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Article ; Online: Hb Nivaria: A New Hemoglobin Variant with a Shortened

Ropero Gradilla, Paloma / Raya, José María / González, Fernando Ataúlfo / Rochas, Sara / Ferrer-Benito, Sara / Nieto, Jorge M / Martín-Santos, Taida / Barrios, Marcelo / Gutiérrez-Murillo, Lorena / Villegas, Ana / Benavente, Celina

Hemoglobin

2023 Volume 46, Issue 6, Page(s) 344–346

Abstract: We report a novel hemoglobin (Hb) variant found in a Spanish individual from Santa Cruz de Tenerife, the Canary Islands in Spain. The proband was a 39-year-old male. High performance liquid chromatography (HPLC) displayed an unknown peak (19.3%) at a ... ...

Abstract	We report a novel hemoglobin (Hb) variant found in a Spanish individual from Santa Cruz de Tenerife, the Canary Islands in Spain. The proband was a 39-year-old male. High performance liquid chromatography (HPLC) displayed an unknown peak (19.3%) at a retention time of 1.3 min. eluting before Hb A
MeSH term(s)	Male ; Humans ; Adult ; Glycated Hemoglobin ; Lysine ; Hemoglobins ; Chromatography, High Pressure Liquid ; Electrophoresis, Capillary
Chemical Substances	Glycated Hemoglobin ; Lysine (K3Z4F929H6) ; Hemoglobins
Language	English
Publishing date	2023-02-27
Publishing country	England
Document type	Case Reports ; Journal Article
ZDB-ID	750615-6
ISSN	1532-432X ; 0363-0269
ISSN (online)	1532-432X
ISSN	0363-0269
DOI	10.1080/03630269.2023.2172430
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Article ; Online: Bone marrow trephine biopsy in Hodgkin's lymphoma. Comparison with PET-CT scan in 65 patients.

Lakhwani, Sunil / Cabello-García, Dolores / Allende-Riera, Ana / Cárdenas-Negro, Carlos / Raya, José María / Hernández-Garcia, Miguel T

Medicina clinica

2017 Volume 150, Issue 3, Page(s) 104–106

Abstract: Background and objectives: To compare bone marrow biopsy (BMB) and PET/CT in detecting bone marrow involvement in Hodgkin's lymphoma MATERIAL AND METHODS: Retrospective analysis of 65 patients with both tests in the initial staging or in relapse with ... ...

Title translation	Biopsia de médula ósea en el linfoma de Hodgkin. Comparación con la PET-TC en 65 pacientes.
Abstract	Background and objectives: To compare bone marrow biopsy (BMB) and PET/CT in detecting bone marrow involvement in Hodgkin's lymphoma MATERIAL AND METHODS: Retrospective analysis of 65 patients with both tests in the initial staging or in relapse with special attention to the PET/CT uptake pattern. Results: In 3 patients (4.6%), the BMB showed bone marrow involvement with the PET/CT being positive in them all: 2 with diffuse+multifocal pattern and one diffuse only. In 11 additional patients (total 14/65, 21%), bone marrow involvement was diagnosed by PET/CT because bone marrow uptake was above hepatic one. The pattern was focal only in 2 cases, multifocal in 5, diffuse in 3 and diffuse+multifocal in one. In these last 4 cases the BMB showed an unspecific myelopathy. Conclusions: PET/CT detects all cases with BMB affected and many that escape to biopsy, however when the uptake pattern is diffuse it could be by involvement or reactive hyperplasia and in those cases the BMB should be done.
MeSH term(s)	Adolescent ; Adult ; Aged ; Biopsy ; Bone Marrow/pathology ; Female ; Hodgkin Disease/diagnostic imaging ; Hodgkin Disease/pathology ; Humans ; Male ; Middle Aged ; Positron Emission Tomography Computed Tomography ; Retrospective Studies ; Young Adult
Language	Spanish
Publishing date	2017-08-31
Publishing country	Spain
Document type	Comparative Study ; Journal Article
ZDB-ID	411607-0
ISSN	1578-8989 ; 0025-7753
ISSN (online)	1578-8989
ISSN	0025-7753
DOI	10.1016/j.medcli.2017.06.060
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Article ; Online: Thrombopoietin receptor agonist switch in adult primary immune thrombocytopenia patients: A retrospective collaborative survey involving 4 Spanish centres.

