Thrombotic Thrombocytopenic Purpura Masquerading as Hemolysis, Elevated Liver Enzymes, Low Platelets (HELLP) Syndrome in Late Pregnancy

Rehberg, Jonathan F.; Briery, Christian; Hudson, William T.; Bofill, James A.; Martin, James N.

doi:10.1097/01.aog.0000215994.25958.31

Cited by 44 publications

(22 citation statements)

References 5 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…6 In contrast, a review of 23 previously published cases of pregnancy-related TMA associated with ADAMTS13 deficiency [21][22][23][24][25][26][27][28][29][30] clearly shows this type of TMA occurs predominantly in the late second and third trimesters. This is in accordance with a recent review of 166 pregnancyrelated TTP cases in which the median time of TTP occurrence was 23 weeks.…”

Section: Discussionmentioning

confidence: 97%

Pregnancy-Associated Hemolytic Uremic Syndrome Revisited in the Era of Complement Gene Mutations

Fakhouri¹,

Roumenina²,

François³

et al. 2010

Journal of the American Society of Nephrology

408

395

View full text Add to dashboard Cite

In contrast to pregnancy-associated thrombotic thrombocytopenic purpura, the pathogenesis and presentation of pregnancy-associated atypical hemolytic uremic syndrome (P-aHUS) remain ill-defined. We conducted a retrospective study to assess the presentation and outcomes of patients presenting with P-aHUS and the prevalence of alternative C3 convertase dysregulation. P-aHUS occurred in 21 of the 100 adult female patients with atypical HUS, with 79% presenting postpartum. We detected complement abnormalities in 18 of the 21 patients. The outcomes were poor: 62% reached ESRD by 1 month and 76% by last follow-up. The risk for P-aHUS was highest during a second pregnancy. Thirty-five women, 26 (74%) of whom had complement abnormalities, had at least one pregnancy before the onset of a non-pregnancy-related aHUS. Outcomes did not differ between patients with pregnancy-related and non-pregnancy-related aHUS. Mutations in the SCR19-20 domains of factor H were less frequent in P-aHUS patients compared with non-pregnancy-related aHUS. Pregnancies in female patients with complement abnormalities (n ϭ 44) were complicated by fetal loss and preeclampsia in 4.8% and 7.7%, respectively. Better understanding of complement dysregulation in pregnancy complications is essential, especially to guide development of pharmacologic agents to modulate this system.

show abstract

Section: Discussionmentioning

confidence: 97%

Pregnancy-Associated Hemolytic Uremic Syndrome Revisited in the Era of Complement Gene Mutations

Fakhouri¹,

Roumenina²,

François³

et al. 2010

Journal of the American Society of Nephrology

408

395

View full text Add to dashboard Cite

show abstract

“…Analysis of papers published from 2002 to 2011 shows 17 detailed case-reports [8,31,[37][38][39][40][41][42][43][44][45][46], of acquired TTP where ADAMTS13 was documented (Supplementary Table 3). …”

Section: Pregnancy-associated Ttp In Women With Acquired Autoimmune Ttpmentioning

confidence: 99%

“…In 14/17 patients (82%), the TTP boot occurred during the first pregnancy including 11 patients with no antecedent of TTP and 3 patients with either previous TTP boots or autoimmunity [29,31,40,44]. In 3/17 patients (18%), the TTP boot occurred during the second or the third pregnancy including either patients with antecedent of TTP [8,41] or not [42]. At presentation, in all patients, ADAMTS13 activity was lower than 10% and associated with detectable auto-antibodies in a large majority of cases (n=12); the acquired feature of TTP was however attested by later normalization of ADAMTS13 activity in most cases.…”

