Laman

▼

Minggu, 25 September 2011

TROMBOSITOPENIA dalam KEHAMILAN

LATAR BELAKANG
Trombositopenia sering terjadi pada seorang ibu dan neonatus yang selalu disebabkan oleh destruksi trombosit (platelet destruction). Kadar trombosit ibu tidak hamil dan neonatus adalah 150.000 – 400.000 / µL ; dan pada wanita hamil umumnya lebih rendah.
Trombositopenia dalam Kehamilan , dapat disebabkan oleh berbagai penyebab:
  • Trombositopenia Gestasional
  • Infeksi virus dan bakteri
  • Preeklampsia dengan komplikasi sindroma HELLP (hemolisis-Elevated Liver Enzyme dan Low Platelet.
Berikut akan dibahas tentang imune trombositopenia , imune trombositopenik purpura (ITP) dan NAIT – neonatal alloimmune thrombocytopenia.

PATOFISIOLOGI
Trombositopenia pada ITP terjadi oleh karena destruksi trombosit yang di mediasi oleh autoantibodi trombosit langsung terhadap antigen permukaan sel. Sistem retikuendotelial merusak antibodi-trombotis komplek. Autoantibodi ini dapat menembus plasenta sehingga dapat mengganggu ibu dan anak.
NAIT disebabkan oleh imunisasi maternal terhadap antigen “fetal paternally plateler-specific antigen ( mirip dengan penyakit Rhesus ). Ibu memiliki jumlah trombosit normal, namun janinnya mengalami trombositopenia berat.
EPIDEMIOLOGI
Angka Kejadian :
Amerika
Angka kejadian ITP : 1 – 2 kasus per 1000 persalinan[2]
Diagnosa ITP ditegakkan saat pemeriksaan antenatal pada pasien dengan riwayat kelainan darah.
Angka kejadian NAIT : 1 – 2 kasus per 1000 persalinan
Internasional
Angka kejadian ITP : 1.8 kasus per 1000 persalinan di Helsinki, Finlandia. [3]
Angka kejdian NAIT : 0.5 kasus per 1000 persalinan dan 1.5 kasus per 1000 neonatus hidup di Inggris dan Perancis. [4]
Di Jepang angka kejadian NAIT 0.3 kasus per 1000 lahir hidup dan adanya inkompatibilitas HPA (human Platelet Antigen) – 4 merupakan etiologi dari 80% kasus. [6] Rekurensi NAIT sangat tinggi (mendekati 100%) [7]
image
Immune thrombocytopenia. An infant born with neonatal lupus syndrome and severe thrombocytopenia. Note extensive bruising and petechiae.
image
Immune thrombocytopenia. An infant born with a cephalohematoma.

MORTALITAS dan MORBIDITAS
  • Resiko ibu bersalin dengan ITP adalah perdarahan, terutama bila jumlah trombosit < 20.000. Trombositopenia neonatus akibat transportasi aktif antibodi trombosit transplasenta menimbulkan masalah klinik yang bermakna dan terjadi 9 dari 66 kehamilan dengan ITP (13.6%). Dari kehamilan tersebut, 5 neonatus dari 66 kehamilan menderita trombositopenia dengan jumlah trombosit < 50.000/µL
  • Trombositopenia neonatus yang hebat menempatkan neonatus dalam resiko perdarahan intrakranial atau viseral.
  • Morbiditas neonatus lebih sering terjadi pada NAIT dengan 10% kematian pada neonatus yang menderita dan 10% menderita kelainan neurologis akibat perdarahan intrakranial. Neonatus yang terkena menunjukkan petechiae generalisata, perdarahan inraabominal dan perdarahan lainnya.
Hemaotoma
Immune thrombocytopenia. Neonatal brain at autopsy showing extensive subdural hemorrhage.

RAS
  • ITP dapat terjadi pada semua ras
  • Lebih dari 50% kasus NAIT terjadi pada ras kulit putih
SEX
  • ITP lebih sering terjadi pada wanita (rasio 3:1).[12]
  • NAIT pada neonatus dapat terjadi pada kedua jenis kelamin
USIA
  • Diagnosa ITP seringkali ditegakkan pada dekade II dan III kehidupan
  • NAIT terjadi dalam kehidupann janin dengan 25 – 50% perdaeahan intrakranial terdeteksi pada pemeriksaan USG prenatal sebelum onset persalinan[13]

