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1.
Rev. bras. anal. clin ; 53(1): 90-96, 20210330. tab, ilus
Artículo en Portugués | LILACS | ID: biblio-1291778

RESUMEN

Descrição: Relato de caso de um paciente com um transcrito raro (e1a2) na Leucemia Mieloide Crônica (LMC) e outro com uma translocação rara na Síndrome Mielodisplásica (SMD). Discussão: O transcrito e1a2 possui frequência de 1% entre os casos de LMC, já a translocação t(11,17)(q23;q21) não foi evidenciada em paciente com SMD do tipo Anemia Refratária com Excesso de Blastos (AREB) do tipo 2. Conclusão: Ambos os casos apresentados possuem associação incomum entre fenótipo e genótipo. A correlação da clínica com os achados laboratoriais é importante para a determinação fidedigna do diagnóstico e prognóstico destes pacientes.


Description: Case report of a patient with a rare transcript (e1a2) in Chronic Myeloid Leukemia (CML) and another with a rare translocation in Myelodysplastic Syndrome (SMD). Discussion: The transcript e1a2 has a frequency of 1% in CML cases, whereas t (11,17) (q23; q21) translocation was not observed in a patient with type of Refractory Anemia with Excess Blasts (AREB) type 2. Conclusion: Both cases reported have unusual association between phenotype and genotype. The correlation of the clinic with the laboratory findings is important for the reliable determination of the diagnosis and prognosis of these patients.


Asunto(s)
Humanos , Femenino , Persona de Mediana Edad , Fenotipo , Translocación Genética , Anemia Refractaria , Leucemia Mielógena Crónica BCR-ABL Positiva , Leucemia , Neoplasias Hematológicas , Genotipo
3.
Korean Journal of Medicine ; : 11-16, 2019.
Artículo en Coreano | WPRIM | ID: wpr-759923

RESUMEN

Hypoxia inducible factor (HIF)-stabilizers are being developed for the renal anemia treatment. This small molecules inhibit prolyl hydroxylase domain (PHD)-containing enzymes, causing HIF activation instead of degradation under the state of normoxia, finally increase production of intrinsic erythropoiesis. Current treatment guidelines suggest that renal anemia should be treated mainly with iron and erythropoiesis stimulating agents (ESAs). But there are several complications and concerns such as hypertension, ESA refractory anemia and increased cardiovascular mortality in using ESAs. Advantages of HIF stabilizers over ESAs are orally available, no dose-up requirement for inflammation. So far new HIF stabilizers showed efficacy and safety in renal anemia treatment. This new therapeutic agent may emerge as a standard treatment option for renal anmia treatment.


Asunto(s)
Humanos , Anemia , Anemia Refractaria , Hipoxia , Eritropoyesis , Hematínicos , Hepcidinas , Hipertensión , Inflamación , Hierro , Mortalidad , Prolil Hidroxilasas , Insuficiencia Renal Crónica
4.
Journal of Experimental Hematology ; (6): 515-519, 2019.
Artículo en Chino | WPRIM | ID: wpr-771927