Section: Pregnancy-associated Ttp In Women With Acquired Autoimmune Ttpmentioning

confidence: 99%

See 1 more Smart Citation

ADAMTS, Thrombotic Thrombocytopenic Purpura and Pregnancy

Veyradier¹,

Stépanian²,

Coppo³

2013

Hereditary Genetics

View full text Add to dashboard Cite

Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy (TMA) which pathophysiology mainly relies on a severe deficiency (either acquired or inherited) of ADAMTS13, the specific von Willebrand factor (VWF) protease. TTP is characterized by a feminine predominance and pregnancy is a precipitating factor for TTP boots. Obstetrical TTP represents at least 20% of all TTP occurring in child-bearing age women.In this review, an analyze of the English-language literature from 1955 to 2011 found about 350 cases of obstetrical TTP including about 40 case-reports/-series with well documented ADAMTS13 investigation (32 inherited and 17 acquired TTP with severe ADAMTS13 deficiency). In the 32 patients with inherited TTP, the first pregnancy was systematically associated with a TTP boot, mostly occurring during the third trimester; curative plasma therapy (PT) allowed a good maternal outcome although the fetal outcome was almost systematically bad. In the 17 patients with acquired TTP, TTP also occurred mostly de novo during the first pregnancy and after 20 weeks gestation; curative PT usually allowed a good maternal outcome and the birth of an alive baby in about 2 cases/3.The diagnosis of obstetrical TTP is challenging because it mostly occurs in women with no antecedent of TTP and it has no specific clinical/biological symptoms except a severely deficient ADAMTS13. However, because of the severity of the prognosis in the absence of urgent treatment, any thrombocytopenia +/-hemolytic anemia in a pregnant woman with no alternative diagnosis to TMA should be considered as TTP.The management of an obstetrical TTP boot consists in a blood collection for ADAMTS13 investigation followed by an emergency first-line treatment with PT yielding a maternal response rate of about 80% although the global stillbirth rate is likely to be close to 50%.The follow-up of women who recovered from an obstetrical TTP boot should include a complete ADAMTS13 investigation to distinguish between the inherited and the acquired form of TTP, in order to both estimate the risk for relapse and optimize prophylaxis indication during subsequent pregnancies. The relapse rate appears to be 100% in inherited TTP and about 20% in acquired TTP. Early prophylactic PT is thus indicated systematically in inherited TTP as it is clearly beneficial for both the mother and the fetus outcomes. In contrast, the optimal management is still debated in subsequent pregnancies of women with acquired TTP whose clinical and biological monitoring should be very careful.TTP is a very rare complication of pregnancy (about 1/100,000 pregnancies) but it is a life-threatening disease for both the mother and the fetus. Many advances have been performed in the last 10 years in terms of diagnosis and treatment. However, clear guidelines are still needed to optimize the management of subsequent pregnancies, which may be significantly different as a function of the pathophysiology for ADAMTS13 deficiency.

show abstract

“…Many papers have dealt with this question that, whether sever HELLP syndrome particularly in the postpartum period is a form of TMA or not? [5,6,9,[15][16][17][18][19]. Having detailed knowledge of natural history of each disease process and the considerable overlap of them, which make them seem to be "disease syndrome" rather than single disease, will certainly help us to distinguish these related peculiar conditions [6].…”

Section: Discussionmentioning

confidence: 99%

Management of Concurrent Severe Preeclampsia and Thrombotic Thrombocytopenic Purpura (TTP)

kheirkhah¹

2018

ICPJL

View full text Add to dashboard Cite

Thrombotic thrombocytopenic purpura (TTP) is a devastating disease involving the micro circulating (capillary wall damage) of multiple organs and could be seriously fatal if not diagnosed and treated promptly [1]. TTP is a rare complication of pregnancy with a poor prognosis and high fetal mortality, especially when it occurs during the first trimester [2]. But most of TTP cases occur in the late trimester of pregnancy. Untreated TTP is associated with more than 90% mortality rate [3]. Plasma replacement, even in pregnancy, remains as the corner stone of prevention and treatment of TTP, especially those with autoantibody-driven ADAMTS-13-deficient TTP [4].

show abstract

Thrombotic Thrombocytopenic Purpura Masquerading as Hemolysis, Elevated Liver Enzymes, Low Platelets (HELLP) Syndrome in Late Pregnancy

Cited by 44 publications

References 5 publications

Pregnancy-Associated Hemolytic Uremic Syndrome Revisited in the Era of Complement Gene Mutations

Pregnancy-Associated Hemolytic Uremic Syndrome Revisited in the Era of Complement Gene Mutations

ADAMTS, Thrombotic Thrombocytopenic Purpura and Pregnancy

Management of Concurrent Severe Preeclampsia and Thrombotic Thrombocytopenic Purpura (TTP)

Contact Info

Product

Resources

About