• Title/Summary/Keyword: 골수형성이상증후군

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Diagnosis and Treatment of Myelodysplastic Syndrome in the Era of Genetic Testing (유전자 검사 시대 골수형성이상증후군의 진단과 치료)

  • Junshik Hong
    • The Korean Journal of Medicine
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    • v.99 no.1
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    • pp.11-16
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    • 2024
  • Myelodysplastic syndrome (MDS) is a heterogeneous disorder with diverse prognoses influenced by cytopenias, genetic variants, and myeloblast proportions in the bone marrow. Accurate prognosis prediction and tailored treatment plans are essential. The International Prognostic Scoring System-Molecular (IPSS-M), which additionally reflects the impact of MDS-related genetic mutations to the clinical and laboratory information, is anticipated to offer superior prognostic accuracy compared to existing systems like the Revised International Prognostic Scoring System (IPSS-R). Despite its statistical complexity, its web-based calculation and ease of discussing results with patients using intuitive data sets provide notable advantages. Progress in MDS treatment, exemplified by effective anemia correction with an erythropoiesis-maturation agent in SF3B1-mutated cases and efforts to refine poor prognoses in TP53-mutated cases, reflects the evolving landscape of genetic-based interventions in MDS. Advancements in genetic diagnostic technology, combined with enhanced knowledge of the bone marrow niche, are anticipated to lead to significant improvement in MDS treatment outcomes in the future.

Analysis of Prenatal and Postnatal Factors Associated with Complications and Prognosis in Premature Infants with Leukemoid Reaction (백혈병양반응을 보인 미숙아의 합병증과 예후에 관한 산전과 산후 인자에 대한 분석)

  • Kwon, Se-Ho;Lee, Byoung-Kook;Lee, Heun-Ji;Na, So-Young;Lee, Jung-Ha;Park, Su-Yeon;Kang, Eun-Kyeung;Kim, Do-Hyeon;Lee, Kwan;Kim, Hee-Sup
    • Neonatal Medicine
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    • v.15 no.2
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    • pp.151-159
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    • 2008
  • Purpose : This study determined the prenatal and postnatal factors associated with complications and prognosis in premature infants with leukemoid reaction. Methods : We retrospectively reviewed the medical records of premature infants with gestational ages <37 weeks and low birth weights (<2,500 g) who were admitted immediately after birth to the neonatal intensive care unit at the Dongguk University Ilsan Hospital between June 2005 and July 2006. A leukemoid reaction was defined as an absolute neutrophil count (ANC) >30,000/$mm^3$. The infants who had leukemoid reaction comprised the study group, while the remainder of infants made up the control group. The relationships between maternal and neonatal variables and ANC were studied. Results : Leukemoid reaction was detected in 3.1% of the study infants (8 of 252). Factors more frequently associated with infants with leukemoid reaction were as follows: maternal chorioamnionitis, high levels of maternal and infant C-reactive protein, gestational age <37 weeks, birth weight <2,500 g, low Apgar score, prolonged ventilator support, and a high incidence of bronchopulmonary dysplasia (BPD). However, there were no significant differences with respect to the antenatal usage of steroids, the incidences of patent ductus arteriosus, necrotizing enterocolitis, intraventricular hemorrhage, retinopathy of prematurity, and mortality between the two groups. Conclusion : Leukemoid reaction in premature infants was associated with chorioamnionitis and high levels of serum C-reactive protein in mothers and infants, and BPD in infants. These findings suggest that leukemoid reaction is secondary to inflammation caused by infection.

Continual Reassessment Method in Phase I Clinical Trials for Leukemia Patients (백혈병환자 대상의 제1상임상시험 연속재평가방법)

  • Lee, Joo-Hyoung;Song, Hae-Hiang
    • Communications for Statistical Applications and Methods
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    • v.18 no.5
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    • pp.581-594
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    • 2011
  • The traditional method of 3+3 standard design and model-based Bayesian continual reassessment method (CRM) are commonly used in Phase I clinical trials to identify the maximal tolerated dose(MTD) of a new drug. In this paper we review clinical examples of Phase I trials that were carried out in patients with refractory or relapsed leukemia and myelodysplastic syndrome. The recently proposed 3+1+1 design and rolling-6 design can shorten the trial duration, when a very slow accrual of patients with a simple 3+3 standard design may result in the untimely termination of trials. Too conservative approaches in determining the dose levels in Phase I clinical trials can leave clinical investigators unable to accurately determine the MTD. When determining future patient doses, the designs that use a time-to-event CRM can cooperate late toxicities by accounting for the proportion of the observation period of each enrolled patient. With the CRM design, simulations under different scenarios during the trial are important in detecting the under- or over-estimation of the initial estimate of the dose-limiting toxicity rate for each dose level. We present the advantages and drawbacks of the designs used in Phase I clinical trials for leukemia patients.

The Prognostic Indicies of Pneumocystis Carinii Pneumonia in Immunocompromised Patients other than Acquired Immune Deficiency Syndrome (비 AIDS 면역 결핍 환자들에서 발생한 주폐포자충 폐렴의 예후인자)

  • Park, Wann;Kim, Yoo-Kyum;Lee, Jin-Seong;Ahn, Jong-Jun;Hong, Sang-Bum;Shim, Tae-Sun;Lim, Chae-Man;Lee, Sang-Do;Kim, Woo-Sung;Kim, Dong-Soon;Kim, Won-Dong;Koh, Youn-Suck
    • Tuberculosis and Respiratory Diseases
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    • v.45 no.4
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    • pp.805-812
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    • 1998
  • Background: Among the variety of opportunistic infections, pneumonia comprises the major morbidity in immunocompromised patients. Pneumocystis carnii pneumonia (PCP) and cytomegalovirus (CMV) pneumonia are common infectious illness of immunocompromised hosts. Although there are many reports regarding to the co-infection of PCP and CMV diagnosed by bronchoalveolar lavage (BAL) fluid examination, the effects of CMV co-infection on the outcome of PCP is still controversial. The purpose of this investigation is to evaluate the effects of CMV detected by BAL fluid examination on the clinical course of PCP in the immunocompromised patients other than human immunodeficiency virus infection. Method: Ten patients with PCP were enrolled and retrospective analysis of their medical records were done. HIV infected persons were excluded. The PCP was diagnosed by BAL fluid examination with Calcofluor-White staining. CMV was detected in BAL fluid by Shell-vial culture system. Chest radiographic findings were reviewed. We used Fisher's exact test and Mann-Whitney U test for statistical analysis of data. Results: The underlying disorders of patients were idiopathic pulmonary fibrosis (n=1), renal transplantation (n=4), necrotizing vasculitis (n=l), systemic lupus erythematosus (n=1), brain tumor (n=1), chronic myelogenous leukemia (n=1), unidentified (n=1). There were no difference in clinical course, APACHE III score, arterial blood gas analysis, white blood cell count, lymphocyte count, serum albumin concentration, chest radiographic findings and mortality between patients with PCP alone (n=4) and those with CMV co-infection (n=6). Univariate analysis regarding to the factors that associated with mortality of PCP were revealed that the application of mechanical ventilation (p=0.028), the level of APACHE III score (p=0.018) and serum albumin concentration (p=0.048) were related to the mortality of patients with PCP. Conclusion: The clinical course of PCP patients co-infected by CMV were not different from PCP only patients. Instead, accompanied respiratory failure, high APACHE III score and poor nutritional status were associated with poor outcome of PCP.

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