 ANAMNESA

  • Ibu hamil dengan ITP dapat tanpa gejala atau dengan gejala ringan (epistaksis atau perdarahan gusi, atau petekiae))
  • Dapat terjadi riwayat menorrhagia atau menometrorrhagia sebelum kehamilan. ·
  • Seorang wanita dengan riwayat persalinan neonatus dengan trombositopenia, perdarahan viseral atau intrkranial patut diduga menderita NAIT. Namun, 50% neonatus dengan NAIT adalah anak pertama .
DIAGNOSA BANDING
Rujukan Kepustakaan
  1. Giers G, Wenzel F, Fischer J, et al. Retrospective comparison of maternal vs. HPA-matched donor platelets for treatment of fetal alloimmune thrombocytopenia. Vox Sang. Oct 27 2009;[Medline].
  2. Burrows RF, Kelton JG. Thrombocytopenia at delivery: a prospective survey of 6715 deliveries. Am J Obstet Gynecol. Mar 1990;162(3):731-4. [Medline].
  3. Sainio S, Jarvenpaa AL, Renlund M. Thrombocytopenia in term infants: a population-based study. Obstet Gynecol. Mar 2000;95(3):441-6. [Medline].
  4. Blanchette VS, Chen L, de Friedberg ZS. Alloimmunization to the PlA1 platelet antigen: results of a prospective study. Br J Haematol. Feb 1990;74(2):209-15. [Medline].
  5. Dreyfus M, Kaplan C, Verdy E. Frequency of immune thrombocytopenia in newborns: a prospective study. Immune Thrombocytopenia Working Group. Blood. Jun 15 1997;89(12):4402-6. [Medline].
  6. Davis GL. Platelet specific alloantigens. Clin Lab Sci. Nov-Dec 1998;11(6):356-61. [Medline].
  7. Bussel JB. Immune thrombocytopenia in pregnancy: autoimmune and alloimmune. J Reprod Immunol. Dec 15 1997;37(1):35-61. [Medline].
  8. Yamada H, Kato EH, Kobashi G. Passive immune thrombocytopenia in neonates of mothers with idiopathic thrombocytopenic purpura: incidence and risk factors. Semin Thromb Hemost. 1999;25(5):491-6. [Medline].
  9. Biswas A, Arulkumaran S, Ratnam SS. Disorders of platelets in pregnancy. Obstet Gynecol Surv. Aug 1994;49(8):585-94. [Medline].
  10. Durand-Zaleski I, Schlegel N, Blum-Boisgard C. Screening primiparous women and newborns for fetal/neonatal alloimmune thrombocytopenia: a prospective comparison of effectiveness and costs. Immune Thrombocytopenia Working Group. Am J Perinatol. Oct 1996;13(7):423-31. [Medline].
  11. ACOG practice bulletin, American College of Obstetricians and Gynecologists. Thrombocytopenia in pregnancy. Number 6, September 1999. Clinical management guidelines for obstetrician- gynecologists. Int J Gynaecol Obstet. Nov 1999;67(2):117-28. [Medline].
  12. George JN, el-Harake MA, Raskob GE. Chronic idiopathic thrombocytopenic purpura. N Engl J Med. Nov 3 1994;331(18):1207-11. [Medline].
  13. Herman JH, Jumbelic MI, Ancona RJ. In utero cerebral hemorrhage in alloimmune thrombocytopenia. Am J Pediatr Hematol Oncol. Winter 1986;8(4):312-7. [Medline].
  14. Christiaens GC, Nieuwenhuis HK, von dem Borne AE. Idiopathic thrombocytopenic purpura in pregnancy: a randomized trial on the effect of antenatal low dose corticosteroids on neonatal platelet count. Br J Obstet Gynaecol. Oct 1990;97(10):893-8. [Medline].
  15. Cohen DL, Baglin TP. Assessment and management of immune thrombocytopenia in pregnancy and in neonates. Arch Dis Child Fetal Neonatal Ed. Jan 1995;72(1):F71-6. [Medline].
  16. Cines DB, Blanchette VS. Immune thrombocytopenic purpura. N Engl J Med. Mar 28 2002;346(13):995-1008. [Medline].
  17. Yamada H, Kato EH, Kishida T. Risk factors for neonatal thrombocytopenia in pregnancy complicated by idiopathic thrombocytopenic purpura. Ann Hematol. May 1998;76(5):211-4. [Medline].
