DOCSLIB.ORG

Myelodysplastic Syndrome After Acute Promyelocytic Leukemia

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Myelodysplastic Syndrome After Acute Promyelocytic Leukemia Leukemia (2003) 17, 1600–1604 & 2003 Nature Publishing Group All rights reserved 0887-6924/03 $25.00 www.nature.com/leu Myelodysplastic syndrome after acute promyelocytic leukemia: the European APL group experience I Lobe1, F Rigal-Huguet2, A Vekhoff3, B Desablens4, D Bordessoule5, C Mounier6, A Ferrant7, M Sanz8, M Fey9, C Chomienne10, S Chevret11, L Degos12 and P Fenaux13 for the European APL group 1Service des Maladies du Sang, CHU Lille, France; 2Service d’He´matologie, CHU Toulouse, France; 3De´partement d’He´matologie et Oncologie Me´dicale, Hoˆpital Hoˆtel-Dieu, Paris, France; 4Service des Maladies du Sang, CHU Amiens, France; 5Service d’He´matologie Clinique, CHU Limoges, France; 6De´partement d’He´matologie, CHU Saint-Etienne, France; 7Service d’He´matologie, UCL Bruxelles, Belgium; 8Hospital Clinico de Valencia, Espagne; 9Medical Institute (INSEL), Bern, Switzerland; 10Laboratoire de Biologie Cellulaire He´matopoı¨e´tique, Hoˆpital Saint-Louis, Paris, France; 11DBIM, Hoˆpital Saint-Louis, Paris, France; 12Institut d’He´matologie, Hoˆpital Saint-Louis, Paris, France; and 13Service d’he´matologie clinique, Hoˆpital Avicenne, Bobigny, France With improved treatment of acute promyelocytic leukemia major emerging problem in the follow-up of APL. Those findings (APL) by all trans retinoic acid (ATRA) combined to anthracy- prompted us to review cases of MDS or AML (non-APL) cline–aracytin chemotherapy (CT), a larger number of those patients may be at risk of late complications. Recently, the occurring during the evolution of APL patients included in Rome group reported five cases of myelodysplastic syndrome multicenter trials of our European group. (MDS) or acute myeloid leukemia (AML, non-APL) occurring during the course of 77 APL patients (6.5%) in complete remission (CR). From 1991 to 1998, we treated 677 newly Patients and treatment diagnosed cases of APL, and 617 of them achieved CR with ATRA combined to CT (n ¼ 579) or CT alone (n ¼ 38); 246 of Patients them received subsequent maintenance CT with 6 mercapto- purine and methotrexate. With a median follow-up of 51 months, 6 patients (0.97%) developed MDS, 13–74 months after Between 1991 and 1998, we included 677 newly diagnosed the diagnosis of APL. In all six cases, t(15;17) and PML- cases of APL in APL 91 and APL 93 trials. Inclusion criteria were: RARalpha rearrangement were absent at the time of MDS diagnosis of APL based on morphological criteria and subse- diagnosis, and karyotype mainly showed complex cytogenetic quently confirmed by presence of t(15;17) or PML-RAR alpha abnormalities involving chromosomes 5 and/or 7, typical of gene rearrangement,8 age 75 years or less, and a minimum MDS observed after treatment with alkylating agents, although follow-up of 3 years. none of the six patients had received such agents for the treatment of APL. Our findings suggest that MDS can indeed be a long-term complication in APL, although probably at lower 9–11 incidence than that previously reported. Treatment Leukemia (2003) 17, 1600–1604. doi:10.1038/sj.leu.2403034 Keywords: acute promyelocytic leukemia-myelodysplastic APL 91 trial design: Patients were randomized to receive syndrome; therapy related chemotherapy alone (CT group) or ATRA followed by CT (ATRA group). In the CT group, patients received two successive courses of daunorubicin (DNR) 60 mg/m2/day for 3 days and AraC 200 mg/m2/day for 7 days (courses I and II). Those who Introduction achieved complete remission (CR) after course I received a final