She has no knowledge of blood counts done at any other time. What is the most probable etiology of the thrombocytopenia? The mother's platelet count at delivery was 52 × 109/L. The long-term consequences of thrombotic microangiopathy (thrombotic thrombocytopenic purpura and hemolytic uremic syndrome) in pregnancy. Differential diagnosis of gestational thrombocytopenia versus ITP. In approximately 15%-20% of cases, no symptoms are present. Close liaison with an obstetrician with expertise in maternal-fetal medicine is required. In patients with ITP, we consider safe for anticoagulation a platelet count stably > 50 × 109/L. A mild thrombocytopenia is relatively frequent during pregnancy and has generally no consequences for either the mother or the fetus. The thrombocytopenia of ITP is by definition an isolated hematologic abnormality, although anemia of pregnancy or iron deficiency may also be present. Until it is determined that the neonate has a safe platelet count, intramuscular injections, such as vitamin K, should be avoided. Platelet transfusion is not appropriate to prepare for spinal anesthesia; post-transfusion increments may be inadequate or short-lived and should be reserved to treat bleeding. A review of the clinical courses of 92 women with ITP during 119 pregnancies over an 11-year period found women with previously diagnosed ITP were less likely to require therapy for ITP than those with newly diagnosed ITP, although the frequency of bleeding complications did not differ between the 2 groups.18  Platelet counts at delivery were < 150 × 109/L in 89% of women. Plasma exchange is initially performed daily until the platelet count is > 150 × 109/L for at least 3 days and serum LDH concentrations have returned to normal, or nearly normal, levels. Our anesthesiologists will generally withhold spinal anesthesia for women with platelet counts < 80 × 109/L. Severe gestational (incidental) thrombocytopenia: to treat or not to treat. The patient stated she preferred epidural anesthesia, and 10 mg of prednisone daily was begun without a response. Awareness of these many causes facilitates proper diagnosis and management of thrombocytopenia in the pregnant setting. Blood 2013; 121:38. ITP is the second most common cause of an isolated low platelet count in pregnancy, accounting for ∼ 3% of women who are thrombocytopenic at delivery. A 36-year-old woman, gravida 2 para 2 (G2P2), with a history of ITP since age 28 asks whether she can safely become pregnant again. International consensus report on the investigation and management of primary immune thrombocytopenia. For individuals with a prior history of ITP, the platelet count may decline further during pregnancy and improve after delivery [20]. Neuraxial techniques in obstetric and non-obstetric patients with common bleeding diatheses. Clinical management guidelines for obstetrician-gynecologists. Clinical and laboratory features of microangiopathies of pregnancy. 2013; 121(1):38-47; The ASH 2011 evidence-based practice guideline for immune thrombocytopenia; and other sources. Other less common causes of thrombocytopenia in pregnancy … There are very few data on the proportion of preeclampsia that occurs antepartum versus postpartum, but reports of the incidence of new-onset postpartum hypertension or preeclampsia range from 0.3% to 27.5%.49  The diagnosis of eclampsia is made with the onset of seizures that cannot be attributed to other causes in a woman with preeclampsia. As a rule of thumb, developing a platelet count < 100 × 109/L early in pregnancy, with declining platelet counts as gestation progresses, is most consistent with ITP. Pregnancy in patients with idiopathic thrombocytopenic purpura: assessing the risks for the infant at delivery. Two months later, her platelet count was 127 × 109/L. Does gestational hypertension become pre-eclampsia? T.G., A.H.J., and R.S. Laboratory findings reveal evidence of microangiopathic hemolytic anemia, a negative (with rare exceptions) direct antiglobulin test, and normal coagulation tests (prothrombin time, activated partial thromboplastin time, fibrinogen and D-dimers). Postpartum plasma exchange for atypical preeclampsia-eclampsia as HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome. Although representing no threat in the majority of patients, thrombocytopenia may result from a range of pathologic conditions requiring closer monitoring and possible therapy. British guidelines suggest starting aspirin 75 mg/d and low molecular weight heparin at prophylactic doses (eg, dalteparin 5000 IU/d subcutaneously) once the platelet count is > 50 × 109/L66 ; these recommendations, however, are not routinely followed in other countries. Management of the pregnant patient with idiopathic thrombocytopenic purpura. Most cases of preeclampsia are managed entirely by the obstetrician with minimal, if any, input from the hematologist, whose role is usually confined to confirming the presence of thrombocytopenia and ruling out other possible causes of the thrombocytopenia. 