diagnosis

Current State of Diagnosis and Treatment of Acute Myeloid Leukemias in Adult Patients in the Republic of Kazakhstan

AUTOIMMUNE THROMBOCYTOPENIA ,

AA Klodzinskii1, IA Pivovarova1, LG Turgunova2, AZh Anafina2, AV Zinchenko1

1 Center for Hematology, branch of Karaganda Center for Hematology, 41/43 Erubaeva str., Karaganda, Republic of Kazakhstan, 100012

2 Medical University of Karaganda, 40 Gogolya str., Karaganda, Republic of Kazakhstan, 100008

For correspondence: Aimzhan Zharkynovna Anafina, 40 Gogolya str., Karaganda, Republic of Kazakhstan, 100008; Tel.: +7(701)493-54-16; e-mail: aimzhan_31_08@mail.ru

For citation: Klodzinskii AA, Pivovarova IA, Turgunova LG, et al. Current State of Diagnosis and Treatment of Acute Myeloid Leukemias in Adult Patients in the Republic of Kazakhstan. Clinical oncohematology. 2022;15(1):69–75. (In Russ).

DOI: 10.21320/2500-2139-2022-15-1-69-75

ABSTRACT

Background. In recent years, the incidence of acute myeloid leukemias (AML) globally has continued to increase. Current approaches to AML treatment remain a challenge for the healthcare in many countries. There are only single studies on the analysis of AML state in adult patients in Kazakhstan. Over the last 10 years in Kazakhstan, no results of AML monitoring in adult patients have been available.

Aim. To study the characteristics of clinical course and treatment outcomes in AML in the Central Kazakhstan and in the city of Ust-Kamenogorsk, East Kazakhstan Region.

Materials & Methods. The study enrolled 86 AML patients (46 men and 40 women), the median age was 60.5 years (range 19–86 years); 64 (74.4 %) patients were from Karaganda Region, 15 (17.4 %) patients were from Ust-Kamenogorsk, and 7 (8.1 %) patients were from other regions of Kazakhstan. The analysis covered the structure and treatment outcomes in newly diagnosed AML patients within the period from 2018 to June, 2021. Statistical analysis of data was made using SPSS Statistics 23.0.

Results. The analysis of diagnostic techniques showed that myelogram and immunophenotyping were used in 98.8 %, cytogenetic assay was made in 18 %, and molecular analysis was performed in 59.3 % of patients. The “7+3” remission induction was administered in 54.6 % of patients, 20.9 % of patients were treated with hypomethylating agents and low doses of cytarabine, and 24.4 % of patients were on palliative and supportive therapy. Out of 47 patients treated with the “7+3” remission induction, complete clinical hematological remission was reached in 29 (61.7 %) patients. Primary resistance was reported in 21.3 % of patients. Early mortality (death within 30 days from the start of induction) rate was 17 %. High-dose cytarabine consolidation (1.5–3 g/m2 twice every other day, 2–3 courses) was administered to 75.8 % of patients. All the allogeneic bone marrow transplantations (n = 7) were performed at the National Research Center for Oncology and Transplantology in Nur-Sultan. The median overall survival in the group of standard “7+3” chemotherapy recipients was 11 months (range 1–83 months), and the median disease-free survival was 9 months (range 2–79 months).

Conclusion. The study presents the characteristics and short-term outcomes of treatment of adult AML patients in Kazakhstan. The study limitations were a short follow-up period and enrollment of patients only from two regions of Kazakhstan. It is necessary to continue improving the current standards of AML diagnosis and treatment of adult patients.

Keywords: Kazakhstan, acute myeloid leukemias, diagnosis, treatment.

