NOT OTHERWISE SPECIFIED T-CELL LYMPHOMA: OUTCOMES OF A SINGLE CENTER STUDY.

BACKGROUND: The peripheral T-cell lymphoma not otherwise specified (PTCL-NOS) is the most common subtype of peripheral T-cell lymphoma (PTCL). It constitutes approximately 25% of all PTCLs and accounts for more than 15% of all lymphomas. The results of the first Ukrainian prospective study of patients with PTCL-NOS are presented in the article. The aim of the study was to analyze the morbidity of PTCL patients and the treatment performed, to evaluate overall survival and progression-free survival, and to determine the factors that predict the treatment response. PATIENTS AND METHODS: An analysis was performed on the data of 31 patients diagnosed with peripheral PTCL-NOS from February 2018 to the present. T-cell lymphoid neoplasms were diagnosed according to the 2016 WHO classification. The treatment regimens were in alignment with ESMO and NCCN guidelines. More than 90% of patients were prescribed anthracycline-based regimens (CHOP; CHOEP - cyclophosphamide, doxorubicin, etoposide, vincristine, prednisone). An initial treatment was performed with CHOP-based regimens in 38.70% (n = 12) of patients, with the addition of etoposide in 58.06% of patients (n = 18). RESULTS: The response was assessed according to the response criteria for malignant lymphoma (Cheson, 2008, 2014). The overall response to therapy was 58.06% (n = 18), with complete responses in 29.03% of patients and partial responses in 29.03% of patients. The stabilization of the disease occurred in 3.44%, while the disease progression in 41.37% of patients. The 12-month and 24-month survival rates were 75.44% and 50.81%, respectively. The 12-month and 24-month progression-free survivals were 47.68% and 33.1%, respectively. Ki-67 overexpression (> 65%) was a negative prognostic factor. CONCLUSIONS: The results of the treatment of PTCL obtained in a Ukrainian population study are similar to those in other European studies, all of which remain unsatisfactory. Further research is required to develop a new strategy for examination and therapy to improve treatment outcomes. The emphasis should be placed on the pragmatic clinical trials comparing the efficacy of first-line treatment in PTCL patients with both favorable and unfavorable clinical factors.

THE ROLE OF RADIATION THERAPY IN THE TREATMENT OF PTCL-NOS. / РОЛЬ ПРОМЕНЕВОЇ ТЕРАПІЇ У ЛІКУВАННІ PTCL-NOS.

Peripheral T-cell lymphomas (PTCLs) are a heterogeneous group of lymphoproliferative disorders, accounting for about 10 % of all non-Hodgkin lymphomas. The most common subtype is peripheral T-cell lymphoma, unclassified (PTCL-NOS), accounting for about 26 % of all PTCLs. PTCL-NOS is associated with less favorable overall survival (OS) and progression-free survival (PFS) compared with aggressive B-cell lymphomas. The role of radiation therapy in the treatment of PTCL-NOS is still not definitively defined. The results of many studies show that the addition of radiation therapy to the treatment regimen is associated with a significant improvement in survival in patients with early-stage PTCL-NOS, but in the later stages, the benefit of radiation therapy is not obvious. OBJECTIVE: peripheral T-cell lymphoma, unspecified is a clinically and biologically heterogeneous disease with a poor prognosis. Since the role of radiation therapy is still unclear, a study was conducted to evaluate the effectiveness of radiation therapy in peripheral T-cell lymphoma, unspecified. MATERIALS AND METHODS: The work is based on clinical observations and treatment results of patients who were diagnosed between 2013 and 2023 at the National Cancer Institute (in the period from 2020 to 2023, patients were observed and treated as part of research). 56 patients were included in the study. RESULTS: The work analyzed the immediate results of the treatment of patients with peripheral T-cell lymphoma, unspecified depending on the stage and type of treatment, as well as the overall survival of these patients. When analyzing the overall response to the treatment of patients with I/II stages of the disease, it was proven that this indicator is higher in the group of patients who received chemoradiotherapy, compared to patients who received only chemotherapy (100 % versus 83.3 %), and this indicator was higher due to patients who demonstrated a complete response to therapy (75 % vs. 50 %). Analyzing the response to treatment of patients with III/IV stages of the pathological process, it was established that there was no difference in the overall response to treatment, the level of complete and partial response to treatment. Analysis of the overall survival of patients with I/II stages of the disease, with a median follow-up of 60 months, demonstrated a significant improvement in overall survival in the group of patients who received chemoradiotherapy compared to the group of patients who received only radiation therapy (median 48 vs. 22 months). Overall 1-year (78 % vs. 69 %), 3-year (64 % vs. 40 %), and 5-year (48 % vs. 35 %) were also higher in the chemoradiotherapy group. In the group of patients with III/IV stages of the disease, there was no difference in overall survival between patients who received chemoradiotherapy and patients who received only chemotherapy (median 16 vs. 13 months, 1-year survival 54 vs. 52, 3-year survival 33 vs. 30 and 5-year overall survival of 23 vs. 20 %. CONCLUSIONS: The addition of radiation therapy to the treatment plan demonstrated a significant improvement in the overall response and overall survival of patients with peripheral T-cell lymphomas, unspecified with I and II stages of the pathological process, but in III and IV stages of the disease, the benefit of radiation therapy has not been proven.

