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Myelodysplastic Syndrome, Juvenile Myelomonocytic Leukemia, and Acute Myeloid Leukemia Associated with Complete Or Partial Monos

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Myelodysplastic Syndrome, Juvenile Myelomonocytic Leukemia, and Acute Myeloid Leukemia Associated with Complete Or Partial Monos Leukemia (1999) 13, 376–385 1999 Stockton Press All rights reserved 0887-6924/99 $12.00 http://www.stockton-press.co.uk/leu Myelodysplastic syndrome, juvenile myelomonocytic leukemia, and acute myeloid leukemia associated with complete or partial monosomy 7 H Hasle1, M Arico` 2, G Basso3, A Biondi4, A Cantu` Rajnoldi4, U Creutzig5, S Fenu6, C Fonatsch7, OA Haas8, J Harbott9, G Kardos10, G Kerndrup11, G Mann8, CM Niemeyer12, H Ptoszkova13, J Ritter5, R Slater14, J Stary´15, B Stollmann-Gibbels16, AM Testi6, ER van Wering17 and M Zimmermann5 for the European Working Group on MDS in Childhood (EWOG-MDS) 1Department of Pediatrics, Aarhus University Hospital, Denmark; 2Department of Pediatrics, University IRCCS S Matteo, Pavia, Italy; 3Department of Pediatrics, University of Padova, Italy; 4Department of Pediatrics, University of Milan, Italy; 5Department of Pediatrics, University of Mu¨nster, Germany; 6La Sapienza, Rome, Italy; 7Institute of Medical Biology, University of Vienna, Austria; 8St Anna Children’s Hospital, Vienna, Austria; 9Oncogenetic Laboratory, Children’s Hospital, Giessen, Germany; 10Department of Pediatrics, Free University of Amsterdam, The Netherlands; 11Department of Pathology, Odense University Hospital, Denmark; 12Department of Pediatrics, University of Freiburg, Germany; 13Department of Pediatrics, Ostrava, Czech Republic; 14Netherlands Working Party on Cancer Genetics and Cytogenetics, Rotterdam, The Netherlands; 152nd Department of Pediatrics, Prague, Czech Republic; 16Department of Pediatrics, University of Essen, Germany; 17Dutch Childhood Leukemia Study Group, The Hague, The Netherlands We reviewed the clinical features, treatment, and outcome of The most conflicting area in the classification of childhood 100 children with myelodysplastic syndrome (MDS), juvenile MDS has been the pediatric equivalent of what the FAB group myelomonocytic leukemia (JMML), and acute myeloid leukemia (AML) associated with complete monosomy 7 (−7) or deletion termed CMML. These children have most often been referred of the long arm of chromosome 7 (7q−). Patients with therapy- to as juvenile chronic myeloid leukemia (JCML) in the British induced disease were excluded. The morphologic diagnoses and American literature,7,8 whereas others have favored the according to modified FAB criteria were: MDS in 72 (refractory FAB term CMML.4,9–11 An International Working Group con- anemia (RA) in 11, RA with excess of blasts (RAEB) in eight, cluded that the different terminology did not reflect the exist- RAEB in transformation (RAEB-T) in 10, JMML in 43), and AML ence of different disorders and proposed the term juvenile in 28. The median age at presentation was 2.8 years (range 2 myelomonocytic leukemia (JMML). The term has attained months to 15 years), being lowest in JMML (1.1 year). Loss of 6,12–14 chromosome 7 as the sole cytogenetic abnormality was international acceptance and will be used throughout observed in 75% of those with MDS compared with 32% of this paper. Although we acknowledge that JMML often shows those with AML. Predisposing conditions (including familial myeloproliferative features different from other MDS types, we MDS/AML) were found in 20%. Three-year survival was 82% in have included JMML in the group of childhood MDS in RA, 63% in RAEB, 45% in JMML, 34% in AML, and 8% in RAEB- accordance with the practice by other cooperative groups. T. Children with −7 alone had a superior survival than those Loss of chromosome 7 material, either as complete mono- with other cytogenetic abnormalities: this was solely due to a − − better survival in MDS (3-year survival 56 vs 24%). The reverse somy 7 ( 7) or as deletion of the long arm (7q ), is the most was found in AML (3-year survival 13% in −7 alone vs 44% in common cytogenetic abnormality in childhood MDS seen in other cytogenetic groups). Stable disease for several years was approximately 30% of cases.4,5,15 Only 4–5% of childhood documented in more than half the patients with RA or RAEB. AML cases show −7/7q−.16–19 AML patients with −7/7q− have Patients with RA, RAEB or JMML treated with bone marrow a very poor prognosis,16,17,19 but due to the infrequency of the transplantation (BMT) without prior chemotherapy had a 3-year survival of 73%. The morphologic diagnosis was the strongest association there are very few data on the clinical character- prognostic factor. Only patients with a diagnosis of JMML fitted istics of these patients. what has previously been referred to as the monosomy 7 syn- Children with MDS and −7 have often been considered a drome. Our data give no support to the concept of monosomy distinct hematologic disorder described as the monosomy 7 7 as a distinct syndrome. syndrome, characterized by young age, male predominance, Keywords: myelodysplastic syndrome (MDS); acute myeloid leu- hepatosplenomegaly, and