RESUMEN

OBJECTIVE@#To investigate the efficacy and safely of DAC and CAG/HAG preexcitation chemotherapy regimens for the treatment of patients with MDS-RAEB (refractory anemia with excess blasts, RAEB).@*METHODS@#The clinical data of 86 MDS-RAEB patients were analyzed retrospectively from February 2014 to February 2018. According to therapeutic regimem, the 86 patients were divided into 2 groups: group A (41 patients) with DAC preexcitation chemotherapy regimen, and group B (45 patients) with CAG/HAG preexcitation chemotherapy regimen; and the disease control effect, effective treatment course, median survival time and incidence of adverse reactions were compared between these 2 groups.@*RESULTS@#The CR rate and ORR rate were not significantly different between these 2 groups (P>0.05). The mCR rate in group A was significantly higher than that in group B (P<0.05). The numbers of cases obtained therapeutic efficacy at 2 rd and 3 rd conrse in group A significantly more than those in group B (P<0.05), but the number of cases obtained efficacy at 1 st course in group B was significantly higher than that in group A (P<0.05). The median OS time was not significanly different between 2 groups (P>0.05). The duration of neutrophils deficiency in group A was significantly shorter than that in group B (P<0.05). The transfusion volume of red blood cells and platelets in group A was significantly less than that of group B (P<0.05). The incidence of neutropenia, anemia and thrombocytopenia of III-IV grade at different treatment courses of group A were significantly lower than that in group B (P<0.05). The incidence of infection of III-IV grade in group A at 3rd treatment course was significantly lower than that in group B (P<0.05).@*CONCLUSION@#Preexcitation chemotherapy regimens of DAC and CAG/HAG for the treatment of MDS-RAEB possess the same effects for disease control; application of DAC regimen can efficiently reduce the risk of adverse reaction, but CAG/HAG regimen can be helpful to accelerate the effective process of treatment.


Asunto(s)
Humanos , Anemia Refractaria , Anemia Refractaria con Exceso de Blastos , Quimioterapia , Síndromes Mielodisplásicos , Quimioterapia , Estudios Retrospectivos , Resultado del Tratamiento
5.
Journal of Experimental Hematology ; (6): 1215-1219, 2019.
Artículo en Chino | WPRIM | ID: wpr-775739

RESUMEN

OBJECTIVE@#To evaluate the clinical efficacy of low dose combined chemotherapy(LDCC) for patients with relapsed and refractory aplastic anemia-paroxysmal nocturnal hemoglobinuria(AA-PNH) syndrome, and to analyze the advantages of LDCC in the treatment of AA-PNH syndrome.@*METHODS@#The clinical characteristics and the curative effect of LDCC in 9 patients with relapsed and refractory AA-PNH syndrome were retrospectively analyzed. Five patients were treated with MP therapy[melphalan 2 mg/(m·d); prednisone 0.5 mg/(kg·d)], and the other 4 patients were treated with HA therapy(HHT 2 mg/d iv drip, for 5 days; Ara-C 100 mg/d iv drip, for 5 days). The changes of PNH clone, dosage of corticosteroid, hemolysis and the relapse of disease, hematological parameters and adverse reactions were compared before and after therapy. All patients were treated for 1-2 courses.@*RESULTS@#Seven out of 9 patients responded well, the dosage of corticosteroid and the bilirubin concentration decreased significantly and anemia was relieved in 7 patients (P<0.05). One patient relapsed in one year. PNH clone of 3 patients turned negative. Five patients did not rely on blood transfusion in 1 year. There was no bone marrow failure to be found in all patients.@*CONCLUSION@#The LDCC has better efficacy and safety in the treatment of patients with AA-PNH syndrome, moreover, the patients is more tolerant to LDCC, thus the LDCC may be a selection for treatment of patients with relapsed and refractory AA-PNH syndrome.


Asunto(s)
Humanos , Anemia Aplásica , Anemia Refractaria , Hemoglobinuria Paroxística , Hemólisis , Estudios Retrospectivos
6.
Hematol., Transfus. Cell Ther. (Impr.) ; 40(3): 219-225, July-Sept. 2018. tab, graf
Artículo en Inglés | LILACS | ID: biblio-953833