2013 Jan 3. What can the patient expect during a subsequent pregnancy? For pregnancies of < 34 weeks' gestation and in which the maternal and fetal status is reassuring, glucocorticoids are recommended to accelerate fetal pulmonary maturity followed by delivery within 48 hours. Would you like email updates of new search results? The urine dipstick revealed significant proteinuria (2+). Number 6, September 1999. DITP indicates drug-induced thrombocytopenia; HIT, heparin-induced thrombocytopenia; and GT, gestational thrombocytopenia. Evaluation and management of severe preeclampsia before 34 weeks' gestation. As reported on the official package inserts (http://www.accessdata.fda.gov/scripts/cder/drugsatfda/index.cfm), although no studies have been published. HELLP should be differentiated from AFLP, which is a rare, life-threatening complication of the third trimester of pregnancy, although it is not always diagnosed before delivery. First-line therapy is similar to that of nonpregnant women with newly diagnosed ITP.6,19  We usually start with oral prednisone or prednisolone at a low dose (10 mg daily). We consider expectant management performed under close maternal and fetal surveillance, only in selected cases for no more than 48-72 hours and only in patients < 34 weeks of gestation. 2011 Apr 21;117(16):4190-207, partial 2019 update can be found in Blood Adv 2019 Dec 10;3(23):3829, correction can by found in Blood Adv 2020 Jan 28;4(2):252; Gernsheimer T, James AH, Stasi R. How I treat thrombocytopenia in pregnancy. In most cases, it is an acquired disorder because of autoantibodies neutralizing ADAMTS13 activity.65  VWF-cleaving protease deficiency can also occur in DIC, HUS, preeclampsia, and HELLP.66  However, a severe deficiency (< 5% of the activity in normal plasma) appears to be specific for TTP.67, HUS is a more heterogeneous disease. ACOG practice bulletin: Thrombocytopenia in pregnancy. This condition can be determined through an early pregnancy test as there is no specific diagnostic test to determine the cause of low platelet count in the body. Abnormalities of thyroid function are not uncommon in both ITP and pregnancy and associated with significant pregnancy-related complications and fetal risk,13  and we do this testing routinely. There has been no report of transmission of TTP to the infant. Effectiveness of combining plasma exchange with continuous hemodiafiltration on acute Fatty liver of pregnancy complicated by multiple organ dysfunction. 121(1):38-47. . We recommend plasma exchange if thrombocytopenia, hemolysis, or renal failure continues to worsen 48-72 hours postpartum and differentiating between preeclampsia/HELLP/AFLP and thrombotic thrombocytopenic purpura (TTP)/atypical hemolytic uremic syndrome that can follow a normal pregnancy becomes difficult, if not impossible. Differentiating it from gestational thrombocytopenia may be problematic, if not impossible, in the absence of prepregnancy platelet counts or a previous history of ITP, as both entities are diagnoses of exclusion. Her first child was delivered at term without complications. Ther Adv Hematol. Blood . 18 Therefore, in critically ill patients suspected of having HIT, we are disinclined to reflexively recommend suspension of heparin or … On the other hand, there is no robust evidence to suggest that neonates from women with ITP poorly responsive or refractory to treatment have a higher risk of severe thrombocytopenia. Treatment goals change with the dynamic state of parturition and in particular during delivery, when surgical risks and the neonate's passage through the birth canal need be considered. Splenectomy may induce a remission and has been reported to be associated with few complications if performed during the second trimester,29,30  when risks of anesthesia to the fetus are minimal and uterine size will not complicate the procedure. If the diagnosis is ITP or uncertain, we