Publications (10)19.18 Total impact
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Article: Potential link between MHC-self-peptide presentation and hematopoiesis; the analysis of HLA-DR expression in CD34-positive cells and self-peptide presentation repertoires of MHC molecules associated with paroxysmal nocturnal hemoglobinuria.
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ABSTRACT: The mechanisms of MHC allele associations with paroxysmal nocturnal hemoglobinuria (PNH) and its aplastic anemia subtype (AA/PNH) remain unclear. It might be dependent on MHC molecule functional properties, such as a scope and frequency of antigen sampling and presentation. For documented PNH-associated MHC alleles we analyzed current reference databases on MHC molecule-eluted peptide presentation repertoires and searched for a range of presented peptides. MHC class II expression was measured on CD34+ cells and appeared to be increased in PNH patients. Two class I alleles (HLA-A*24:02 and B*18:01) have been previously confirmed to associate with protection and increased risk of AA/PNH, respectively. Their product molecules presented immunodominant epitopes derived from proapoptotic (serine/threonine-protein phosphatase) and antiapoptotic (phospholipase D), respectively, intracellular enzymes dependent on phosphoinositide (PI) content. For total PNH and non-aplastic PNH (n/PNH) subtype-associated DRB1*15:01 and DRB1*04:01 class II molecules presentation of exceptionally broad arrays of their own peptide fragments has been found. We conclude that self antigen peptides presented with high frequency in the context of MHC molecules of increased expression may be involved in the immune recognition and the regulation of HSC in the periphery. The block in the normal plasma membrane PI production due to the PIG-A mutation can help explain the differences in the activation of intracellular regulatory pathways observed between PNH and normal HSC. This is evident in the variation in MHC association patterns and peptide presentation repertoires between these two groups of patients.Cell biochemistry and biophysics 10/2012; · 3.34 Impact Factor -
Article: The patterns of MHC association in aplastic and non-aplastic paroxysmal nocturnal hemoglobinuria.
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ABSTRACT: The deficiency of glycosyl-phosphatidylinositol (GPI)-anchored proteins in plasma membranes of PIG-A gene mutated hematopoietic stem cells (HSCs) is so far insufficient to explain the domination of paroxysmal nocturnal hemoglobinuria (PNH) clone over the normal HSC. We attempted to elucidate possible link between MHC and initial severe aplastic anemia (ISAA/PNH) type and non-aplastic (n/PNH) outcome of PNH. In 50 PNH patients assigned as ISAA/PNH (n = 13), n/PNH (n = 33) or nonassigned (n = 4) and 200 ethnically matched controls we analyzed MHC associations. Our data confirmed strong associations of DRB1*15:01 (RR = 3.51, p = 0.0011) and DQB1*06:02 (RR = 7.09, p = 0.000026) alleles, especially with n/PNH subtype. B*18:01 allele was associated with increased risk of ISAA/PNH subtype (RR = 5.25, p = 0.0028). We conclude that both class II and class I MHC alleles are associated with different subsets of PNH. Clonal selection of PIG-A mutated cells with cognate metabolic block is associated with MHC class II alleles DRB1*15:01 and DQB1*06:02 independent from initial severe AA clone selection. MHC class I molecule B*18:01 can additionally influence the domination of PNH clone in PNH subjects with initial severe aplastic anemia.Archivum Immunologiae et Therapiae Experimentalis 03/2011; 59(3):231-8. · 2.54 Impact Factor -
Article: Diversity of thalassemia variants in Poland - screening by real-time PCR.
