Browsing by Author "LIMA, M."
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- An unusual acute myeloid leukemia associated with hyper IgE: another case of AML‐M5c?Publication . LIMA, M.; ORFAO, A.; COUTINHO, J.; FERREIRA, G.; FREITAS, I.; SILVESTRE, F; JUSTICA, B.Haematologica. 2001 Feb;86(2):216-7. An unusual acute myeloid leukemia associated with hyper IgE: another case of AML-M5c? Lima M, Orfão A, Coutinho J, Ferreira G, Freitas I, Silvestre F, Justiça B. PMID: 11224498 [PubMed - indexed for MEDLINE]
- Association of CD4+/CD56+/CD57+/CD8+(dim) large granular lymphocytic leukemia, splenic B‐cell lymphoma with circulating villous lymphocytes, and idiopathic erythrocytosisPublication . LIMA, M.; GONCALVES, C.; MARQUES, L.; MARTIN, M.C.; TEIXEIRA, M.A.; QUEIROS, M.L.; SANTOS, A.H.; BALANZATEGUI, A.; GARCIA‐SANZ, R.; PINTO‐RIBEIRO, A.C.; JUSTICA, B.; ORFAO, A.Ann Hematol. 2001 Nov;80(11):685-90. Association of CD4+/CD56+/CD57+/CD8+(dim) large granular lymphocytic leukemia, splenic B-cell lymphoma with circulating villous lymphocytes, and idiopathic erythrocytosis. Lima M, Gonçalves C, Marques L, Martin MC, Teixeira MA, Queirós ML, Santos AH, Balanzategui A, Garcia-Sanz R, Pinto-Ribeiro AC, Justiça B, Orfão A. Service of Clinical Hematology, Hospital Geral Santo António, Porto, Portugal. mmc.lima@clix.pt Abstract In this paper we report a rare association of a splenic marginal zone B-cell lymphoma with villous lymphocytes and a T-cell large granular lymphocytic leukemia coexpressing CD4 and CD8 as well as CD56 and CD57 natural killer-associated markers in an asymptomatic patient investigated because of an occasional finding of erythrocytosis and leukocytosis in routine blood analysis. We also discuss the possible reasons for this particular association. PMID: 11757730 [PubMed - indexed for MEDLINE]
- CD8 (+)/V beta 5.1(+) large granular lymphocyte leukemia associated with autoimmune cytopenias, rheumatoid arthritis and vascular mammary skin lesions: successful response to 2‐deoxycoformycin.Publication . GRANJO, E.; LIMA, M.; CORREIA, T.; LISBOA, C.; MAGALHAES, C; CUNHA, N.; TEIXEIRA, M.A.; QUEIROS, M.L.Hematol Oncol. 2002 Jun;20(2):87-93. Cd8(+)/V beta 5.1(+) large granular lymphocyte leukemia associated with autoimmune cytopenias, rheumatoid arthritis and vascular mammary skin lesions: successful response to 2-deoxycoformycin. Granjo E, Lima M, Correia T, Lisboa C, Magalhães C, Cunha N, Teixeira MA, Queirós ML, Candeias J, Matutes E. Department of Clinical Haematology, Hospital Geral de São João, Porto, Portugal. npp46740@mail.telepac.pt Abstract We report a case of CD8(+)/V beta 5.1(+) T-cell large granular lymphocyte leukemia (T-LGL leukemia) presenting with mild lymphocytosis, severe autoimmune neutropenia, thrombocytopenia, polyarthritis and recurrent infections with a chronic disease course. Immunophenotyping showed an expansion of CD3(+)/TCR alpha beta(+)/CD8(+bright)/CD11c(+)/CD57(-)/CD56(-) large granular lymphocytes with expression of the TCR-V beta 5.1 family. Southern blot analysis revealed a clonal rearrangement of the TCR beta-chain gene. Hematopoietic growth factors, high dose intravenous immunoglobulin and corticosteroids were of limited therapeutic benefit to correct the cytopenias. During the disease course, the patient developed a severe cutaneous leg ulcer and bilateral vascular mammary skin lesions. Treatment with 2-deoxycoformycin resulted in both clinical and hematological complete responses, including the resolution of vascular skin lesions. Combined immuno-staining with relevant T-cell associated and anti-TCR-V beta monoclonal antibodies proved to be a sensitive method to assess the therapeutic effect of 2-deoxycoformicin and to evaluate the residual disease. Copyright 2002 John Wiley & Sons, Ltd. PMID: 12111871 [PubMed - indexed for MEDLINE