  18. Moise KJ Jr, Patton DE, Cano LE. Misdiagnosis of a normal fetal platelet count after coagulation of intrapartum scalp samples in autoimmune thrombocytopenic purpura. Am J Perinatol. Sep 1991;8(5):295-6. [Medline].
  19. Berry SM, Leonardi MR, Wolfe HM. Maternal thrombocytopenia. Predicting neonatal thrombocytopenia with cordocentesis. J Reprod Med. May 1997;42(5):276-80. [Medline].
  20. Cook RL, Miller RC, Katz VL. Immune thrombocytopenic purpura in pregnancy: a reappraisal of management. Obstet Gynecol. Oct 1991;78(4):578-83. [Medline].
  21. Bussel J, Kaplan C. The fetal and neonatal consequences of maternal alloimmune thrombocytopenia. Baillieres Clin Haematol. Jun 1998;11(2):391-408. [Medline].
  22. Martí-Carvajal AJ, Peña-Martí GE, Comunián-Carrasco G. Medical treatments for idiopathic thrombocytopenic purpura during pregnancy. Cochrane Database Syst Rev. Oct 7 2009;CD007722. [Medline].
  23. Sukenik-Halevy R, Ellis MH, Fejgin MD. Management of immune thrombocytopenic purpura in pregnancy. Obstet Gynecol Surv. Mar 2008;63(3):182-8. [Medline].
  24. Howman RA, Barr AL, Shand AW, Dickinson JE. Antenatal intravenous immunoglobulin in chronic immune thrombocytopenic purpura: case report and literature review. Fetal Diagn Ther. 2009;25(1):93-7. [Medline].
  25. Bussel JB, Graziano JN, Kimberly RP. Intravenous anti-D treatment of immune thrombocytopenic purpura: analysis of efficacy, toxicity, and mechanism of effect. Blood. May 1 1991;77(9):1884-93. [Medline].
  26. Scaradavou A, Woo B, Woloski BM. Intravenous anti-D treatment of immune thrombocytopenic purpura: experience in 272 patients. Blood. Apr 15 1997;89(8):2689-700. [Medline].
  27. Copel JA, Gollin YG, Grannum PA. Alloimmune disorders and pregnancy. Semin Perinatol. Jun 1991;15(3):251-6. [Medline].
  28. Bussel JB, Zabusky MR, Berkowitz RL. Fetal alloimmune thrombocytopenia. N Engl J Med. Jul 3 1997;337(1):22-6. [Medline].
  29. Kaplan C, Daffos F, Forestier F. Management of alloimmune thrombocytopenia: antenatal diagnosis and in utero transfusion of maternal platelets. Blood. Jul 1988;72(1):340-3. [Medline].
  30. Nicolini U, Tannirandorn Y, Gonzalez P. Continuing controversy in alloimmune thrombocytopenia: fetal hyperimmunoglobulinemia fails to prevent thrombocytopenia. Am J Obstet Gynecol. Oct 1990;163(4 Pt 1):1144-6. [Medline].
  31. Murphy MF, Pullon HW, Metcalfe P. Management of fetal alloimmune thrombocytopenia by weekly in utero platelet transfusions. Vox Sang. 1990;58(1):45-9. [Medline].
  32. Lynch L, Bussel JB, McFarland JG. Antenatal treatment of alloimmune thrombocytopenia. Obstet Gynecol. Jul 1992;80(1):67-71. [Medline].
  33. Bussel JB, Berkowitz RL, Lynch L. Antenatal management of alloimmune thrombocytopenia with intravenous gamma-globulin: a randomized trial of the addition of low-dose steroid to intravenous gamma-globulin. Am J Obstet Gynecol. May 1996;174(5):1414-23. [Medline].
  34. Radder CM, Brand A, Kanhai HH. A less invasive treatment strategy to prevent intracranial hemorrhage in fetal and neonatal alloimmune thrombocytopenia. Am J Obstet Gynecol. 2001;185(3):683-8.
  35. Burrows RF, Kelton JG. Incidentally detected thrombocytopenia in healthy mothers and their infants. N Engl J Med. Jul 21 1988;319(3):142-5. [Medline].
  36. Greinacher A, Eichler P, Lubenow N. Drug-induced and drug-dependent immune thrombocytopenias. Rev Clin Exp Hematol. 2001;5(3):166-200.
  37. McCrae KR, Bussel JB, Mannucci PM, et al. Platelets: an update on diagnosis and management of thrombocytopenic disorders. Hematology Am Soc Hematol Educ Program. 2001;282-305.
di

Tidak ada komentar:

Posting Komentar