المؤلفون: Cárdenas, María C., García-Sanz, Ramón, Puig, Noemí, Pérez-Surribas, David, Flores-Montero, Juan, Ortiz-Espejo, María, de la Rubia, Javier, Cruz-Iglesias, Elena

المصدر: Clinical Chemistry & Laboratory Medicine; Nov2023, Vol. 61 Issue 12, p2115-2130, 16p

مصطلحات موضوعية: MONOCLONAL gammopathies, MONOCLONAL antibodies, IMMUNOGLOBULIN light chains, BONE marrow examination, BLOOD transfusion, HEMATOLOGY

مستخلص: Monoclonal gammopathies (MG) are characterized by the proliferation of plasma cells that produce identical abnormal immunoglobulins (intact or some of their subunits). This abnormal immunoglobulin component is called monoclonal protein (M-protein), and is considered a biomarker of proliferative activity. The identification, characterization and measurement of M-protein is essential for the management of MG. We conducted a systematic review of the different tests and measurement methods used in the clinical laboratory for the study of M-protein in serum and urine, the biochemistry and hematology tests necessary for clinical evaluation, and studies in bone marrow, peripheral blood and other tissues. This review included literature published between 2009 and 2022. The paper discusses the main methodological characteristics and limitations, as well as the purpose and clinical value of the different tests used in the diagnosis, prognosis, monitoring and assessment of treatment response in MG. Included are methods for the study of M-protein, namely electrophoresis, measurement of immunoglobulin levels, serum free light chains, immunoglobulin heavy chain/light chain pairs, and mass spectrometry, and for the bone marrow examination, morphological analysis, cytogenetics, molecular techniques, and multiparameter flow cytometry. [ABSTRACT FROM AUTHOR]

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المؤلفون: Cárdenas, María C., García-Sanz, Ramón, Puig, Noemí, Pérez-Surribas, David, Flores-Montero, Juan, Ortiz-Espejo, María, De la Rubia, Javier, Cruz-Iglesias, Elena

المصدر: Clinical Chemistry & Laboratory Medicine; Nov2023, Vol. 61 Issue 12, p2131-2142, 12p

مصطلحات موضوعية: MONOCLONAL gammopathies, BLOOD transfusion, PATHOLOGICAL laboratories, CLINICAL pathology, HEMATOLOGY, MONOCLONAL antibodies

مستخلص: Monoclonal gammopathies (MG) are a group of clinical entities characterized by the clonal expansion of monoclonal immunoglobulin (M-protein) secreting plasma cells (PC). This document presents the consensus recommendations of the Spanish Society of Laboratory Medicine (SEQC^ML) and the Spanish Society of Hematology and Hemotherapy (SEHH) for the study of MG. The recommendations were established based on scientific evidence and the opinion of experts in MG from the clinical laboratory and clinical hematology fields. Recommendations are proposed for the diagnosis of MG and for patient follow-up according to the type of MG and whether or not the patient is undergoing treatment, and to monitor the disease stability, response to therapy and disease progression. With respect to the diagnosis, we describe the most recent criteria and classification established by the International Myeloma Working Group (IMWG) for multiple myeloma (MM), smoldering MM, monoclonal gammopathy of undermined significance (MGUS) and other related entities. Indications are given about the analytical requirements and application of the different serum and urine laboratory tests (study, detection, identification and measurement of M-protein) and the bone marrow study. Recommendations on the clinical laboratory results report model are established to harmonize and ensure that all relevant information is available, including its content, expression, and interpretive comments. [ABSTRACT FROM AUTHOR]

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المؤلفون: Solves, Pilar, Marco-Ayala, Javier, Sanz, Miguel Ángel, Gómez-Seguí, Inés, Balaguer-Roselló, Aitana, Facal, Ana, Villalba, Marta, Montoro, Juan, Sanz, Guillermo, de la Rubia, Javier, Sanz, Jaime

المصدر: Journal of Clinical Medicine; May2023, Vol. 12 Issue 10, p3467, 10p

مصطلحات موضوعية: HEMATOPOIETIC stem cell transplantation, CORD blood transplantation, BLOOD transfusion

