Um caso de síndrome Hellp associada a síndrome anti-fosfolípida
Palavras-chave:
trombocitopenia, pré-eclampsia, anticorpos anti-cardiolipina, síndrome anti-fosfolípida, abortos espontâneos, hemóliseResumo
Os autores apresentam o caso clínico de uma
mulher de 32 anos, com 20 semanas de gestação,
internada no Serviço de Medicina por naúseas,
vómitos, epigastralgia e trombocitopenia.
A evolução clínico-laboratorial conduziu ao
diagnóstico de síndrome HELLP, ao fim de uma
semana. Com o aparecimento de pré-eclampsia
grave e de insuficiência hepática aguda foi
pedida a interrupção terapêutica urgente da
gravidez, após o que se assistiu à normalização
dos quadros clínico e analítico.
Discute-se o diagnóstico diferencial da síndrome HELLP, no contexto de outras patologias
da gravidez, caracterizando-se a sua evolução e as atitudes terapêuticas.
A propósito deste caso, os autores fazem
uma revisão da síndrome HELLP (patologia
normalmente diagnosticada nos Serviços de
Obstetrícia) e da síndrome antifosfolípida. São
ainda tecidas considerações sobre a associação destas síndromes.
Downloads
Referências
Nagayama K, Izumi N, Miyasaka Y, Saito K, Ono K, Noguchi O et al. Hemolysis, elevated liver enzymes and low platelets syndrome
with primary anti-phospholipid antibody syndrome. Intern Med 1997;36(9):661-666.
Weinstein L. Syndrome of hemolysis, elevated liver enzymes, and low platelet count: a severe consequence of hypertension in pregnancy. Am J Obstet Gynecol 1982;142:159-167.
Pritchard JA, Weisman R Jr, Ratnoff OD, Vosburgh GJ. Intravascular hemolysis, thrombocytopenia, and other hematological abnormalities associated with severe toxemia of pregnancy. N Engl J Med 1954;250:89-98.
Sibai BM. The HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets): much ado about nothing? Am J Obstet Gynecol
;162:311-316.
Sibai BM, Ramadan MK, Usta I, Salama M, Mercer BM, Friedman AS. 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.
Wilson WA, Gharavi AE, Koike T et al. International consensus statement on preliminary classification criteria for definite antiphospholipid syndrome: report of an international workshop. Arthritis Rheum 1999;42:1309-1311.
Asherson RA, Cervera R, de Groot PG et al. Catastrophic antiphospholipid syndrome: international consensus statement on classification
criteria and treatment guidelines. Lupus 2003;12:530-534.
Harris N. Antiphospholipid syndrome. In Klippel J, Dieppe P (ed): Rheumatology. Mosby Year Book Europe Limited 1994: 6.32.1-6.
Levine JS, Branch DW, Rauch J. The Antiphospholipid Syndrome. N Engl J Med 2002; 346:752-763.
Laurell AB, Nilsson IM. Hypergamma-globulinemia circulating anti-coagulant, and biologic false-positive Wasserman reaction. J Lab Clin
Med 1957;49:694-707.
Harris EN, Chan JKA, Asherson RA, Aber VR, Gharavi AE, Hughes GRV. Thrombosis, recurrent fetal loss and thrombocytopenia. Predictive value of the anticardiolipin antibody test. Arch Intern Med 1986;146:2153-2156.
Cowchock S. Prevention of fetal death in the antiphospholipid antibody syndrome. Lupus 1996;5(5):467-472.
Knox TA, Olans LB. Liver disease in pregnancy. N Engl J Med 1996;335:569-576.
Kincaid-Smith P. The renal lesion of preeclampsia revisited. Am J Kidney Dis 1991;17:144-148.
Seligman SP, Buyon JP, Clancy RM, Young BK, Abramson SB. The role of nitric oxide in the pathogenesis of preeclampsia. Am J Obstet
Gynecol 1994;171:944-948.
Taylor RN, Varma M, Teng NNH, Roberts JM. Women with preeclampsia have higher plasma endothelin levels than women with normal
pregnancies. J Clin Endocrinol Metab 1990;71:1675-1677.
de Wolf F, Brosens I, Renaer M. Fetal growth retardation and the maternal arterial supply of the human placenta in the absence of sustained hypertension. Br J Obstet Gynaecol 1980;87:678-685.
McCrae KR, Cines DB. Thrombotic microangiopathy during pregnancy. Semin Hematol 1997;34:148-158.
Branch DW, Silver RM, Blackwell JL, Reading JC, Scott JR. Outcome of treated pregnancies in women with antiphospholipid syndrome: an
update of the Utah experience. Obstet Gynecol 1992;80:614-620.
de Boer K, Büller HR, Ten Cate JW, Treffers PE. Coagulation studies in the syndrome of hemolysis, elevated liver enzymes and low platelets.