- Chronic eosinophilic leukaemia presenting with erythroderma, mild eosinophilia and hyper‐IgE: clinical, immunological and cytogenetic features and therapeutic approach. A case report.Publication . GRANJO, E.; LIMA, M.; LOPES, J.M.; DORIA, S.; ORFAO, A.; YING, S.; BARATA, L.T.; MIRANDA, M.; CROSS, N.C.; BAIN, B.J.Acta Haematol. 2002;107(2):108-12. Chronic eosinophilic leukaemia presenting with erythroderma, mild eosinophilia and hyper-IgE: clinical, immunological and cytogenetic features and therapeutic approach. A case report. Granjo E, Lima M, Lopes JM, Dória S, Orfão A, Ying S, Barata LT, Miranda M, Cross NC, Bain BJ. Department of Clinical Haematology, Hospital Geral de São João, Porto, Portugal. elisagranjo@netc.pt Abstract A 23-year-old, white male metallurgist presented with pruritic erythematous maculo-papules over the trunk and upper limbs and 6 months later developed erythroderma, eosinophilia and multi-organ dysfunction. A diagnosis of chronic eosinophilic leukaemia was made on the basis of myeloproliferative involvement of both peripheral blood and bone marrow, associated with eosinophilic differentiation and a t(5;12)(q33;p13) translocation. The initial therapeutic approach was interferon alfa-2b plus cytosine arabinoside, for 13 months, followed by hydroxyurea plus vincristine. There was improvement of skin lesions, disappearance of eosinophilia and decrease of serum immunoglobulin E, towards normal values. Copyright 2002 S. Karger AG, Basel PMID: 11919392 [PubMed - indexed for MEDLINE]
- Clinicobiological, immunophenotypic, and molecular characteristics of monoclonal CD56‐/+dim chronic natural killer cell large granular lymphocytosis.Publication . LIMA, M.; ALMEIDA, J.; MONTERO, A.G.; TEIXEIRA M DOS, A.; QUEIROS, M.L.; SANTOS, A.H.; BALANZATEGUI, A.; ESTEVINHO, A.; ALGUERO MDEL, C.; BARCENA, P.; FONSECA, S.; AMORIM, M.L.; CABEDA, J.M.; PINHO, L.; GONZALEZ, M.; SAN MIGUEL, J.; JUSTICA, B.; ORFAO, A.Am J Pathol. 2004 Oct;165(4):1117-27. Clinicobiological, immunophenotypic, and molecular characteristics of monoclonal CD56-/+dim chronic natural killer cell large granular lymphocytosis. Lima M, Almeida J, Montero AG, Teixeira Mdos A, Queirós ML, Santos AH, Balanzategui A, Estevinho A, Algueró Mdel C, Barcena P, Fonseca S, Amorim ML, Cabeda JM, Pinho L, Gonzalez M, San Miguel J, Justiça B, Orfão A. Serviço de Hematologia, Unidade de Citometria, Hospital Geral de Santo António, Rua D Manuel II, s/n, 4099-001 Porto, Portugal. mmc.lima@clix.pt. Abstract Indolent natural killer (NK) cell lymphoproliferative disorders include a heterogeneous group of patients in whom persistent expansions of mature, typically CD56(+), NK cells in the absence of any clonal marker are present in the peripheral blood. In the present study we report on the clinical, hematological, immunophenotypic, serological, and molecular features of a series of 26 patients with chronic large granular NK cell lymphocytosis, whose NK