مستخلص: Introduction: Transfusion plays a main role in supportive treatment for patients who receive an allogeneic hematopoietic stem cell transplantation (HSCT). In this study, we compare the transfusion requirements of patients undergoing different modalities of HSCT according to different time periods. The objective is to assess the evolution of HSCT transfusion requirements over time, from a single institution. Methods: The clinical charts and transfusion records of patients who underwent HSCT of different modalities at La Fe University Hospital during a twelve-year period were reviewed (2009–2020). For analysis, we divided the overall time into three periods: 1 from 2009 to 2012, 2 from 2013 to 2016 and 3 from 2017 to 2020. The study included 855 consecutive adult HSCT: 358 HLA-matched related donors (MRD), 134 HLA-matched unrelated donors (MUD), 223 umbilical cord blood transplantation (UCBT) and 140 haploidentical transplants (Haplo-HSCT). Results: There were no significant differences in RBC and PLT requirements or transfusion independence among the three time periods for MUD and Haplo-HSCT. However, the transfusion burden increased significantly for MRD HSCT during the 2017–2020 period. Conclusion: despite HSCT modalities having evolved and changed over time, overall transfusion requirements have not significantly decreased and continue to be a cornerstone of transplantation-supportive care. [ABSTRACT FROM AUTHOR]

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المؤلفون: Piñana, José Luis, Vázquez, Lourdes, Martino, Rodrigo, de la Cámara, Rafael, Sureda, Anna, Rodríguez-Veiga, Rebeca, Garrido, Ana, Sierra, Jorge, Ribera, José-María, Torrent, Anna, Mateos, María Victoria, de la Rubia, Javier, Tormo, Mar, Díez-Campelo, María, García-Gutiérrez, Valentín, Álvarez-Larrán, Alberto, Sancho, Juan-Manuel, MartínGarcía-Sancho, Alejandro, Yañez, Lucrecia, Pérez Simón, José Antonio

المصدر: Leukemia & Lymphoma; Mar2022, Vol. 63 Issue 3, p538-550, 13p

مصطلحات موضوعية: BLOOD transfusion, COVID-19 vaccines, VACCINATION, SARS-CoV-2, VACCINE effectiveness

مستخلص: In the midst of the COVID-19 pandemic, different vaccines in front of SARS-CoV-2 have been approved and administered in different vulnerable populations. As patients with cancer were excluded from pivotal trials of vaccination, little is known on their immunogenic response to these vaccines, particularly in patients with severely impaired immune system. In response to that uncertainty, the Spanish Society of Hematology and Hemotherapy launched an initiative aimed to provide recommendations for vaccination of the main hematological conditions. This document is based on the available information on COVID-19 outcomes, prior knowledge on vaccination in hematological patients, recent published data on serological response in oncohematological patients and expert opinions. New information about SARS-CoV-2 vaccination will be gathered in the near future, providing new scientific grounds to delineate the most adequate management of vaccination in patients with hematological diseases. The current limited data on SARS-CoV-2 vaccines in hematological patients represents a major limitation of this expert consensus opinion. In fact, the speed in which this field evolves may reduce their validity in the near future. [ABSTRACT FROM AUTHOR]

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المؤلفون: Alvarez-Larrán, Alberto, del Río-Garma, Julio, Pujol, Misericòrdia, de la Rubia, Javier, Hernández-Jodra, Manuel, Borrell, Montserrat, González-Porras, José R., García-Gala, José M., Viejo, Aurora, Enríquez, Lourdes, Arbona, Cristina, García-Erce, José A., Noblejas, Ana G., Pereira, Arturo

المصدر: Annals of Hematology; Oct2009, Vol. 88 Issue 10, p973-978, 6p, 2 Charts

مصطلحات موضوعية: THROMBOTIC thrombocytopenic purpura, PLASMA exchange (Therapeutics), BLOOD transfusion, HEMOLYTIC anemia, HEMOLYSIS & hemolysins, DISEASE relapse

مستخلص: The remission rate with plasma exchange (PE) in thrombotic thrombocytopenic purpura (TTP) exceeds 80%, but the disease relapses in up to 20–30% of the cases. Clinical characteristics and response to treatment of relapsed TTP are not well defined. The objective of the present study was to compare the clinical and biological characteristics at presentation and the response to treatment between de novo and relapsed TTP. For such purpose, a total of 102 episodes of idiopathic TTP (70 de novo and 32 relapses) included in a recent multicentric prospective cohort study were analysed. All patients were homogeneously treated with daily PE and costicosteroids. In comparison with de novo TTP, episodes of relapsed TTP showed a higher Hb level (median, 122 g/l versus 91 g/l, p < 0.001) and lower serum lactate dehydrogenase (2.2- versus 4.5-fold above the upper limit of normality, p < 0.001). Neurological symptoms and fever were less frequently observed in patients with relapsed TTP than in patients with de novo TTP. Patients with relapsed TTP needed fewer PE sessions (five versus ten, p = 0.02) and a smaller volume of plasma (221 ml/kg versus 468 ml/kg, p = 0.004) to achieve remission than those with de novo TTP. There was no significant difference in the rate of recrudescence under treatment, the need of complementary treatments or the frequency of refractoriness to PE therapy. In conclusion, relapsed TTP has a milder clinical profile and responds more easily to PE than de novo TTP. [ABSTRACT FROM AUTHOR]