Br J Obstet Gynaecol 1991;98:42-47.
Usta IM, Barton JR, Amon EA, Gonzalez A, Sibai BM. Acute fatty liver of pregnancy: an experience in the diagnosis and management of
fourteen cases. Am J Obstet Gynecol 1994;171:1342-1347.
Goodlin RC. Preeclampsia as the great impostor. Am J Obstet Gynecol 1991;164:1577-1581.
Hinchey J, Chaves C, Appignani B et al. A reversible posterior leukoencephalopathy syndrome. N Engl J Med 1996;334:494-500.
Chobanian AV, Bakris GL, Black HR, Cushman WC, Green LA, Izzo JL Jr, et al. The National High Blood Pressure Education Program.
Seventh Report of the Joint National Committee on Prevention, Detection, Evaluation, and Treatment of High Blood Pressure. Hypertension
;42:1206-1252.
Belfort MA, Anthony J, Saade GR, Allen JC. A comparison of magnesium sulfate and nimodipine for the prevention of eclampsia. N Engl Med 2003;348:304-311.
Martin JN Jr, Files JC, Blake PG et al. Plasma exchange for preeclampsia, I: postpartum use for persistently severe preeclampsia-eclampsia
with HELLP syndrome. Am J Obstet Gynecol 1990;162:126-137.
Martin JN Jr, Perry KG Jr, Blake PG, May WA, Moore A, Robinette L. Better maternal outcomes are achieved with dexamethasone therapy
for postpartum HELLP (hemolysis, elevated liver enzymes, and thrombocytopenia) syndrome. Am J Obstet Gynecol 1997;177:1011-1017.
Rai R, Backos M, Baxter N, Chilcott I, Regan L. Recurrent miscarriage – an aspirin a day? Hum Reprod 2000;10:2220-2223.
Rushton DI. Placental pathology in spontaneous miscarriage. In: Beard RW, Sharp F (eds). Early pregnancy loss: mechanisms and treatment. Royal College of Obstetrics and Gynecology. London 1988:149-158.
Rand JH, Wu X-X, Andree H, Lockwood CJ, Guller S, Scher J et al. Pregnancy loss in the antiphospholipid-antibody syndrome: a possible
thrombogenic mechanism. N Engl J Med 1997;337(3):154-160.
Piette J-C, Wechsler B, Frances C, Papo T, Godeau P. Exclusion criteria for primary antiphospholipid syndrome. J Rheumatol 1993;20:1802-
Nilsson IM, Astedt B, Hedner U, Berezin D. Intrauterine death and circulating anticoagulant (“antithromboplastin”). Acta Med Scand
;197:153-159.
Lockwood CJ, Romero R, Feinberg RF, Clyne LP, Coster B, Hobbins JC. The prevalence and biologic significance of lupus anticoagulant and
cardiolipin antibodies in a general obstetric population. Am J Obstet Gynecol 1989;161:369-373.
Rote NS, Lockwood C. Antiphospholipid antibodies, annexin V, and pregnancy loss. N Engl J Med 1997;337:1630-1631. (letter).
Lockshin MD, Erkan D. Treatment of the Antiphospholipid Syndrome. N Engl J Med 2003;349:1177-1179 (editorial).
Tulppala M, Marttunen M, Söderström-Anttila V et al. Low-dose aspirin in prevention of miscarriage in women with unexplained or
autoimmune related recurrent miscarriage: effect on prostacyclin and thromboxane A2 production. Hum Reprod 1997;12:1567-1572.
Erkan D, Merrill JT, Yazici Y, Sammaritano L, Buyon JP, Lockshin MD. High thrombosis rate after fetal loss in antiphospholipid syndrome:
effective prophylaxis with aspirin. Arthritis Rheum 2001;44:1466-1467.
Jilma B, Kamath S, Lip GYH. Antithrombotic therapy in special circumstances – pregnancy and cancer. BMJ 2003;326:37-40.
Farquharson RG, Quenby S, Greaves M. Antiphospholipid syndrome in pregnancy: a randomised, controlled trial of treatment. Obstet Gynecol 2002;100:408-413.
Empson M, Lassere M, Craig JC, Scott JR. Recurrent pregnancy loss with antiphospholipid antibody: a systematic review of therapeutic
trials. Obstet Gynecol 2002;99:135-144.
Kutteh WH, Ermel LD. A clinical trial for the treatment of antiphospholipid antibody-associated recurrent pregnancy loss with lower dose
heparin and aspirin. Am J Reprod Immunol 1996;35:402-407.