Acta Haematologica 12/2008; 120(3):153-7. · 1.35 Impact Factor -
Article: Diagnostic and therapeutic quandaries in NK-cell lymphomas — a report of two cases
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ABSTRACT: In the present paper we describe clinical course of natural killer (NK) cell lymphomas. In the first patient, diagnosis of nasal NK/T cell lymphoma was based on histopathologic examination of tumor lesion within the base of nose (Ann Arbor IIA). The patient received chemotherapy according to CN3OP (cyclophos- phamide, mitoxantrone, vincristine, prednisone) and IVAC (etoposide, ifosfamide, cytarabine) protocols. His initial response to chemotherapy was good, but rapid disease progression within skin and lungs subsequently occured. The patient died due to cardio-pulmonary insufficiency 7 months after lymphoma diagnosis. In the second patient, diagnosis of blastic NK cell lymphoma was based on histopathologic findings in skin and trephine biopses. The patient was treated according to COP (cyclophosphamide, vincristine, prednisone) and CC (cyclophosphamide, cladribine) protocols and after disease progression he received 2 additional courses of mitoxantrone, etoposide and cytarabine. His initial response to che- motherapy was good, but rapid disease progression within bone marrow and skin occured. The patient died due to central nervous system hemorrhage in the course of disseminated intravascular coagulopa- thy 9 months after lymphoma diagnosis. These two case reports reveal diagnostic and therapeutic diffi- culties of the very rare NK cell lymphomas.Onkologia w Praktyce Klinicznej. 01/2007; 3(4):217-222. -
Article: Molecular analysis of three novel G6PD variants: G6PD Pedoplis-Ckaro, G6PD Piotrkow and G6PD Krakow.
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ABSTRACT: We present three novel mutations in the G6PD gene and discuss the changes they cause in the 3-dimensional structure of the enzyme: 573C-->G substitution that predicts Phe to Leu at position 191 in the C-terminus of helix alphae, 851T-->C mutation which results in the substitution 284Val--> -->Ala in the beta+alpha domain close to the C-terminal part of helix alphaj, and 1175T-->C substitution that predicts Ile to Thr change at position 392.Acta biochimica Polonica 01/2007; 54(4):877-81. · 1.49 Impact Factor -
Article: Molecular and haematological studies of four families with hereditary spherocytosis resulting from band 3 deficiency.
Acta Haematologica 02/2006; 116(2):143-5. · 1.35 Impact Factor -
Article: First two successful unrelated donor bone marrow transplantations for paroxysmal nocturnal hemoglobinuria in Poland.
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ABSTRACT: The experience with bone marrow transplantation (BMT) from matched unrelated donors (MUD) for paroxysmal nocturnal hemoglobinuria (PNH) is limited and optimal preparative regimen has been not established. We report first two MUD BMTs for patients with PNH in Poland. Preparative regimen consisted of Treosulfan, Fludarabine and Thymoglobulin. We also present the review of published reports on allogeneic transplantations for PNH and discuss important transplant-related issues. Both patients are alive and are doing well over 12 and over 4 months following BMT. Regeneration is complete with full 100% donor chimerism and the eradication of PNH clone. MUD BMT is an effective treatment for PNH. Treosulfan, Fludarabine and Thymoglobulin treatment can be safely and effectively used for conditioning in PNH.Annals of transplantation: quarterly of the Polish Transplantation Society 02/2005; 10(3):26-30. · 2.02 Impact Factor -
Article: Heterozygosity of CDAN II (HEMPAS) gene may be detected by the analysis of erythrocyte membrane glycoconjugates from healthy carriers.
