47 research outputs found

    Coinheritance of HbD-Punjab/Ξ²+-thalassemia (IVSI+5 G-C) in patient with Gilbert's syndrome

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    Thalassemia and qualitative hemoglobinopathy are hereditary disorders of Hb synthesis that lead to change in the Hb conformation or a decrease in the synthesis of structurally normal Hb, and consequently, to erythron pathology. Many variants of Hb are unstable or have altered affinity for oxygen, and, in heterozygous form can be associated with clinical and hematological manifestations (hemolytic anemia, hypochromic microcytic anemia, erythrocytosis). HbD-Punjab [Ξ²121 (GH4) Glu β†’ Gln; HBB: C.364G> C] is variant of Hb carrying the amino acid substitution in the 121 position of Ξ²-globin chain. In all cases reported so far, patients with HbD-Punjab/Ξ²+-thalassemia (IVSI+5 G-C) combination experienced typical thalassemia with hypochromic microcytosis. HbD-Punjab was detected by electrophoresis from 37 to 94% of total Hb. The article describes rare clinical case of the cohabitation of HbD-Punjab/Ξ²+-thalassemia (IVSI+5 G-C) in a patient with homozygous variant of Gilbert's syndrome observed in AS Loginov Moscow Clinical Scientific Center

    CUTANEOUS MANIFESTATIONS OF ANGIOIMMUNOBLASTIC T-CELL LYMPHOMA

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    Background: Angioimmunoblast T-cell lymphoma (AITL) is a rare T-cell lymphoproliferative disease that is accompanied by generalized lymphadenopathy, hepatosplenomegaly, intoxication symptoms and extranodal lesions. The extranodal manifestations of the disease frequently involve various skin changes. One of the first such manifestations is maculopapular rashes observed in about half of AITL patients and usually preceding the appearance of lymphadenopathy. Other forms of skin lesions accompany the disease considerably less frequently.Aim: To characterize the range of skin changes in patients suffering from AITL, to establish a correspondence between the nature of skin changes and their histological picture.Materials and methods: 54 AITL patients were being treated at the National Research Centre for Hematology from 2000 to 2017, with the male/female ratio being 30/24. The median age was 61 (29–81) years.Results: Changes in the skin were observed in 24 (44.4 %) of 54 AITL patients, out of whom 18 (75 %) and 6 (25 %) were male and female patients, respectively. Maculopapular rash was observed in 22 (91.7 %) out of 24 patients. The morphological and molecular investigations of skin biopsy specimens exhibiting maculopapular rash demonstrated nonspecific reactive changes. Patients with maculopapular rash demonstrated an increase in the level of total (polyclonal) IgE. Specific skin lesions detected in 8 (14.8 %) cases were represented by a β€˜livedo reticularis’, focal skin hyperpigmentation, erythroderma, left eyelid tumour and tumour in 3, 2, 1, 1 and 1 cases, respectively.Conclusion: Maculopapular rash frequently observed in AITL patients is a reactive process not associated with a specific skin lesion. Specific skin lesions in AITL are much less common and can be represented by various forms. In some AITL cases, skin changes of the reactive and tumour nature can be simultaneously observed

