Hematologic Disorders in Pregnancy
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Questions and Answers

What is thrombophilia?

A disorder with a genetic predisposition toward venous embolism that alters clotting factors.

What is thromboembolism?

A product of increased risk of a venous thromboembolism (VTE) in pregnancy.

Which type of thromboembolism is more common prenatally?

  • Deep vein thrombosis (DVT) (correct)
  • Pulmonary embolism (PE)
  • Which type of thromboembolism is more common during labor and delivery, and in the postpartum period?

    <p>Pulmonary embolism (PE) (A)</p> Signup and view all the answers

    What is thrombocytopenia?

    <p>A disorder of platelet alteration, resulting in a low platelet count.</p> Signup and view all the answers

    What platelet count defines thrombocytopenia?

    <p>Less than 150,000 per microliter of blood.</p> Signup and view all the answers

    What platelet count is associated with disordered bleeding?

    <p>Less than 50,000 per microliter of blood.</p> Signup and view all the answers

    What is antiphospholipid syndrome?

    <p>A disorder of abnormal antibody production that leads to thrombotic events.</p> Signup and view all the answers

    What are the clinical presentations of antiphospholipid syndrome?

    <p>Recurrent pregnancy loss (A), Severe or early-onset preeclampsia (B), Abnormal fetal growth without explanation (C), Unexplained clot formation (D)</p> Signup and view all the answers

    What management strategies are implemented for antiphospholipid syndrome in pregnancy?

    <p>Monthly fetal growth ultrasounds (A), Twice-weekly testing at term (B), Low-molecular-weight heparin (LMWH), heparin, or low-dose aspirin (C)</p> Signup and view all the answers

    When should fetal surveillance begin for inherited thrombophilias?

    <p>At 36 weeks gestation.</p> Signup and view all the answers

    What hemoglobin and hematocrit values are likely indicative of poor volume expansion and are associated with poor outcomes?

    <p>Hemoglobin &gt; 15 g/dL (A), Hematocrit &gt; 45% (B)</p> Signup and view all the answers

    What is extremely high hemoglobin or hematocrit associated with for the fetus?

    <p>Intrauterine growth restriction (IUGR).</p> Signup and view all the answers

    What are the consequences of anemia in pregnancy?

    <p>Maternal death (A), Increased preterm birth (B), Pregnancy loss (C), Fetal growth restriction (D)</p> Signup and view all the answers

    What type of anemia results from an RBC enzyme defect or deficiency that is episodic, induced by drugs, infections, or surgery?

    <p>Glucose-6-phosphate dehydrogenase (G6PD) deficiency.</p> Signup and view all the answers

    What is the hereditary pattern for Glucose-6-phosphate dehydrogenase (G6PD) deficiency?

    <p>X-linked, meaning it is more common in males.</p> Signup and view all the answers

    Which drugs can induce G6PD deficiency?

    <p>Nitrofurantoins (A), Analgesics (pain relievers) (B), Antimalarial compounds (C), Antipyretics (fever reducers) (D), Sulfonamides (E)</p> Signup and view all the answers

    What type of anemia results from genetically based structural defects in erythrocyte membranes, causing RBCs to be spherical in shape?

    <p>Hereditary spherocytosis.</p> Signup and view all the answers

    What is the inheritance pattern for hereditary spherocytosis?

    <p>Autosomal dominant, with variable penetrance.</p> Signup and view all the answers

    What differentiates hereditary spherocytosis from G6PD deficiency?

    <p>Hereditary spherocytosis is not dependent on a provoking agent or exposure.</p> Signup and view all the answers

    What type of anemia is a result of a genetic based defect in the polarization of hemoglobin molecules, making RBCs contract and the axis becomes rod-shaped?

    <p>Hereditary elliptocytosis.</p> Signup and view all the answers

    What is the inheritance pattern for hereditary elliptocytosis?

    <p>Autosomal dominant.</p> Signup and view all the answers

    What is more common in G6PD and how often should screening occur?

    <p>Every trimester (A), Urinary tract infections (UTIs) (B)</p> Signup and view all the answers

    What is the management for G6PD?

    <p>Physician consultation (A), Discontinuation of the suspected exposure (B), Avoid oxidant medications (sulfonamides, nitrofurantoin) (C)</p> Signup and view all the answers

    What is the clinical presentation for hereditary spherocytosis?