Comparison of the efficiency of peripheral blood stem cell apheresis on the blood cell separators.

BACKGROUND: Stem cells apheresis is a key step in the process of the autologous stem cell transplantation. Available blood cell separators (BCS) have different efficiency due to the technical characteristics and influence of the operator. MATERIALS AND METHODS: Retrospectively, data were collected of the peripheral blood stem cells apheresis performed using available BCS manufactured by Fresenius (ComTec and Amicus) in the National Cancer Institute Ukraine from 2017 to 2020. The collection efficiency coefficient (CEC) was calculated, the formula for predicting the total volume of processed blood (TVPB) was adapted for each separator. RESULTS: The analysis included data from 60 patients (total of 92 apheresis procedures). The mean CEC was established at the level of (53.8 ± 36.6) % for the Amicus device and (44.2 ± 37.3) % for the ComTec device; P = 0.22. The lower product volume was obtained using the Amicus device compared to the ComTec device; P = 2×10-7. The amount of collected stem cells was comparable in both groups (5.8 ± 5.7) ×106/kg and (4.1 ± 3.1) ×106/kg, respectively; P = 0.064. The adaptation of the formula for predicting the TVPB to achieve the optimum amount of stem cells was performed. CONCLUSION: The CEC for each device was within the generally accepted limits of 30-50%, and did not differ significantly. Nevertheless, using of the Amicus BCS allowed to collect lower volumes of the product, maintaining other characteristics of the product competitive.

Ukrainian prospective study in patients with T-cell non-Hodgkin lymphomas.

BACKGROUND: T-cell lymphoma (TCL) is a heterogeneous group of lymphoproliferative diseases that account for 10-15% of all non-Hodgkin lymphomas. The aim of the study was to analyze the incidence of TCL in Ukraine, distribution according to subtypes and to assess the results of treatment of patients with TCL depending on lymphoma subtype and clinical-and-laboratory risk factors. PATIENTS AND METHODS: Data from 70 patients with TCL were analyzed from February 2018 to May 2021. T-cell lymphoid neoplasms were diagnosed according to the 2016 WHO classification. The patients were divided into 4 groups: 1st (A) - leukemic forms (n = 13) (received SMILE or HyperCVAD +/- auto/alloSCT); 2nd (B) - nodal T-cell lymphomas (n = 43) (CHOP-like regimens); 3rd (C) - cutaneous T-cell lymphomas (n = 9) (PUVA therapy, interferon, and methotrexate); 4th (D) - extranodal T-cell lymphomas (n = 5) (CHOP-like regimens). The response was determined according to the Lugano 2014 criteria. RESULTS: According to the study results, 5-6% of all non-Hodgkin lymphoma registered in Ukraine in 2018-2020 were T-cell lymphomas. The most common subtype was peripheral TCL (61%). In the studied groups of TCL patients, the overall response rate was 50% (n = 35). 2-years event-free survival rate was 62.27%. 2-years overall survival rate was 65.76%. 18-month progression-free and overall survival in group B was higher versus groups A, C and D. The factors of unfavorable prognosis were bone marrow involvement and the expression of Ki67 > 65% (p = 0.03 and p = 0.006, respectively). CONCLUSIONS: Histologic subtype of T-cell non-Hodgkin lymphoma influence the treatment outcome. The best overall response rate, overall survival rate, progression-free survival were in group of patients with nodal T-cell non-Hodgkin lymphomas, the worst - in patients from leukemic group. Poor prognostic factors are bone marrow involvement, and Ki-67 expression > 65%.

