Clinical features and outcome of children with first marrow relapse of acute lymphoblastic leukemia expressing BCR-ABL fusion transcripts. BFM Relapse Study Group.

نویسندگان

  • B Beyermann
  • A G Agthe
  • H P Adams
  • K Seeger
  • C Linderkamp
  • G Goetze
  • W D Ludwig
  • G Henze
چکیده

Although the Philadelphia chromosome (Ph1) has been identified as an adverse prognostic factor in acute lymphoblastic leukemia (ALL), little is known about the incidence and clinical course of relapsed Ph1-positive ALL in children. The incidence was determined by screening of 170 consecutive children with first bone marrow relapse of ALL using the reverse transcriptase-polymerase chain reaction (RT-PCR) and comparison, with cytogenetic analysis. Among these 170 children, 20 (12%) were found to be BCR-ABL-positive, representing a rate that is about three times higher than that reported for newly diagnosed ALL. Ten of the cases were identified by RT-PCR only. In none of the 21 patients with T-cell immunophenotypes could an expression of the BCR-ABL mRNA be detected. BCR-ABL positivity was associated with a significantly shorter duration of first remission (P = .0086) and higher white blood cell (P = .0157) and blast cell counts (P = .0304) at relapse diagnosis. All patients were treated according to the ALL-REZ BFM 87 and 90 relapse trials of the BFM Relapse Study Group. The intensive multiagent chemotherapy induced a second complete remission in only 60% of children with BCR-ABL-positive ALL compared with in 91% of those without BCR-ABL expression (P = .0023). The prognosis of BCR-ABL-positive ALL in children is poor, with a probability of event-free survival at 2 years of 8% versus 50% in those without BCR-ABL mRNA or cytogenetic analysis should become part of the routine diagnostic panel for children with newly diagnosed ALL and is fundamental for children presenting with an early bone marrow relapse.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Frequency of BCR-ABL Fusion Transcripts in Iranian Azeri Turkish patients with Chronic Myeloid Leukemia

Background: The Philadelphia chromosome (Ph) characterized by t (9; 22) (q34; q11.2) is a reciprocal translocation giving rise to a chimeric BCR-ABL fusion gene. Incidence of Ph chromosome is over 98% in Patients with Chronic Myeloid Leukemia (CML) and around 20% in acute lymphoblastic leukemia (ALL). The finding of this fusion gene is essential for diagnosis of CML by detection of various fusi...

متن کامل

Clinical Features and Outcome of Children With First Marrow Relapse of Acute Lymphoblastic Leukemia Expressing BCR-ABL Fusion Transcripts

http://bloodjournal.hematologylibrary.org/site/misc/rights.xhtml#repub_requests Information about reproducing this article in parts or in its entirety may be found online at: http://bloodjournal.hematologylibrary.org/site/misc/rights.xhtml#reprints Information about ordering reprints may be found online at: http://bloodjournal.hematologylibrary.org/site/subscriptions/index.xhtml Information abo...

متن کامل

Central nervous system relapse prophylaxis in acute lymphoblastic leukemia (ALL) intrathecal chemotherapy with and without cranial irradiation

Background: Central Nervous System (CNS) relapse in acute lymphoblastic leukemia was significantly decreased due to the use of new chemotherapyeutic agents, Intrathecal chemotherapy and cranial irradiation. The purpose of this study was to compare the effectiveness of intrathecal (IT) CNS chemotherapy alone versus combination of IT chemotherapy with cranial irradiation for prevention of CNS rel...

متن کامل

BCR-ABL fusion genes and laboratory findings in patients with chronic myeloid leukemia in northeast Iran

Background: A specific chromosomal abnormality, the Philadelphia chromosome (BCR-ABL fusion), is present in all patients with chronic myeloid leukemia (CML). The b2a2 and b3a2 fusion mRNAs encode p210 fusion protein p210 and e1a2 encode p190. The aim of this study was to evaluate the frequency of BCR-ABL fusion transcript variants in Northeast of Iranian CML patients and to compare the laborato...

متن کامل

Medium and High-Dose Methotrexate-Induced Toxicity in Children with Acute Lymphoblastic Leukemia

Background: Methotrexate has an important role in treatment of acute leukemia. We measured methotrexate level in CSF and blood of children with acute lymphoblastic leukemia to determine complications and outcomes. Materials and Methods: One hundred and twenty patients (73 male, 47 female), with mean age at diagnosis of 6.1 years (11mo_15years) entered the study. Patients were divided into two...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:
  • Blood

دوره 87 4  شماره 

صفحات  -

تاریخ انتشار 1996