    Assessment of bone marrow biopsy in patients with masked polycythemia vera

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    Masked polycythemia vera (PV) is characterized by presence ot Jak2 mutation, specific morphological pattern in the bone marrow biopsy, and the lack demanding levels of Hb in accordance with criteria WHO, 2008 for PV. The purpose of this study was assessment of the pathomorphologic peculiarities of bone marrow biopsies in patients with masked PV. The group of 24 patients with masked PV was formed on the basis of morphological picture, clinical, laboratory and molecular data. Histological examination of bone marrow trephine biopsy in most cases showed hypercellular marrow 18/24 (75%) (age adujsted). Enlargement and rejuvenation of erythroid lineage was observed in 23/24 and 22/24 cases (95.9% and 91.7%). The histotopography of megakaryocytes in the majority of cases, 20/24 (83.3%) was characterized by discrete arrangement of megakaryocytes and forming intertrabecular loose clusters (3-16 cells). Typical for PV morphology of megakaryocytes was detected in the majority of cases, 19/24 (79.2%). There were 5/24 (20,8%) cases with characteristic features of essential thrombocythemia (Π•Π’-like features). The grade of stroma fibrosis in all cases was MF-0. Morphological picture in bone marrow biopsy of patients with masked PV was characteristic for PV in most cases. However, in some cases of masked PV the morphology part of megakaryocytes was similar to essential thrombocythemia or pre-fibrotic/early stage of primary myelofibrosis.