Received: September 7, 2021

Accepted: December 10, 2021

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Статистика Plumx английский

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Systemic Т-Cell Lymphoproliferative Disease Associated with Epstein-Barr Virus: A Literature Review and a Case Report

AUTOIMMUNE THROMBOCYTOPENIA , ,

EA Shalamova, AM Kovrigina, IA Shupletsova, EE Nikulina, VD Latyshev, NV Tsvetaeva

National Research Center for Hematology, 4 Novyi Zykovskii pr-d, Moscow, Russian Federation, 125167

For correspondence: Alla Mikhailovna Kovrigina, PhD in Biology, 4 Novyi Zykovskii pr-d, Moscow, Russian Federation, 125167; e-mail: kovrigina.alla@gmail.com

For citation: Shalamova EA, Kovrigina AM, Shupletsova IA, et al. Systemic Т-Cell Lymphoproliferative Disease Associated with Epstein-Barr Virus: A Literature Review and a Case Report. Clinical oncohematology. 2021;14(4):477–87. (In Russ).

DOI: 10.21320/2500-2139-2021-14-4-477-487

ABSTRACT

Epstein-Barr virus (EBV) is ubiquitous, being identified in 90–95 % of adults. Its reactivation in immunodeficiency conditions often leads to clonal transformation of В-lymphocytes and development of В-cell lymphoproliferative diseases (LPD) and В-cell lymphomas. At the same time, in the countries of North-East and East Asia, as well as Latin America, non-immunocompromised patients sometimes demonstrate the development of EBV-associated Т-cell lymphoproliferative diseases. The present paper reports a rare case of EBV-associated systemic T-LPD with lymphadenopathy, splenomegaly as well as acute autoimmune hemolytic anemia in a man of Caucasian race. Complex analysis of anamnestic, pathomorphological, and laboratory data allowed to distinguish this disease from Т-cell lymphoma and choose the appropriate patient management strategy.

Keywords: lymphoproliferative disease, Epstein-Barr virus, EBV+ T-LPD, diagnosis, pathomorphology.

Received: May 30, 2021

Accepted: September 2, 2021

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Статистика Plumx английский

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Clinical and Immunomorphological Characteristics of Lymphomatoid Papulosis Type E (Literature Review and Case Report)

AUTOIMMUNE THROMBOCYTOPENIA ,

TT Valiev1, AM Kovrigina2, TS Belysheva1

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

2 National Research Center for Hematology, 4 Novyi Zykovskii pr-d, Moscow, Russian Federation, 125167

For correspondence: Timur Teimurazovich Valiev, MD, PhD, 24 Kashirskoye sh., Moscow, Russian Federation, 115478; e-mail: timurvaliev@mail.ru

For citation: Valiev TT, Kovrigina AM, Belysheva TS. Clinical and Immunomorphological Characteristics of Lymphomatoid Papulosis Type E (Literature Review and Case Report). Clinical oncohematology. 2020;13(4):389–94. (In Russ).

DOI: 10.21320/2500-2139-2020-13-4-389-394

ABSTRACT

Lymphomatoid papulosis (LP) is a rare variant of benign lymphoproliferative disease with skin involvement. Based on clinical, morphological, and immunobiological characteristics, WHO hematopoietic and lymphoid tissue tumor classification (2016) differentiates between several LP types: А, В, С, D, Е, and with 6p25.3 rearrangement. The present article reviews the literature on clinical course, pathomorphological, immunological, and biomolecular characteristics of LP in adults and children. For the first time in the domestic literature, it provides a case report of LP, type E, in a 2-year-old child. Differential diagnosis and optimal disease management of LP are also described in detail.

Keywords: lymphomatoid papulosis, clinical features, diagnosis, treatment.