Lakhwani, Sunil / Perera, María / Fernández-Fuertes, Fernando / Ríos de Paz, Mario A / Torres, Melisa / Raya, José María / Hernández, Miguel T

European journal of haematology

2017 Volume 99, Issue 4, Page(s) 372–377

Abstract: Objective: To describe the reasons for and result of thrombopoietin receptor agonists (TPO-RA) switching in adult immune thrombocytopenia (ITP) patients of 4 Spanish centres.: Methods: We retrospectively analysed all patients who received sequential ... ...

Abstract	Objective: To describe the reasons for and result of thrombopoietin receptor agonists (TPO-RA) switching in adult immune thrombocytopenia (ITP) patients of 4 Spanish centres. Methods: We retrospectively analysed all patients who received sequential treatment with both TPO-RA between 2010 and 2015 recording clinical and biological parameters. Results: Twenty-six patients were included; 17 received first romiplostim and 9 received first eltrombopag. Reasons for switching were inefficacy (n = 10), patient preference (n = 8), side effects (n = 5) and excessive platelet count fluctuation (n = 3). When the switch was due to inefficacy, 100% of patients who received romiplostim first and 66% who received eltrombopag first responded to the second drug. It is significant that none of the patients who received romiplostim first reached the maximum recommended dose before switching. When the change was due to patient preference or because of side effects, 100% of the patients responded to both TPO-RA. Three patients changed from romiplostim to eltrombopag due to platelet count fluctuation; one did not respond and the fluctuation persisted in the remaining 2 patients. We also found 4 sustained remissions after administering the second TPO-RA, 2 of these with inefficacy of the first drug. Conclusion: TPO-RA switching is a feasible strategy in different scenarios with high probability of success.
Language	English
Publishing date	2017-10
Publishing country	England
Document type	Journal Article
ZDB-ID	392482-8
ISSN	1600-0609 ; 0902-4441
ISSN (online)	1600-0609
ISSN	0902-4441
DOI	10.1111/ejh.12932
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Article ; Online: Integrating AIPSS-MF and molecular predictors: A comparative analysis of prognostic models for myelofibrosis.

Mosquera-Orgueira, Adrián / Arellano-Rodrigo, Eduardo / Garrote, Marta / Martín, Iván / Pérez-Encinas, Manuel / Gómez-Casares, María-Teresa / Hernández-Sánchez, Alberto / Ferrer-Marín, Francisca / Mora, Elvira / Velez, Patricia / Ayala, Rosa / Angona, Anna / Heras, Natalia de Las / Magro, Elena / Pérez-Míguez, Carlos / Crucitti, Davide / Mata-Vázquez, María-Isabel / Fox, María-Laura / González de Villambrosía, Sonia /

Ramírez, María-José / García, Ana / García-Gutiérrez, Valentín / Cáceres, Amparo / Durán, María-Antonia / Senín, María-Alicia / Raya, José-María / González, José A / Cuevas, Beatriz / Xicoy, Blanca / Nangalia, Jyoti / Hernández-Rivas, Jesús M / Bellosillo, Beatriz / Álvarez-Larrán, Alberto / Hernández-Boluda, Juan C

HemaSphere

2024 Volume 8, Issue 3, Page(s) e60

Language	English
Publishing date	2024-03-20
Publishing country	United States
Document type	Journal Article
ISSN	2572-9241
ISSN (online)	2572-9241
DOI	10.1002/hem3.60
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Article ; Online: The prognostic impact of non-driver gene mutations and variant allele frequency in primary myelofibrosis.

Hernández-Sánchez, Alberto / Villaverde-Ramiro, Ángela / Arellano-Rodrigo, Eduardo / Garrote, Marta / Martín, Iván / Mosquera-Orgueira, Adrián / Gómez-Casares, María-Teresa / Ferrer-Marín, Francisca / Such, Esperanza / Velez, Patricia / Ayala, Rosa / Angona, Anna / de Las Heras, Natalia / Magro, Elena / Mata-Vázquez, María-Isabel / Fox, María-Laura / de Villambrosía, Sonia González / Ramírez, María-José / García, Ana /

García-Gutiérrez, Valentín / Cáceres, Amparo / Durán, María-Antonia / Senín, Alicia / Raya, José-María / González, José Antonio / Cuevas, Beatriz / Xicoy, Blanca / Pérez-Encinas, Manuel / Bellosillo, Beatriz / Álvarez-Larrán, Alberto / Hernández-Rivas, Jesús María / Hernández-Boluda, Juan Carlos

American journal of hematology

2024 Volume 99, Issue 4, Page(s) 755–758

Abstract: Prognostic impact of non-MPN driver gene mutations in primary myelofibrosis. MIPSS70: Mutation-Enhanced International Prognostic Score System. ...