ΠœΠ°ΡΠΊΠΈΡ€ΠΎΠ²Π°Π½Π½Π°Ρ истинная полицитСмия (ИП) являСтся клиничСской Ρ„ΠΎΡ€ΠΌΠΎΠΉ истинной ΠΏΠΎΠ»ΠΈΡ†ΠΈΡ‚Π΅ΠΌΠΈΠΈ, для ΠΊΠΎΡ‚ΠΎΡ€ΠΎΠΉ Ρ…Π°Ρ€Π°ΠΊΡ‚Π΅Ρ€Π½ΠΎ Π½Π°Π»ΠΈΡ‡ΠΈΠ΅ ΠΌΡƒΡ‚Π°Ρ†ΠΈΠΈ JAK2, ΡΠΎΠΎΡ‚Π²Π΅Ρ‚ΡΡ‚Π²ΡƒΡŽΡ‰Π΅ΠΉ морфологичСской ΠΊΠ°Ρ€Ρ‚ΠΈΠ½Ρ‹ Π² костном ΠΌΠΎΠ·Π³Π΅, ΠΈ нСдостаточный для установлСния Π΄ΠΈΠ°Π³Π½ΠΎΠ·Π° ΠΏΠΎ критСриям Π’ΠžΠ— 2008 ΡƒΡ€ΠΎΠ²Π΅Π½ΡŒ НЬ. ЦСлью Π΄Π°Π½Π½ΠΎΠ³ΠΎ исслСдования Π±Ρ‹Π»Π° ΠΎΡ†Π΅Π½ΠΊΠ° ΠΌΠΎΡ€Ρ„ΠΎΠ»ΠΎΠ³ΠΈΠΈ костного ΠΌΠΎΠ·Π³Π° Π½Π° ΠΌΠ°Ρ‚Π΅Ρ€ΠΈΠ°Π»Π΅ Ρ‚Ρ€Π΅ΠΏΠ°Π½ΠΎΠ±ΠΈΠΎΠΏΡ‚Π°Ρ‚ΠΎΠ² костного ΠΌΠΎΠ·Π³Π° Ρƒ ΠΏΠ°Ρ†ΠΈΠ΅Π½Ρ‚ΠΎΠ² с маскированной Ρ„ΠΎΡ€ΠΌΠΎΠΉ ИП. ΠœΠ΅Ρ‚ΠΎΠ΄Ρ‹. На основании клиничСских Π΄Π°Π½Π½Ρ‹Ρ… ΠΏΡ€ΠΈ динамичСском наблюдСнии, патоморфологичСского Π΄ΠΈΠ°Π³Π½ΠΎΠ·Π°, Π΄Π°Π½Π½Ρ‹Ρ… молСкулярного исслСдования Π±Ρ‹Π»Π° сформирована Π³Ρ€ΡƒΠΏΠΏΠ° ΠΈΠ· 24 ΠΏΠ°Ρ†ΠΈΠ΅Π½Ρ‚ΠΎΠ² с маскированной ИП. Π‘ ΠΏΠΎΠΌΠΎΡ‰ΡŒΡŽ Ρ€Π°Π·Ρ€Π°Π±ΠΎΡ‚Π°Π½Π½ΠΎΠ³ΠΎ гистологичСского ΠΊΠΎΠ΄ΠΈΡ„ΠΈΠΊΠ°Ρ‚ΠΎΡ€Π° ΠΏΡ€ΠΎΠ²ΠΎΠ΄ΠΈΠ»ΠΎΡΡŒ Π΄Π΅Ρ‚Π°Π»ΡŒΠ½ΠΎΠ΅ гистологичСскоС исслСдованиС ΠΏΠ΅Ρ€Π²ΠΈΡ‡Π½Ρ‹Ρ… Ρ‚Ρ€Π΅ΠΏΠ°Π½ΠΎΠ±ΠΈΠΎΠΏΡ‚Π°Ρ‚ΠΎΠ² костного ΠΌΠΎΠ·Π³Π°. ΠŸΡ€ΠΈ ΠΎΡ†Π΅Π½ΠΊΠ΅ стСпСни Ρ€Π΅Ρ‚ΠΈΠΊΡƒΠ»ΠΈΠ½ΠΎΠ²ΠΎΠ³ΠΎ Ρ„ΠΈΠ±Ρ€ΠΎΠ·Π° срСзы ΠΎΠΊΡ€Π°ΡˆΠΈΠ²Π°Π»ΠΈ ΠΏΠΎ Π“ΠΎΠΌΠΎΡ€ΠΈ. Π Π΅Π·ΡƒΠ»ΡŒΡ‚Π°Ρ‚Ρ‹: ΠŸΡ€ΠΈ гистологичСском исслСдовании Ρ‚Ρ€Π΅ΠΏΠ°Π½ΠΎΠ±ΠΈΠΎΠΏΡ‚Π°Ρ‚ΠΎΠ² костного ΠΌΠΎΠ·Π³Π° Π² Π±ΠΎΠ»ΡŒΡˆΠΈΠ½ΡΡ‚Π²Π΅ случаСв опрСдСлялся Π³ΠΈΠΏΠ΅Ρ€ΠΊΠ»Π΅Ρ‚ΠΎΡ‡Π½Ρ‹ΠΉ (ΠΎΡ‚Π½ΠΎΡΠΈΡ‚Π΅Π»ΡŒΠ½ΠΎ возрастной Π½ΠΎΡ€ΠΌΡ‹) костный ΠΌΠΎΠ·Π³ 18/24 (75%). Π’ΠΎ всСх случаях выявлСна пролифСрация ΠΌΠ΅Π³Π°ΠΊΠ°Ρ€ΠΈΠΎΡ†ΠΈΡ‚ΠΎΠ², ΠΏΠΎΠ»ΠΈΠΌΠΎΡ€Ρ„Π½Ρ‹Ρ… ΠΏΠΎ Ρ€Π°Π·ΠΌΠ΅Ρ€Ρƒ ΠΈ ΠΌΠΎΡ€Ρ„ΠΎΠ»ΠΎΠ³ΠΈΠΈ, с