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Objectives The treatment of choice for extramedullary plasmacytoma (EMP) is radiotherapy (RT). It is under discussion whether the management of an anaplastic form of EMP requires the addition of systemic therapy. Case presentation We present a case of a 66-year-old male who was diagnosed with anaplastic plasmacytoma of the maxillary sinus. After the exclusion of multiple myeloma, Dara-VMP (daratumumab, bortezomib, melphalan, and prednisolone) regimen was initiated. During the third cycle of Dara-VMP, a progression of the tumor was observed. RT and BRd (bendamustine, dexamethasone, and lenalidomide) regimen were initiated. After 4 cycles of BRd, disease progression was established. KRd (carfilzomib, lenalidomide, and dexamethasone) regimen was initiated. The first cycle of KRd was not completed, as the patient was diagnosed with COVID-19. After the infection, the progression of EMP was observed. Conclusions In this case, the anaplastic EMP was resistant to RT and chemotherapy regimens with novel agents, including a monoclonal antibody, an immunomodulatory drug, and proteasome inhibitors.

The morbidity and mortality of BCR-ABL-negative myeloproliferative neoplasia (MPN) patients is highly dependent on thrombosis that may be affected by antiphospholipid antibodies (aPLA) and lupus anticoagulant. Our aim was to evaluate the association of the aPLA together with platelet receptor glycoprotein (GP) Ia/IIa c.807C>T CT/TT genotypes and thrombotic complications in patients with MPNs. The study included 108 patients with BCR-ABL-negative MPN with data of previous thrombosis. Two different screening and one confirmatory test for the lupus anticoagulant were performed. Thrombotic complications were present in 59 (54.6%) subjects. aPLA were more frequently found in MPN patients with thrombosis vs no thrombosis (25.4 and 6.1%; p = 0.007). MPN patients with arterial thrombosis were more frequently positive for aPLA vs no arterial thrombosis (38.8 and 11.9%; p = 0.001). aPLA were more frequently found in patients with cerebrovascular events vs other arterial thrombotic complications or no thrombosis, respectively (39.3, 6.1, and 12.9%; p < 0.001). MPN patients with thrombosis were more frequently positive with aPLA and had platelet receptor GP Ia/IIa c.807C>T CT/TT genotypes compared to MPN patients without thrombosis (18.6 and 2.0%; p = 0.006). aPLA alone or with coexistence with platelet receptor GP Ia/IIa c.807C>T CT/TT polymorphism could be associated with thrombotic complications in patients with MPN.

IntroductionPatients with Philadelphia-negative (Ph-) myeloproliferative neoplasms: primary myelofibrosis, essential thrombocythemia and polycythemia vera; often develop thrombotic events. It was suggested that the genetic variants that are responsible for blood coagulation and elevated homocysteine level play causal role in the occurrence of thrombosis. Our aim was to evaluate the single nucleotide polymorphisms in PROS1, EPCR, PROC, MTHFR, MS genes and their associations with the risk of developing thrombosis as well as clinical characteristics in patients with myeloproliferative disorders.Material and methodsThe screening of PROS1 g.66847T>C, EPCR c.4678G>C, EPCR c.6936A>G, PROC c.565C>T, MTHFR c.677C>T, MTHFR c.1298A>C, MS c.2756A>G polymorphisms was performed at Lithuanian University of Health Sciences, Kaunas, Lithuania. The PCR-RFLP method was applied.ResultsAfter genotyping 88 patients with Ph- myeloproliferative disorders, the association was found between venous thrombosis and MTHFR c.1298A>C (p=0.019). Regression analysis revealed that carriers of PROS1 66847TC, EPCR 4678GC, 6936AG or GG, PROC 565CT or TT, MTHFR 677CT, MS 2756AG genotypes were associated with a lower risk of developing venous thrombosis. EPCR 6936AG genotype could be considered as a protective factor against arterial thrombosis. Genotypes PROC 565CT and 565TT were associated with a lower risk of decreased levels of MPV, 565TT carriers were less likely to develop arterial or venous thrombosis, compared with those carrying the CC or CT genotype.ConclusionsIt can be concluded that more research into these polymorphisms needs to be performed, since there are many conflicting results published regarding the complexity of the possible interactions between these gene variants and predisposition to thrombotic events.

The most important complications of Philadelphianegagive (non BCR-ABL) myeloproliferative neoplasms (MPNs) are vascular events. Our aim was to evaluate the effects of single nucleotide polymorphisms (SNPs), platelet glycoproteins (GPs) (Ia/IIa, Ibα, IIb/IIIa and VI), von Willebrand factor (vWF), coagulation factor VII (FVII), β-fibrinogen, and the risk of thrombosis in patients with non BCR-ABL MPNs at the Lithuanian University of Health Sciences. Kaunas, Lithuania. Genotyping was done for 108 patients. The TT genotype of the GP Ia/IIa c.807C>T polymorphism was more frequently found in the group of MPN patients with arterial thrombosis compared to MPN patients who were thrombosis-free [26.5 vs. 11.5%, p = 0.049; odds ratio (OR) 2.68; 95% confidence interval (95% CI) 1.01-7.38]. The CT genotype of the β-fibrinogen c.-148C>T polymorphism occurred more frequently in MPN patients with arterial, and total thrombosis compared to the wild or homozygous genotype (57.7 vs. 40.0 vs. 12.5%; p = 0.027), (64.7 vs. 44.4 vs. 25%; p = 0.032), respectively. The carrier state for the c.-323P10 variant of FVII SNP (summation of P10/10 and P0/10) was more frequent in MPN patients with thrombosis compared to the wild-type genotype carriers (71.4 vs. 43.4%; p = 0.049; OR 3.26; 95% CI 1.01-11.31). The coexistence of heterozygous β-fibrinogen c.-148C>T and FVII c.-323P0/10 SNP, increased the risk of arterial thrombosis (21.1 vs. 3.7%, p = 0.008; OR 6.93; 95% CI 1.38-34.80). The TT genotype of GP Ia/IIa c.807C>T, the CT genotype of β-fibrinogen c.-148C>T and FVII c.-323P0/10 SNP could be associated with risk of thrombosis in MPN patients.