RESUMEN

ABSTRACT Background: Refractory or unexplained iron deficiency anemia accounts for about 15% of all cases. The endoscopic gastrointestinal workup sometimes fails to establish the cause of iron deficiency anemia and a considerable proportion of patients regardless of risk category fail to respond to oral iron supplementation. The aim of the present study was to assess the etiological role of Helicobacter pylori infection in adult Egyptian patients with unexplained or refractory iron deficiency anemia. Methods: A case controlled study was composed of 104 iron deficiency anemia cases and 70 age- and gender-matched healthy controls. Patients were diagnosed with iron deficiency anemia according to hemoglobin, mean corpuscular volume, serum ferritin, and transferrin saturation. Upper and lower endoscopies were performed and active H. pylori infection was investigated by testing for the H. pylori antigen in stool specimens. Hematological response to H. pylori treatment with triple therapy together with iron therapy (n = 32) or only iron therapy (n = 32) were assessed in patients with H. pylori infection. Results: H. pylori infection was more prevalent in patients with unexplained or refractory iron deficiency anemia (61.5%). Of the different hematological parameters investigated, there was a significant correlation only between H. pylori infection and mean corpuscular volume (p-value 0.046). Moreover, there was a significant correlation between receiving triple therapy together with iron supplementation and improvements in the hematological parameters [hemoglobin (p-value < 0.001), mean corpuscular volume (p-value < 0.001), iron (p-value < 0.001) and serum ferritin (p-value < 0.001)] compared to receiving iron supplementation alone. Conclusions: Failing to test for H. pylori infection could lead to a failure to identify a treatable cause of anemia and could lead to additional and potentially unnecessary investigations. Furthermore, treatment of H. pylori infection together with iron supplementation gives a more rapid and satisfactory response.


Asunto(s)
Humanos , Masculino , Femenino , Adulto , Persona de Mediana Edad , Anciano , Anemia Refractaria , Helicobacter pylori , Anemia Ferropénica , Síntomas sin Explicación Médica
7.
Blood Research ; : 8-8, 2017.
Artículo en Inglés | WPRIM | ID: wpr-97880

RESUMEN

No abstract available.


Asunto(s)
Anemia Refractaria , Trombocitosis
8.
Korean Journal of Medicine ; : 63-67, 2016.
Artículo en Coreano | WPRIM | ID: wpr-149386

RESUMEN

Glomerulonephritis associated with malignancy is deemed to be paraneoplastic glomerulonephritis. Myelodysplastic syndrome (MDS) is a group of heterogeneous clonal hematopoietic stem cell disorders characterized by impaired hematopoietic cell differentiation and cytopenia. The pathophysiology of MDS is thought to be immune-mediated in part. A few reports have documented various forms of glomerulonephritis in patients with MDS and suggested that immune dysregulation is important in the development of paraneoplastic glomerulonephritis. Here, we report a patient with MDS and refractory anemia with excess blast-2 accompanied by minimal change nephrotic syndrome. The patient was treated with prednisolone, and the nephrotic-range proteinuria and pancytopenia improved markedly.


Asunto(s)
Humanos , Anemia Refractaria , Diferenciación Celular , Glomerulonefritis , Células Madre Hematopoyéticas , Síndromes Mielodisplásicos , Nefrosis Lipoidea , Síndrome Nefrótico , Pancitopenia , Prednisolona , Proteinuria , Esteroides
9.
Korean Journal of Medicine ; : 460-463, 2016.
Artículo en Inglés | WPRIM | ID: wpr-101313

RESUMEN

Here, we report on a 20-year-old patient with a primary nonseminomatous mediastinal germ cell tumor (MGCT) who developed myelodysplastic syndrome (MDS) 2 months following chemotherapy with cisplatin, etoposide, ifosfamide, and paclitaxel. Bone marrow examinations revealed that the MDS was a refractory anemia with excess type II blasts and complex chromosomal abnormalities. With the onset of MDS occurring rapidly following chemotherapy, it is unlikely to have been caused by the therapy. We discuss the association between primary nonseminomatous MGCTs and hematological malignancies, including the possibility of a common clonal origin.


