The Role of BAALC-Expressing Leukemia Precursor Cells in the Pathogenesis of Myelodysplastic Syndromes

NN Mamaev, MV Latypova, AI Shakirova, TL Gindina, MM Kanunnikov, NYu Tsvetkov, IM Barkhatov, SN Bondarenko, MD Vladovskaya, EV Morozova

RM Gorbacheva Scientific Research Institute of Pediatric Oncology, Hematology and Transplantation; IP Pavlov First Saint Petersburg State Medical University, 6/8 L’va Tolstogo str., Saint Petersburg, Russian Federation, 197022

For correspondence: Prof. Nikolai Nikolaevich Mamaev, MD, PhD, 6/8 L’va Tolstogo str., Saint Petersburg, Russian Federation, 197022; e-mail: nikmamaev524@gmail.com

For citation: Mamaev NN, Latypova MV, Shakirova AI, et al. The Role of BAALC-Expressing Leukemia Precursor Cells in the Pathogenesis of Myelodysplastic Syndromes. Clinical oncohematology. 2022;15(1):62–8. (In Russ).

DOI: 10.21320/2500-2139-2022-15-1-62-68


ABSTRACT

The present paper provides evidence for a high detection rate of BAALC gene overexpression, also combined with WT1 gene overexpression, in patients with myelodysplastic syndromes (MDS) and FISH-verified chromosome defects. The BAALC and WT1 gene expression profiling in 16 MDS patients (6 out of them received allogeneic hematopoietic stem cell transplantation) showed an increased BAALC expression in 14 patients. The expression level in 2 patients was near the cut-off. Low expression levels were identified in a female patient with isolated 5q deletion in karyotype and also with its combination with complex karyotype. On the other hand, the highest expression levels were reported in patients with normal karyotype and 3q26 locus rearrangement, which was associated with EVI1 gene overexpression. Since the BAALC expression level, at least in patients with the major (except for М3 and М7) FAB-variants of acute myeloid leukemias (AML), was closely associated with BAALC-producing precursor cells of leukemia clone, a profound study of this phenomenon in MDS patients seems to be important for understanding the finest mechanisms underlying the pathogenesis of AML and AML relapses on the level of precursor cells.

Keywords: myelodysplastic syndromes, BAALC and WT1 genes, overexpression, post-transplantation relapses, BAALC-producing precursor cells, pathogenesis, prognosis.

Received: July 7, 2021

Accepted: November 4, 2021

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Статистика Plumx английский

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Stevens-Johnson Syndrome after Treatment of Female Patient with Small Lymphocytic B-Cell Lymphoma, Autoimmune Hemolytic Anemia and Antiphospholipid Antibody Syndrome with Rituximab

AL Melikyan, IN Subortseva, AM Kovrigina, TI Kolosheinova, EK Egorova, EI Pustovaya

Hematology Research Center under the Ministry of Health of the Russian Federation, 4а Novyi Zykovskii pr-d, Moscow, Russian Federation, 125167

For correspondence: Irina Nikolaevna Subortseva, PhD, 4а Novyi Zykovskii pr-d, Moscow, Russian Federation, 125167; Tel.: +7(495)612-44-71; e-mail: soubortseva@yandex.ru

For citation: Melikyan AL, Subortseva IN, Kovrigina AM, et al. Stevens-Johnson Syndrome after Treatment of Female Patient with Small Lymphocytic B-Cell Lymphoma, Autoimmune Hemolytic Anemia and Antiphospholipid Antibody Syndrome with Rituximab Clinical oncohematology. 2017;10(1): 120–7 (In Russ).

DOI: 10.21320/2500-2139-2017-10-1-120-127


ABSTRACT

Stevens-Johnson syndrome is a severe delayed type systemic allergic reaction which affects the skin and mucous membranes. In adults, Stevens-Johnson syndrome is usually caused by the administration of drugs or a malignant process. The paper presents a case of Stevens-Johnson syndrome after the treatment of a female patient with small lymphocytic B-cell lymphoma, autoimmune hemolytic anemia and antiphospholipid antibody syndrome with rituximab. A rare combination of Stevens-Johnson syndrome and small lymphocytic B-cell lymphoma of small lymphocytes, as well as the development of severe delayed type systemic allergic reaction to introduction of rituximab are of special interest. A detailed medical history and the clinical manifestations of the disease allowed to diagnose Stevens-Johnson syndrome at early stages and prescribe an adequate therapy. As a result of the treatment, the patient’s condition has improved considerably. Symptoms of general toxicity were arrested completely; there was a complete epithelization of erosive defects. Therefore, the presented clinical observation shows that timely diagnosis, complex drug therapy, and comprehensive care can cure the diseases as soon as possible and prevent complications.