Abstract	Prognostic impact of non-MPN driver gene mutations in primary myelofibrosis. MIPSS70: Mutation-Enhanced International Prognostic Score System.
MeSH term(s)	Humans ; Prognosis ; Primary Myelofibrosis/diagnosis ; Primary Myelofibrosis/genetics ; Mutation ; Janus Kinase 2/genetics ; Gene Frequency
Chemical Substances	Janus Kinase 2 (EC 2.7.10.2)
Language	English
Publishing date	2024-01-30
Publishing country	United States
Document type	Letter
ZDB-ID	196767-8
ISSN	1096-8652 ; 0361-8609
ISSN (online)	1096-8652
ISSN	0361-8609
DOI	10.1002/ajh.27203
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Article ; Online: Cytomegalovirus-associated hemophagocytic syndrome in a patient with Crohn's disease receiving azathioprine.

Hernández-Camba, Alejandro / Lakhwani, Sunil / Ramos, Laura / Raya, José Maria / Quintero, Enrique

Journal of gastrointestinal and liver diseases : JGLD

2013 Volume 22, Issue 4, Page(s) 471–472

MeSH term(s)	Adult ; Anti-Infective Agents/adverse effects ; Antiviral Agents/therapeutic use ; Azathioprine/adverse effects ; Bone Marrow Examination ; Crohn Disease/diagnosis ; Crohn Disease/drug therapy ; Crohn Disease/immunology ; Cytomegalovirus Infections/chemically induced ; Cytomegalovirus Infections/diagnosis ; Cytomegalovirus Infections/drug therapy ; Cytomegalovirus Infections/immunology ; Gastrointestinal Agents/adverse effects ; Humans ; Immunocompromised Host ; Lymphohistiocytosis, Hemophagocytic/chemically induced ; Lymphohistiocytosis, Hemophagocytic/diagnosis ; Lymphohistiocytosis, Hemophagocytic/drug therapy ; Lymphohistiocytosis, Hemophagocytic/immunology ; Male ; Treatment Outcome
Chemical Substances	Anti-Infective Agents ; Antiviral Agents ; Gastrointestinal Agents ; Azathioprine (MRK240IY2L)
Language	English
Publishing date	2013-12-19
Publishing country	Romania
Document type	Case Reports ; Letter
ZDB-ID	2427021-0
ISSN	1842-1121 ; 1841-8724
ISSN (online)	1842-1121
ISSN	1841-8724
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Article ; Online: Real-world analysis of main clinical outcomes in patients with polycythemia vera treated with ruxolitinib or best available therapy after developing resistance/intolerance to hydroxyurea.

Alvarez-Larrán, Alberto / Garrote, Marta / Ferrer-Marín, Francisca / Pérez-Encinas, Manuel / Mata-Vazquez, M Isabel / Bellosillo, Beatriz / Arellano-Rodrigo, Eduardo / Gómez, Montse / García, Regina / García-Gutiérrez, Valentín / Gasior, Mercedes / Cuevas, Beatriz / Angona, Anna / Gómez-Casares, María Teresa / Martínez, Clara M / Magro, Elena / Ayala, Rosa / Del Orbe-Barreto, Rafael / Pérez-López, Raúl /

Fox, Maria Laura / Raya, José-María / Guerrero, Lucía / García-Hernández, Carmen / Caballero, Gonzalo / Murillo, Ilda / Xicoy, Blanca / Ramírez, M José / Carreño-Tarragona, Gonzalo / Hernández-Boluda, Juan Carlos / Pereira, Arturo

Cancer

2022 Volume 128, Issue 13, Page(s) 2441–2448

Abstract: Background: Ruxolitinib is approved for patients with polycythemia vera (PV) who are resistant/intolerant to hydroxyurea, but its impact on preventing thrombosis or disease-progression is unknown.: Methods: A retrospective, real-world analysis was ... ...