Asunto(s)
Humanos , Adulto Joven , Anemia Refractaria , Examen de la Médula Ósea , Aberraciones Cromosómicas , Cisplatino , Quimioterapia , Etopósido , Células Germinativas , Neoplasias Hematológicas , Ifosfamida , Síndromes Mielodisplásicos , Neoplasias de Células Germinales y Embrionarias , Paclitaxel
10.
Journal of Experimental Hematology ; (6): 801-805, 2016.
Artículo en Chino | WPRIM | ID: wpr-246864

RESUMEN

<p><b>OBJECTIVE</b>To investigate the clinical significance of bone marrow morphological differences in the differential diagnosis of megaloblastic anemia (MM) and refractory anemia (R4).</p><p><b>METHODS</b>A total of 60 anemia patients selected from our hospital between April 2004 and April 2015 were divided into MA group (30 cases) and RA group (30 cases) in accordance with their clinical diagnosis. Clinical manifestations, results of bone marrow morphology test, blood examination, peripheral blood smear, erythroid megaloblastic variability rate and nucleated red blood cell level in the 2 groups were compared and analyzed.</p><p><b>RESULTS</b>Incidence of fever, hemorrhage, digestive reaction, splenomegaly and fatigue as well as hemoglobin level, platelets and white blood cell counts in patients of MA group were similar to those of RA group, there was no statistically significant difference between 2 groups (P>0.05). The percentages of dysplastic hematopoiesis in erythroid cells, granulocytic cells, magakaryoajtic cells, the PAS-positive rate and red blood cell distribution in the MA patients were obviously lower than those in the RA patients, while the erythroid megaloblastic variability rate (90%) in MA group was obviously higher than that in RA patients (10%) and with statistically significant difference (P<0.05). The percentage of immature red blood cells was similar between MA group (53.33%) and RA group (60.00%), without significant difference (P>0.05).</p><p><b>CONCLUSION</b>Most of clinical manifestations and peripheral blood smear results are consistent in MA patients and RA patients, bone marrow morphology detection in RA group should be focused on lymphocytoid micromegakaryocytes, while the erythroid megaloblastic cell body is the focus in MA group, PAS can be used as a diagnostic criteria.</p>


Asunto(s)
Humanos , Anemia Megaloblástica , Diagnóstico , Anemia Refractaria , Diagnóstico , Médula Ósea , Patología , Diagnóstico Diferencial , Recuento de Eritrocitos , Recuento de Leucocitos , Megacariocitos , Biología Celular
12.
Chinese Journal of Hematology ; (12): 297-301, 2015.
Artículo en Chino | WPRIM | ID: wpr-282047

RESUMEN

<p><b>OBJECTIVE</b>To investigate the relationship between cytogenetic markers with World Health Organization (WHO) classification, disease progress and prognosis in cases with primary myelodysplastic syndromes (MDS).</p><p><b>METHODS</b>298 patients with de novo MDS from the first affiliated hospital of medical school, Zhejiang University were enrolled in the retrospective analysis of WHO classification, karyotype, and prognosis. Follow-up study was also conducted.</p><p><b>RESULTS</b>The WHO classifications at first diagnosis were as follows: refractory cytopenia with unilineage dysplasia (RCUD), 18 cases; refractory anemia with ring sideroblasts (RARS), 8 cases; refractory cytopenia with multiline dysplasia (RCMD), 104 cases; refractory anemia with excess blasts-1, 76 cases; refractory anemia with excess blasts-2, 85 cases; MDS unclassified (MDS-U), 5 cases involved; and single del (5q), 2 cases. 39.6% of MDS patients carried karyotypic abnormalities. Among them, the frequency of numerical abnormalities, structural abnormalities and the existence of composite abnormalities were 45, 31, and 42, respectively. The composite abnormalities were unbalanced translocations and complex chromosomal abnormalities. The incidence of both karyotypic abnormalities and complex chromosomal abnormalities in RAEB group was higher than that in non-RAEB group (P<0. 05). An analysis based on IPSS-R Scoring System showed that advanced risk stratification (except the low-risk group) gradually enhanced the incidence of karyotypic abnormalities (P<0.05). In addition, the probability of evolution to leukemia increased with the higher IPSS-R score (P<0.05). In RAEB group, the cases with +8 chromosome, accounting for 19.5% of karyotypic abnormalities, had worse prognosis than those with normal chromosomes.</p><p><b>CONCLUSION</b>Karyotype was identified with an independent risk factor in MDS patients. Therefore, the information on cytogenetic analysis was critical for diagnosis, prognosis and individual treatment. MDS patients presenting+8 chromosome, an intermediate risk factor, were associated with a poorer outcome compared to cases with normal chromosomes in RAEB group.</p>