Π½Π°Π»ΠΈΡ‡ΠΈΠ΅ΠΌ Π°Ρ‚ΠΈΠΏΠΈΡ‡Π½Ρ‹Ρ… Ρ„ΠΎΡ€ΠΌ с гипСрсСгмСнтированными ядрами, со Π·Ρ€Π΅Π»ΠΎΠΉ ΠΌΠΎΡ€Ρ„ΠΎΠ»ΠΎΠ³ΠΈΠ΅ΠΉ. Π Π°ΡΡˆΠΈΡ€Π΅Π½ΠΈΠ΅ ΠΈ ΠΎΠΌΠΎΠ»ΠΎΠΆΠ΅Π½ΠΈΠ΅ эритроидного ростка наблюдалось соотвСтствСнно Π² 23/24 ΠΈ 22/24 случаях (95,9% ΠΈ 91,7%). ΠŸΡ€ΠΈ ΠΎΡ†Π΅Π½ΠΊΠ΅ гистотопографии ΠΌΠ΅Π³Π°ΠΊΠ°Ρ€ΠΈΠΎΡ†ΠΈΡ‚ΠΎΠ² Π² Π±ΠΎΠ»ΡŒΡˆΠΈΠ½ΡΡ‚Π²Π΅ случаСв 20/24 (83,3%) ΠΎΠΏΡ€Π΅Π΄Π΅Π»ΡΠ»ΠΎΡΡŒ сочСтаниС Ρ€Π°Π·Ρ€ΠΎΠ·Π½Π΅Π½Π½ΠΎΠ³ΠΎ располоТСния ΠΌΠ΅Π³Π°ΠΊΠ°Ρ€ΠΈΠΎΡ†ΠΈΡ‚ΠΎΠ² ΠΈ Ρ„ΠΎΡ€ΠΌΠΈΡ€ΠΎΠ²Π°Π½ΠΈΠ΅ Ρ€Ρ‹Ρ…Π»Ρ‹Ρ… кластСров (3-16 ΠΊΠ»Π΅Ρ‚ΠΎΠΊ). Π₯арактСрная для истинной ΠΏΠΎΠ»ΠΈΡ†ΠΈΡ‚Π΅ΠΌΠΈΠΈ морфология элСмСнтов ΠΌΠ΅Π³Π°ΠΊΠ°Ρ€ΠΈΠΎΡ†ΠΈΡ‚Π°Ρ€Π½ΠΎΠ³ΠΎ ростка наблюдалась Π² Π±ΠΎΠ»ΡŒΡˆΠΈΠ½ΡΡ‚Π²Π΅ случаСв 19/24 (79,2%). Π’ 5/24 (20,8) случаСв Π±Ρ‹Π»ΠΈ выявлСны ΠΏΡ€ΠΈΠ·Π½Π°ΠΊΠΈ, Ρ…Π°Ρ€Π°ΠΊΡ‚Π΅Ρ€Π½Ρ‹Π΅ для ΡΡΡΠ΅Π½Ρ†ΠΈΠ°Π»ΡŒΠ½ΠΎΠΉ Ρ‚Ρ€ΠΎΠΌΠ±ΠΎΡ†ΠΈΡ‚Π΅ΠΌΠΈΠΈ (Β«Π•Π’-likeΒ» ΠΏΡ€ΠΈΠ·Π½Π°ΠΊΠΈ). Π‘Ρ‚Π΅ΠΏΠ΅Π½ΡŒ Ρ„ΠΈΠ±Ρ€ΠΎΠ·Π° стромы Π²ΠΎ всСх случаях составляла MF-0. Π’Ρ‹Π²ΠΎΠ΄Ρ‹: ΠœΠΎΡ€Ρ„ΠΎΠ»ΠΎΠ³ΠΈΡ‡Π΅ΡΠΊΠ°Ρ ΠΊΠ°Ρ€Ρ‚ΠΈΠ½Π° костного ΠΌΠΎΠ·Π³Π° ΠΏΠ°Ρ†ΠΈΠ΅Π½Ρ‚ΠΎΠ² с маскированной Ρ„ΠΎΡ€ΠΌΠΎΠΉ ИП Π² Π±ΠΎΠ»ΡŒΡˆΠΈΠ½ΡΡ‚Π²Π΅ ΠΏΡ€ΠΎΠ°Π½Π°Π»ΠΈΠ·ΠΈΡ€ΠΎΠ²Π°Π½Π½Ρ‹Ρ… Π½Π°ΠΌΠΈ случаСв Π±Ρ‹Π»Π° Ρ…Π°Ρ€Π°ΠΊΡ‚Π΅Ρ€Π½ΠΎΠΉ для истинной ΠΏΠΎΠ»ΠΈΡ†ΠΈΡ‚Π΅ΠΌΠΈΠΈ. ВмСстС с Ρ‚Π΅ΠΌ, Π² части наблюдСний ΠΏΡ€ΠΈ маскированной ИП Π² ΠΌΠΎΡ€Ρ„ΠΎΠ»ΠΎΠ³ΠΈΠΈ ΠΈ гистотопографии ΠΌΠ΅Π³Π°ΠΊΠ°Ρ€ΠΈΠΎΡ†ΠΈΡ‚Π°Ρ€Π½ΠΎΠ³ΠΎ ростка ΠΎΠΏΡ€Π΅Π΄Π΅Π»ΡΠ»ΠΈΡΡŒ ΠΏΡ€ΠΈΠ·Π½Π°ΠΊΠΈ, Ρ…Π°Ρ€Π°ΠΊΡ‚Π΅Ρ€Π½Ρ‹Π΅ для ΡΡΡΠ΅Π½Ρ†ΠΈΠ°Π»ΡŒΠ½ΠΎΠΉ Ρ‚Ρ€ΠΎΠΌΠ±ΠΎΡ†ΠΈΡ‚Π΅ΠΌΠΈΠΈ ΠΈΠ»ΠΈ ΠΏΡ€Π΅-Ρ„ΠΈΠ±Ρ€ΠΎΠ·Π½ΠΎΠΉ/Ρ€Π°Π½Π½Π΅ΠΉ стадии ΠΏΠ΅Ρ€Π²ΠΈΡ‡Π½ΠΎΠ³ΠΎ ΠΌΠΈΠ΅Π»ΠΎΡ„ΠΈΠ±Ρ€ΠΎΠ·Π°

