Hematological complications of Gaucher's disease in pregnancy

被引:7
作者
Clarkson, CP [1 ]
Magann, EF [1 ]
Siddique, SA [1 ]
Morrison, JC [1 ]
机构
[1] USN Hosp, Dept Obstet & Gynecol, Camp Pendleton, CA 92055 USA
关键词
D O I
10.1093/milmed/163.7.499
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
A case is presented of a 31-year-old Filipino female, gravida 5 para 2, at 38 weeks plus 5 days gestation, with known type I Gaucher's disease who underwent repeat cesarean delivery. After cesarean delivery, the patient developed disseminated intravascular coagulation and required transfusion of eight 6-packs of platelets, 6 units of fresh frozen plasma, two 10-packs of cryoprecipitate, and 6 units of packed red blood cells. pregnancy is generally well tolerated in patients with type i Gaucher's disease, an autosomal recessive lysosomal storage disorder in which lipid deposits accumulate in the liver, spleen, and bone marrow. Hemorrhagic problems secondary to severe thrombocytopenia may develop postpartum in pregnancies complicated by Gaucher's disease, requiring significant support with blood and blood products.
引用
收藏
页码:499 / 501
页数:3
相关论文
共 18 条
[1]   Gaucher's disease and pregnancy [J].
Ayhan, A ;
Tuncer, ZS ;
Simsek, H .
EUROPEAN JOURNAL OF OBSTETRICS GYNECOLOGY AND REPRODUCTIVE BIOLOGY, 1996, 66 (01) :69-70
[2]   CIRCULATING ANTI-COAGULANT IN PATIENTS WITH GAUCHERS-DISEASE [J].
BENJAMIN, D ;
JOSHUA, H ;
DOUER, D ;
SHAKLAI, M ;
KRUGLIAC, Y ;
PINKHAS, J .
ACTA HAEMATOLOGICA, 1979, 61 (04) :233-234
[3]  
Beutler E, 1997, Curr Opin Hematol, V4, P19
[4]  
BEUTLER E, 1991, NEW ENGL J MED, V325, P1354
[5]   PREGNANCY AND GAUCHERS DISEASE [J].
BROMBERG, YM ;
TOAFF, R ;
DIENGOTT, D .
BRITISH MEDICAL JOURNAL, 1953, 2 (4839) :761-763
[6]   SOUTHWESTERN INTERNAL-MEDICINE CONFERENCE - GAUCHER DISEASE - A HETEROGENEOUS CLINICAL COMPLEX FOR WHICH EFFECTIVE ENZYME REPLACEMENT HAS COME OF AGE [J].
FRENKEL, EP .
AMERICAN JOURNAL OF THE MEDICAL SCIENCES, 1993, 305 (05) :331-344
[7]   OBSTETRIC ASPECTS OF GAUCHER DISEASE [J].
GOLDBLATT, J ;
BEIGHTON, P .
BRITISH JOURNAL OF OBSTETRICS AND GYNAECOLOGY, 1985, 92 (02) :145-149
[8]  
GOLDMAN L, 1983, ANN INTERN MED, V98, P54
[9]   GYNECOLOGIC AND OBSTETRIC ASPECTS OF GAUCHERS-DISEASE - A SURVEY OF 53 PATIENTS [J].
GRANOVSKYGRISARU, S ;
ABOULAFIA, Y ;
DIAMANT, YZ ;
HOROWITZ, M ;
ABRAHAMOV, A ;
ZIMRAN, A .
AMERICAN JOURNAL OF OBSTETRICS AND GYNECOLOGY, 1995, 172 (04) :1284-1290
[10]   ADULT GAUCHERS DISEASE, WITH SPECIAL REFERENCE TO THE VARIATIONS IN ITS CLINICAL COURSE AND THE VALUE OF STERNAL PUNCTURE AS AN AID TO ITS DIAGNOSIS [J].
GROEN, J ;
GARRER, AH .
BLOOD, 1948, 3 (11) :1221-1237