course of DNR 45 mg/m2/day for 3 days and AraC 1 g/m2 every Acute promyelocytic leukemia (APL) is a specific type of acute 12 h for 4 days (course III). Patients who achieved CR only after myeloid leukemia (AML) characterized by the morphology of course II received two cycles of course III. blast cells (M3 according to FAB classification), t(15;17) In the ATRA group, patients received ATRA 45 mg/m2/day translocation that fuses the PML gene on chromosome 15 to orally until CR, or for a maximum of 90 days. After CR the RAR alpha gene on chromosome 17, and by specific achievement, they received the same three CT courses as in the 1,2 coagulopathy. Anthracycline–aracytin (AraC) chemotherapy CT group. (CT), introduced in the 1960s, has yielded cure rates of 35–40% in APL, whereas combination of anthracycline–Ara C or APL 93 trial design: Induction treatment was stratified on anthracycline alone CT to all trans retinoic acid (ATRA), age and initial WBC. Patients aged less than 65 years with WBC introduced in the early 1990s, has improved this cure rate to 3 less than 5000/mm were randomized between ATRA followed 65–70%,3–6 thereby increasing the number of patients poten- by CT (ATRA-CT group) similar to the ATRA group of APL 91 tially at risk of late side effects of treatments. In the last 2 years, trial, and ATRA plus CT (ATRA+CT) differing from ATRA-CT several case reports of myelodysplastic syndrome (MDS) or AML by the fact that CT was started on day 3 of ATRA treatment. (different from APL), occurring during the course of APL, have 3 7 Patients with WBC45000/mm at presentation or aged 66 to been made. Recently, the Rome group reported five cases of 3 75 years and with WBC less than 5000/mm were not MDS in 77 (6.5%) APL patients treated with CT alone or ATRA randomized and received ATRA plus CT course I from day 1 combined to CT, suggesting that MDS/AML was possibly a and the same treatment as in the ATRA-CT group, respectively. Patients who achieved CR received two CT consolidation ´ Correspondence: Dr P Fenaux, Service d’hematologie clinique/ Paris courses (similar to courses II and III of APL 91 trial). The elderly XIII Universite´,Hoˆpital Avicenne 125 rue de Stalingrad, 93009 Bobigny, France; Fax :+33 0 140875487/0148955499; pierre.fe- group, however, only received course II. Patients were then [email protected] offered a second randomization testing maintenance treatment Received 11 February 2003; accepted 8 April 2003 with intermittent ATRA (45 mg/m2/day, 15 days every 3 months) Myelodysplastic syndrome after APL I Lobe et al 1601 and continuous CT with 6 mercaptopurine (6 MP, 90 mg/m2/day and 22, dup (12)(q12;q22), and patient no. 5 had monosomy 8 orally) and methotrexate (MTX, 15 mg/m2/week), both sched- and t(8;11)(q32;q21). At the time of MDS diagnosis, no t(15;17) uled for 2 years. was found, and PML-RARalpha was negative in five patients. In the remaining patient (patient no. 3), where the research of the Follow-up transcript was a failure, FISH analysis was negative for t(15;17). Three of the six patients progressed to AML (MoAML in all three cases) 1, 6 and 18 months after diagnosis of MDS. Patients were followed up until January 1st 2000 (sixth interim Treatment of MDS/AML was symptomatic in four patients and analysis) for APL 91 trial and January 1st 2002 (third interim consisted of intensive chemotherapy in the remaining two cases analysis) for APL 93 trial. MDS and AML were classified (nos. 1 and 6). Patient no. 1 did not achieve CR and died a few according to FAB group criteria.12 months later of progressive disease; patient no. 6 achieved CR and was receiving consolidation courses. Results After diagnosis of RA, one patient (no.2) relapsed from her APL. Karyotype showed both t(15;17) and rearrangements In total, 677 patients were included in APL 91 trial (n ¼ 101) and observed during the MDS phase. PML-RARalpha rearrangement APL 93 trial (n ¼ 576). Of these 617 of them achieved CR, after was present. The patient was treated by ATRA and mitoxantrone chemotherapy alone (n ¼ 38) or ATRA combined to or followed AraC. APL blasts, t(15;17) and PML-RARalpha rearrangement by chemotherapy (n ¼ 579). After consolidation chemotherapy, disappeared, but cytopenias, myelodysplastic features in bone 117, 76 and 129 of the complete remitters received main- marrow and cytogenetic rearrangements other than t(15;17) tenance treatment with 6MP+MTX, intermittent ATRA or both persisted, the diagnosis being still that of RA; she evolved 3 treatment modalities, respectively. The other patients received months later to RAEB and died of progressive disease. no maintenance treatment. With a median follow-up of 51 months (range: 39–118), six patients (0.97%) developed MDS or AML including one of the Discussion patients included in APL 91 trial and five of the patients included in APL 93 trial. The incidence of MDS/AML after APL we report, close to 1%, Clinical and hematological characteristics of the patients who was lower than in the Rome group experience, although the developed MDS or AML are shown in Table 1. Median age was follow-up was similar. 56.5 years (range: 52–73) at diagnosis of APL, and 59.5 years If one includes the five patients reported by the Rome group, (range: 57–76) at diagnosis of MDS/AML and there were two the six patients presented here and previous case reports, 22 females and four male patients. Five patients initially had cases of MDS or AML (other than APL) occurring during the classical APL and one had the microgranular variant APL. course of APL have been reported,14–25 and their characteristics Patient 1 had been previously reported.13 are shown in Table 2. Cytogenetic study at diagnosis of APL showed isolated Median age of those 22 patients at diagnosis of APL was 52 t(15;17) translocation in three patients. Patients nos.
Load more

Recommended publications
  • Updates in Mastocytosis
    Updates in Mastocytosis Tryptase PD-L1 Tracy I. George, M.D. Professor of Pathology 1 Disclosure: Tracy George, M.D. Research Support / Grants None Stock/Equity (any amount) None Consulting Blueprint Medicines Novartis Employment ARUP Laboratories Speakers Bureau / Honoraria None Other None Outline • Classification • Advanced mastocytosis • A case report • Clinical trials • Other potential therapies Outline • Classification • Advanced mastocytosis • A case report • Clinical trials • Other potential therapies Mastocytosis symposium and consensus meeting on classification and diagnostic criteria for mastocytosis Boston, October 25-28, 2012 2008 WHO Classification Scheme for Myeloid Neoplasms Acute Myeloid Leukemia Chronic Myelomonocytic Leukemia Atypical Chronic Myeloid Leukemia Juvenile Myelomonocytic Leukemia Myelodysplastic Syndromes MDS/MPN, unclassifiable Chronic Myelogenous Leukemia MDS/MPN Polycythemia Vera Essential Thrombocythemia Primary Myelofibrosis Myeloproliferative Neoplasms Chronic Neutrophilic Leukemia Chronic Eosinophilic Leukemia, NOS Hypereosinophilic Syndrome Mast Cell Disease MPNs, unclassifiable Myeloid or lymphoid neoplasms Myeloid neoplasms associated with PDGFRA rearrangement associated with eosinophilia and Myeloid neoplasms associated with PDGFRB abnormalities of PDGFRA, rearrangement PDGFRB, or FGFR1 Myeloid neoplasms associated with FGFR1 rearrangement (EMS) 2017 WHO Classification Scheme for Myeloid Neoplasms Chronic Myelomonocytic Leukemia Acute Myeloid Leukemia Atypical Chronic Myeloid Leukemia Juvenile Myelomonocytic