Asunto(s)
Humanos , Cariotipo Anormal , Anemia Refractaria , Aberraciones Cromosómicas , Cromosomas Humanos Par 8 , Estudios de Seguimiento , Cariotipificación , Síndromes Mielodisplásicos , Pronóstico , Estudios Retrospectivos , Factores de Riesgo , Organización Mundial de la Salud
13.
Biosci. j. (Online) ; 30(4): 1269-1277, july/aug. 2014. tab
Artículo en Portugués | LILACS | ID: biblio-967573

RESUMEN

Estudos epidemiológicos sobre Síndromes Mielodisplásicas (SMD) não são encontrados na literatura brasileira, o que requer investigação dessa doença prevalente em idosos e com incidência maior com o aumento da idade. Esse trabalho objetivou investigar o perfil sociodemográfico e clínico dos pacientes portadores de SMD. Tratase de um corte transversal, desenvolvido no Rio Grande do Norte, realizado de janeiro de 2000 a dezembro de 2010. Para análise descritiva foi utilizado o programa Epi Info 2002, versão 3.5.2. Os cálculos da probabilidade de associação entre as características analisadas e o gênero foram realizados pelos Testes do qui-quadrado, de Fisher e Exato de Fisher. O nível de significância considerado foi de 0,05. O trabalho foi aprovado em seus aspectos éticos e metodológico pelo Comitê de Ética em Pesquisa CEP/HUOL protocolo 432/10. Dos 29 pacientes selecionados, houve predomínio de idosos, do sexo masculino, com baixa escolaridade, que apresentaram anemia como sintoma inicial. A maior parte foi de pessoas de pele branca, residentes em casa própria, moradores em zona urbana e com renda inferior a dois salários mínimos. Todos utilizaram terapia com hemoderivados, principalmente o concentrado de hemácias, numa frequência de quatro ou mais unidades por mês de consumo, sendo que 20% realizou dosagem de ferritina sérica, todos com valores acima do normal referenciado. Conclui-se que se faz necessário a realização de pesquisas com maiores populações, de caráter multicêntrico a fim de melhor evidenciamento dos dados sociodemográficos e clínicos com possibilidade de avaliação por regiões do país.


Epidemiological studies on Myelodysplastic Syndromes (MDS) are not found in Brazilian literature, which requires investigation of this prevalent disease in the elderly and higher incidence with increasing age. This study aimed to investigate the sociodemographic and clinical characteristics of patients with MDS to characterize this population at a referral center for high complexity. It is a cross-performed from January 2000 to December 2010. For descriptive analysis was conducted using Epi Info 2002, version 3.5.2. The calculations of the likelihood of association between the characteristics analyzed and gender were performed using the chi-square, Fisher and Fisher's Exact. The level of significance was 0.05. The study was approved in its ethical aspects and the methodological Ethics Committee in Research ECR/HUOL Protocol 432/10. We selected 29 patients. The sample was characterized mainly by elderly male with lower education, who had anemia as initial symptom. Most were white-skinned people living in their own homes, residents in urban areas and with income less than two minimum wages. All blood products used therapy, especially red blood cells, a frequency of four or more units per month of consumption. Only 20% performed dosage of serum ferritin, all with values referenced above normal. We conclude that it is necessary to conduct research with larger populations, multicenter character in order to best evidence on the demographic data and clinical evaluation with the possibility of the country.


