Acute myeloid leukemia (AML) is a rare type of childhood acute leukemia, which has a worse prognosis than childhood acute lymphoblastic leukemia. Over the past decade, significant progress has been made in the treatment of childhood AML and the 5-year event-free survival rate may be as high as 70% in developed countries. This survival improvement is largely attributable to risk-stratified treatments, therapies tailored to individual patients based on the biological characteristics of the disease, and continuously improving supportive care. An accurate diagnosis is the prerequisite for risk stratification, prognostic evaluation and therapeutic decision making. How to reduce early mortality and thus improve overall survival, how to implement appropriate supportive treatment to reduce treatment-associated complications, and how to reduce treatment-related mortality are the key to the improvement of therapies for childhood acute myeloid leukemia.
Child ; Humans ; Leukemia, Myeloid, Acute ; diagnosis ; drug therapy ; genetics ; mortality ; Leukemia, Promyelocytic, Acute ; drug therapy ; genetics

Early diagnosis of acute promyelocytic leukemia (APL) depends primarily on morphological recognition before the presence of t(15;17) or PML-RAR gene rearrangement is confirmed. But the diagnosis is difficult to be made, if typical APL morphologic features are not found. Here, we describe a 32- year old man who had been diagnosed as APL. He relapsed with AML M1 like phenotype, lacking the typical features of APL. At relapse, t(15;17) and PML-RAR alpha gene rearrangement were detected. After 14 days of chemotherapy and all-trans retinoic acid, the phenotype changed from the AML M1 like features to the typical hypergranular APL. Awareness of atypical morphologic subtypes found in APL is important. And identification of t(15;17) or PML/RAR alpha rearrangement will be helpful in diagnosis of atypical APL.
Diagnosis ; Drug Therapy ; Early Diagnosis ; Gene Rearrangement ; Leukemia, Promyelocytic, Acute* ; Phenotype ; Recurrence* ; Tretinoin

We describe a patient with acute promyelocytic leukemia (APL) with no detectable cytogenetic abnormality of either chromosomes 15 or 17 who nevertheless had juxtaposition of promyelocytic leukemia (PML) and retinoic acid receptor-alpha (RARA) and expressed a chimeric transcript. Conventional cytogenetics showed the 46, XX. The metaphase fluorescence in situ hybridization (FISH) with a 5' PML and 3' RARA probe showed a juxtaposed PML-RARA fusion signal on one chromosome 17 homologue, an RARA signals on the other chromosome 17 homologue, and one PML signal on each chromosome 15 homologue. Our patient is presently in remission and doing well after chemotherapy with idarubicin and all trans retinoic acid (ATRA) treatment. Our results show that APL patients with cytogenetically normal chromosome 15 and 17 may, nevertheless, have involvement of both PML and RARA genes and as the prognostic outcome in APL is associated with the presence of a PML-RARA fusion, it is necessary to perform RT-PCR or FISH to aid diagnosis.
Chromosome Aberrations ; Chromosomes, Human, Pair 15* ; Chromosomes, Human, Pair 17* ; Cytogenetics ; Diagnosis ; Drug Therapy ; Fluorescence ; Humans ; Idarubicin ; In Situ Hybridization ; Leukemia ; Leukemia, Promyelocytic, Acute* ; Metaphase ; Tretinoin

All-trans retinoic acid (ATRA) therapy induces complete remission in acute promyelocytic leukemia (APL) via differentiation of the APL cells. Recent clinical trials in China and United states showed that arsenic trioxide (ATO) is an effective and relatively safe drug in the treatment of APL. The patient was 29-year-old woman with APL who had been treated heavily with allogeneic bone marrow transplantation (BMT) in 4 years ago and reinduction chemotherapy plus donor lymphocyte infusion (DLI) after relapse in 2 years ago. After diagnosis for relapse, she had been treated with ATRA, but unfortunately failed. She was treated with ATO at the dose of 10 mg/day intravenously for 6 weeks. Complete remission was achieved at 3 weeks and the cumulative dose of ATO during induction was 420mg. She had complete remission without severe adverse effects except mild impairment of liver function and is following up for 6 months. We report a case of remission induction by ATO in ATRA refractory APL patient who experienced multiple relapse after allogeneic BMT, chemotherapy and DLI.
Adult ; Arsenic* ; Bone Marrow Transplantation* ; Bone Marrow* ; China ; Diagnosis ; Drug Therapy ; Female ; Humans ; Leukemia, Promyelocytic, Acute* ; Liver ; Lymphocytes ; Recurrence ; Remission Induction* ; Tissue Donors ; Tretinoin ; United States