Received: June 9, 2020

Accepted: September 15, 2020

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Primary Bone Lymphomas: 18F-FDG PET and PET-CT as Methods of Diagnosis and Efficacy Estimation of Antitumor Treatment

AUTOIMMUNE THROMBOCYTOPENIA , , ,

AK Smol’yaninova1, ER Moskalets2, GA Yatsyk1, IE Kostina1, AS Bogolyubskaya3, NG Gabeeva1, EG Gemdzhian1, SA Tatarnikova1, DS Badmadzhapova1, EE Zvonkov1

1 National Research Center for Hematology, 4 Novyi Zykovskii pr-d, Moscow, Russian Federation, 125167

2 European Medical Center, 35 Shchepkina str., Moscow, Russian Federation, 129090

3 NI Pirogov Russian National Research Medical University, 1 Ostrovityanova str., Moscow, Russian Federation, 117997

For correspondence: Anna Konstantinovna Smol’yaninova, MD, PhD, 4 Novyi Zykovskii pr-d, Moscow, Russian Federation, 125167; Tel.: +7(926)912-31-16; e-mail: annmo8@mail.ru

For citation: Smol’yaninova AK, Moskalets ER, Yatsyk GA, et al. Primary Bone Lymphomas: 18F-FDG PET and PET-CT as Methods of Diagnosis and Efficacy Estimation of Antitumor Treatment. Clinical oncohematology. 2020;13(1):33–49 (In Russ).

DOI: 10.21320/2500-2139-2020-13-1-33-49

ABSTRACT

Background. Primary bone lymphoma (PBL) is a rare malignant tumor. Initial examination aimed at detecting all primary lesions is an indispensable prerequisite for the choice of optimal antitumor treatment. Standard methods of diagnosis (X-ray, CT, and MRI) are not always adequate to measure the real tumor mass. Another well-known characteristic feature of PBL is a challenge in evaluating the effect of its treatment because of residual changes in the bones of most patients. However, the data on using 18F-FDG PET, another method of metabolic imaging, in PBL are rather rare in accessible literature.

Aim. To study the specific use of PET with 18F-FDG at initial examination and efficacy estimation of PBL treatment.

Materials & Methods. The trial included 21 PBL patients who received PET with 18F-FDG at initial examination and a month after the end of treatment. The results of 18F-FDG PET imaging were compared with the data obtained by means of structural diagnostic methods (CT and MRI) and the analysis of biopsy samples with pathologic lesions.

Results. Intensive uptake of 18F-FDG (SUVmax 8.6–40.1, mean SUVmax 23.5), according to PET data, was reported in all patients in those tumor lesions which were identified by the structural diagnostic methods and confirmed by biopsies. Besides, each of 21 cases showed pathologic infiltration of adjacent soft tissues with high metabolic activity. In PET-CT with 18F-FDG 13 further tumor localizations were revealed in 8 (38 %) patients. On completing the therapy, according to CT and MRI data, residual changes were observed in all (n = 21, 100 %) patients. The residual metabolic activity in the involved bones was identified in 13 (62 %) patients (SUVmax 2.91–8.7, mean SUVmax 4.2). In 4 of them the residual lesions were subjected to biopsy. None of 4 cases was reported to show tumors. Only in 1 out of 13 patients with residual metabolic changes a tumor relapse was detected. Overall 10-year survival in the groups of patients with and without FDG+ residual changes was 91 % and 100 %, respectively, with insignificant differences (p = 0.39).

Conclusion. PET-CT with 18F-FDG is a highly sensitive technique for evaluating the primary lesion volumes in PBL patients. In 100 % of bone and soft tissue lesions an intensive uptake of 18F-FDG was observed. At the same time our study showed persistent metabolic activity on completing antitumor treatment in more than a half of patients, and in most of them it was not caused by tumor. Therefore, in our view, ongoing residual metabolic activity in PBL cannot always be regarded as an indication for continued treatment or consolidation radiotherapy.

Keywords: primary bone lymphoma, survival, positron emission tomography, diagnosis, efficacy estimation of antitumor treatment.

