Paroxysmal Nocturnal Hemoglobinuria in Patients with Aplastic Anemia: Challenges, Characteristics, and Analysis of Clinical Experience

Shilova, Elena; Глазанова, Т В; Чубукина, Ж В; Розанова, О Е; Zenina, Marina; Сельцер, А В; Ругаль, В И; Balashova, V. A.; Kobilyanskaya, V.; Кробинец, И. И.; Udaleva, Vera; Zotova, Irina; Стельмашенко, Л. В.; Романенко, Н. А.; Zamotina, Tatyana; Хоршева, И В; Voloshin, S.

doi:10.21320/2500-2139-2019-12-3-319-328

Клиническая онкогематология

2019

DOI: 10.21320/2500-2139-2019-12-3-319-328

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Paroxysmal Nocturnal Hemoglobinuria in Patients with Aplastic Anemia: Challenges, Characteristics, and Analysis of Clinical Experience

Elena Shilova

Т В Глазанова

Ж В Чубукина

et al.

Abstract: Актуальность и цели. Пароксизмальная ночная гемоглобинурия (ПНГ)-заболевание, обусловленное приобретенным клональным нарушением гемопоэтических стволовых клеток с повышенной чувствительностью мембраны клеток клона к комплементу. ПНГ может быть как самостоятельным заболеванием, так и связанным с другими патологическими состояниями, характеризующимися костномозговой недостаточностью, в первую очередь с апластической анемией (АА). При АА, ассоциированной с ПНГ (АА/ПНГ), величина патологического клона исходно може… Show more

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Cited by 3 publications

References 26 publications

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Case of Paroxysmal Nocturnal Hemoglobinuria. Problems of Laboratory Diagnostics

Sosnin¹,

Щекотова²,

Pavlenina³

et al. 2022

Laboratornaya I Klinicheskaya Meditsina. Farmatsiya

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The presented case demonstrates the importance of a comprehensive analysis of all the examination data in a patient with paroxysmal nocturnal hemoglobinuria (PNH). A comparison was made of anamnesis data, the clinical picture of the disease, the results of biochemical blood analysis and hematological analyzer data with morphological assessment of cells in a stained blood smear. The interest of this case for hematologists and laboratory specialists is due to the rarity of this disease, difficulties in diagnostics, the presence of 2 types of hemolysis, features of morphological changes in peripheral blood erythrocytes, as well as analysis of the dynamics of manifestations, including against the background of targeted therapy of PNH.

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Case of Paroxysmal Nocturnal Hemoglobinuria. Problems of Laboratory Diagnostics

Sosnin¹,

Щекотова²,

Pavlenina³

et al. 2022

Laboratornaya I Klinicheskaya Meditsina. Farmatsiya

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Clonal Evolution of Aplastic Anemia: A Brief Literature Review and a Case Report

Shilova

Глазанова

Kostroma

et al. 2022

Клиническая онкогематология

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Aplastic anemia (AA) is a non-neoplastic hematological disease closely associated with bone marrow failure which is typical of paroxysmal nocturnal hemoglobinuria (PNH) and myelodysplastic syndrome (MDS). The PNH clones can be detected in more than a half of AA patients at onset of the disease, and there is a probability for AA/PNH co-variants to progress to classic hemolytic PNH. At the same time, the AA patients treated by immunosuppressive therapy undergo the risk of disease transformation to MDS and acute myeloid leukemia. Currently known risk factors and possible precursors of such transformation are considered in the brief literature review. In addition to that, the paper provides a case report of AA/PNH transformation to MDS during complete AA remission after immunosuppressive therapy combined with a successful haploidentical transplantation of hematopoietic stem cells.

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Long-Term Outcomes of Immunosuppressive Therapy for Aplastic Anemia: A Single-Center Experience

Шилова,

Романенко,

Чебыкина

et al. 2024

Clinical Oncohematology

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Background. Bone marrow transplantation-ineligible aplastic anemia (AA) is most effectively treated with combined immunosuppressive therapy (IST). It yields remissions in most patients. However, it has such disadvantages as frequent relapses, incomplete hematologic recovery, and clonal evolution risk. Besides, АА is not always treated according to standard regimens. For different reasons, some AA patients receive delayed therapy or IST mono-treatment predominantly with cyclosporine A (CsA). Aim. To assess long-term IST outcomes in AA patients followed-up at the Russian Research Institute of Hematology and Transfusiology for 5 years after therapy onset. Materials & Methods. The study enrolled 30 AA patients who received IST for more than 5 years (continuous follow-up of 5.5–33 years) with monitoring of the main hemogram parameters and PNH clone size. Patients were aged 19–73 years (median 29 years). There were 8 women and 12 men. Based on international criteria, severe AA (SAA) was initially diagnosed in 18 patients, and non-severe АА (NAA) was diagnosed in 12 patients. Combined IST was administered to 22 patients (18 SAA patients and 4 NAA patients), the remaining 8 patients received ATG (n = 1) and CsA (n = 7). Results. A response to IST was achieved in 28 (93.3 %) out of 30 patients, 16 (53.3 %) of them showed complete remission. This paper documents the characteristics of hematologic recovery depending on the compliance with standard therapy regimens, as well as on the disease variant, development of late complications and clonal evolution, characteristics of pregnancy and childbirth in 4 female patients in remission. PNH clone increased in more than a half (10 out of 16) patients whose clone was initially > 2.6 %. Long-term clonal evolution to myeloid neoplasia (13 years after IST onset) was registered in 2 (6.7 %) patients with complete AA remission. Aseptic (avascular) osteonecrosis as complication was reported in 6 (20 %) followed-up patients. Conclusion. The results of the study highlight the importance of and the need for early start and adherence to standard combined IST regimens aimed at optimum therapeutic effect in both SAA and NAA patients, as well as for long-term follow-up of patients after completing IST.

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Paroxysmal Nocturnal Hemoglobinuria in Patients with Aplastic Anemia: Challenges, Characteristics, and Analysis of Clinical Experience

Cited by 3 publications

References 26 publications

Case of Paroxysmal Nocturnal Hemoglobinuria. Problems of Laboratory Diagnostics

Case of Paroxysmal Nocturnal Hemoglobinuria. Problems of Laboratory Diagnostics

Clonal Evolution of Aplastic Anemia: A Brief Literature Review and a Case Report

Long-Term Outcomes of Immunosuppressive Therapy for Aplastic Anemia: A Single-Center Experience

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