No abstract available.
Antineoplastic Agents/*adverse effects/*therapeutic use ; Bone Marrow Cells/metabolism ; Humans ; Karyotyping ; Leukemia, Myeloid, Acute/*chemically induced/*diagnosis/genetics ; Leukemia, Promyelocytic, Acute/*drug therapy ; Male ; Middle Aged ; Oncogene Proteins, Fusion/genetics ; Remission Induction ; Tretinoin/therapeutic use

All-trans retinoic acid (ATRA) is the standard induction treatment for acute promyelocytic leukemia (APL). Quite many ATRA-related side effects, including retinoic acid syndrome, were reported. So far, it has rarely been reported that Sweet's syndrome, characterized by fever, neutrophilia, painful erythematous cutaneous plaques, dense dermal infiltrates of mature neutrophils and rapid response to steroid therapy, is associated with ATRA. In the case that Sweet's syndrome associated with ATRA is found, physicians will have to face a great challenge over the possibility of infectious conditions. We present here a case of Sweet's syndrome associated with ATRA. A 35-year-old female with APL developed fever, painful erythematous cutaneous plaques on both cheeks, right wrist and both shins during induction chemotherapy with ATRA. A skin biopsy revealed a dense dermal infiltrate, consisting of mature neutrophils without vasculitis or cutaneous immunoglobulin deposits, which is compatible with Sweet's syndrome. Oral prednisone was administered and the lesions started to improve within 48 hours
Adult ; Biopsy, Needle ; Case Report ; Female ; Follow-Up Studies ; Human ; Leukemia, Promyelocytic, Acute/diagnosis/*drug therapy ; Prednisone/administration & dosage ; Risk Assessment ; Sweet's Syndrome/*chemically induced/drug therapy/*pathology ; Tretinoin/*adverse effects/therapeutic use

BACKGROUND: To see whether there has been improvement in the survival of patients with acute leukemia over the last 14 years, a retrospective analysis was performed. METHODS: Clinical and laboratory data were obtained form the medical records. Patient survival data was obtained from the hospital records, national cancer registry or by direct phone contacts. RESULTS: Between June, 1989 and August 2002, 714 adult patients were diagnosed with acute leukemia at Asan Medical Center in Seoul. Fourteen patients were lost to follow-up within 100 days of the diagnosis and excluded. There were 535 patients with acute myelogenous leukemia (AML) and 165 with acute lymphoblastic leukemia (ALL). There were 65 patients with acute promyelocytic leukemia (APL) among 535 patients with AML. Patients with non-APL AML and ALL were divided into 3 cohorts according to the year of the diagnosis: cohort I, 1989~1994; cohort II, 1995~1998; cohort III, 1999~2002. Patients with APL were also divided into 3 cohorts: cohort I, pre-all-transretinoic acid (ATRA) period (1989~1994. 2); cohort II, ATRA with or without chemotherapy (1994. 3~2000. 8); and cohort III, ATRA plus idarubicin (2000. 9~2002). Univariate analysis showed significant improvement in patient survival in non-APL AML (4-year projected survival rates of 10%, 19%, and 33% for cohorts I, II, and III, respectively, P=0.0000), in ALL (27%, 28%, and 52%, P=0.03), and in APL (36%, 56%, and 80%, P=0.04). Multivariate analysis showed that the year of diagnosis was a significant independent variable for patient survival in non-APL AML and ALL. CONCLUSION: Our study showed significant survival improvement in acute leukemia over the last 14 years. This improvement is not likely due to change in patient demographics. Rather, it is likely that introduction of newer methods of treatment of acute leukemia, such as multi-cycle combination chemotherapy for ALL, high dose cytarabine consolidation for AML, ATRA for APL, and wider application of allogeneic hematopoietic cell transplantation, has resulted in a better patient survival.
Adult ; Cell Transplantation ; Chungcheongnam-do ; Cohort Studies ; Cytarabine ; Demography ; Diagnosis ; Drug Therapy ; Drug Therapy, Combination ; Hospital Records ; Humans ; Idarubicin ; Leukemia* ; Leukemia, Myeloid, Acute ; Leukemia, Promyelocytic, Acute ; Lost to Follow-Up ; Medical Records ; Multivariate Analysis ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Retrospective Studies ; Seoul ; Survival Rate ; Transplants

