
| title_full | Successful treatment of synchronous hairy cell leukaemia and diffuse large B cell lymphoma in a patient with severe hypercalcemia and extensive osteolytic lesions |
| title_fullStr | Successful treatment of synchronous hairy cell leukaemia and diffuse large B cell lymphoma in a patient with severe hypercalcemia and extensive osteolytic lesions |
| title_full_unstemmed | Successful treatment of synchronous hairy cell leukaemia and diffuse large B cell lymphoma in a patient with severe hypercalcemia and extensive osteolytic lesions |
| title_short | Successful treatment of synchronous hairy cell leukaemia and diffuse large B cell lymphoma in a patient with severe hypercalcemia and extensive osteolytic lesions |
| title_sort | successful treatment of synchronous hairy cell leukaemia and diffuse large b cell lymphoma in a patient with severe hypercalcemia and extensive osteolytic lesions |
| topic | lymphoma, large b-cell, diffuse drug therapy gene expression leukemia, hairy cell hypercalcemia immunotherapy magnetic resonance imaging mutation tomography, emission-computed |
| url | http://www.doiserbia.nb.rs/img/doi/0042-8450/2022/0042-84502100073M.pdf |
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