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Context CD23 expression in follicular lymphoma (FL) is associated with better overall survival (OS). It is known that FL cases with inguinal lymph node lesions and large-cell morphology are characterized by CD23 expression, which is a distinctive feature of these patients whose prognosis is also favorable. However, the CD23 prognostic significance in various FL morphological grades and other tumor lesion localization is unclear. Objective To determine the CD23 expression prognostic value in different FL grades. Design CD23 expression pattern was evaluated by immunohistochemistry. Three groups of patients were identified: expression is absent (CD23neg), intense expression in all cells (CD23bright), expression is weak and/or only present in a portion of tumor cells (CD23dim). Patients The study included 206 FL patients, including 93 FL1–2 grade, 68 FL3A, 45 FL3B, and transformation into DLBCL. In 102 patients, inguinal region involvement was noted. Results In general, OS was similar for the CD23neg, CD23bright, and CD23dim groups. There was also no correlation between OS and CD23 expression in patients with and without iliac region involvement. However, we found that CD23 prognostic value depends on FL grade. In FL1–2 and aggressive FL, there was no association between OS and CD23 expression. In FL3A patients, OS in the CD23dim group was lower than in the CD23neg and CD23 bright groups. The 5-year OS in the CD23neg, CD23bright, and CD23dim groups were 91%, 87%, and 65%, respectively. The level of these differences tended to be significant (log-rank test, p=0.09). Conclusions The previously shown association between CD23 expression, lesion localization, and OS was not confirmed. However, CD23 expression pattern analysis revealed a fact not previously known. The intermediate CD23 expression pattern is an unfavorable prognostic factor in FL3A patients. It is worth noting that combining CD23dim with CD23neg into a single group resulted in a “favorable” status compared with the CD23bright group. Thus, expression cutoff is important, and we assume this phenomenon has not been previously noted because the true CD23bright value was not identified. The heterogeneous CD23 expression in the tumor population probably indicates clonal tumor heterogeneity, which correlates with an unfavorable disease course.

Context: Composite lymphomas (CL) can occur simultaneously or sequentially and are of a particular interest in hematology. Objective: Approaches to the diagnosis and treatment of CL are complex with a full range of modern laboratory methods. Design: Analysis of cases of CL with histological and molecular features of these cases. Setting: Study setting can be used in hematological inpatient and outpatient departments. Patients: There were 37 cases of CL (M:F=2:1, median age 64 (38-83 years)). Interventions: All patients underwent a full range of diagnostic procedures, including histological, immunophenotypic, cytogenetic and molecular tests. Main outcomes measures: Study the combination types of CL. Results: Metachronous CL were observed in 17 cases; synchronous CL in 20 cases. Metachronous CL cases: the most common type of the second lymphoma was diffuse B-large cell lymphoma (DBLCL) with 58.8% and Hodgkin's lymphoma (HL) with 29.4%. In 30% of cases, DBLCL developed after HL therapy, and in 30% - after therapy of angioimmunoblastic lymphoma. The median time to development of the second lymphoproliferative disease (LD) was 6 years (range 7 mo - 40 yrs). Synchronous CL were observed in 55% of cases; the most common were the combination of chronic lymphocytic leukemia (CLL) with other LD, hairy cell leukemia (HCL) 54.5%, large granular lymphocytic leukemia, marginal zone lymphoma (MZL), follicular lymphoma, multiple myeloma, and fungal mycosis. Combination of HL with other LD was observed in 25% of cases (HL+MZL represent 60% of cases). It is not clear whether HL in this combination represents a clonal transformation from MZL cells or it is a secondary tumor that develops de novo after MZL treatment. Since common IGVH gene rearrangements and somatic mutation status could be detected for both types of cells; this may indicate that MZL and HL cell clones may originate from a single B–cell of the follicular germinal center. Conclusions: Careful examination is needed in atypical clinical course of lymphomas to exclude the second LD, including clonally related. CL are excellent models for studying the complex process of tumor cells evolution during the course of the disease. Treatment of CL is a non-trivial task and aims to control both diseases.

Background: Anaplastic large cell ALK-positive lymphoma (ALCL ALK+) is a lymphoproliferative disease of the “young” age. ALCL ALK+ has a favourable prognosis. A failure in therapy (relapse) is diagnosed within the first 1.5-2 years of the follow up period. Aim: The aim of the present study was to compare the effectiveness of the NHL BFM-90 and CHOEP protocols intended for the treatment of adult patients presenting with the ALCL ALK+. Materials and methods: This prospective comparative randomized study was carried out based at the National Medical Research Centre of Hematology, Moscow, during the period from June 2014 to December 2019. It involved 23 patients with the ALCL ALK+ disease randomly allocated to two groups. One of them included 11 patients given a CHOEP, the other consisted of 12 patients treated using a high-dose CT in accordance with the NHL BFM-90 protocol (age medians 33 and 40 years respectively). Results: The 3-year OS and EFS rates among the patients receiving CHOEP was 91%, with all the members of this study group having completed the treatment regimen. All patients comprising the latter (NHL BFM-90) group achieved complete remission. However, only 9 of the 12 patients fully complied with the treatment protocol because of its toxic effects. It can be inferred that the two study groups are comparable in terms of the 3-year OS and EFS rates among the patients treated in accordance with the CHOEP and NHL BFM-90 programs, the differences between them being insignificant. Conclusion: High-dose CT based on the NHL BFM-90 protocol has no advantages over the less toxic CHOEP regimen as far as the treatment of the ALCL ALK+ is concerned (if the efficacy of both modalities is estimated in terms of the 3-year OS and EFS rates). However, additional studies with larger patient samples are needed to obtain statistically significant results before a definitive conclusion can be made as regards clinical feasibility of the use of high-dose therapeutic regimens for the treatment of adult patients presenting with the ALCL ALK+ disease.