    Diagnostics and treatment challenges of Ph-like acute lymphoblastic leukemia: a description of 3 clinical cases

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    B-cell acute lymphoblastic leukemia (B-ALL) is a diverse group of malignant blood disorders both with regard to the biological properties of the tumor and to therapeutic approaches. Immunophenotyping, molecular genetic techniques, whole-genome sequencing characterize B-ALL as a very diverse group for sensitivity to chemotherapy and prognosis. We present three clinical cases of patients with B-ALL and expected good response to standard therapy, in whom standard protocol treatment failured: refractoriness, persistence of minimal residual disease (MRD), and progression (MRD increase). The remission in these patients was achieved after chemotherapy change to immunological targeted therapy. Nowadays a unified therapeutic approach to all primary patients of the B-ALL is considered generally outdated. Great efforts are carrying out to develop molecular genetic classifications. The molecular dissection of subtypes of B-ALL goes on, and new protocols for selective treatment with targeting are clearly outlined for each subtype of B-ALL

    First experience of using Brentuximab vedotin and modified program NHL-BFM-90 in the front-line treatment of patient with anaplastic large-cell lymphoma: a case report and a review of literature

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    Nodal anaplastic ALK-negative large cell lymphoma (nALCL, ALK-) is a В-cell lymphoma that is characterized by aggressive clinical course and low sensitivity to БНОР (cyclophosphamide, doxorubicin, vincristine, prednisolone) and other chemotherapy regimen. In the article we present a literature review and describe our clinical case of nALCL, ALK-. For the first time a combination of Brentuximab vedotin with modified program NHL-BFM-90 was used as a first-line therapy. As a result of immunochemotherapy a complete antineoplastic effect was obtained. For consolidation of this effect high-dose chemotherapy with following autologous blood stem cell transplantation was performed. The chosen treatment tactics allowed to achieve a complete remission in a medium risk group patient

    Π˜ΠΌΠΌΡƒΠ½ΠΎΠ³ΠΈΡΡ‚ΠΎΡ…ΠΈΠΌΠΈΡ‡Π΅ΡΠΊΠ°Ρ ΠΈ ΡƒΠ»ΡŒΡ‚Ρ€Π°ΡΡ‚Ρ€ΡƒΠΊΡ‚ΡƒΡ€Π½Π°Ρ визуализация коронавируса SARS-CoV-2 Π² тканях Π»Π΅Π³ΠΊΠΈΡ…, лимфатичСских ΡƒΠ·Π»ΠΎΠ² ΠΈ ΠΏΠΎΡ‡Π΅ΠΊ ΡƒΠΌΠ΅Ρ€ΡˆΠΈΡ… ΠΏΠ°Ρ†ΠΈΠ΅Π½Ρ‚ΠΎΠ²