Asunto(s)
Preleucemia , Síndromes Mielodisplásicos , Anemia Refractaria , Dinámica Poblacional , Epidemiología
16.
s.l; CONITEC; [2014].
No convencional en Portugués | LILACS, BRISA | ID: biblio-875102

RESUMEN

A SÍNDROME: Síndrome mielodisplásica ou mielodisplasia (MDS, sigla em inglês) é uma desordem da célula-tronco hematopoiética caracterizada pela displasia em uma ou mais linhagens e pela hematopoese ineficaz. O resultado é uma pancitopenia (diminuição global de todos os elementos do sangue ­ hemácias, leucócitos e plaquetas) levando à anemia dependente de transfusões e a um aumento do risco de infecções ou hemorragia, além do aumento do risco de desenvolver leucemia mieloide aguda refratária com excesso de blastos-1 (RAEB-1), anemia refratária com excesso de blastos-2 (RAEB-2), citopenia refratária com displasia multilinhagem (CRDM), síndrome mielodisplásica não-classificada e mielodisplasia associada com a deleção isolada (5q). Além destes, a leucemia mielomonocítica crônica (CMML) e a leucemia mielomonocítica juvenil são tipos de cânceres de sangue que a Organização Mundial da Saúde (OMS) classifica como "doenças mielodisplásicas/mieloproliferativas mistas". Os diferentes tipos de cânceres de sangue possuem manifestações diferentes e também exibem diferenças no prognóstico e na mortalidade. TRATAMENTO: As opções terapêuticas para MDS incluem: cuidados de suporte, terapia de baixa intensidade e terapia de alta intensidade. Os cuidados de suporte requerem transfusões de células vermelhas do sangue ou transfusões plaquetárias para trombocitopenia grave ou hemorragia trombocitopênica. A talidomida está sendo proposta como uma opção terapêutica de baixa intensidade no tratamento da Síndrome Mielodisplásica (CID: D46.0 ­ Anemia refratária sem sideroblastos, D46.1 ­ Anemia refratária com sideroblastos e D46.4 ­ Anemia refratária NE ­ não especificada) para os pacientes refratários à eritropoetina. A TECNOLOGIA: A talidomida é um derivado do ácido glutâmico e estruturalmente contém dois anéis amida e um único centro quiral. Este composto existe na forma de mistura equivalente dos isômeros S(-) e R(-) que se interconvertem rapidamente em condições fisiológicas. O enantiômero S está relacionado com os efeitos teratogênicos da talidomida, enquanto o enantiômero R é responsável pelas propriedades sedativas do fármaco. EVIDÊNCIAS CIENTÍFICAS: Foram priorizados, entre todos os artigos publicados até a data da busca: 1) ensaios clínicos randomizados, revisões sistemáticas, meta-análises, estudos multicêntricos e ensaios clínicos, 2) nas línguas portuguesa, inglesa ou espanhola, 3) que avaliaram a Mielodisplasia ou a Síndrome Mielodisplásica e 4) com desfechos clínicos de eficácia: avaliação da resposta hematológica e tolerância à droga. Entretanto, não foram encontrados estudos com boa qualidade metodológica e bom grau de recomendação, sendo o estudo de Fase II de Bouscary e colaboradores (2005) e o estudo Fase II de Moreno-Aspitia e colaboradores (2006) os que apresentaram melhor qualidade. Os estudos de Fase II, também chamados de estudos de avaliação de dose, são fundamentais para se avaliar uma nova indicação médica para a talidomida, pois o medicamento não foi desenvolvido para esta finalidade e há a incerteza quanto às doses que favorecem a melhor resposta clínica livre de eventos ou com eventos adversos menores e toleráveis. CONCLUSÕES: A evidência atualmente disponível sobre eficácia e segurança da talidomida para tratamento da Síndrome Mielodisplásica ou Mielodisplasia é baseada em estudos de Fase II de estabelecimento de doses e séries de casos, com qualidade média, produzida por equipes de pesquisadores diversas. Neste sentido, os resultados apresentados pelos estudos sugerem uma recomendação fraca a favor do medicamento. DECISÃO: PORTARIA SCTIE-MS N.º 45, de 16 de DEZEMBRO de 2014 - Torna pública a decisão de ampliar o uso da talidomida para tratamento da síndrome mielodisplásica no âmbito do Sistema Único de Saúde ­ SUS.


