Browsing by Author "Čemerikić, Vesna (6602796339)"

Introduction Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and very aggressive hematological malignancy derived from precursor of the plasmacytoid dendritic cell. We present a case with cervix uteri involvement without skin lesions, which is, to the best of our knowledge, the first case of BPDCN localized in the cervix. Case outline A 66-year-old previously healthy women initially presented with a four-week history of vaginal bleeding. Gynecologic examination revealed a tumorous bleeding formation on cervix uteri. Except paleness of the skin, physical examination results were normal. Complete blood counts showed anemia and thrombocytopenia. Computed tomography scans showed an expansive tumorous formation at the level of the isthmus and cervix uteri, 60 × 42 mm in size. Cervical biopsy was done and final pathohistological diagnosis was BPDCN. Karyotype analysis results from the bone marrow aspiration specimen demonstrated tetrasomy of chromosome 2 and monosomy of chromosome 16. The patient did not accept treatment and died two months after the initial diagnosis was established. Conclusion Attributes such as aggressive clinical course of BPDCN, demonstrated unusual localization, infrequency, and the absence of consensus about standard treatment options, demand constructive clinical reasoning and tight cooperation between medical professionals of various fields. © 2020, Serbia Medical Society. All rights reserved.

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. © 2022 Inst. Sci. inf., Univ. Defence in Belgrade. All rights reserved.