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    The objective: immunohistochemical and electron microscopic imaging of the SARS-CoV-2 coronavirus in lung, lymph nodes and kidney tissues of patients who died of COVID-19.Subjects and Methods. For immunohistochemical tests, specimens of sections of formalin-fixed and paraffin-embedded tissues of the lungs, lymph nodes and kidneys of patients who died from COVID-19 were used. Quantitative assessment of the SARS-CoV-2 viral load level was carried by the original RT qPCR and calculated by the formula: SARS-CoV-2 copy number/ABL1 Ρ… 100 copies, expressed as the ratio of the true number of SARS-CoV-2 cDNA copies per 100 copies cDNA of the ABL1 gene. For morphological tests, samples of native lung, lymph node and kidney tissues were taken.Results. Immunohistochemical and electron microscopic tests revealed particles of the SARS-CoV-2 coronavirus in the cytoplasm of endothelial cells of air-blood barrier of the lungs, the vascular glomerulus of the kidneys, in the cytoplasm of macrophages of the lymph node, and also in cytoplasm of lymphocytes in the lumen of lung capillaries.ЦСль исслСдования: иммуногистохимичСская ΠΈ элСктронно-микроскопичСская визуализация коронавируса SARS-CoV-2 Π² тканях Π»Π΅Π³ΠΊΠΈΡ…, лимфатичСских ΡƒΠ·Π»ΠΎΠ² ΠΈ ΠΏΠΎΡ‡Π΅ΠΊ ΠΏΠ°Ρ†ΠΈΠ΅Π½Ρ‚ΠΎΠ², ΡƒΠΌΠ΅Ρ€ΡˆΠΈΡ… ΠΎΡ‚ COVID-19.ΠœΠ°Ρ‚Π΅Ρ€ΠΈΠ°Π»Ρ‹ ΠΈ ΠΌΠ΅Ρ‚ΠΎΠ΄Ρ‹. Для иммуногистохимичСских исслСдований Π² качСствС ΠΌΠ°Ρ‚Π΅Ρ€ΠΈΠ°Π»Π° ΠΈΡΠΏΠΎΠ»ΡŒΠ·ΠΎΠ²Π°Π½Ρ‹ срСзы фиксированных Π² Ρ„ΠΎΡ€ΠΌΠ°Π»ΠΈΠ½Π΅ ΠΈ Π·Π°ΠΊΠ»ΡŽΡ‡Π΅Π½Π½Ρ‹Ρ… Π² ΠΏΠ°Ρ€Π°Ρ„ΠΈΠ½ Ρ‚ΠΊΠ°Π½Π΅ΠΉ Π»Π΅Π³ΠΊΠΈΡ…, лимфатичСских ΡƒΠ·Π»ΠΎΠ² ΠΈ ΠΏΠΎΡ‡Π΅ΠΊ ΠΏΠ°Ρ†ΠΈΠ΅Π½Ρ‚ΠΎΠ², ΡƒΠΌΠ΅Ρ€ΡˆΠΈΡ… ΠΎΡ‚ COVID-19. ΠšΠΎΠ»ΠΈΡ‡Π΅ΡΡ‚Π²Π΅Π½Π½Π°Ρ ΠΎΡ†Π΅Π½ΠΊΠ° уровня вирусной Π½Π°Π³Ρ€ΡƒΠ·ΠΊΠΈ SARS-CoV-2 ΠΏΡ€ΠΎΠ²Π΅Π΄Π΅Π½Π° с использованиСм ΠΎΡ€ΠΈΠ³ΠΈΠ½Π°Π»ΡŒΠ½ΠΎΠ³ΠΎ способа кПЦР Π Π’ ΠΈ рассчитана ΠΏΠΎ Ρ„ΠΎΡ€ΠΌΡƒΠ»Π΅: число ΠΊΠΎΠΏΠΈΠΉ SARS-CoV-2/число ΠΊΠΎΠΏΠΈΠΉ ABL1 Ρ… 100, Π²Ρ‹Ρ€Π°ΠΆΠ΅Π½Π° ΠΎΡ‚Π½ΠΎΡˆΠ΅Π½ΠΈΠ΅ΠΌ истинного числа ΠΊΠΎΠΏΠΈΠΉ ΠΊΠ”ΠΠš SARS-CoV-2 Π½Π° 100 ΠΊΠΎΠΏΠΈΠΉ ΠΊΠ”ΠΠš Π³Π΅Π½Π° ABL1. Для морфологичСских исслСдований взяты Π½Π°Ρ‚ΠΈΠ²Π½Ρ‹Π΅ ΠΎΠ±Ρ€Π°Π·Ρ†Ρ‹ Ρ‚ΠΊΠ°Π½Π΅ΠΉ Π»Π΅Π³ΠΊΠΈΡ…, лимфатичСских ΡƒΠ·Π»ΠΎΠ² ΠΈ ΠΏΠΎΡ‡Π΅ΠΊ.Π Π΅Π·ΡƒΠ»ΡŒΡ‚Π°Ρ‚Ρ‹. ΠŸΡ€ΠΈ иммуногистохимичСском ΠΈ элСктронно-микроскопичСском исслСдовании частицы коронавируса SARS-CoV-2 Π±Ρ‹Π»ΠΈ ΠΎΠ±Π½Π°Ρ€ΡƒΠΆΠ΅Π½Ρ‹ Π² Ρ†ΠΈΡ‚ΠΎΠΏΠ»Π°Π·ΠΌΠ΅ ΡΠ½Π΄ΠΎΡ‚Π΅Π»ΠΈΠ°Π»ΡŒΠ½Ρ‹Ρ… ΠΊΠ»Π΅Ρ‚ΠΎΠΊ аэрогСматичСского Π±Π°Ρ€ΡŒΠ΅Ρ€Π° Π»Π΅Π³ΠΊΠΈΡ…, сосудистого ΠΊΠ»ΡƒΠ±ΠΎΡ‡ΠΊΠ° ΠΏΠΎΡ‡Π΅ΠΊ, Π² Ρ†ΠΈΡ‚ΠΎΠΏΠ»Π°Π·ΠΌΠ΅ ΠΌΠ°ΠΊΡ€ΠΎΡ„Π°Π³ΠΎΠ² лимфатичСского ΡƒΠ·Π»Π°, Π° Ρ‚Π°ΠΊΠΆΠ΅ Π² Ρ†ΠΈΡ‚ΠΎΠΏΠ»Π°Π·ΠΌΠ΅ Π»ΠΈΠΌΡ„ΠΎΡ†ΠΈΡ‚ΠΎΠ² Π² просвСтС капилляров Π»Π΅Π³ΠΊΠΈΡ…