Ukraine Data on Prognostic Factors and Treatment Outcomes in Patients with Peripheral T-Cell Lymphomas.

BACKGROUND: Peripheral T-cell lymphomas (PTCLs) is a diverse group of lymphomas (10-15% of all non-Hodgkins lymphomas) with aggressive behavior. Despite the standard of 1st line anthracycline-containing regimens, clinical outcomes are poor compared to B-cell lymphomas. In addition, there are still debates about specific prognostic factors (PF) in PTCLs. AIMS: Primary endpoints - event-free survival (EFS) and overall survival (OS). To evaluate the prognostic significance of five PTCLs scores (International Prognostic Index - IPI, International Peripheral T-cell lymphoma Project Score - IPTCL, Prognostic Index for T-cell lymphoma - PIT, modified Prognostic Index for T-cell lymphoma - mPIT and T-cell score). PATIENTS AND METHODS: From 67 enrolled patients, only 50 were included: PTCL not otherwise specified (22, 44%), anaplastic large cell lymphoma ALK+ (anaplastic lymphoma kinase-positive) (10, 20%) and ALK (anaplastic lymphoma kinase-negative) (18, 36%). Patients received CHOP-like regimens (CHOP, CHOEP, EPOCH). RESULTS: The overall rate response was observed in 66% of cases (complete response 78%). There were 48% of relapses after the 1st line therapy during follow-up (median 11 months; range 1-85 months). Median age 57 (range 22-80) with male predominance 62%. In total, 40% of patients were > 60 years old, 48% had stage III-IV. Majority of patients were assessed by five prognostic scores. IPI (45 patients): the 3-year EFS and OS were higher for IPI 1 vs. IPI > 2 (80 vs. 18% and 87 vs. 27%, respectively; p = 0.0002). Receiver operating characteristic analysis confirmed poor clinical outcome to patients with PF > 1 (Se = 88 %; Sp = 68 %; AUC = 0.7; p = 0.0081). IPTCLP (41 patients): the presence of PF = 1-2 showed EFS and OS reduction. A 3-year EFS rate for 1-2 PF was 25 vs. 70% for PF = 0 (p = 0.003). Thus, 3-year OS in patients with PF = 0 vs. PF = 1-2 was 100 vs. 20% (p = 0.0001). PIT (42 patients): better 3-year EFS and OS in patients with PF = 0 vs. PF = 1-3 (88 vs. 28% and 100 vs. 34%, respectively, p = 0.001). Patients with PF = 1-3 have a higher rate of relapses vs. PF = 0 (p = 0.0005 by Cox-test). mPIT (21 patients): no significant difference between PF and clinical outcomes. T-cell score (18 patients): higher survival rates with PF 2. More than 2 PF have an impact on EFS (p = 0.005). The 3-years OS in patients with PF 2 was 77 vs. 25% in cases with PF 3 (p = 0.001). CONCLUSION: IPI, PIT, IPTCLP are still very useful in defining risk stratification. As to mPIT and T-cell score, more patients to evaluate their prognostication possibility are needed.

[PROGNOSTIC RISK FACTORS FOR NEWLY DIAGNOSED PATIENTS WITH ADVANCED STAGES CLASSICAL HODGKIN'S LYMPHOMA].

Determination the complex of new prognostic risk factors will optimize and improve treatment and survival of patients with primary Hodgkin's Lymphoma.

[Primary bone lymphoma].

Presented literature data on the occurrence, diagnosis and treatment of primary lymphoma of bone. Case reports described in medical practice.

[Efficiency therapy with rituximab in patients with non-Hodgkin's lymphomas].

Management of non-Hodgkin's lymphomas (NHL) has been significantly changed during last 40 years from radiotherapy to modern polychemotherapy with application of monoclonal antibodies. A ten year experience of the treatment of patients with B-cell NHL with rituximab application was accumulated in National Cancer Institute. The group of patients with NHL, who were treated in oncohematology department in the period from 2001 to 2010, was chosen for study of the efficacy of the treatment with rituximab. The immediate and distant results of the treatment were evaluated in this group of patients. The usage of the polychemotherapy with rituximab was highly efficient in the treatment of primary and relapsed patients with NHL. This group shows high rate of the immediate and distant results of treatment.