Laskin CA, Bombardier C, Hannah ME, Mandel FP, Ritchie K, Farewell V et al. Prednisone and aspirin in women with autoantibodies and
unexplained recurrent fetal loss. N Engl J Med 1997;337:148-154.
Cowchock FS, Reece EA, Balaban D, Branch DW, Plouffe L. Repeated fetal losses associated with antiphospholipid antibodies: a collaborative randomized trial comparing prednisone with low-dose heparin treatment. Am J Obstet Gynecol 1992;166:1318-1323.
Branch DW, Peaceman AM, Druzin M et al. A multicenter, placebo-controlled pilot study of intravenous immune globulin treatment of
antiphospholipid syndrome during pregnancy. Am J Obstet Gynecol 2000;182:122-127.
Dreyfus M, Tissier I, Baldauf JJ, Ritter J. HELLP syndrome. Review and update. J Gynecol Obstet Biol Reprod (Paris) 1997;26(1):9-15.
Reubinoff BE, Schenker JG. HELLP syndrome - a syndrome of hemolysis, elevated liver enzymes, and low platelet count - complicating
preeclampsia/eclampsia. Int J Gynaecol Obstet 1991;36(2):95-102.
Sibai BM, Taslimi MM, El-Nazer A, Amon E, Mabie BC, Ryan GM. Maternal-perinatal outcome associated with the syndrome of hemolysis,
elevated liver enzymes, and low platelets in severe preeclampsia/eclampsia. Am J Obstet Gynecol 1986;155:501-509.
Barton JR, Riely CA, Adamec TA, Shanklin DR, Khoury AD, Sibai BM. Hepatic histopathological condition does not correlate with laboratory
abnormalities in HELLP syndrome (hemolysis, elevated liver enzymes, and low platelet count). Am J Obstet Gynecol 1992;167:1538-1543.
Sibai BM, Spinnato JA, Watson DL, Hill GA, Anderson GD. Pregnancy outcome in 303 cases with severe preeclampsia. Obstet Gynecol
;64:319-325.
Martin JN Jr, Blake PG, Perry KG Jr, McGaul JF, Hess LW, Martin LW. The natural history of HELLP syndrome: patterns of disease progression
and regression. Am J Obstet Gynecol 1991;164:1500-1513.
Visser W, Wallenburg HCS. Temporizing management of severe pre-eclampsia with and without the HELLP syndrome. Br J Obstet Gynaecol 1995;102:111-117.
Magann E, Martin R, Isaacs J, Blake P, Morrison J, Martin J. Corticosteroids for the enhancement of fetal lung maturity: impact on the
gravida with preeclampsia and the HELLP syndrome. Aust N Z J Obstet Gynecol 1993;33:127-131.
Sibai BM, Ramadan MK, Chari RS, Friedman AS. Pregnancies complicated by HELLP syndrome (hemolysis, elevated liver enzymes, and
low platelets): subsequent pregnancy outcome and long-term prognosis. Am J Obstet Gynecol 1995;172:125-129.
Rüdiger G Osmers, Ekkehard Schültz, Falko Diedrich, Birgit Wehry, Thomas Krauss, Michael Oellerich, Walther Kuhn. Increased serum
levels of hialuronic acid in pregnancies complicated by preeclampsia or hemolysis, elevated liver enzymes, and low platelets syndrome. Am
J Gynecol 1998;178(2):341-345.
Iioka H. Clinical use of human hepatocyte growth factor in the early detection of HELLP syndrome. Gynecol Obstet Invest 1996;41(2):103-
Martin JN Jr, Blake PG, Perry KG Jr, McGaul JF, Hess LW, Martin LW. The natural history of HELLP syndrome: patterns of disease progression
and regression. Am J Obstet Gynecol 1991;164:1500-1513.
Branch DW, Khamashta MA. Antiphospholipid syndrome: obstetric diagnosis, management, and controversies. Obstet Gynecol
;101:1333-1344.
Erkan D, Yazici Y, Harrison MJ, Peterson M, Sammaritano L, Lockshin MD. APLASA study: primary thrombosis prevention in asymptomatic
antiphospholipid antibody (APL) patients with low-dose aspirin (ASA). Lupus 2002;11:573.
Cooper C, Choy E, Arthritis Research Campaign’s Clinical Trials Committee. The blossoming of evidence-based clinical rheumatology: the
Arthritis Research Campaign’s Clinical Trials Collaboration in association with the MRC Clinical Trials Unit, BSR and BOA. Rheumatology
;42:713-715
Ficheiros Adicionais
Publicado
Como Citar
Edição
Secção
Licença
Este trabalho encontra-se publicado com a Licença Internacional Creative Commons Atribuição 4.0.
Direitos de Autor (c) 2023 Medicina Interna
Acesso livre