Received: August 2, 2019

Accepted: December 5, 2019

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EBV-Positive Lymphoproliferative Diseases: A New Concept and Differential Diagnosis (Literature Review and Case Reports)

LYMPHOID TUMORS , , , , , , , ,

АM Kovrigina

National Research Center for Hematology, 4 Novyi Zykovskii pr-d, Moscow, Russian Federation, 125167

For correspondence: Professor Alla Mikhailovna Kovrigina, PhD in Biology, 4 Novyi Zykovskii pr-d, Moscow, Russian Federation, 125167; e-mail: kovrigina.alla@gmail.com

For citation: Kovrigina AM. EBV-Positive Lymphoproliferative Diseases: A New Concept and Differential Diagnosis (Literature Review and Case Reports). Clinical oncohematology. 2018;11(4):326–37.

DOI: 10.21320/2500-2139-2018-11-4-326-337

ABSTRACT

In recent years increasing attention focuses on the concept of EBV-positive lymphoproliferative diseases related to primary or secondary immunodeficiency resulting from immunosuppressive therapy and persistent infections. Due to the progress of treatment methods in oncohematology and oncology this pathology also occurs as a delayed event when new surgical and therapeutic approaches are applied. The paper presents proof for the pathogenetic significance of Epstein-Barr virus (EBV) in the pathology under consideration with its various clinical manifestations and describes the evolution of knowledge on posttransplant lymphoproliferative disorders with their morphological classification underlying EBV+ lymphoproliferative diseases. The WHO Classification of Tumours of Hematopoietic and Lymphoid Tissues revised in 2017 includes new forms of EBV+ lymphoproliferative diseases (mucocutaneous ulcer, T- and NK-cell childhood lymphoproliferative disorders including cutaneous and systemic forms of chronic active EBV infection) and EBV+ large B-cell lymphomas (unspecified and fibrin-associated diffuse large B-cell lymphomas). The paper summarizes major characteristics of these diseases and exemplifies them by the author’s own experience.

Keywords: B-, T-, NK-cell lymphoproliferative diseases, Epstein-Barr virus (EBV), immunodeficiency, immune imbalance, immunosuppression, morphology, diagnosis.

Received: July 20, 2018

Accepted: September 25, 2018

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  13. Cohen M, De Matteo E, Narbaitz M, et al. Epstein-Barr virus presence in pediatric diffuse large B-cell lymphoma reveals a particular association and latency patterns: Analysis of viral role in tumor microenvironment. Int J Cancer. 2013;132(7):1572–80. doi: 10.1002/ijc.27845.

  14. Uccini S, Al-Jadiry MF, Scarpino S, et al. Epstein-Barr virus-positive diffuse large B-cell lymphoma in children: A disease reminiscent of Epstein-Barr virus-positive diffuse large B-cell lymphoma of the elderly. Hum Pathol. 2015;46(5):716–24. doi: 10.1016/j.humpath.2015.01.011.

  15. Nicolae A, Pittaluga S, Abdullah S, et al. EBV-positive large B-cell lymphomas in young patients: A nodal lymphoma with evidence for a tolerogenic immune environment. Blood. 2015;126(7):863–72. doi: 10.1182/blood-2015-02-630632.

  16. Kunitomi A, Hasegawa Y, Asano N, et al. EBV-positive reactive hyperplasia progressed into EBV-positive diffuse large B-cell lymphoma of the elderly over a 6-year period. Intern Med. 2018;57(9):1287–90. doi: 10.2169/internalmedicine.9112-17.

  17. de la Hera Magallanes AI, Montes-Moreno S, Hernandez SG, et al. Early phase of Epstein-Barr virus (EBV)-positive diffuse large B cell lymphoma of the elderly mimicking EBV-positive reactive follicular hyperplasia. Histopathology. 2011;59(3):571–5. doi: 10.1111/j.1365-2559.2011.03950.x.

  18. Roberts TK, Chen X, Liao JJ. Diagnostic and therapeutic challenges of EBV-positive mucocutaneous ulcer: a case report and systematic review of the literature. Exp Hematol Oncol. 2016;5(1):13. doi: 10.1186/s40164-016-0042-5.