Asunto(s)
Humanos , Talidomida/administración & dosificación , Síndromes Mielodisplásicos/tratamiento farmacológico , Sistema Único de Salud , Anemia Refractaria , Brasil , Análisis Costo-Beneficio
17.
Laboratory Medicine Online ; : 110-114, 2013.
Artículo en Coreano | WPRIM | ID: wpr-82591

RESUMEN

Translocation between chromosomes 1 and 19 is well documented in ALL. Here, we report a case of refractory anemia with ring sideroblasts associated with marked thrombocytosis with der(19)t(1;19). A 67-yr-old man was admitted to our hospital with anemia and thrombocytosis. The aspirated bone marrow showed erythroid and megakaryocytic hyperplasia and dyspoiesis. Iron staining showed that the ring sideroblasts increased in number. Bone-marrow cell karyotyping showed 46,XY,der(19)t(1;19)(q23;p13)[9]/46,XY,del(5)(q21)[2]/46,XY[9]. PCR analysis showed the absence of the TCF3-PBX1 rearrangement. The patient was treated with hydroxyurea and intermittent blood transfusion. It is known that t(1;19)(q23;p13) leads to a TCF3-PBX1 fusion gene, whose product is a powerful transcriptional activator that plays a key role in the development of ALL. However, t(1;19) has rarely been reported in myeloid neoplasms and the TCF3-PBX1 fusion gene has not been detected. This implies that other genes might be involved in the TCF3-PBX1 rearrangement, or an alternative TCF3-PBX1 fusion transcript with a different breakpoint has not been detected to date. Further research and case studies, including the use of molecular analysis techniques, are required to evaluate the clinical and prognostic significance of t(1;19) in the development of myeloid neoplasms.


Asunto(s)
Humanos , Anemia , Anemia Refractaria , Transfusión Sanguínea , Médula Ósea , Hidroxiurea , Hiperplasia , Hierro , Cariotipificación , Reacción en Cadena de la Polimerasa , Trombocitosis
18.
Journal of Laboratory Medicine and Quality Assurance ; : 1-7, 2013.
Artículo en Coreano | WPRIM | ID: wpr-225314

RESUMEN

BACKGROUND: Hepcidin plays a central role in the regulation of iron metabolism, and hepatic iron production is stimulated by iron load and inflammation. Recent animal studies have shown that hepcidin levels increase when hematopoiesis is blocked. We aimed to monitor pre- and post-stem cell transplantation hepcidin levels and evaluate its association with hematologic recovery. METHODS: The study group comprised 12 patients with hematologic malignancies (7 with AML, 4 with ALL, and 1 with refractory anemia with excess blasts-2) undergoing allogeneic peripheral blood stem cell transplantation (PBSCT). One day before and 3 days, 1 week, 2 weeks, 4 weeks, and 8 weeks after PBSCT, reticulocyte count and levels of Hb, ferritin, and C-reactive protein were monitored; serum hepcidin-25 was measured by ELISA. RESULTS: The median serum hepcidin-25 levels (ng/mL) were significantly higher until 1 week after PBSCT (103.6, 103.3, and 96.5) than those at 2, 4, and 8 weeks after PBSCT (63.9, 53.9, and 56.6, respectively). The reticulocyte count also significantly increased from 2 weeks after PBSCT. The hepcidin level showed an inverse correlation with reticulocyte count (r=-0.56, P or =63.9) tended to demonstrate lower Hb recovery at 8 weeks than patients with low hepcidin levels did (P=0.15), but without any differences in the incidence of complications. CONCLUSIONS: These findings indicate that hepcidin production is associated with erythropoietic activity and that hepcidin level may be used as an early marker of hematopoietic recovery in PBSCT.