[Treatment of elderly patients with non-Hodgkin's lymphoma].

The analysis of the specifics of the flow of Non-Hodgkin's lymphomas in elderly patients is presented. Comparative analysis of the treatment efficacy and toxicity with schemes CHOP and CNOP in patients is presented.

[Thromboprophylaxis in patients with multiply myeloma who receives chemotherapy with thalidomide].

Achievements of world practice about development and prophilaxis of tromboembolic events (VTE) in cancer patients. Particular attention is paid to prevention of VTE in patients with multiply myeloma who receive chemotherapy with Thalidomide.

[The treatment of patients with refractory forms of Hodgkin's lymphomas].

The treatment of patients with refractory forms of Hodgkin's lymphomas in combination with chemo-radiotherapy is priority. Nevertheless, chemotherapy has to include multicomponent schemes and radiation therapy should be an addition and consolidative method to chemotherapy.

[Main issues of diagnosis and treatment of lymphomas in Ukraine].

Number of patients with lymphomas in Ukraine increases annual. The increase of morbidity during 2007-2008 years is 4.3-8.1%. The rate of effectiveness of the treatment and patient's survival in Ukraine is lower then the world rate. Overview of main issues of the lymphoma's diagnosis and treatment in Ukraine is presented in this article.

[Experience of using thalidomide in the treatment of patients with multiple myeloma].

The results of treatment of patients with multiple myeloma with thalidomide and complications of this treatment are presented. Monotherapy with thalidomide and its combinations with corticosteroids and cytostatics is an effective as first line treatment and as a treatment of patients with refractory disease. The most common side effect is toxic neuropathy. Other complications are less common and are easily controlled with medications.

[Expression of PRAME gene in multiple myeloma]. / Ekspressiia gena PRAME pri mnozhestvennoi mielome.

AIM: To determine clinical significance of PRAME gene expression in multiple myeloma (MM) and feasibility of its use as a marker of residual tumor clone. MATERIAL AND METHODS: 35 MM patients, of them 15 were newly diagnosed and 20 had resistance to previous therapy. PRAME was made if the patients received programmed therapy with high-dose chemotherapy (VD) and autologous transplantation of peripheral cell stem cells. 12 PRAME-positive patients were examined on the day +100, 5 patients--a year later. Monoclonal paraprotein was detected by electrophoresis and radial immunodiffusion of blood serum. Bone marrow affection was assessed at roentgenography and/or MRI. PRAME gene expression in bone marrow biopsy was measured by reverse transcription and PCR amplification. RESULTS: Activation of expression of PRAME gene in MM was found in 68.57% patients. It was higher in patients with MM duration more than 1 year and if they were treated before (85%) than in new cases (46.67%). Expression of PRAME tended to associate with activity of LDP of blood serum. After the above chemotherapy and autotransplantation transcript PRAME did not disappear in 8 of 12 cases. One year after the treatment, of 5 PRAME-positive patients 2 died, 1 had recurrence, 2 are in a compete clinicohematological remission. CONCLUSION: Frequent activation of transcription of the gene PRAME in MM, its assay can be used for monitoring of the disease course, assessment of remission completeness, detection of tumor cell contamination of preparations of autologous stem cells of peripheral blood.

[Long-term results of treatment of chronic B-cell lymphoid leukocytes in persons who participated in the elimination of the effects of the nuclear accident at the Chernobyl nuclear station]. / Rezul'taty likuvannia khronichnoho B-klitynnoho limfoleikozu v uchasnykiv likvidatsiï naslidkiv avariï na ChAES u viddalenomu periodi.

Clinical-and-hematological characteristics are presented of B-cell chronic lymphoid leukosis in those persons who took part in the elimination of the effects of the Chornobyl accident in the remote period. Results are highlighted of treatment of 16 patients with making use of different chemical drug preparations. Employment of fludarabin and cyclophosphan combined in treatment of the medical condition in question has been shown to promote long-term complete and partial remissions in a major proportion of patients, which effect was not achievable with standard means of remediation. A side effect of fludarabin was leukocytopenia that in the presence of changed immunity threatened the patients with development and exacerbation of infections complications. Use of manax and erbisol moderated the immunosuppressive and toxic action of fludarabin, due to which fact infectious complications and drug-induced hepatitis have came to be less common, the patients' quality of life gotten better.