cells were either CD56(-) or expressed very low levels of CD56 (CD56(-/+dim) NK cells), in the context of an aberrant activation-related mature phenotype and proved to be monoclonal using the human androgen receptor gene polymerase chain reaction-based assay. As normal CD56(+) NK cells, CD56(-/+dim) NK cells were granzyme B(+), CD3(-), TCRalphabeta/gammadelta(-), CD5(-), CD28(-), CD11a(+bright), CD45RA(+bright), CD122(+), and CD25(-) and they showed variable and heterogeneous expression of both CD8 and CD57. Nevertheless, they displayed several unusual immunophenotypic features. Accordingly, besides being CD56(-/+dim), they were CD11b(-/+dim) (heterogeneous), CD7(-/+dim) (heterogeneous), CD2(+) (homogeneous), CD11c(+bright) (homogeneous), and CD38(-/+dim) (heterogeneous). Moreover, CD56(-/+dim) NK cells heterogeneously expressed HLA-DR. In that concerning the expression of killer receptors, CD56(-/+dim) NK cells showed bright and homogeneous CD94 expression, and dim and heterogeneous reactivity for CD161, whereas CD158a and NKB1 expression was variable. From the functional point of view, CD56(-/+dim) showed a typical Th1 pattern of cytokine production (interferon-gamma(+), tumor necrosis factor-alpha(+)). From the clinical point of view, these patients usually had an indolent clinical course, progression into a massive lymphocytosis with lung infiltration leading to death being observed in only one case. Despite this, they frequently had associated cytopenias as well as neoplastic diseases and/or viral infections. In summary, we describe a unique and homogeneous group of monoclonal chronic large granular NK cell lymphocytosis with an aberrant activation-related CD56(-/+dim)/CD11b(-/+dim) phenotype and an indolent clinical course, whose main clinical features are related to concomitant diseases. PMID: 15466379 [PubMed - indexed for MEDLINE]PMCID: PMC1618630
- Galactosemia: the genotype and phenotype of seven patientsPublication . MARTINS, E.; TEIXEIRA, J.; CARDOSO, M.; LIMA, M.; BRIONES‐GODINO, P.; BARBOT, C.Rev Neurol. 2004 Jun 16-30;38(12):1132-5. [Galactosemia: the genotype and phenotype of seven patients]. [Article in Spanish] Martins E, Teixeira J, Cardoso ML, Lima MR, Briones-Godino P, Barbot C. SourceUnidad de Metabolismo, Hospital de Niños Maria Pia, Oporto, Portugal. npo38871@mail.telepac.pt Abstract INTRODUCTION: Despite early dietary therapy, many patients with galactosemia show a neurodegenerative disease specially evident in speech impairment and movement disorders. Magnetic resonance imaging of the brain, show cerebral white matter changes with hypomielinization bilateral and symetrical periventricular hypersignal in T2. PATIENTS AND METHODS: We presented clinical and neuroradiological data of seven children (3 to 12 years of age) with classical galactosemia. All had a typical presentation in neonatal period. Two children had normal development (10 and 12 years-old), four presented developmental delay (10, 7, 4 and 3 years-old), and one showed a dystonic cerebral palsy (kernicterus). RESULTS: The brain MRI showed the typical involvement of white matter, in five children, and basal ganglia abnormalities in the kernicterus patient. Three patients are homozygous for Q188R mutation and two are compound heterozygous. CONCLUSION: We found a positive correlation among developmental delay, white matter involvement and Q188R mutation.