    <p>Acute hemolytic crisis that can be precipitated by infection, trauma, or pregnancy.</p> Signup and view all the answers

    What diagnoses are used in hereditary spherocytosis aside from family history?

    <p>Osmotic fragility test (A), Hyperproliferative anemia (changes in CBC, reticulocytes) (B)</p> Signup and view all the answers

    What is the prenatal care management for hereditary spherocytosis?

    <p>Folate supplementation (A), Hemolytic crisis monitoring (B)</p> Signup and view all the answers

    What is the clinical presentation for hereditary elliptocytosis?

    <p>Mild hemolytic state, similar to spherocytosis.</p> Signup and view all the answers

    What diagnostics are used for hereditary elliptocytosis aside from family history?

    <p>Hyperproliferative anemia (changes in CBC, blood smear) (A), Osmotic fragility test (B)</p> Signup and view all the answers

    What type of hemoglobinopathy is hereditable but does not always result in pathology, where normal hemoglobin synthesis occurs at a slower rate?

    <p>Thalassemia syndromes (quantitative changes in hemoglobin production).</p> Signup and view all the answers

    What type of hemoglobinopathy is hereditable but does not always result in pathology, where a genetic alteration affects the amino acid content in the hemoglobin molecule?

    <p>Structural hemoglobinopathies (sickle cell, Hb C, Hb E - qualitative changes in hemoglobin structure).</p> Signup and view all the answers

    What hemoglobin variants are present in the embryo?

    <p>Hb Gower II (A), Hb Gower I (B)</p> Signup and view all the answers

    What hemoglobin variants are present in the fetal period?

    <p>HbF (most abundant) (A), Hb Portland (B)</p> Signup and view all the answers

    What is there a disordered synthesis of in the hemoglobin molecule for thalassemia syndromes?

    <p>Alpha or beta peptide chains.</p> Signup and view all the answers

    What thalassemia results in the fetus being severely affected (hydrops or stillbirth), and females are typically sterile?

    <p>Alpha major thalassemia.</p> Signup and view all the answers

    What thalassemia results in minimal to moderate hypochromic microcytic anemia in pregnancy, and the fetus should be monitored for hydrops?

    <p>Alpha minor thalassemia.</p> Signup and view all the answers

    What thalassemia results in a healthy neonate who declines after 6 months, and females are typically sterile?

    <p>Beta major thalassemia.</p> Signup and view all the answers

    What thalassemia is the most common in NM practice, and typically maternal and fetal outcomes are satisfactory?

    <p>Beta minor thalassemia.</p> Signup and view all the answers

    What beta thalassemia syndrome results in severe disease, fractures, and bone distortion, along with hepatosplenomegaly?

    <p>Homozygous beta thalassemia major (Cooley's Anemia).</p> Signup and view all the answers

    What beta thalassemia syndrome has severity dependent on beta chain production, and may be asymptomatic?

    <p>Heterozygous beta thalassemia minor.</p> Signup and view all the answers

    What will be seen for hemoglobin, MCV, and iron supplementation in beta thalassemia minor?

    <p>Hemoglobin 8-10 g/dL (A), Low MCV (&lt;80 fL) (B), Unresponsive to iron supplementation (C)</p> Signup and view all the answers

    What are the general principles for management of thalassemia syndromes in pregnancy?

    <p>Prophylactic iron and folate (A), Second and third trimester antenatal fetal assessment (B), IUGR and oligohydramnios monitoring (C)</p> Signup and view all the answers

    What is the most common presentation for thalassemia disorders in pregnancy?

    <p>Low serum hemoglobin (A), Low MCV (B)</p> Signup and view all the answers

    What is there an increased risk for with sickle cell disease in pregnancy?

    <p>Preterm labor (PTL) (A), Intrauterine growth restriction (IUGR) (B)</p> Signup and view all the answers

    What are symptoms of sickle cell disease?

    <p>Vascular occlusion (A), Hemolysis (red blood cell destruction) (B), Increased susceptibility to infection (C)</p> Signup and view all the answers

    What supplementation is needed for individuals with sickle cell disease in pregnancy?

    <p>Folic acid supplementation.</p> Signup and view all the answers

    What is there an increased risk for amongst individuals with sickle cell trait in pregnancy?

    <p>Pyelonephritis (kidney infection) (A), Urinary tract infections (UTIs) (quarterly screening) (B), Asymptomatic bacteriuria (C)</p> Signup and view all the answers

    What determines an individual's genetic hemoglobin makeup and helps identify hemoglobinopathies?