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المؤلفون: del Río-Garma, Julio, Alvarez-Larrán, Alberto, Martínez, Clara, Muncunill, Josep, Castell, Dolors, de la Rubia, Javier, Zamora, Concepción, Corral, Mercedes, Viejo, Aurora, Peña, Francisco, Rodríguez-Vicente, Pilar, Contreras, Enric, Arbona, Cristina, Ramírez, Consuelo, Garcia-Erce, José A., Alegre, Adrián, Mateo, Jos, Pereira, Arturo

المصدر: British Journal of Haematology; Oct2008, Vol. 143 Issue 1, p39-45, 7p, 3 Charts

مصطلحات موضوعية: THROMBOTIC thrombocytopenic purpura, HEMOLYTIC anemia, PLASMA exchange (Therapeutics), BLOOD transfusion, COMMUNICABLE diseases, BLOOD plasma

مستخلص: Plasma exchange (PE) with plasma infusion is the treatment of choice for thrombotic thrombocytopenic purpura (TTP) but doubts remain as to whether all kinds of plasma are equally effective. A multicentric cohort study was conducted to compare methylene blue-photoinactivated plasma (MBPIP) with quarantine fresh frozen plasma (qFFP) in the treatment of TTP. One hundred and two episodes of idiopathic TTP were included; MBPIP was used in 63 and qFFP in 39. The treatment schedule consisted of daily PE and costicosteroids, and the main end-point was remission status on day 8. Patients treated with MBPIP required more PEs (median: 11 vs. 5, P = 0·002) and a larger volume of plasma (median: 485 ml/kg vs. 216 ml/kg, P = 0·007) to achieve a remission, and presented more recrudescences while on PE therapy (29 of 63 vs. 8 of 39, P = 0·02) than those receiving qFFP. After adjustment for possible confounding factors, the use of MBPIP was associated with a lower likelihood of remission on day 8 [Odds ratio (OR): 0·17; 95% confidence interval (CI): 0·06–0·47] and a higher risk of recrudescence while on treatment (OR: 4·2; 95% CI: 1·6–10·8). In conclusion, MBPIP is less effective than qFFP in the treatment of TTP. [ABSTRACT FROM AUTHOR]

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المؤلفون: Sanz, Jaime^1,2 sanz_jai@gva.es, Montesinos, Pau¹, Saavedra, Silvana¹, Lorenzo, Ignacio¹, Senent, Leonor¹, Planelles, Dolores³, Larrea, Luis³, Martín, Guillermo¹, Palau, Javier¹, Jarque, Isidro¹, Martínez, Jesús¹, de la Rubia, Javier¹, Moscardó, Federico¹, Martinez, David¹, Gómez, Inés¹, López, María¹, Sanz, Miguel A.¹, Sanz, Guillermo F.¹

المصدر: Biology of Blood & Marrow Transplantation. Nov2010, Vol. 16 Issue 11, p1589-1595. 7p.

مصطلحات موضوعية: *CORD blood, *CHRONIC myeloid leukemia, *BLOOD transfusion, *STEM cell transplantation, *HEALTH outcome assessment, *GRAFT versus host disease, *MEDICAL statistics, *PATIENTS

مستخلص: Clinical studies focused on outcomes of umbilical cord blood transplantation (UCBT) for patients with chronic myelogenous leukemia (CML) in need of allogeneic stem cell transplantation and lacking an HLA-matched adult donor are limited. We analyzed the outcome of 26 adults with CML receiving single-unit UCBT from unrelated donors after myeloablative conditioning at a single institution. Conditioning regimens were based on combinations of thiotepa, busulfan, cyclophospamide or fludarabine, and antithymocyte globulin. At the time of transplantation, 7 patients (27%) were in first chronic phase (CP), 11 (42%) were in second CP, 2 (8%) were in accelerated phase (AP), and 6 (23%) were in blast crisis (BC). The cumulative incidence (CI) of myeloid engraftment was 88% at a median time of 22 days and was significantly better for patients receiving higher doses of CD34+ cells. The CI of acute graft-versus-host disease (GVHD) grade II-IV was 61%, that of acute GVHD grade III-IV was 39%, and that of chronic extensive GVHD was 60%. Treatment-related mortality (TRM) was 41% for patients undergoing UCBT while in first or second CP and 100% for patients in AP or BC (P < .01). After a median follow-up of 8 years, none of the patients relapsed, giving an overall disease-free survival (DFS) at 8 years of 41%. The DFS for patients undergoing UCBT while in any CP was 59%. These results demonstrate that UCBT from unrelated donors can be a curative treatment for a substantial number of patients with CML. Advances in supportive care and better selection of cord blood units and patients are needed to improve TRM. [ABSTRACT FROM AUTHOR]