- Guess what: Chronic 13q14.3+/CD5‐ /CD23+ lymphocytic leukemia in blood and t(11;14)(q13;q32)+/CD5+/CD23‐ mantle cell lymphoma in lymph nodes!Publication . LIMA, M.; PINTO, L.; DOS ANJOS TEIXEIRA, M.; CANELHAS, A.; MOTA, A.; CABEDA, J.M.; SILVA, C.; QUEIROS, M.L.; FONSECA, S.; SANTOS, A.H.; BROCHADO, P.; JUSTICA, B.Cytometry B Clin Cytom. 2003 Jan;51(1):41-4. Guess what: Chronic 13q14.3+/CD5-/CD23+ lymphocytic leukemia in blood and t(11;14)(q13;q32)+/CD5+/CD23- mantle cell lymphoma in lymph nodes! Lima M, Pinto L, Dos Anjos Teixeira M, Canelhas A, Mota A, Cabeda JM, Silva C, Queirós ML, Fonseca S, Santos AH, Brochado P, Justiça B. Service of Clinical Hematology, Hospital Geral de Santo António, Porto, Portugal. mmc.lima@clix.pt Abstract We report a case of a patient with two B-cell lymphoproliferative disorders: CD5(-)/CD23(+) B-cell chronic lymphocytic leukemia and CD5(+)/CD23(-) mantle cell lymphoma. These disorders were diagnosed simultaneously based on flow cytometry, immunohistochemistry, fluorescence in situ hybridization, and polymerase chain reaction-based molecular studies. The B-cell lymphocytic leukemia clone predominated in the blood and bone marrow, whereas the mantle cell clone predominated in lymph nodes. Copyright 2002 Wiley-Liss, Inc. PMID: 12500296 [PubMed - indexed for MEDLINE]
- Immunophenotype and TCR‐Vbeta repertoire of peripheral blood T‐cells in acute infectious mononucleosis.Publication . LIMA, M.; TEIXEIRA, M.; QUEIROS, M.L.; SANTOS, A.H.; GONCALVES, C.; CORREIA, J.; FARINHA, F.; MENDONCA, F.; SOARES, J.M.; ALMEIDA, J.; ORFAO, A.; JUSTICA, B.Blood Cells Mol Dis. 2003 Jan-Feb;30(1):1-12. Immunophenotype and TCR-Vbeta repertoire of peripheral blood T-cells in acute infectious mononucleosis. Lima M, Teixeira Mdos A, Queirós ML, Santos AH, Gonçalves C, Correia J, Farinha F, Mendonça F, Soares JM, Almeida J, Orfão A, Justiça B. Service of Clinical Haematology, Hospital Santo António, Porto, Portugal. mmc.lima@clix.pt Abstract Although a number of studies on the phenotypic changes that occur after T-cell activation have already been published, the specific immunophenotypic features of T-lymphocytes and the frequency at which TCR-variable region (TCR-V) restricted T-cell expansions occur "in vivo" during acute viral infection still remains to be established. We report on the immunophenotype and TCR-V repertoire of peripheral blood T-cells from 28 patients with acute infectious mononucleosis. Immunophenotypic studies were performed by flow cytometry using direct immunofluorescence techniques and stain-and-then-lyse sample preparation protocols with three- and four-colour combinations of monoclonal antibodies directed against a large panel of T- and NK-cell associated markers, activation- and adhesion-related molecules and TCR-Vbeta, -Vgamma and -Vdelta families. Nearly all patients (27/28) showed a massive expansion of CD8(+)/TCRalphabeta(+) T cells, the majority (>90%) of which displayed an immunophenotype compatible with T-cell activation: CD2(+high), CD7(+low), CD11a(+high), CD38(+high), HLA-DR(+high), CD28(+/-low), CD45RO(+high), CD45RA(-/+low), CD11b(-/+low), CD11c(+/-low), CD16(-), CD56(-), CD57(-), CD62L(-), CD94(-), CD158a(-), CD161(-), NKB1(-). Additionally, the levels of both CD3 and CD5 were slightly decreased compared to those found in normal individuals. Late-activation antigens, such as CD57, were found in small proportions of CD8(+)/TCRalphabeta(+) T-cells. Increased numbers of