    Results of program acute myeloid leukemia therapy use in National Medical Research Center for Hematology of the Ministry of Health of Russian Federation

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    Objective. To analyze treatment results of 172 patients with acute myeloid leukemia (AML) aged 18-60 years in National Medical Research Center for Hematology of MHRF. Materials and methods. Inductive and consolidation program for 139 (80%) patients was based on a standardized protocol: 4 courses β€œ7+3” with different anthracycline use (2 courses of daunorubicin, idarubicin, mitoxantrone) and continuous use of cytarabine on the second inductive course. In 20% of patients cytarabine courses at the dose of 1 g/m2 2 times a day for 1-3 days combined with idarubicin and mitoxantrone were used as two consolidation courses. Allogenic bone marrow transplantation was performed in the first complete remission (CR) period in 40% of patients. Results. The frequency of CR achievement in all patients was 78.6%, refractory forms were observed in 13.9% of patients, early mortality - in 7.5% of patients. Seven-year overall survival (OS) rate was 40.7%, relapse free survival (RFS) - 43.2%. When estimating effectiveness depending on cytogenetic risk group it was demonstrated that 5-year OS and RFS in patients with translocation (8; 21) cannot be considered as satisfying, it accounted for 50 and 34%, respectively. At the same time in patients with 16th chromosome inversion (inv16) these characteristics accounted for 68.6 and 63.5%. Acquired results forced reconsidering of the consolidation program in AML patients of this subgroup. The median time to allogenic blood stem cells transplantation (allo-BSCT) in patients with first CR was 6.5 months that was taken as a reference point in landmark analysis of patients in whom allo-BSCT was not performed. Landmark analysis showed that in AML patients of favorable prognosis group allo-BSCT does not significantly reduce the probability of relapse (0 and 36%) and does not influence RFS (33 and 64%). In patients of border-line and poor prognosis allo-BSCT significantly reduces relapse probability (26 and 66%; 20 and 100%) and significantly increases a 7-year RFS (68.7 and 30%; 45.6 and 0%). Allo-BSCT also results in significant RFS increase and reduces the probability of relapse (25 and 78%) in patients in whom CR was achieved only after the second induction course. At the same time allo-BSCT does not influence patients who achieved CR after the first treatment course: 55 and 50%. Conclusion. Multivariate analysis showed that cytogenetic risk group (HR=2.3), time of CR achievement (HR=2.9), and allo-BSCT transplantation (HR=0.16) are independent factors for disease relapse prognosis after achieving CR

    BRAF and MAP2K1 mutations in hairy cell leukemia and splenic marginal zone B-cell lymphoma

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    DNA samples from patients with hairy cell leukemia (HCL) and splenic marginal zone B-cell lymphoma (MZBCL) for BRAF andΒ MAP2K1 activating mutations were analyzed. V600E mutation of BRAF was detected in 39 (98 %) of 40 patients with hairy cell leukemia,Β and no patient with MZBCL. In none of the patients in this study any other activating mutations than V600E in exons 11 and 15Β of BRAF gene were revealed. MAP2K1 Q56P mutation characterized by IGHV4-34 expression was detected in 1 (2 %) of 40 patients withΒ HCL. In none of the patients with MZBCL activating mutations in the 2, 3 or 11 exons of MAR2K1 gene have been identified, includingΒ those with IGHV4-34 expression
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