HELLP Sendromunun Yönetiminde Terapötik Plazma Değişiminin Etkinliğinin Değerlendirilmesi: Tek Merkez Deneyimi

Yıl 2023, Cilt: 37 Sayı: 2, 153 - 160, 06.09.2023

İbrahim Halil Açar , Birol Güvenç

https://doi.org/10.18614/deutip.1277434

Öz

HELLP sendromu, hemoliz, yüksek karaciğer enzimleri ve düşük trombosit sayısı ile karakterize, gebelikle ilişkili ciddi bir komplikasyondur. Kompleman düzensizliği, HELLP sendromunun etiyopatogenezine katkıda bulunur. Terapötik plazma değişimi (TPE), anormal kompleman yolu bileşenlerini ortadan kaldırır ve bunları normal fizyolojik bileşenlerle değiştirir. Bu çalışma, destekleyici tedavi ve kortikosteroidlere yanıt vermeyen HELLP sendromlu hastalarda TPE'nin hastalığın ilerlemesi üzerindeki etkisini değerlendirmeyi amaçladı. Bu retrospektif çalışma, Mississippi sistemine göre Sınıf 1 HELLP sendromu teşhisi konan 13 hastayı içermektedir. Bu hastalara 2012-2015 yılları arasında doğum sonrası dönemde TPE uygulandı. On üç hastadan üçü çoklu organ yetmezliğinden öldü. TPE sonrası hemoglobin ve trombosit sayıları artarken AST, ALT ve LDH seviyeleri azaldı. Bu değişiklikler istatistiksel olarak anlamlıydı (p<0.05). TPE sonrası ölen hastalarda hastaneye yatış ile TPE uygulanması arasındaki süre daha uzundu. TPE, konservatif tedaviye yanıt vermeyen kompleks doğum sonrası HELLP sendromlu hastalarda klinik sonuçları iyileştiren etkili bir tedavi stratejisidir. Bu tür vakalarda erken tanı ve TPE'nin hastalık yönetimindeki rolü giderek önem kazanmaktadır.

Anahtar Kelimeler

HELLP sendromu, terapötik plazma değişimi, klinik sonuçlar

Destekleyen Kurum

YOK

Proje Numarası

YOK

Teşekkür

Çukurova Üniversitesi Tıp Fakültesi Hematoloji kliniği ve Patoloji kliniğine teşekkür ederiz

Evaluating the Efficacy of Therapeutic Plasma Exchange in the Management of HELLP Syndrome: A Single-Center Experience

Öz

HELLP syndrome is a severe pregnancy-related complication characterized by hemolysis, elevated liver enzymes, and low platelet count. Complement dysregulation contributes to the etiopathogenesis of HELLP syndrome. Therapeutic plasma exchange (TPE) removes abnormal complement pathway components and replaces them with normal physiological components. This study aimed to evaluate the impact of TPE on disease progression in HELLP syndrome patients unresponsive to supportive therapy and corticosteroids. This retrospective study involved 13 patients diagnosed with Class 1 HELLP syndrome based on the Mississippi system. These patients underwent TPE in the postpartum period between 2012 and 2015. Of the thirteen patients, three succumbed to multiorgan failure. After TPE, hemoglobin and platelet counts increased, while AST, ALT, and LDH levels decreased. These changes were statistically significant (p<0.05). In patients who died after TPE, the duration between hospital admission and TPE initiation was longer. TPE is an effective treatment strategy that improves clinical outcomes in patients with complex postpartum HELLP syndrome who do not respond to conservative management. Early diagnosis and the role of TPE in disease management are increasingly important in such cases.