CD4(+)/TCRalphabeta(+) T-cells, TCRgammadelta(+) T-cells and NK-cells were also noticed in 17, 16 and 13 of the 28 cases studied, respectively. Evidence for activation of CD4(+)/TCRalphabeta(+) and TCRgammadelta(+) T-cells relied on changes similar to those described for CD8(+)/TCRalphabeta(+) although less pronounced, except for higher levels of both CD5 and CD28 in the absence of reactivity for CD11c on CD4(+)/TCRalphabeta(+) T-cells and higher levels of CD161 and CD94 on TCRgammadelta(+) T-cells. Small expansions of one or more TCR-Vbeta families accounting for 12 +/- 7% of either the CD8(+)/TCRalphabeta(+) or the CD4(+)/TCRalphabeta(+) T-cell compartment were found in 12 of 14 patients studied, whereas the distribution of the TCR-Vgamma and -Vdelta repertoires tested in 2 of the individuals with expanded TCRgammadelta(+) T-cells was similar to that observed in control individuals. The results presented here provide evidence for an extensive T-cell activation during acute viral infection and establish the immunophenotype patterns associated with this condition. PMID: 12667982 [PubMed - indexed for MEDLINE]
- Immunophenotypic aberrations, DNA content, and cell cycle analysis of plasma cells in patients with myeloma and monoclonal gammopathiesPublication . LIMA, M.; TEIXEIRA MDOS, A.; FONSECA, S.; GONCALVES, C.; GUERRA, M.; QUEIROS, M.L.; SANTOS, A.H.; COUTINHO, A.; PINHO, L.; MARQUES, L.; CUNHA, M.; RIBEIRO, P.; XAVIER, L.; VIEIRA, H.; PINTO, P.; JUSTICA, B.Blood Cells Mol Dis. 2000 Dec;26(6):634-45. Immunophenotypic aberrations, DNA content, and cell cycle analysis of plasma cells in patients with myeloma and monoclonal gammopathies. Lima M, Teixeira Mdos A, Fonseca S, Gonçalves C, Guerra M, Queirós ML, Santos AH, Coutinho A, Pinho L, Marques L, Cunha M, Ribeiro P, Xavier L, Vieira H, Pinto P, Justiça B. Service of Clinical Hematology, Hospital Geral de Santo António, Rua D Manual II, s/n, 4050 Porto, Portugal. m.lima@ip.pt Abstract We describe the immunophenotypic and gross DNA defects in 55 patients with myeloma and 50 patients with monoclonal gammopathy and review the literature on this subject (MedLine, 1994-2000). Our data confirmed previous reports indicating that in myeloma nearly all marrow plasma cells are abnormal (98.7 +/- 8.1%). In monoclonal gammopathy the fraction of abnormal plasma cells was 35.0 +/- 32.8%. In both myeloma and monoclonal gammopathy, the most frequent aberrant phenotypic features consisted of absence of expression of CD19, strong expression of CD56, and decreased intensity of expression of CD38; aberrant expression of CD10, CD20, CD22, or CD28 was observed in less than one-third of myeloma cases. The vast majority of cases had two or more phenotypic aberrations. In the DNA studies, 7% of myeloma cases were biclonal and 93% of cases were monoclonal. In those studies with only one plasma cell mitotic cycle, 37% had normal DNA content and 63% were aneuploid (hyperploid, 61%; hypoploid, 2%). The mean percentages of plasma cells in S- and G2M phases were 4.9 +/- 8.5 and 4.4 +/- 6.9%, respectively. Thirty-eight percent of cases had more than 3% of plasma cells in S phase. In monoclonal gammopathy, the DNA index of abnormal plasma cells ranged from 0.89 to 1.30 and the percentage of diploid (31%) and aneuploid (69%) cases was not different from the results found in myeloma. The differences in percentage of abnormal plasma cells in S- (7.4 +/- 8.6%) and G2M-phases (2.4 +/- 1.7%) in patients with monoclonal gammopathy were not statistically significant. Copyright 2000 Academic Press. PMID: 11358356 [PubMed - indexed for MEDLINE]