    [Show full text]
  • The Clinical Management of Chronic Myelomonocytic Leukemia Eric Padron, MD, Rami Komrokji, and Alan F
    The Clinical Management of Chronic Myelomonocytic Leukemia Eric Padron, MD, Rami Komrokji, and Alan F. List, MD Dr Padron is an assistant member, Dr Abstract: Chronic myelomonocytic leukemia (CMML) is an Komrokji is an associate member, and Dr aggressive malignancy characterized by peripheral monocytosis List is a senior member in the Department and ineffective hematopoiesis. It has been historically classified of Malignant Hematology at the H. Lee as a subtype of the myelodysplastic syndromes (MDSs) but was Moffitt Cancer Center & Research Institute in Tampa, Florida. recently demonstrated to be a distinct entity with a distinct natu- ral history. Nonetheless, clinical practice guidelines for CMML Address correspondence to: have been inferred from studies designed for MDSs. It is impera- Eric Padron, MD tive that clinicians understand which elements of MDS clinical Assistant Member practice are translatable to CMML, including which evidence has Malignant Hematology been generated from CMML-specific studies and which has not. H. Lee Moffitt Cancer Center & Research Institute This allows for an evidence-based approach to the treatment of 12902 Magnolia Drive CMML and identifies knowledge gaps in need of further study in Tampa, Florida 33612 a disease-specific manner. This review discusses the diagnosis, E-mail: [email protected] prognosis, and treatment of CMML, with the task of divorcing aspects of MDS practice that have not been demonstrated to be applicable to CMML and merging those that have been shown to be clinically similar. Introduction Chronic myelomonocytic leukemia (CMML) is a clonal hemato- logic malignancy characterized by absolute peripheral monocytosis, ineffective hematopoiesis, and an increased risk of transformation to acute myeloid leukemia.
    [Show full text]
  • Philadelphia Chromosome Unmasked As a Secondary Genetic Change in Acute Myeloid Leukemia on Imatinib Treatment
    Letters to the Editor 2050 The ELL/MLLT1 dual-color assay described herein entails 3Department of Cytogenetics, City of Hope National Medical Center, Duarte, CA, USA and co-hybridization of probes for the ELL and MLLT1 gene regions, 4 each labeled in a different fluorochrome to allow differentiation Cytogenetics Laboratory, Seattle Cancer Care Alliance, between genes involved in 11q;19p chromosome translocations Seattle, WA, USA E-mail: [email protected] in interphase or metaphase cells. In t(11;19) acute leukemia cases, gain of a signal easily pinpoints the specific translocation breakpoint to either 19p13.1 or 19p13.3 and 11q23. In the References re-evaluation of our own cases in light of the FISH data, the 19p breakpoints were re-assigned in two patients, underscoring a 1 Harrison CJ, Mazzullo H, Cheung KL, Gerrard G, Jalali GR, Mehta A certain degree of difficulty in determining the precise 19p et al. Cytogenetic of multiple myeloma: interpretation of fluorescence in situ hybridization results. Br J Haematol 2003; 120: 944–952. breakpoint in acute leukemia specimens in the context of a 2 Thirman MJ, Levitan DA, Kobayashi H, Simon MC, Rowley JD. clinical cytogenetics laboratory. Furthermore, we speculate that Cloning of ELL, a gene that fuses to MLL in a t(11;19)(q23;p13.1) the ELL/MLLT1 probe set should detect other 19p translocations in acute myeloid leukemia. Proc Natl Acad Sci 1994; 91: 12110– that involve these genes with partners other than MLL. Accurate 12114. molecular classification of leukemia is becoming more im- 3 Tkachuk DC, Kohler S, Cleary ML.