  19. Dojcinov SD, Venkataraman G, Raffeld M, et al. EBV positive mucocutaneous ulcer–a study of 26 cases associated with various sources of immunosuppression. Am J Surg Pathol. 2010;34(3):405–17. doi: 10.1097/PAS.0b013e3181cf8622.

  20. Docinov SD, Venkataraman G, Pittaluga S, et al. Age-related EBV-associated lymphoproliferative disorders in the Western population: a spectrum of reactive lymphoid hyperplasia and lymphoma. Blood. 2011;117(8):4726–35. doi: 10.1182/blood-2010-12-323238.

  21. Gratzinger D, Jong D, Jaffe ES, et al. T- and NK-Cell Lymphomas and Systemic Lymphoproliferative Disorders and the Immunodeficiency Setting: 2015 SH/EAHP Workshop Report—Part 4. Am J Clin Pathol. 2017;147(2):188–203. doi: 10.1093/AJCP/AQW213.

A Case Report of Myeloid Sarcoma in a Child

MYELOID TUMORS , ,

TT Valiev1, AM Kovrigina2, TR Panferova1, TL Ushakova1, IN Serebryakova3, NN Tupitsyn3, LYu Grivtsova3, II Matveeva3, EV Mikhailova1, AV Popa1, GL Menkevich1

1 Institute of Pediatric Oncology and Hematology, NN Blokhin Russian Cancer Research Center, 24 Kashirskoye sh., Moscow, Russian Federation, 115478

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

3 Institute of Clinical Oncology, NN Blokhin Russian Cancer Research Center, 24 Kashirskoye sh., Moscow, Russian Federation, 115478

For correspondence: Timur Teimurazovich Valiev, DSci, 24 Kashirskoye sh., Moscow, Russian Federation, 115478; Tel: +7(499)324-42-87; е-mail: timurvaliev@mail.ru

For citation: Valiev TT, Kovrigina AM, Panferova TR, et al. A Case Report of Myeloid Sarcoma in a Child. Clinical oncohematology. 2017;10(2):218–26 (In Russ).

DOI: 10.21320/2500-2139-2017-10-2-218-226

ABSTRACT

The diagnosis of myeloid tumors is based on a complex approach and causes significant difficulties especially in young children. Morphologic, immunologic, cytogenetic, molecular and biologic data on myeloid sarcoma are presented based on the literature data and own clinical case. Treatment results of myeloid sarcoma (especially in the high risk group) are unsatisfactory and should be improved.

Keywords: myeloid sarcoma, diagnosis, children.

Received: November 14, 2016

Accepted: February 9, 2017

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REFERENCES

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Role of Superficial CD200 Marker in Differential Diagnosis of Malignant B-Cell Lymphoproliferative Diseases

LYMPHOID TUMORS , , , , ,

YuV Mirolyubova, EA Stadnik, TS Nikulina, VV Strugov, TO Andreeva, YuV Virts, RV Grozov, AYu Zaritskey

Federal Almazov North-West Medical Research Centre, 2 Akkuratova str., Saint Petersburg, Russian Federation, 197341

For correspondence: Yuliya Vladimirovna Mirolyubova, 2 Akkuratova str., Saint Petersburg, Russian Federation, 197341; e-mail: juli9702@yandex.ru

For citation: Mirolyubova YuV, Stadnik EA, Nikulina TS, et al. Role of Superficial CD200 Marker in Differential Diagnosis of Malignant B-Cell Lymphoproliferative Diseases. Clinical oncohematology. 2017;10(2):169–75 (In Russ).

DOI: 10.21320/2500-2139-2017-10-2-169-175

ABSTRACT

Background & Aims. Flow cytometry is successfully used for diagnosis of malignant lymphoproliferative disorders. However, there are atypical cases that are difficult to interpret; thus, new markers relevant for the differential diagnosis are to be searched for. The aim is to analyze CD200 expression in patients with B-cell lymphoproliferative disorders.