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ABSTRACT: Congenital dyserythropoietic anemia (CDA) type I, II, and III, is associated with abnormalities of erythrocyte membrane glycoconjugates that are most pronounced in type II CDA or hereditary erythroblastic multinuclearity with a positive acidified-serum test (HEMPAS). The abnormalities consist in hypoglycosylation of polylactoaminoglycans linked to proteins (as in band 3 glycoprotein) and ceramides (known under the name of polyglycosylceramides) as well as in accumulation of some oligoglycosylceramides: lactotriaosylceramide, neolactotetraosylceramide, and sometimes globotetraosylceramide. Glycophorin A is partially unglycosylated with respect to O-linked glycans. Types I and II of the disease are inherited in an autosomal recessive fashion. The aim of the present study was to investigate a possibility that heterozygosity with respect to CDAN2 gene in healthy carriers could be detected by analysis of erythrocyte membrane glycoconjugates. We examined a family which consisted of heterozygous parents and their two sons, one of whom was afflicted with CDA II (proband) while the other was healthy. In all family members the glycosylation status of band 3 glycoprotein, polyglycosylceramides and glycophorin A was evaluated from their carbohydrate molar composition. In addition we determined erythrocyte membrane contents of oligo- and polyglycosylceramides, and agglutinability of erythrocytes by anti-i antibody. We found that the heterozygous parents showed, but about 50% less pronounced, most of the typical abnormalities of erythrocyte membrane glycoconjugates that were present in the proband. These abnormalities included: hypoglycosylation of band 3, accumulation and hypoglycosylation of polyglycosylceramides, and accumulation of lactotriaosylceramide. The level of neolactotetraosylceramide in the erythrocyte membranes of the parents was, however, normal. Globotetraosylceramide content was elevated in erythrocytes from the proband and, surprisingly, even more so in the parents. Glycophorin A in the proband was only slightly abnormal. Erythrocytes from both the parents and the proband expressed increased agglutinability with anti-i antibody. All glycoconjugates examined were normal in erythrocytes from the healthy son. Individuals heterozygous with respect to CDAN2 gene can be identified through determination of the carbohydrate molar composition of band 3 and polyglycosylceramides as well as by an elevated erythrocyte content of polyglycosylceramides. In the parents these abnormalities show dosage effects. Determination of the carbohydrate molar composition of glycophorin A and of oligoglycosylceramides seems to be less promising. These findings indicate that the analysis of erythrocyte membrane glycoconjugates may be a valuable addition to the repertoire of methods used in studies on the genetics of CDA.Haematologica 03/2002; 87(2):126-30. · 6.42 Impact Factor -
Article: [A case of hereditary over-hydrated stomatocytosis with stomatospherocytes and spherocytes in the blood].
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ABSTRACT: A patient of 31 years of age with an atypical overhydrated hereditary stomatocytosis is described. The diagnosis was established on the basis of a markedly increased red cell volume with low MCHC, high osmotic fragility of red cells, but increased binding of eosin-5-maleimide (EMA) to red cells, presence of stomatospherocytes and large spherocytes in blood and a high sodium and low potassium concentration in erythrocytes. A double band 7 was found by SDS-PAGE of the erythrocyte membrane, but even when only one them was taken into account, the level of stomatin was normal. Expression of stomatospherocytes in patient's blood was erratic: in blood films prepared in 2005, both stomatospherocytes and large spherocytes were present but in those from 2008 large erythrocytes of spherocyte morphology predominated. Clinically, the disease symptoms were typical for haemolytic anemia. When heparinized blood of the patient was kept at 0 degrees Celsius for 24 h, the haemolysis of red cells amounted only to 2%. The patient's son, 5 years old, suffers from the same disease. CONCLUSION: In spite of its rarity, hereditary stomatocytosis and allied disorders should be taken into consideration in differential diagnosis of haemolytic anemia including newborns. The diagnosis is supported by finding increased binding of eosin-5-maleimide (EMA) dye to patients' erythrocytes associated with their elevated osmotic fragility. Absence of a significant count of stomatocytes in the blood does not exclude the diagnosis of overyhydrated hereditary stomatocytosis.Medycyna wieku rozwojowego 13(2):131-5. -
Article: Efficacy and safety of thalidomide in the treatment of multiple myeloma.
Acta poloniae pharmaceutica 65(6):771-4. · 0.66 Impact Factor
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Institutions
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2008
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Polish Academy of Sciences
- Instytut Chemii Fizycznej
Warsaw, Masovian Voivodeship, Poland
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2006–2007
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Institute of Biochemistry and Biophysics Polish Academy of Sciences
Warsaw, Masovian Voivodeship, Poland
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