Anaphylactic transfusion reactions are rare complications of blood transfusions. Anhaptoglobinemia, a condition that has high incidence in Asia, can cause allergic transfusion reactions or anaphylaxis in severe cases. A 50-yr-old Korean woman was diagnosed with relapsed acute promyelocytic leukemia. She developed thrombocytopenia during chemotherapy and an anaphylactic transfusion reaction on the 4th and 5th platelet transfusions immediately after the transfusion of the platelet concentrates was initiated. Blood analysis showed no detectable serum haptoglobin. We examined her genetic phenotype and detected anhaptoglobinemia, which occurs because of an allelic deletion in the Hp gene cluster. The presence of an antibody against haptoglobin was detected by performing ELISA. To prevent anaphylactic reactions, apheresis platelets were transfused after washing. Consequently, anaphylactic transfusion reactions did not develop. Here, we report the first case of anhaptoglobinemia causing anaphylactic transfusion reaction in Korea.
Alleles ; Anaphylaxis/*etiology ; Antineoplastic Agents/therapeutic use ; Female ; Gene Deletion ; Haptoglobins/*genetics/immunology ; Humans ; Isoantibodies/immunology ; Leukemia, Promyelocytic, Acute/complications/*diagnosis/drug therapy ; Middle Aged ; Phenotype ; Platelet Transfusion/*adverse effects ; Recurrence ; Republic of Korea ; Thrombocytopenia/complications/diagnosis

The early molecular kinetics during all-trans retinoic acid (ATRA) plus arsenic-based induction therapy and its prognostic value for acute promyelocytic leukemia (APL) remain unclear. This study was purposed to investigate the molecular and cytogenetic kinetics and its clinical significance in treatment of APL with ATRA plus arsenic-based induction. The molecular and cytogenetic kinetics was assessed by real-time quantitative RT-PCR and interphase fluorescence in situ hybridization (FISH) in 32 newly diagnosed APL patients. The results showed that the median PML-RARα transcript levels (PML-RARα/ABL) were very significantly up-regulated at 14 days of induction therapy compared with that of pre-treatment (40.10% vs 57.74%, P < 0.01), and then decreased at 28 days of induction therapy and at the end of consolidation therapy (6.97% and 0%), respectively. The total of 65.62% and 31.25% patients showed up-regulation of PML-RARα transcript at 14 and 28 days after induction, as compared with pretreatment. The PML-RARα copies per APL cell before treatment, and at 14 and 28 days after induction were calculated as 0.9, 2.2, 1.4 by the formula of PML-RARA/ABL(%)×2/APL cells (%). With the median follow-up time of 22 months, 32 patients were still in continuous clinical remission and no molecular relapse occurred. Up-regulation of PML-RARa expression during the induction had no effect on outcomes of APL patients. It is concluded that up-regulation of PML-RARa expression is a common event during induction therapy with ATRA plus arsenics. Up-regulation of PML-RARa expression during induction therapy hasn't influenced the long-term prognosis of APL.
Adolescent ; Adult ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Arsenicals ; administration & dosage ; Female ; Humans ; Leukemia, Promyelocytic, Acute ; diagnosis ; drug therapy ; metabolism ; Male ; Middle Aged ; Oncogene Proteins, Fusion ; metabolism ; Prognosis ; Tretinoin ; administration & dosage ; Up-Regulation ; drug effects ; Young Adult

In general, a 2-yr disease-free duration is recommended before kidney transplantation (KT) in end-stage renal disease (ESRD) patients who also have acute leukemia. However, the optimal disease-free interval has not been specified for all subtypes of acute leukemia. Among these subtypes, acute promyelocytic leukemia (APL) shows a favorable prognosis and low relapse rate compared to other types of leukemia. We here report KT after complete remission (CR) of APL in an ESRD patient. Irreversible kidney injury developed in a 23-yr-old man with APL. First, we induced CR and subsequently performed KT 7 months after the achievement of CR. The patient's clinical course after KT was favorable, without allograft rejection or relapse of APL up to1 yr after KT. On the basis of our clinical experience, it is suggested that a long wait may not be necessary before KT in patients with ESRD and APL.
Adult ; Antineoplastic Agents/therapeutic use ; Arsenicals/therapeutic use ; Bone Marrow Cells/pathology ; Humans ; Kidney Failure, Chronic/*therapy/ultrasonography ; *Kidney Transplantation ; Leukemia, Promyelocytic, Acute/*diagnosis/drug therapy ; Male ; Oxides/therapeutic use ; Receptors, Retinoic Acid/genetics/metabolism ; Remission Induction