Anahtar Kelimeler

HELLP syndrome, therapeutic plasma exchange, clinical outcomes

Proje Numarası

YOK

Kaynakça

• 1.Audibert F, Friedman SA, Frangieh AY, Sibai BM. Clinical utility of strict diagnostic criteria for the HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome. Am J Obstet Gynecol. 1996;175:460–464. doi: 10.1016/S0002-9378(96)70162-X
• 2. Lieshout V, Koek GH, Spaanderman MA, Heimel R. Placenta derived factors involved in the pathogenesis of the liver in the syndrome of haemolysis, elevated liver enzymes and low platelets (HELLP): A review. Pregnancy Hypertens. 2019 Oct;18:42-48
• 3. Vaught AJ, Gavriilaki E, Hueppchen N, et al. Direct evidence of complement activation in HELLP syndrome: A link to atypical hemolytic uremic syndrome. Exp Hematol. 2016 May;44(5):390-8.
• 4. Regal JF, Burwick RM, Fleming SD. The Complement System and Preeclampsia. Curr Hypertens Rep. 2017 Oct 18; 19(11): 87.
• 5. Sibai BM, Ramadan MK, Usta I, et al. Maternal morbidity and mortality in 442 pregnancies with hemolysis, elevated liver enzymes, and low platelets (HELLP syndrome). Am J Obstet Gynecol. 1993;169:1000–1006.
• 6. Abildgaard U, Heimdal K. Pathogenesis of the syndrome of hemolysis, elevated liver enzymes, and low platelet count (HELLP): a review. Eur J Obstet Gynecol Reprod Biol. 2013 Feb;166(2):117-23
• 7. Jiang R, Wang T, Li B, He J. Clinical characteristics and pregnancy outcomes of atypical hemolysis, elevated liver enzymes, and low platelets syndrome: A case series. Medicine (Baltimore). 2020 May;99(18):e19798.
• 8. Sibai BM: The HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets): much ado about nothing? Am J Obstet Gynecol 1990, 162:311-316.
• 9. Sibai BM: Imitators of severe pre-eclampsia/eclampsia. Clin Perinatol 2004, 31:835-852.
• 10. Martin JN Jr, Rinehart BK, May WL, Magann EF, Terrone DA, Blake PG: The spectrum of severe preeclampsia: comparative analysis by HELLP (hemolysis, elevated liver enzyme levels, and low platelet count) syndrome classification. Am J Obstet Gynecol 1999, 180:1373-1384.
• 11. Haeger M, Unander M, Norder-Hansson B, Tylman M, Bengtsson A. Complement, neutrophil, and macrophage activation in women with severe preeclampsia and the syndrome of hemolysis, elevated liver enzymes, and low platelet count. Obstet Gynecol. 1992;79(1):19-26.
• 12. Mihu D, Costin N, Mihu CM, Seicean A, Ciortea R. HELLP syndrome - a multisystemic disorder. J Gastrointestin Liver Dis 2007; 16: 419- 24.
• 13. Fang CJ, Richards A, Liszewski MK, Kavanagh D, Atkinson JP. Advances in understanding of pathogenesis of aHUS and HELLP. British journal of haematology. 2008;143:336–348.
• 14. Haram K, Svendsen E, Abildgaard U. The HELLP syndrome: clinical issues and management. A Review. BMC Pregnancy Childbirth . 2009 Feb 26;9:8.
• 15. Haeger M, Unander M, Norder-Hansson B, Tylman M, Bengtsson A. Complement, neutrophil, and macrophage activation in women with severe preeclampsia and the syndrome of hemolysis, elevated liver enzymes, and low platelet count. Obstet Gynecol. 1992;79(1):19-26.
• 16. Schwartz J, Padmanabhan A, Aqui N, et al. Guidelines on the use of therapeutic apheresis in clinical practice—evidence-based approach from the Writing Committee of the American Society for Apheresis: the Seventh Special Issue. J Clin Apher. 2016;31(3):149-162.
• 17. Baxter JK, Weinstein L: HELLP syndrome: the state of the art. Obstet Gynecol Surv. 2004, 59: 838-845.
• 18. Goodlin RC: Severe pre-eclampsia: another great imitator. Am J Obstet Gynecol 1976, 125:747-753.
• 19. Pokharel SM, Chattopadhyay SK, Jaiswal R, Shakya P. HELLP syndrome--a pregnancy disorder with poor prognosis. Nepal Med Coll J . 2008 Dec;10(4):260-263.
• 20. Zeidman LA, Videnovic A, Bernstein LP, Pellar CA. Lethal pontine hemorrhage in postpartum syndrome of hemolysis, elevated liver enzyme levels, and low platelet count. Arch Neurol . 2005 Jul;62(7):1150-1153.
• 21. Fitzpatrick KE, Hinshaw K, Kurinczuk JJ, Knight M. Risk factors, management, and outcomes of hemolysis, elevated liver enzymes, and low platelets syndrome and elevated liver enzymes, low platelets syndrome. Obstet Gynecol. 2014 Mar;123(3):618-627.
• 22. Sibai BM. Diagnosis, controversies, and management of the syndrome of hemolysis, elevated liver enzymes, and low platelet count. Obstet Gynecol. 2004 May;103(5 Pt 1):981-991
• 23. American College of Obstetricians and Gynecologists Committee Opinion, Committee on Obstetric Practice. Antenatal corticosteroid therapy for fetal maturation (number 273). Obstet Gynecol 2002;99:871-873.
• 24. Schroder W, Heyl W. HELLP-syndrome. Difficulties in diagnosis and therapy of a severe form of preeclampsia. Clin Exp Obstet Gynecol. 1993;20:88–94