Introduction. At the initiative of the Russian Hematology Society, the research group for the study of idiopathic aplastic anemia has developed clinical recommendations for the diagnosis and treatment of idiopathic aplastic anemia.Aim: to standardize diagnostic and therapeutic approaches for the treatment of acquired aplastic anemia in Russia.Methods. The methodological approaches used are based on the principles of evidence-based medicine, based on the recommendations of the Russian council of experts on the diagnosis and treatment of patients with idiopathic aplastic anemia, Russian and international experience in managing patients, and the recommendations of the European group for the study of aplastic anemia.Results. A new revised and updated version of the national clinical guidelines is presented.Conclusion. These recommendations are intended for doctors of various specialties, health administrators, and medical school students.Conflict of interest: the authors declare no conflict of interest.Financial disclosure: this study did not have sponsorship.
Breast implant-associated anaplastic large-cell lymphoma will be identified as a separate nosological entity in the 2017 adapted WHO classification due to differences in its clinical presentations, pathogenesis, and prognosis with those of nodal and cutaneous anaplastic large-cell lymphomas. The paper gives a review of the literature and describes the authors' own clinical case of common breast implant-associated anaplastic large-cell lymphoma involving breast tissue, axillary lymph nodes, anterior chest muscles, and bone marrow. The treatment policy chosen by the authors could achieve complete remission.
Introduction. Induction chemotherapy (CT) for primary diffuse large B-cell lymphoma (DLBCL) of the central nervous system (CNS) is based on the use of methotrexate in high doses. An optimal consolidation strategy involves high-dose chemotherapy followed by autologous haematopoietic stem cell transplantation (auto-HSCT). The most effective conditioning regimen comprises a combination of chemotherapy agents including thiotepa.Aim. To present the authors’ experience of applying auto-HSCT/TBC in patients with primary DLBCL of the CNS.Methods. The prospective study CNS-2015 was carried out among 20 patients aged 20–52 years (median 42 years old) from 2015 to 2019. The male/female ratio came to 13/7. The somatic status of 17 (85 %) patients was 0–1 on the ECOG scale. Only 3 (15 %) patients showed the somatic status of 4 points. According to the criteria of the MSKCC prognostic system, 18 (90 %) and 2 (10 %) patients were assigned to the low-risk and medium-risk groups, respectively.Results. All patients included in the study received 3–5 cycles of chemotherapy with high doses of methotrexate, vincristine, procarbazine and rituximab (R-MPV), as well as underwent auto-HSCT following TBC-based conditioning regimen (thiotepa, busulfan, cyclophosphamide). Prior to auto-HSCT, 15 and 5 out of 20 patients having completed induction chemotherapy achieved complete remission and partial remission, respectively. Following auto-HSCT, complete remission was achieved in 5 patients with an initial partial response to treatment. All patients underwent temozolomide maintenance therapy for 2 years. With a median follow-up of 17 (1–46) months, 18 patients are alive and in remission. Two patients, who relapsed 4 and 5 months after auto-HSCT and achieved no response to the second line of chemotherapy and radiation therapy, died 24 and 26 months after auto-HSCT.Conclusion. R-MPV is an effective treatment for patients with primary DLBCL of CNS, which is not accompanied by severe toxicity. The use of high-dose chemotherapy with TBC allows a high remission rate to be achieved. The mortality associated with treatment in the group of patients included in the study came to 0 %.
Introduction. Rodenticides are pesticides used in the control of rodents. In Russia, only anticoagulant rodenticides are allowed to be used.Aim: describe a case of mass poisoning with anticoagulant rodenticides.Main findings. An observation is given of poisoning with anticoagulant rodenticides in 80 people due to the consumption of sunflower oil produced from seeds that have been treated with rodenticides. The victims had a pronounced hemorrhagic syndrome: all had ecchymosis, 79 % had macrohematuria, 1 had uterine bleeding, 3 had intra-abdominal hemorrhages, 16 had nosebleeds, 2 had gastrointestinal bleeding, and 2 had intracerebral hemorrhages. The international normalized ratio (INR) was not definable in 56 patients, while the remaining patients had a median INR of 3.9 (fluctuations from 1.29 to 16.2). Activated partial thromboplastin time (APTT) was not definable in 7 patients; the remaining patients had the median APTT of 65 seconds. Three of the victims died of hemorrhagic syndrome. This article analyzes the conducted therapy. In life-threatening hemorrhagic syndrome induced by rodenticide poisoning or warfarin overdose the drugs of choice are prothrombin complex concentrates and recombinant activated clotting factor VII, but not fresh frozen plasma and vicasol. For long-term therapy, vitamin K1 should be used.Conflict of interest: the authors declare no conflict of interest.
Сytokine release syndrome is the common complication of CAR-T therapy. We report a case of patient with B-cell acute lymphoblastic leukemia developing сytokine release syndrome with shock and multiple organ failure and requiring cytokine removal and hemodiafiltration. Remission of the disease was achieved after CAR-T therapy.
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