    [Show full text]
  • Acute Myeloid Leukemia Evolving from JAK 2-Positive Primary Myelofibrosis and Concomitant CD5-Negative Mantle Cell
    Hindawi Publishing Corporation Case Reports in Hematology Volume 2012, Article ID 875039, 6 pages doi:10.1155/2012/875039 Case Report Acute Myeloid Leukemia Evolving from JAK 2-Positive Primary Myelofibrosis and Concomitant CD5-Negative Mantle Cell Lymphoma: A Case Report and Review of the Literature Diana O. Treaba,1 Salwa Khedr,1 Shamlal Mangray,1 Cynthia Jackson,1 Jorge J. Castillo,2 and Eric S. Winer2 1 Department of Pathology and Laboratory Medicine, Rhode Island Hospital, The Warren Alpert Medical School, Brown University, Providence, RI 02903, USA 2 Division of Hematology/Oncology, The Miriam Hospital, The Warren Alpert Medical School, Brown University, Providence, RI 02904, USA Correspondence should be addressed to Diana O. Treaba, [email protected] Received 2 April 2012; Accepted 21 June 2012 Academic Editors: E. Arellano-Rodrigo, G. Damaj, and M. Gentile Copyright © 2012 Diana O. Treaba et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Primary myelofibrosis (formerly known as chronic idiopathic myelofibrosis), has the lowest incidence amongst the chronic myeloproliferative neoplasms and is characterized by a rather short median survival and a risk of progression to acute myeloid leukemia (AML) noted in a small subset of the cases, usually as a terminal event. As observed with other chronic myeloproliferative neoplasms, the bone marrow biopsy may harbor small lymphoid aggregates, often assumed reactive in nature. In our paper, we present a 70-year-old Caucasian male who was diagnosed with primary myelofibrosis, and after 8 years of followup and therapy developed an AML.
    [Show full text]
  • Mirna182 Regulates Percentage of Myeloid and Erythroid Cells in Chronic Myeloid Leukemia
    Citation: Cell Death and Disease (2017) 8, e2547; doi:10.1038/cddis.2016.471 OPEN Official journal of the Cell Death Differentiation Association www.nature.com/cddis MiRNA182 regulates percentage of myeloid and erythroid cells in chronic myeloid leukemia Deepak Arya1,2, Sasikala P Sachithanandan1, Cecil Ross3, Dasaradhi Palakodeti4, Shang Li5 and Sudhir Krishna*,1 The deregulation of lineage control programs is often associated with the progression of haematological malignancies. The molecular regulators of lineage choices in the context of tyrosine kinase inhibitor (TKI) resistance remain poorly understood in chronic myeloid leukemia (CML). To find a potential molecular regulator contributing to lineage distribution and TKI resistance, we undertook an RNA-sequencing approach for identifying microRNAs (miRNAs). Following an unbiased screen, elevated miRNA182-5p levels were detected in Bcr-Abl-inhibited K562 cells (CML blast crisis cell line) and in a panel of CML patients. Earlier, miRNA182-5p upregulation was reported in several solid tumours and haematological malignancies. We undertook a strategy involving transient modulation and CRISPR/Cas9 (clustered regularly interspersed short palindromic repeats)-mediated knockout of the MIR182 locus in CML cells. The lineage contribution was assessed by methylcellulose colony formation assay. The transient modulation of miRNA182-5p revealed a biased phenotype. Strikingly, Δ182 cells (homozygous deletion of MIR182 locus) produced a marked shift in lineage distribution. The phenotype was rescued by ectopic expression of miRNA182-5p in Δ182 cells. A bioinformatic analysis and Hes1 modulation data suggested that Hes1 could be a putative target of miRNA182-5p. A reciprocal relationship between miRNA182-5p and Hes1 was seen in the context of TK inhibition.
    [Show full text]
  • Outcomes for Patients with Chronic Lymphocytic Leukemia and Acute Leukemia Or Myelodysplastic Syndrome
    Leukemia (2016) 30, 325–330 © 2016 Macmillan Publishers Limited All rights reserved 0887-6924/16 www.nature.com/leu ORIGINAL ARTICLE Outcomes for patients with chronic lymphocytic leukemia and acute leukemia or myelodysplastic syndrome FP Tambaro1, G Garcia-Manero2, SM O'Brien2, SH Faderl3, A Ferrajoli2, JA Burger2, S Pierce2, X Wang4, K-A Do4, HM Kantarjian2, MJ Keating2 and WG Wierda2 Acute leukemia (AL) and myelodysplastic syndrome (MDS) are uncommon in chronic lymphocytic leukemia (CLL). We retrospectively identified 95 patients with CLL, also diagnosed with AL (n = 38) or MDS (n = 57), either concurrently (n =5)or subsequent (n = 90) to CLL diagnosis and report their outcomes. Median number of CLL treatments prior to AL and MDS was 2 (0–9) and 1 (0–8), respectively; the most common regimen was purine analog combined with alkylating agent±CD20 monoclonal antibody. Twelve cases had no prior CLL treatment. Among 38 cases with AL, 33 had acute myelogenous leukemia (AML), 3 had acute lymphoid leukemia (ALL; 1 Philadelphia chromosome positive), 1 had biphenotypic and 1 had extramedullary (bladder) AML. Unfavorable AML karyotype was noted in 26, and intermediate risk in 7 patients. There was no association between survival from AL and number of prior CLL regimens or karyotype. Expression of CD7 on blasts was associated with shorter survival. Among MDS cases, all International Prognostic Scoring System (IPSS) were represented; karyotype was unfavorable in 36, intermediate in 6 and favorable in 12 patients; 10 experienced transformation to AML. Shorter survival from MDS correlated with higher risk IPSS, poor-risk karyotype and increased number of prior CLL treatments.