leukocytosis.20–23 The monosomy 7 kemia (AML); children; monosomy 7 syndrome has many similarities with JMML and the distinction between the two nosological entities has not been clear-cut. In a previous study we did not find any major clinical differences Introduction between JMML in children with and without −7.11 Further- more, −7 has been considered to represent a late event or an Myelodysplastic syndrome (MDS) comprises a heterogeneous opportunistic cytogenetic abnormality.24 Therefore, it may be group of clonal stem cell disorders characterized by ineffec- questioned whether a classification solely based on the loss tive hematopoiesis often with prominent morphologic abnor- of chromosome 7 is of clinical relevance. malities. MDS is rare in childhood with an annual incidence The aim of the present study was to describe the clinical 1 of only four/million. The French–American–British (FAB) characteristics, the predisposing conditions, survival and group proposed a classification of MDS comprising five sub- response to treatment of a large number of children with groups: refractory anemia (RA), RA with ringed sideroblasts −7/7q−. (RARS), RA with excess of blasts (RAEB), RAEB in transform- ation (RAEB-T), and chronic myelomonocytic leukemia 2,3 (CMML). The FAB classification has become widely Materials and methods accepted for MDS in adults. The classification of childhood 4–6 MDS has remained rather controversial and inconsistent. Data on children with −7/7q− and myeloid malignancies were collected retrospectively through members of the European Correspondence: H Hasle, Department of Pediatrics, Aarhus Univer- Working Group on MDS in Childhood (EWOG-MDS). Patients sity Hospital Skejby, 8200 Aarhus N, Denmark; Fax: 45 8949 6023 previously exposed to chemotherapy or radiation, as well as Received 13 July 1998; accepted 20 November 1998 patients with Fanconi anemia or congenital granulocytopenia Monosomy 7 and myeloid leukemia H Hasle et al 377 were excluded. The study group consisted of 100 children Results from Austria (n = 5), Czech Republic (n = 8), Denmark (n = 20), Germany (n = 40), Italy (n = 15) and the Netherlands (n = Clinical characteristics 12). Data on 28 of the patients have been included in previous studies from EWOG-MDS.11,25 RA was diagnosed in 11 patients, RAEB in eight, RAEB-T in Inclusion required a diagnosis of MDS or AML and bone 10, JMML in 43 and AML in 28 (MO two, M1 four, M2 12, marrow (BM) or peripheral blood (PB) karyotype by standard M4 two, M5 one, M6 three, M7 two, unclassified two). The technique showing at least three cells with loss of a whole clinical characteristics according to morphologic diagnosis chromosome 7 or at least two cells with identical structural are shown in Table 1. The median age at presentation was abnormalities leading to loss of chromosome 7q material. 2.8 years (range 2 months to 15 years), being lowest in JMML Confirmatory fluorescence in situ hybridization (FISH) studies (1.1 year) (P Ͻ 0.001). JMML predominated among the young- were performed in a few cases, but the classification of the est children accounting for 83% of the 23 cases occurring in patients relied solely on standard cytogenetics. The first infants below 1 year of age (Figure 1). Boys dominated in all reported abnormal karyotype involving chromosome 7 was MDS subgroups with an overall boy/girl ratio of 2.4. In con- used to classify the patients as −7 alone, −7 plus other abnor- trast, a predominance of girls with AML resulted in a boy/girl malities (−7 + other), 7q− alone, or 7q− plus other abnormali- ratio of 0.75 (P Ͻ 0.01). ties (7q−+other). Karyotypes were described according to the At presentation 49 patients had fever Ͼ38°C, an active International System for Human Cytogenetic Nomenclature infection was documented in 34 children. Hepatosplenome- 1995.26 galy and lymphadenopathy were associated with JMML. Of All cases were categorized according to the FAB classi- the seven children with JMML who did not present with fication of MDS and AML, with the following two modifi- splenomegaly, five developed splenomegaly during the clini- cations: for a diagnosis of JMML up to 20% myeloblasts in the cal course. Skin rash was described in 16 children, nine of blood was accepted.11,27,28 AML was diagnosed when these had JMML. Blasts in the cerebrospinal fluid at presen- myeloblasts in PB were Ͼ30%, regardless of the number of tation were noticed in five patients, all with AML. Diabetes myeloblast in BM.29 insipidus was present in two patients, both had JMML with − White blood cell count (WBC) was corrected for the pres- 7. Chloroma occurred in five children with JMML, two with ence of nucleated red blood cells in PB. Reference values for RAEB-T, and two with AML. corpuscular volume (MCV) were taken from Dallman and Siimes30 and for hemoglobin F (HbF) from Huehns and Beaven.31 Due to the retrospective nature of the study some Hematologic findings data could not be retrieved. The number of patients with eval- = = Hematologic characteristics at diagnosis are given in Table uable data was: hemoglobin (n 99), MCV (n 68), HbF (n Ͻ = 41), WBC (n = 100), platelets (n = 99), complete PB differen- 2.
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