Materials & Methods. 187 patients with chronic lymphocytic leukemia (CLL), 14 patients with mantle cell lymphoma (MCL), 9 patients with marginal zone lymphoma (MZL), and 5 patients with hairy cell leukemia (HCL) were enrolled in the study. Neoplasm was not confirmed in 12 subjects. The patients underwent the following tests: CBC, immunophenotyping of peripheral blood or bone marrow lymphocytes, and a cytogenetic test. In some cases, an additional immunohistochemical test of bone marrow trepanobiopsy or lymph node biopsy samples was required.

Results. In all cases of CLL and HCL, the CD200 expression was positive; mean fluorescence intensity was higher in these cases as compared to other groups. Negative expression of CD200 prevailed in MCL patients; however, at the same time 2 cases of intermediate and positive expression were reported, both showing moderate fluorescence intensity values. CD200 expression was heterogeneous in MZL patients.

Conclusion. The CD200 negative expression excludes typical HCL and CLL. Additional cytogenetic and immunnohistoсhemical tests should be performed in such cases to verify the diagnosis, first of all, MCL or MZL.

Keywords: CD200, flow cytometry, diagnosis, chronic lymphocytic leukemia, mantle cell lymphoma, marginal zone lymphoma, hairy cell leukemia.

Received: September 7, 2016

Accepted: January 3, 2017

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REFERENCES

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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

COMPLICATIONS OF ANTITUMOR TREATMENT , , , , ,

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

REVIEWS , , , , , ,

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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Modern Aspects of Diagnosis and Treatment of Anaplastic Large Cell Lymphoma in Children (Literature Review)

LYMPHOID TUMORS , , ,

AS Levashov1, TT Valiev1, AM Kovrigina2, AV Popa1, GL Mentkevich1

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

2 Hematology Research Center, 4а Novyi Zykovskii pr-d, Moscow, Russian Federation, 125167

For correspondence: Andrei Sergeevich Levashov, scientific worker, 24 Kashirskoye sh., Moscow, Russian Federation, 115478; Tel.: +7(916)233-05-75; e-mail: andreyslevashov@mail.ru

For citation: Levashov AS, Valiev TT, Kovrigina AM, et al. Modern Aspects of Diagnosis and Treatment of Anaplastic Large Cell Lymphoma in Children (Literature Review). Clinical oncohematology. 2016;9(2):199–207 (In Russ).

DOI: 10.21320/2500-2139-2016-9-2-199-207

ABSTRACT

Anaplastic large cell lymphoma (ALCL) includes different types of the disease that are heterogeneous according to clinical, morphological, immunological, cytogenetic and molecular biological features. The review demonstrates not only main clinical and morphoimmunological characteristics of ALCL, but also presents data about expression and prognostic significance of STAT3, pSTAT3tyr705, and survivin (transcription factor). It demonstrates the value of defining the minimal disseminated disease (the minimal disseminated disease is evaluated using the PCR test before initiation of the treatment, and the minimal residual disease is evaluated during the treatment and after its completion), and clinical and molecular biological prognostic factors are also identified. There is still no a standard therapeutic regimen for pediatric ALCL patients. However, the following therapeutic protocols are considered most effective: NHL-BFM 90/95, CCG5941, SFOP-LM 89/91, UKCCSG, ALCL99-Vinblastine, POG АРО 9315, AIEOP LNH-92/97. Treatment outcomes are presented in this paper. Particular attention is paid to different molecular biological markers that allow further improvement of patients’ stratification in risk groups and possible use of target medications (multikinase inhibitors and monoclonal antibodies) improving the therapy outcomes.

Keywords: anaplastic large cell lymphoma, diagnosis, treatment, children.

Received: February 3, 2016

Accepted: February 10, 2016

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