check the platelet count every 2-4 weeks, depending on the stability of the platelet counts. Delivery is recommended for women who do not respond to plasma exchange.66  Renal failure may require supportive care with dialysis. The effects on pregnancy outcome and on the fetus of other medications used to treat ITP are either: unknown (thrombopoietin receptor agonists), tolerable/associated with some risks but used for other indications in the setting of pregnancy (azathioprine, cyclosporine, and cyclophosphamide), or known to be teratogenic and not used in pregnancy (mycophenylate; danazol). The initial management of TTP/HUS during pregnancy does not differ from that of the nonpregnant patient. Maternal characteristics and risk of severe neonatal thrombocytopenia and intracranial hemorrhage in pregnancies complicated by autoimmune thrombocytopenia. A platelet count between 20 and 30 × 10 9 /L in a nonbleeding patient is … Intravenous anti-D as a treatment for immune thrombocytopenic purpura (ITP) during pregnancy. Limited data are available for rituximab in pregnancy, and the use of this drug for pregnancy-associated ITP cannot be recommended because of its potential for crossing the placenta.34  Short-term therapy with danazol in combination with high-dose IVIg and corticosteroids has been used for refractory thrombocytopenia in the third trimester.35  However, danazol has been observed to cause birth defects and has been designated category X by the FDA; its use should therefore be avoided. Bone marrow examination is rarely necessary to evaluate a thrombocytopenic pregnant patient and is not required to make the diagnosis of ITP. A rare inherited cause of thrombocytopenia is type IIB von Willebrand disease (VWD).11  Women with this condition may develop thrombocytopenia, for the first time, in pregnancy and be misdiagnosed with ITP. Viral screening (HIV, hepatitis C virus [HCV], hepatitis B virus [HBV]) and Helicobacter pylori testing are recommended. 1 Introduction. A platelet count should be obtained at delivery to determine the need for immediate therapy. von Willebrand factor-cleaving protease (ADAMTS13) in thrombocytopenic disorders: a severely deficient activity is specific for thrombotic thrombocytopenic purpura. Blood. FDA designated pregnancy category in brackets. What is the standard management of thrombotic microangiopathies of pregnancy? There are no published data on the use of thrombopoietin receptor agonists in pregnancy; and because their effects on the fetus are unknown, they cannot be recommended. Myers B. She has been treated with and responded to corticosteroids and IVIg in the past. The role of plasma exchange in HELLP syndrome. The use of platelet transfusions before delivery in pregnant women with ITP has been reported in 5%-18.9% of cases, reflecting not only different patient populations but also different practices.18,42. As in the nonpregnant patient, testing for antiplatelet antibodies is of no value in the diagnosis of ITP in pregnancy, it is neither sensitive nor specific, nor is it predictive of neonatal thrombocytopenia.15,16  Type 2B VWD should be included in the differential diagnosis of thrombocytopenia during pregnancy, especially in women with a personal or family history of abnormal bleeding, or if therapy for ITP is ineffective (Table 3). In the case we have described, the platelet count was assessed monthly at her routine prenatal visits. Approximately 50% of patients with AFLP meet the criteria for preeclampsia, but overlap with the HELLP syndrome is also very common. Prednisone and aspirin in women with autoantibodies and unexplained recurrent fetal loss. In unresponsive cases, we recommend the use of azathioprine. Accessibility Two clinical scenarios are particularly relevant for their prevalence and the issues relating to their management. How should delivery be managed? Preeclampsia, the HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets), and acute fatty liver of pregnancy (AFLP) have overlapping clinical and laboratory features with thrombotic microangiopathies that are not specific to pregnancy and may pose considerable diagnostic challenges (Table 5). The incidence of immune thrombocytopenic purpura in children and adults: a critical review of published reports. Estimation of the risk of thrombocytopenia in the offspring of pregnant women with presumed immune thrombocytopenic purpura. Diagnosis is largely dependent on timing of its onset, severity of the