    [Show full text]
  • Mutations and Prognosis in Primary Myelofibrosis
    Leukemia (2013) 27, 1861–1869 & 2013 Macmillan Publishers Limited All rights reserved 0887-6924/13 www.nature.com/leu ORIGINAL ARTICLE Mutations and prognosis in primary myelofibrosis AM Vannucchi1, TL Lasho2, P Guglielmelli1, F Biamonte1, A Pardanani2, A Pereira3, C Finke2, J Score4, N Gangat2, C Mannarelli1, RP Ketterling5, G Rotunno1, RA Knudson5, MC Susini1, RR Laborde5, A Spolverini1, A Pancrazzi1, L Pieri1, R Manfredini6, E Tagliafico7, R Zini6, A Jones4, K Zoi8, A Reiter9, A Duncombe10, D Pietra11, E Rumi11, F Cervantes12, G Barosi13, M Cazzola11, NCP Cross4 and A Tefferi2 Patient outcome in primary myelofibrosis (PMF) is significantly influenced by karyotype. We studied 879 PMF patients to determine the individual and combinatorial prognostic relevance of somatic mutations. Analysis was performed in 483 European patients and the seminal observations were validated in 396 Mayo Clinic patients. Samples from the European cohort, collected at time of diagnosis, were analyzed for mutations in ASXL1, SRSF2, EZH2, TET2, DNMT3A, CBL, IDH1, IDH2, MPL and JAK2. Of these, ASXL1, SRSF2 and EZH2 mutations inter-independently predicted shortened survival. However, only ASXL1 mutations (HR: 2.02; Po0.001) remained significant in the context of the International Prognostic Scoring System (IPSS). These observations were validated in the Mayo Clinic cohort where mutation and survival analyses were performed from time of referral. ASXL1, SRSF2 and EZH2 mutations were independently associated with poor survival, but only ASXL1 mutations held their prognostic relevance (HR: 1.4; P ¼ 0.04) independent of the Dynamic IPSS (DIPSS)-plus model, which incorporates cytogenetic risk. In the European cohort, leukemia-free survival was negatively affected by IDH1/2, SRSF2 and ASXL1 mutations and in the Mayo cohort by IDH1 and SRSF2 mutations.