- Immunophenotypic analysis of the TCR‐Vbeta repertoire in 98 persistent expansions of CD3(+)/TCR‐alphabeta(+) large granular lymphocytes: utility in assessing clonality and insights into the pathogenesis of the diseasePublication . LIMA, M.; ALMEIDA, J.; SANTOS, A.H.; DOS ANJOS TEIXEIRA, M.; ALGUERO, M.C.; QUEIROS, M.L.; BALANZATEGUI, A.; JUSTICA, B.; GONZALEZ, M.; SAN MIGUEL, J.F.; ORFAO, A.Am J Pathol. 2001 Nov;159(5):1861-8. Immunophenotypic analysis of the TCR-Vbeta repertoire in 98 persistent expansions of CD3(+)/TCR-alphabeta(+) large granular lymphocytes: utility in assessing clonality and insights into the pathogenesis of the disease. Lima M, Almeida J, Santos AH, dos Anjos Teixeira M, Alguero MC, Queirós ML, Balanzategui A, Justiça B, Gonzalez M, San Miguel JF, Orfão A. Serviço de Hematologia Clínica, Unidade de Citometria, Hospital Geral de Santo António, Porto, Portugal. mmc.lima@clix.pt Abstract At present, a major challenge in the initial diagnosis of leukemia of large granular lymphocytes (LGLs) is to establish the clonal nature of the expanded population. In the present study we have analyzed by flow cytometry immunophenotyping the TCR-Vbeta repertoire of 98 consecutive cases of persistent expansions of CD4(+) or CD8(+bright) CD3(+)/TCR-alphabeta(+) LGLs and compared the results with those obtained in molecular studies of TCR-beta gene rearrangements. Fifty-eight cases were considered to be monoclonal in molecular studies whereas in the remaining 40 cases there was no evidence for monoclonality (11 cases were considered oligoclonal and 29 polyclonal). The TCR-Vbeta repertoire was biased to the preferential use of one or more TCR-Vbeta families in 96% of cases, a total of 124 TCR-Vbeta expansions being diagnosed: one TCR-Vbeta expansion in 71 cases and two or more TCR-Vbeta expansions in 23 cases. The highest TCR-Vbeta expansion observed in each case was higher among monoclonal (74 +/- 19%) as compared to nonmonoclonal cases (24 +/- 14%) (P = 0.001), as did the fraction of LGLs that exhibited a TCR-Vbeta-restricted pattern (86 +/- 16% and 42 +/- 23%, respectively; P = 0.0001); by contrast, the proportion of cases displaying more than one TCR-Vbeta expansion was higher in the latter group: 7% versus 48%, respectively (P = 0.001). Results obtained in oligoclonal cases were intermediate between those obtained in polyclonal and monoclonal cases and similar results were observed for CD4(+) as for CD8(+bright) T-cell expansions. TCR-Vbeta families expressed in CD8(+bright) T-cell-LGL proliferations showed a pattern of distribution that mimics the frequency at which the individual TCR-Vbeta families are represented in normal peripheral blood T cells. Assuming that a given proliferation of LGLs is monoclonal whenever there is an expansion of a given TCR-Vbeta family of at least 40% of the total CD4(+) or CD8(+bright) T-cell compartment, we were able to predict clonality with a sensitivity of 93% and a specificity of 80%. By increasing the cut-off value to 60%, sensitivity and specificity were of 81% and 100%. In summary, our results suggest that flow cytometry immunophenotypic analysis of the TCR-Vbeta repertoire is a powerful screening tool for the assessment of T-cell clonality in persistent expansions of TCR-alphabeta(+) LGLs. PMID: 11696446 [PubMed - indexed for MEDLINE]PMCID: PMC1867049