thrombocytopenia, and the association with other abnormalities. Thrombopoietin receptor agonist for treatment of immune thrombocytopenia in pregnancy: a narrative review. Counseling for women with ITP wishing to become pregnant is recommended. It develops in roughly 5% to 10% of all pregnancies and can result from a range of conditions that may or may not be unique to pregnancy. How I treat thrombocytopenia in pregnancy. Moderate to severe thrombocytopenia during pregnancy. [2] , [5] , [6] , [7] In this condition, the platelet count usually does not fall below 70 × 10 9 /L, especially during the third trimester, and the count returns to normal within 2–12 weeks of delivery. Pregnancy-related complications, such as preeclampsia, may also cause thrombocytopenia. Secondary ITP includes isolated thrombocytopenia secondary to some infections (HIV, HCV, H pylori) and to other autoimmune disorders, such as SLE. ITP is not an indication for cesarean delivery.6,19  Mode of delivery in a pregnant patient with ITP is based on obstetric indications, with avoidance of procedures associated with increased hemorrhagic risk to the fetus (eg, forceps, vacuum extraction, and fetal scalp electrode/samples).6  Most neonatal hemorrhages occur at 24-48 hours and are actually not related to trauma at the time of delivery.36,37  Determination of the fetal platelet count by fetal scalp vein blood draws or periumbilical blood sampling presents a potential hemorrhagic risk to the fetus and may inaccurately predict a low platelet count.38  For this reason, fetal platelet count measurement is not recommended. The patient denies any history of bleeding or bruising. The presence of DIC is considered a contraindication to conservative treatment. Immune thrombocytopenic purpura in pregnancy. There are no confirmatory laboratory tests of GT and the diagnosis remains one of exclusion. Abnormal laboratory findings included: Hb 14.7 g/dL, WBC 15.3 × 109/L, platelets 87 × 109/L, albumin 25 g/L. The diagnosis of AFLP is usually based on clinical and laboratory findings. There is still ongoing research to determine the reason for the lowering of platelet count in women with a normal pregnancy. Many of the agents frequently used in nonpregnant ITP patients may not be safe during pregnancy. How I treat thrombocytopenia in pregnancy. eCollection 2021. Maternity Guidelines – Thrombocytopenia in Pregnancy (GL927) May 2020 5.2.3 Postnatal: • Platelet count daily until day 2-5 • If count <20x10 9 /L or symptomatic – perform USS of brain and treat with iv IgG; • Platelet transfusion if heavy bleeding (ONLY give platelets with Others may start with a higher dose and adjust downward, but there are no published randomized trials to guide management. Her history and examination are otherwise unremarkable. A mild thrombocytopenia is relatively frequent during pregnancy and has generally no consequences for either the mother or the fetus. The fetus continued to develop normally. It is understood that, if maternal conditions deteriorate, cesarean delivery should be performed immediately. [Article in Chinese] Wang L(1), Hou M(1). Women with no bleeding manifestations and platelet counts > 30 × 109/L do not require any treatment until delivery is imminent. Transplacental passage of circulating maternal antiplatelet antibodies and the risk of neonatal thrombocytopenia are not affected by splenectomy.31-33. Her blood count showed Hb 7.7 g/dL, WBC 18.6 × 109/L, and platelets 42 × 109/L. 35 However, danazol has been observed to … 2019 Dec 14;40(12):977-979. doi: 10.3760/cma.j.issn.0253-2727.2019.12.001. The count should be performed on a cord blood sample or, preferably, a peripheral blood sample. Thrombocytopenia in Pregnancy Presented by ASH in 2013, adapted from Gernsheimer T, James AH, Stasi R, How I treat thrombocytopenia in pregnancy. Amylase and lipase values may be elevated in the setting of concomitant pancreatitis. Reversal of coagulopathy (eg, transfusion of fresh frozen plasma, cryoprecipitate, packed red blood cells, and platelets) may be required before delivery and/or in the immediate postnatal period. The frequency of hematologic monitoring was increased to weekly at 34 weeks' gestation. We discuss these problems using a case-based approach of representative clinical scenarios and examine available evidence to help guide practice. Even if the diagnosis is uncertain, the potential complications of TTP-HUS exceed by far the significant risks of plasma exchange