    [Show full text]
  • The AML Guide Information for Patients and Caregivers Acute Myeloid Leukemia
    The AML Guide Information for Patients and Caregivers Acute Myeloid Leukemia Emily, AML survivor Revised 2012 Inside Front Cover A Message from Louis J. DeGennaro, PhD President and CEO of The Leukemia & Lymphoma Society The Leukemia & Lymphoma Society (LLS) wants to bring you the most up-to-date blood cancer information. We know how important it is for you to understand your treatment and support options. With this knowledge, you can work with members of your healthcare team to move forward with the hope of remission and recovery. Our vision is that one day most people who have been diagnosed with acute myeloid leukemia (AML) will be cured or will be able to manage their disease and have a good quality of life. We hope that the information in this Guide will help you along your journey. LLS is the world’s largest voluntary health organization dedicated to funding blood cancer research, advocacy and patient services. Since the first funding in 1954, LLS has invested more than $814 million in research specifically targeting blood cancers. We will continue to invest in research for cures and in programs and services that improve the quality of life for people who have AML and their families. We wish you well. Louis J. DeGennaro, PhD President and Chief Executive Officer The Leukemia & Lymphoma Society Inside This Guide 2 Introduction 3 Here to Help 6 Part 1—Understanding AML About Marrow, Blood and Blood Cells About AML Diagnosis Types of AML 11 Part 2—Treatment Choosing a Specialist Ask Your Doctor Treatment Planning About AML Treatments Relapsed or Refractory AML Stem Cell Transplantation Acute Promyelocytic Leukemia (APL) Treatment Acute Monocytic Leukemia Treatment AML Treatment in Children AML Treatment in Older Patients 24 Part 3—About Clinical Trials 25 Part 4—Side Effects and Follow-Up Care Side Effects of AML Treatment Long-Term and Late Effects Follow-up Care Tracking Your AML Tests 30 Take Care of Yourself 31 Medical Terms This LLS Guide about AML is for information only.
    [Show full text]
  • Acute Massive Myelofibrosis with Acute Lymphoblastic Leukemia Akut Masif Myelofibrozis Ve Akut Lenfoblastik Lösemi Birlikteliği
    204 Case Report Acute massive myelofibrosis with acute lymphoblastic leukemia Akut masif myelofibrozis ve akut lenfoblastik lösemi birlikteliği Zekai Avcı1, Barış Malbora1, Meltem Gülşan1, Feride Iffet Şahin2, Bülent Celasun3, Namık Özbek1 1Department of Pediatrics, Başkent University Faculty of Medicine, Ankara, Turkey 2Department of Medical Genetics, Başkent University Faculty of Medicine, Ankara, Turkey 3Department of Pathology, Başkent University Faculty of Medicine, Ankara, Turkey Abstract Acute myelofibrosis is characterized by pancytopenia of sudden onset, megakaryocytic hyperplasia, extensive bone mar- row fibrosis, and the absence of organomegaly. Acute myelofibrosis in patients with acute lymphoblastic leukemia is extremely rare. We report a 4-year-old boy who was diagnosed as having acute massive myelofibrosis and acute lym- phoblastic leukemia. Performing bone marrow aspiration in this patient was difficult (a “dry tap”), and the diagnosis was established by means of a bone marrow biopsy and immunohistopathologic analysis. The prognostic significance of acute myelofibrosis in patients with acute lymphoblastic leukemia is not clear. (Turk J Hematol 2009; 26: 204-6) Key words: Acute myelofibrosis, acute lymphoblastic leukemia, dry tap Received: April 9, 2008 Accepted: December 24, 2008 Özet Akut myelofibrozis ani gelişen pansitopeni, kemik iliğinde megakaryositik hiperplazi, belirgin fibrozis ve organomegali olmaması ile karakterize bir hastalıktır. Akut myelofibrozis ile akut lenfoblastik lösemi birlikteliği çok nadir görülmektedir.
    [Show full text]
  • Myelodysplastic Syndromes Overview and Types
    cancer.org | 1.800.227.2345 About Myelodysplastic Syndromes Overview and Types If you have been diagnosed with a myelodysplastic syndrome or are worried about it, you likely have a lot of questions. Learning some basics is a good place to start. ● What Are Myelodysplastic Syndromes? ● Types of Myelodysplastic Syndromes Research and Statistics See the latest estimates for new cases of myelodysplastic syndromes in the US and what research is currently being done. ● Key Statistics for Myelodysplastic Syndromes ● What's New in Myelodysplastic Syndrome Research? What Are Myelodysplastic Syndromes? Myelodysplastic syndromes (MDS) are conditions that can occur when the blood- forming cells in the bone marrow become abnormal. This leads to low numbers of one or more types of blood cells. MDS is considered a type of cancer1. Normal bone marrow 1 ____________________________________________________________________________________American Cancer Society cancer.org | 1.800.227.2345 Bone marrow is found in the middle of certain bones. It is made up of blood-forming cells, fat cells, and supporting tissues. A small fraction of the blood-forming cells are blood stem cells. Stem cells are needed to make new blood cells. There are 3 main types of blood cells: red blood cells, white blood cells, and platelets. Red blood cells pick up oxygen in the lungs and carry it to the rest of the body. These cells also bring carbon dioxide back to the lungs. Having too few red blood cells is called anemia. It can make a person feel tired and weak and look pale. Severe anemia can cause shortness of breath. White blood cells (also known as leukocytes) are important in defending the body against infection.