Abstract	Background: Ruxolitinib is approved for patients with polycythemia vera (PV) who are resistant/intolerant to hydroxyurea, but its impact on preventing thrombosis or disease-progression is unknown. Methods: A retrospective, real-world analysis was performed on the outcomes of 377 patients with resistance/intolerance to hydroxyurea from the Spanish Registry of Polycythemia Vera according to subsequent treatment with ruxolitinib (n = 105) or the best available therapy (BAT; n = 272). Survival probabilities and rates of thrombosis, hemorrhage, acute myeloid leukemia, myelofibrosis, and second primary cancers were calculated according to treatment. To minimize biases in treatment allocation, all results were adjusted by a propensity score for receiving ruxolitinib or BAT. Results: Patients receiving ruxolitinib had a significantly lower rate of arterial thrombosis than those on BAT (0.4% vs 2.3% per year; P = .03), and this persisted as a trend after adjustment for the propensity to have received the drug (incidence rate ratio, 0.18; 95% confidence interval, 0.02-1.3; P = .09). There were no significant differences in the rates of venous thrombosis (0.8% and 1.1% for ruxolitinib and BAT, respectively; P = .7) and major bleeding (0.8% and 0.9%, respectively; P = .9). Ruxolitinib exposure was not associated with a higher rate of second primary cancers, including all types of neoplasia, noncutaneous cancers, and nonmelanoma skin cancers. After a median follow-up of 3.5 years, there were no differences in survival or progression to acute leukemia or myelofibrosis between the 2 groups. Conclusions: The results suggest that ruxolitinib treatment for PV patients with resistance/intolerance to hydroxyurea may reduce the incidence of arterial thrombosis. Lay summary: Ruxolitinib is better than other available therapies in achieving hematocrit control and symptom relief in patients with polycythemia vera who are resistant/intolerant to hydroxyurea, but we still do not know whether ruxolitinib provides an additional benefit in preventing thrombosis or disease progression. We retrospectively studied the outcomes of 377 patients with resistance/intolerance to hydroxyurea from the Spanish Registry of Polycythemia Vera according to whether they subsequently received ruxolitinib (n = 105) or the best available therapy (n = 272). Our findings suggest that ruxolitinib could reduce the incidence of arterial thrombosis, but a disease-modifying effect could not be demonstrated for ruxolitinib in this patient population.
MeSH term(s)	Hemorrhage/chemically induced ; Humans ; Hydroxyurea/adverse effects ; Leukemia, Myeloid, Acute/drug therapy ; Neoplasms, Second Primary/drug therapy ; Nitriles ; Polycythemia Vera/drug therapy ; Primary Myelofibrosis/drug therapy ; Pyrazoles ; Pyrimidines ; Retrospective Studies ; Thrombosis/chemically induced ; Thrombosis/drug therapy ; Thrombosis/prevention & control
Chemical Substances	Nitriles ; Pyrazoles ; Pyrimidines ; ruxolitinib (82S8X8XX8H) ; Hydroxyurea (X6Q56QN5QC)
Language	English
Publishing date	2022-04-13
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	1429-1
ISSN	1097-0142 ; 0008-543X ; 1934-662X
ISSN (online)	1097-0142
ISSN	0008-543X ; 1934-662X
DOI	10.1002/cncr.34195
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Article ; Online: Management of side effects of BCR/ABL-negative chronic myeloproliferative neoplasm therapies. Focus on anagrelide.

Antelo, María Luisa / de Las Heras, Natalia / Gonzalez Porras, Jose Ramón / Kerguelen, Ana / Raya, Jose María

Expert review of hematology

2015 Volume 8, Issue 6, Page(s) 819–835

Abstract: Although hydroxyurea is considered the first-line cytoreductive therapy in high-risk patients with polycythemia vera or essential thrombocythemia, approximately 20-25% of patients develop resistance or intolerance and they need an alternative therapy. ... ...

Abstract	Although hydroxyurea is considered the first-line cytoreductive therapy in high-risk patients with polycythemia vera or essential thrombocythemia, approximately 20-25% of patients develop resistance or intolerance and they need an alternative therapy. Anagrelide is the treatment of choice in patients with essential thrombocythemia intolerant or with resistance to hydroxyurea. Anagrelide is usually well tolerated. Although there is concern about the increased risk of cardiac side effects, in most cases these are mild, and easily manageable. In this paper, the available evidence about the management of patients with myeloproliferative neoplasms, with a special focus on the side effects of drug therapies is reviewed.
MeSH term(s)	Female ; Fibrinolytic Agents/administration & dosage ; Fibrinolytic Agents/therapeutic use ; Fusion Proteins, bcr-abl/adverse effects ; Humans ; Male ; Myeloproliferative Disorders/drug therapy ; Quinazolines/administration & dosage ; Quinazolines/therapeutic use
Chemical Substances	Fibrinolytic Agents ; Quinazolines ; Fusion Proteins, bcr-abl (EC 2.7.10.2) ; anagrelide (K9X45X0051)
Language	English
Publishing date	2015-12
Publishing country	England
Document type	Journal Article ; Research Support, Non-U.S. Gov't ; Review
ZDB-ID	2516804-6
ISSN	1747-4094 ; 1747-4086
ISSN (online)	1747-4094
ISSN	1747-4086
DOI	10.1586/17474086.2015.1088779
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Article ; Online: Low-risk polycythemia vera treated with phlebotomies: clinical characteristics, hematologic control and complications in 453 patients from the Spanish Registry of Polycythemia Vera.