treatment.70  A virology screen on pretreatment blood samples is necessary to exclude HIV-associated TTP, and HBV and HCV before plasma exposure. The majority of available reports are based on observational studies that either examine available epidemiologic evidence or report outcomes of a single therapeutic approach. Consistently large and/or hypogranular platelets may suggest congenital thrombocytopenia. In many reports, the clinical difference between TTP and HUS is based on the presence of acute renal failure, which is minimal or absent in the former and dominant in the latter. Clinical issues and management: a review. To support a diagnosis of GT, women should have no past history of thrombocytopenia (except during a previous pregnancy), and the thrombocytopenia should resolve spontaneously within 1-2 months after delivery. Therapy late in gestation is generally based on the risk of maternal hemorrhage at delivery (see next section). Laboratory investigations reveal several abnormalities. Her last pregnancy was complicated by a platelet count of 20 × 109/L in her third trimester requiring both corticosteroids and IVIg. Author information: (1)Department of Hematology, Qilu Hospital, Shandong University, Jinan 250012, China. Abstract A mild thrombocytopenia is relatively frequent during pregnancy and has generally no consequences for either the mother or the fetus. For women with platelet counts of 50-80 × 109/L, in whom a diagnosis of ITP cannot be excluded, we give 10 mg of prednisone once daily starting 10 days before the anticipated date of delivery. Platelet counts < 50 × 109/L occur in ∼ 10% of newborns of mothers with ITP, whereas platelet counts < 20 × 109/L occur in 5% of cases.18  Intracranial hemorrhage has been reported in 0%-1.5%.18,31,32  There are no indirect ways of measuring the fetal platelet count, and the correlation between maternal and fetal platelet counts is poor.4,31  The best predictor of severe thrombocytopenia at birth is its occurrence in an older sibling,2,43,44  Maternal response to treatment does not automatically protect the newborn from the development of thrombocytopenia. Table 1 .1Causes and Relative Incidence of Thrombocytopenia in Pregnancy *Secondary ITP includes isolated thrombocytopenia secondary to some infections (HIV, HCV, H. pylori) and to other autoimmune disorders such as systemic lupus erythematous.Reference: Adapted from Gernsheimer T, James AH, Stasi R, How I treat thrombocytopenia in pregnancy. [How I treat primary immune thrombocytopenia in pregnancy] [How I treat primary immune thrombocytopenia in pregnancy] [How I treat primary immune thrombocytopenia in pregnancy] Zhonghua Xue Ye Xue Za Zhi. A retrospective 11-year analysis of obstetric patients with idiopathic thrombocytopenic purpura. Clin Adv Hematol Oncol. Some causes of thrombocytopenia are unique to pregnancy and may not be familiar to hematologists. Comparison of platelet counts in first and second newborns of mothers with immune thrombocytopenic purpura. Platelet counts may occasionally fall to levels as low as 10-20 × 109/L at term, typically with nadir value 1-3 days before delivery, but they rapidly improve after delivery.12. If a history of prior spontaneous abortion or thrombosis is present, the patient is placed on low molecular weight heparin. The risk of spinal haematoma following neuraxial anaesthesia or lumbar puncture in thrombocytopenic individuals. Nevertheless, knowing the exact diagnosis at that stage of pregnancy changes the management very little, as will be discussed in the indications for treatment. Epub 2015 Oct 2. Although there are reports of patients with paroxysmal nocturnal hemoglobinuria continuing eculizimab during pregnancy,71,72  there is no published experience of its use for atypical HUS in pregnancy. Table 3 reports the set of laboratory tests we use in our investigation of pregnant patients with thrombocytopenia. A careful review of the peripheral blood smear remains the main diagnostic procedure. Women should also have the blood count repeated 1-3 months after delivery to assess whether spontaneous resolution of the thrombocytopenia has occurred. The VWD panel was normal. Because intrauterine fetal death may occur because of placental infarction caused by thrombosis of the decidual arterioles, serial fetal monitoring with uterine artery Dopplers should be performed. Limited data are available for rituximab in pregnancy, and the use of this drug for pregnancy-associated ITP cannot be recommended because of its potential for crossing the placenta. The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia. Screening for coagulation abnormalities (prothrombin time, activated partial thromboplastin time, fibrinogen, D-dimers), liver function tests (bilirubin, albumin, total protein, transferases, and alkaline phosphatase), antiphospholipid antibodies and lupus anticoagulant, and serologies for systemic lupus erythematosus (SLE) are done if laboratory data, history, and physical examination suggest the thrombocytopenia may be secondary. Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype. Search for other works by this author on: Management of the obstetric patient with thrombocytopenia. What testing should be ordered when a patient presents with thrombocytopenia during pregnancy? Intrauterine death may occur. How often should the patient be monitored and what will be the indication for treatment of thrombocytopenia? We treat ITP in the first and second trimesters when the patient is symptomatic with bleeding, platelet counts are < 30 × 109/L, or a planned procedure requires a higher platelet count. However, in a few patients, the low platelet count may compromise the ability to deliver epidural anesthesia and general anesthesia represents a greater risk. A Cochrane meta-analysis showed that patients receiving steroids had a significantly greater improvement in platelet counts relative to those who did not, but there was no beneficial effect on maternal death or severe maternal morbidity, or perinatal/infant death.57  Dexamethasone achieves a more rapid return of the platelet count to normal than betamethasone.57  Our approach to treating patients with severe HELLP (platelets < 50 × 109/L) is to give intravenous dexamethasone 10 mg every 12 hours for 2-4 doses antepartum and 2 more doses at 10 mg intravenously every 12 hours postpartum. Thrombocytopenia occurs commonly during pregnancy, and may result from diverse etiologies. The frequency of the exchanges is guided by blood counts and serum lactate dehydrogenase (LDH) concentrations. Liver biopsy is deferred because of its inherent risk. Intensive postpartum monitoring is necessary in women with HELLP because laboratory abnormalities frequently worsen 24-48 hours after delivery with peak rise in LDH and platelet nadir. Pregnancy outcomes after recovery from thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from an international working group. She was told her platelet count was 62 × 109/L at that time. Br J Haematol 2012; 158:3. [Differential diagnosis of thrombocytopenia in pregnancy]. Patients may also require additional antihypertensive therapy. Thrombocytopenia may be the only initial manifestation of preeclampsia. The article will focus on the clinical characteristics and management of primary immune thrombocytopenia (ITP) and its distinction from gestational thrombocytopenia, and on the thrombotic microangiopathies of pregnancy. In this review, we describe a systematic approach to the diagnosis and treatment of these disease entities using a case presentation format. Reliable predictors of neonatal immune thrombocytopenia in pregnant women with idiopathic thrombocytopenic purpura. A mild thrombocytopenia is relatively frequent during pregnancy and has generally no consequences for either the mother or the fetus. If the platelet count is normal, there is no need for repeat counts, although parents should be instructed on observation for unexplained bruising or petechiae and a thorough examination performed at 1 week. What treatments are safe during pregnancy? The most common type is pregnancy-associated thrombocytopenia (PAT), which accounts for 65% to 80% of the cases, followed by idiopathic thrombocytopenia (ITP) and hypertensive disorder in pregnancy (PIH). J Clin Apher. Pregnancy outcomes after maternal exposure to rituximab. Cohen DL, Baglin TP. A mild thrombocytopenia is relatively frequent during pregnancy and has generally no consequences for either the mother or the fetus. Although representing no threat in the majority of patients, thrombocytopenia may result from a range of pathologic conditions requiring closer monitoring and possible therapy. Other than an occasional large form, platelets should appear normal. 121(1):38-47. . Thrombocytopenia is typically mild to moderate, with approximately two-thirds of cases having platelet counts 130-150 × 109/L.

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