    [Show full text]
  • Chronic Myelomonocytic Leukemia Early Detection, Diagnosis, and Staging Detection and Diagnosis
    cancer.org | 1.800.227.2345 Chronic Myelomonocytic Leukemia Early Detection, Diagnosis, and Staging Detection and Diagnosis Catching cancer early often allows for more treatment options. Some early cancers may have signs and symptoms that can be noticed, but that is not always the case. ● Signs and Symptoms of Chronic Myelomonocytic Leukemia ● How Is Chronic Myelomonocytic Leukemia Diagnosed? Stages and Outlook (Prognosis) After a cancer diagnosis, staging provides important information about the extent of cancer in the body and anticipated response to treatment. ● How Is Chronic Myelomonocytic Leukemia Staged? ● Survival Rates for Chronic Myelomonocytic Leukemia Questions to Ask About CMML Here are some questions you can ask your cancer care team to help you better understand your CMML diagnosis and treatment options. ● Questions to Ask Your Doctor About Chronic Myelomonocytic Leukemia 1 ____________________________________________________________________________________American Cancer Society cancer.org | 1.800.227.2345 Signs and Symptoms of Chronic Myelomonocytic Leukemia The most common sign of chronic myelomonocytic leukemia (CMML) is having too many monocytes (seen on a blood test). Having too many monocytes also causes many of the symptoms of CMML. These monocytes can settle in the spleen or liver, enlarging these organs. An enlarged spleen (called splenomegaly) can cause pain in the upper left part of the belly (abdomen). It can also cause people to notice they feel full too fast when they eat. If the liver gets too big (called hepatomegaly), it causes discomfort in the upper right part of the abdomen. Low numbers of other types of blood cells1 cause many of the signs and symptoms of CMML: ● A shortage of red blood cells (anemia) can lead to feeling very tired, with shortness of breath and pale skin.
    [Show full text]
  • Acute Myeloid Leukemia (AML)
    Acute Myeloid Leukemia (AML) AML is a blood cancer that starts in the bone marrow but moves quickly into the blood, sometimes spreading to other parts of the body. What is AML? Global Incidence Leukemia is classified based on two attributes—its speed of progression and the type of white blood cells aected. AML is the most common In 2012, the worldwide type of leukemia in adults. incidence of AML was Leukemia is described as being either acute Average age at diagnosis is estimated to be (fast growing) or chronic (slow growing), and either myelogenous (aecting the 68 350,000+ myeloid cells) or lymphocytic (aecting the lymphoid cells, or lymphocytes). Fast Growing Slow Growing Causes and Risk Factors Leukemia Leukemia Today, researchers understand a lot more Acute Lymphocytic Leukemia Chronic Lymphocytic Leukemia about what may cause AML. DNA mutations, which may result from exposure to radiation, Acute Myeloid Chronic Myeloid cancer-causing chemicals or the aging (or Myelogenous) Leukemia (or Myelogenous) Leukemia process, are commonly found in AML cells. Signs and Symptoms Prognosis At first, patients with AML often have non-specific symptoms usually associated with more common In general, prognosis for AML ailments like the flu. Often, signs and symptoms result from a shortage of normal blood cells, which patients is poor. happens when the leukemia cells crowd out the normal blood-making cells in the bone marrow. Prognosis is influenced by patient These signs and symptoms include: age, AML subtype, and other factors Estimated 5-year survival
    [Show full text]