Triguero, Ana / Pedraza, Alexandra / Pérez-Encinas, Manuel / Mata-Vázquez, María Isabel / Vélez, Patricia / Fox, Laura / Gómez-Calafat, Montse / García-Delgado, Regina / Gasior, Mercedes / Ferrer-Marín, Francisca / García-Gutiérrez, Valentín / Angona, Anna / Gómez-Casares, María Teresa / Cuevas, Beatriz / Martínez, Clara / Pérez, Raúl / Raya, José María / Guerrero, Lucía / Murillo, Ilda /

Bellosillo, Beatriz / Hernández-Boluda, Juan Carlos / Sanz, Cristina / Álvarez-Larrán, Alberto

Annals of hematology

2022 Volume 101, Issue 10, Page(s) 2231–2239

Abstract: Hematological control, incidence of complications, and need for cytoreduction were studied in 453 patients with low-risk polycythemia vera (PV) treated with phlebotomies alone. Median hematocrit value decreased from 54% at diagnosis to 45% at 12 months, ... ...

Abstract	Hematological control, incidence of complications, and need for cytoreduction were studied in 453 patients with low-risk polycythemia vera (PV) treated with phlebotomies alone. Median hematocrit value decreased from 54% at diagnosis to 45% at 12 months, and adequate hematocrit control over time (< 45%) was observed in 36%, 44%, and 32% of the patients at 6, 12, and 24 months, respectively. More than 5 phlebotomies per year in the maintenance phase were required in 19% of patients. Worsening thrombocytosis, age > 60 years, and microvascular symptoms constituted the main indications for starting cytoreduction. Median duration without initiating cytoreduction was significantly longer in patients younger than 50 years (< 0.0001). The incidence rate of thrombosis under phlebotomies alone was 0.8% per year and the estimated probability of thrombosis at 10 years was 8.5%. The probability of arterial thrombosis was significantly higher in patients with arterial hypertension whereas there was a trend to higher risk of venous thrombosis in cases with high JAK2V617F allele burden. Rates of major bleeding and second primary neoplasm were low. With a median follow-up of 9 years, survival probability at 10 years was 97%, whereas the probability of myelofibrosis at 10 and 20 years was 7% and 20%, respectively. Progression to acute myeloid leukemia was documented in 3 cases (1%). Current management of low-risk PV patients is associated with low rate of thrombosis and long survival. New treatment strategies are needed for improving hematological control and, in the long term, reducing progression to myelofibrosis.
MeSH term(s)	Humans ; Leukemia, Myeloid, Acute/complications ; Middle Aged ; Phlebotomy/adverse effects ; Polycythemia Vera/complications ; Polycythemia Vera/diagnosis ; Polycythemia Vera/surgery ; Primary Myelofibrosis/diagnosis ; Registries ; Thrombosis/complications ; Thrombosis/etiology
Language	English
Publishing date	2022-08-30
Publishing country	Germany
Document type	Journal Article
ZDB-ID	1064950-5
ISSN	1432-0584 ; 0939-5555 ; 0945-8077
ISSN (online)	1432-0584
ISSN	0939-5555 ; 0945-8077
DOI	10.1007/s00277-022-04963-z
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Article ; Online: Correction to: Low‑risk polycythemia vera treated with phlebotomies: clinical characteristics, hematologic control and complications in 453 patients from the Spanish Registry of Polycythemia Vera.

Triguero, Ana / Pedraza, Alexandra / Perez-Encinas, Manuel / Mata-Vazquez, María Isabel / Vélez, Patricia / Fox, Laura / Gomez-Calafat, Montse / Garcia-Delgado, Regina / Gasior, Mercedes / Ferrer-Marin, Francisca / Garcia-Gutierrez, Valentín / Angona, Anna / Gomez-Casares, María Teresa / Cuevas, Beatriz / Martínez, Clara / Pérez, Raúl / Raya, José María / Guerrero, Lucía / Murillo, Ilda /

Bellosillo, Beatriz / Hernandez-Boluda, Juan Carlos / Sanz, Cristina / Alvarez-Larran, Alberto

Annals of hematology

2022 Volume 101, Issue 12, Page(s) 2819–2820

Language	English
Publishing date	2022-10-20
Publishing country	Germany
Document type	Published Erratum
ZDB-ID	1064950-5
ISSN	1432-0584 ; 0939-5555 ; 0945-8077
ISSN (online)	1432-0584
ISSN	0939-5555 ; 0945-8077
DOI	10.1007/s00277-022-05005-4
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