Keywords: Stevens-Johnson syndrome, pathogenesis, clinical manifestations, diagnosis, treatment, rituximab.

Received: July 28, 2016

Accepted: December 6, 2016

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Primary Mediastinal (Thymic) Large B-Cell Lymphoma

GS Tumyan, IZ Zavodnova, MYu Kichigina, EG Medvedovskaya

NN Blokhin Russian Cancer Research Center, 24 Kashirskoye sh., Moscow, Russian Federation, 115478

For correspondence: Gayane Sergeevna Tumyan, DSci, 24 Kashirskoye sh., Moscow, Russian Federation, 115478; Tel: +7(499)324-98-29; e-mail: gaytum@mail.ru

For citation: Tumyan GS, Zavodnova IZ, Kichigina MYu, Medvedovskaya EG. Primary Mediastinal (Thymic) Large B-Cell Lymphoma. Clinical oncohematology. 2017;10(1):13–24 (In Russ).

DOI: 10.21320/2500-2139-2017-10-1-13-24


ABSTRACT

Primary mediastinal (thymic) large B-cell lymphoma (PMBCL) is one of the primary extranodal tumors and originates from thymic medulla B cells. The disease is more common in young women and declares itself by mainly locally advanced growth within the anterior upper mediastinum with frequent involvement of chest organs. PMBCL has specific morphological, immunological, and genetic characteristics that permit to differentiate it from other similar diseases: diffuse large В-cell lymphoma, nodular sclerosis Hodgkin’s lymphoma, and mediastinal gray zone lymphoma. Immunochemotherapy with subsequent irradiation of the residual mediastinal tumor is the standard treatment of PMBCL. No benefits of one drug therapy over another have been demonstrated to date in controlled studies. Application of new imaging techniques (PET/CT) may result in withdrawal of the radiotherapy in some PMBCL patients without impairment of delayed survival rates.

Keywords: primary mediastinal (thymic) large B-cell lymphoma, primary extranodal lymphomas, diagnosis, pathogenesis, morphological, immunological/genetic characteristics, treatment.

Received: August 22, 2016

Accepted: December 17, 2016

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Lymphomas in HIV-Infected Patients: Literature Review

A.V. Pivnik1, N.V. Seregin2, Yu.G. Parkhomenko3,4, O.A. Tishkevich4, A.M. Kovrigina5, Е.B. Likunov6

1 N.N. Pletnev Moscow Clinical Research Center, Department of Health, Moscow, Russian Federation

2 Moscow Municipal Cancer Dispensary No. 3, Moscow, Russian Federation

3 Research Institute of Human Morphology, RAMS, Moscow, Russian Federation

4 Clinical Infectious Diseases Hospital No. 2, Department of Health, Moscow, Russian Federation

5 Hematology Research Center, RF MH, Moscow, Russian Federation

6 American Medical Center, Moscow, Russian Federation

Address correspondence to: pivnikav@gmail.com

For citation: Pivnik A.V., Seregin N.V., Parkhomenko Yu.G., Tishkevich O.A., Kovrigina A.M., Likunov E.B. Lymphomas in HIV-Infected Patients: Literature Review. Klin. onkogematol. 2014; 7(3): 264–77 (In Russ.).


ABSTRACT

This review presents data on incidence, pathogenesis, diagnosis, and treatment of lymphoid malignancies in HIV-infected patients. Articles published by Russian and foreign authors are being reviewed. The principle role of decreased CD4+ lymphocyte count in development of secondary diseases in HIV patients is emphasized. Data on the structure of death causes in HIV-infected patients published by Russian authors are presented. They demonstrate that lymphoma is the 5th most common death cause (of 6 leading death causes). Hodgkin’s lymphoma in HIV-infected patients is not discussed in the review, because its pathogenesis differs from that of aggressive lymphomas and requires a separate discussion.


Keywords: lymphomas in HIV-infected patients, HIV, AIDS, secondary disorders, death causes, pathogenesis, diagnosis, treatment.

Accepted: May 15, 2014

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