HEMATOLOGIC MALIGNANCIES: DIAGNOSIS, TREATMENT, SUPPORTIVE CARE
Castleman disease (angiofollicular hyperplasia of lymph nodes) – a rare benign lymphoproliferative disease with prolonged asymptomatic course, associated with a wide variety of autoimmune and oncological diseases and the risk of non-Hodgkin’s lymphoma. The rare occurrence of this disease and a variety of clinical course did not allow for a complete and consistent research on the etiology and pathogenesis and the standard therapies development. In recent years, the number of patients with Castleman disease in the Russian Federation has increased, which requires its recognition among non-neoplastic and neoplastic lymphadenopathy. The article provides an overview about clinical and histological variants of Castleman’s disease, its pathogenesis concepts, classification and treatment.
Lineage switch is a rare phenomenon in which a transition from lymphoid to myeloid was observed in relapse of acute leukemia, or vice versa. This paper presents the four clinical case reports of acute lymphoblastic leukemia with MLL gene rearrangement (KMT2A) with myeloid phenotype in relapse.
In this pilot study results of prolonged (up to 74 month) follow-up of 16 CLL patients with del17p13 or del11q22 in the first I-FISH analysis have been presented. It was shown that the presences of del17p13 or del11q22 as well as the rate of the aberrant cells having those abnormalities are significant for the prognosis and clinical course of CLL. The high rate (more than 70 %) of cells with 17p13 deletion means very unfavorable cytogenetic marker and indicates the short survival. On the other hand the low rate of cells with 17p13 deletion may predict disease progression but in case of effective treatment there are no effects on the patient’s survival. The high rate (more than 70 %) of cells with del11q22 indicates the early requiring for treatment. It was revealed the frequency of cells with marker aberrations may change in the same patient including decreasing to the control level as result of treatment and without it too. The frequency of aberrant cells decreases significantly after the finishing of appropriated treatments program and usually returns to its previous level in case of relapse and/or progression of disease. The subclones’ ratio between different marker abnormalities could vary during treatment and after its ending. It’s also possible the occurrence of new leukemic cells subclones. Taking in consideration the wide range of clinical CLL course and the possibility of the clonal evolution, the FISH-analysis should be as integral part of clinical practice both during the CLL staging and the next patient’s follow-up.
Patients with myelofibrosis in blast-phase commonly have a median overall survival of only 3–6 months. Given the older median age of onset and heavy pretreatment, intensive chemotherapy often is not appropriate and has low efficacy with high toxicity. Ruxolitinib (a JAK1/2 inhibitor) has provided significant clinical benefits in patients with chronic phase myelofibrosis. We report our experience of treating blastphase myelofibrosis patients with the combination therapy of ruxolitinib plus low dose mercaptopurine or low-dose cytarabine. The cases presented here demonstrated the feasibility and tolerability of combination continuous ruxolitinib treatment with mercaptopurine or low-dose cytarabine for patients with blast-phase myelofibrosis. The efficacy of these combination regimens is encouraging.
BASIC RESEARCH
The wide range of techniques could be employed to find mismatches in minor histocompatibility antigens between transplant recipients and their donors. In the current study we compared three genotyping methods based on polymerase chain reaction (PCR) for four minor antigens. Three of the tested methods: allele-specific PCR, restriction fragment length polymorphism and real-time PCR with TaqMan probes demonstrated 100% reliability when compared to Sanger sequencing for all of the studied polymorphisms. High resolution melting analysis was unsuitable for genotyping of one of the tested minor antigens (HA-1) as it has linked synonymous polymorphism. Obtained data could be used to select the strategy for large-scale clinical genotyping.
INVITATION TO DISCUSS
In the era of evidence-based medicine the main reason for the implementation of new treatment methods are the results of clinical trials. However, clinical trials is a separate area of knowledge, which is often little known to clinicians, that does not allow them to adequately interpret the results of studies. The article describes the basic principles for the conduct and interpretation of clinical trials in hematology and oncology.
CONGRESSES, CONFERENCES, SYMPOSIA
ISSN 2413-4023 (Online)