Occurrence of myeloproliferative disorder in patients with Noonan syndrome

被引:132
作者
BaderMeunier, B
Tchernia, G
Mielot, F
Fontaine, JL
Thomas, C
Lyonnet, S
Lavergne, JM
Dommergues, JP
机构
[1] HOP BICETRE, HEMATOL LAB, F-94270 LE KREMLIN BICETRE, FRANCE
[2] HOP NECKER ENFANTS MALAD, DEPT PEDIAT, PARIS, FRANCE
[3] TROUSSEAU HOSP, DEPT PEDIAT, PARIS, FRANCE
关键词
D O I
10.1016/S0022-3476(97)70273-7
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
We report four cases of Noonan syndrome associated with chronic myelomonocytic leukemia in childhood, These children shared some hematologic features: thrombocytopenia, splenomegaly in the first months of life, occurrence of chronic myelomonocytic leukemia without abnormalities of the initial bone marrow karyotype, and, in three cases, improvement of the hematologic disease, A common pathophysiologic process in such patients is suggested.
引用
收藏
页码:885 / 889
页数:5
相关论文
共 24 条
[1]  
ALLEN RC, 1992, AM J HUM GENET, V51, P1229
[2]   NOONANS-SYNDROME AND ACUTE LYMPHOBLASTIC-LEUKEMIA [J].
ATTARDMONTALTO, SP ;
KINGSTON, JE ;
EDEN, T .
MEDICAL AND PEDIATRIC ONCOLOGY, 1994, 23 (04) :391-392
[3]   Myelodysplastic syndromes in childhood: Report of 49 patients from a French multicentre study [J].
BaderMeunier, B ;
Mielot, F ;
Tchernia, G ;
Buisine, J ;
Delsol, G ;
Duchayne, J ;
Lemerle, S ;
Leverger, G ;
deLumley, L ;
Manel, AM ;
Nathanson, M ;
Plantaz, D ;
Robert, A ;
Schaison, G ;
Sommelet, D ;
Vilmer, E .
BRITISH JOURNAL OF HAEMATOLOGY, 1996, 92 (02) :344-350
[4]   REFRACTORY-ANEMIA AND MITOCHONDRIAL CYTOPATHY IN CHILDHOOD [J].
BADERMEUNIER, B ;
ROTIG, A ;
MIELOT, F ;
LAVERGNE, JM ;
CROISILLE, L ;
RUSTIN, P ;
LANDRIEU, P ;
DOMMERGUES, JP ;
MUNNICH, A ;
TCHERNIA, G .
BRITISH JOURNAL OF HAEMATOLOGY, 1994, 87 (02) :381-385
[5]  
BOROCHOWITZ Z, 1989, Neurofibromatosis, V2, P309
[6]   CLONALITY IN JUVENILE CHRONIC MYELOGENOUS LEUKEMIA [J].
BUSQUE, L ;
GILLILAND, DG ;
PRCHAL, JT ;
SIEFF, CA ;
WEINSTEIN, HJ ;
SOKOL, JM ;
BELICKOVA, M ;
WAYNE, AS ;
ZUCKERMAN, KS ;
SOKOL, L ;
CASTLEBERRY, RP ;
EMANUEL, PD .
BLOOD, 1995, 85 (01) :21-30
[7]   PLATELET DYSFUNCTION IN NOONANS SYNDROME - A CASE WITH A PLATELET CYCLOOXYGENASE-LIKE DEFICIENCY AND CHRONIC IDIOPATHIC THROMBOCYTOPENIC PURPURA [J].
FLICK, JT ;
SINGH, AK ;
KIZER, J ;
LAZARCHICK, J .
AMERICAN JOURNAL OF CLINICAL PATHOLOGY, 1991, 95 (05) :739-742
[8]   NO EVIDENCE FOR LINKAGE TO THE TYPE-1 OR TYPE-2 NEUROFIBROMATOSIS LOCI IN NOONAN SYNDROME FAMILIES [J].
FLINTOFF, WF ;
BAHUAU, M ;
LYONNET, S ;
GILGENKRANTZ, S ;
LACOMBE, D ;
MARCON, F ;
LEVILLIERS, J ;
KACHANER, J ;
MUNNICH, A ;
LEMERRER, M .
AMERICAN JOURNAL OF MEDICAL GENETICS, 1993, 46 (06) :700-705
[9]   ABDOMINAL ULTRASOUND IN NOONAN SYNDROME - A STUDY OF 44 PATIENTS [J].
GEORGE, CD ;
PATTON, MA ;
ELSAWI, M ;
SHARLAND, M ;
ADAM, EJ .
PEDIATRIC RADIOLOGY, 1993, 23 (04) :316-318
[10]   MYELODYSPLASTIC SYNDROMES IN CHILDHOOD - CLASSIFICATION, EPIDEMIOLOGY, AND TREATMENT [J].
HASLE, H .
LEUKEMIA & LYMPHOMA, 1994, 13 (1-2) :11-26