Asunto(s)
Animales , Humanos , Anemia Refractaria , Péptidos Catiónicos Antimicrobianos , Proteína C-Reactiva , Trasplante de Células , Ferritinas , Neoplasias Hematológicas , Hematopoyesis , Incidencia , Inflamación , Hierro , Compuestos Organotiofosforados , Trasplante de Células Madre de Sangre Periférica , Recuento de Reticulocitos , Trasplante de Células Madre , Trasplantes
19.
Korean Journal of Blood Transfusion ; : 78-83, 2012.
Artículo en Inglés | WPRIM | ID: wpr-76690

RESUMEN

Antiglobulin test-negative hemolytic anemia, thrombophilia, and marrow failure, such as aplastic anemia and myelodysplastic syndrome - refractory anemia (MDS-RA), are the primary clinical manifestations of paroxysmal nocturnal hemoglobinuria (PNH). Here, we report on a case of a 56-year-old male patient diagnosed with PNH, MDS-RA, and immune hemolytic anemia (IHA). The patient was transferred to the hospital with an impression of hemolytic anemia and pulmonary embolism. Positive results were observed on direct and indirect antiglobulin tests, and alloantibody, anti-C and anti-e, autoantibodies were identified. In addition, C and e antigens were found in Rh subgrouping. Therefore, due to the presence of autoantibodies against C and e antigens, we assumed that the cause of IHA was autoimmune reaction. Spherocytosis, increased osmotic fragility test, and positivity on direct and indirect antiglobulin tests were not considered characteristics of PNH. Therefore, without the presence of pulmonary embolism and MDS-RA, it is possible that autoimmune hemolytic anemia was considered the only reason for the hemolytic anemia, and that PNH could be overlooked. In patients with PH, use of washed RBCs during transfusion is not necessary. PNH screening test is recommended for patients who have experienced a thromboembolic event and intravascular hemolysis or MDS-RA. In order to obtain accurate information regarding the percentage of GPI-AP-deficient RBCs, flow cytometric analysis should be performed prior to transfusion.


Asunto(s)
Humanos , Masculino , Persona de Mediana Edad , Anemia Aplásica , Anemia Hemolítica , Anemia Hemolítica Autoinmune , Anemia Refractaria , Autoanticuerpos , Médula Ósea , Prueba de Coombs , Hemoglobinuria Paroxística , Hemólisis , Antígenos e de la Hepatitis B , Concentración de Iones de Hidrógeno , Tamizaje Masivo , Síndromes Mielodisplásicos , Fragilidad Osmótica , Embolia Pulmonar , Trombofilia
20.
Pediatric Gastroenterology, Hepatology & Nutrition ; : 183-187, 2012.
Artículo en Inglés | WPRIM | ID: wpr-207208

RESUMEN

Collagenous gastritis (CG) is a rare disorder that is characterized by the presence of a thick subepithelial collagen band with multiple infiltrated inflammatory cells of the gastric mucosa. CG is divided into two major subsets: first, in children and young adults presenting with severe anemia and abdominal colic pain (pediatric-type CG); and second, in adult patients with chronic watery diarrhea associated with collagenous colitis (adult-type CG). We report two cases of pediatric-type CG, each presenting with refractory anemia and chronic diarrhea.


Asunto(s)
Adulto , Niño , Humanos , Adulto Joven , Anemia , Anemia Ferropénica , Anemia Refractaria , Cólico , Colitis Colagenosa , Colágeno , Diarrea , Mucosa Gástrica , Gastritis , Hierro
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