    <p>Hemoglobin electrophoresis.</p> Signup and view all the answers

    What is the clinical implication for the RBC antibody "Duffy" in pregnancy?

    <p>The fetus dies (hemolytic anemia).</p> Signup and view all the answers

    What is the clinical implication of the RBC antibody "Rh(c)" in pregnancy?

    <p>Hemolytic disease of the newborn (similar to Rh(D)?)</p> Signup and view all the answers

    Flashcards

    thrombophilia

    A genetic disorder increasing risk for venous embolism by altering clotting factors.

    thromboembolism

    A condition causing increased risk of venous thromboembolism (VTE) during pregnancy.

    DVT

    Deep vein thrombosis, more common during pregnancy.

    PE

    Pulmonary Embolism, more common in labor and postpartum patients.

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    thrombocytopenia

    A disorder characterized by low platelet count.

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    < 150000

    The platelet count threshold for diagnosing thrombocytopenia.

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    < 50000

    Platelet count where disordered bleeding becomes likely.

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    antiphospholipid syndrome

    A disorder of abnormal antibody production that results in thrombotic events.

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    recurrent pregnancy loss

    Clinical presentation for antiphospholipid syndrome involving multiple miscarriages.

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    36 weeks

    When fetal surveillance should begin for inherited thrombophilias.

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    hemoglobin > 15

    High hemoglobin levels that may indicate poor volume expansion in pregnancy.

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    IUGR

    Intrauterine growth restriction linked to high hemoglobin or hematocrit in fetuses.

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    glucose 6 phosphate deficiency

    Anemia caused by an enzyme defect in red blood cells, triggered by various factors.

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    autosomal dominant

    Hereditary pattern for disorders like hereditary spherocytosis.

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    hereditary spherocytosis

    Anemia resulting from structural defects in erythrocyte membranes, causing spherical RBCs.

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    hemolytic crisis

    Clinical presentation in hereditary spherocytosis, triggered by infections or stress.

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    thalassemia syndromes

    Hereditary blood disorders causing abnormal hemoglobin production, leading to various symptoms.

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    beta major

    A form of thalassemia causing severe disease in neonates and infertility in females.

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    sickle cell disease

    An inherited disorder leading to sickle-shaped red blood cells and various health complications.

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    hemoglobin electrophoresis

    Test to identify hemoglobin types and detect hemoglobinopathies.

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    RBC antibodies in pregnancy

    Antibodies like Duffy and Kell can hemolyze red blood cells in newborns.

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    folic acid supplementation

    Necessary support for individuals with sickle cell disease during pregnancy.

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    UTIs in G6PD

    More common in G6PD deficiency, with recommended trimester screenings.

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    maternal death risk

    Increased risk in pregnancy with conditions like anemia or sickle cell disease.

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    hyperproliferative anemia

    Anemia marked by changes in complete blood count and reticulocytes.

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    prophylactic iron and folate

    Recommended to prevent anemia in thalassemia during pregnancy.

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    low MCV

    Common presentation in thalassemia disorders indicating microcytic anemia.

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    Hemolytic Disease of Newborn

    A condition where antigens cause issues in newborns, often linked to maternal Rh incompatibilities.

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    Study Notes

    Hematologic Disorders in Pregnancy

    • Thrombophilia: Genetic disorder increasing risk of venous thromboembolism (VTE). Alters clotting factors.
    • Thromboembolism: Increased risk of VTE in pregnancy.
    • DVT (Deep Vein Thrombosis): More common prenatally, of thromboembolisms.
    • PE (Pulmonary Embolism): More common in labor and postpartum patients, of thromboembolisms.
    • Thrombocytopenia: Disorder of platelet alteration with low platelet count.
    • Thrombocytopenia Platelet Count: < 150,000/µL.
    • Disordered Bleeding Thrombocytopenia Platelet Count: < 50,000/µL.
    • Antiphospholipid Syndrome: Disorder of abnormal antibody production causing thrombotic events.
    • Antiphospholipid Syndrome Clinical Presentation: Recurrent pregnancy loss, severe/early-onset preeclampsia, abnormal fetal growth, unexplained clot formation.
    • Antiphospholipid Syndrome Management in Pregnancy: Monthly fetal growth ultrasounds, twice-weekly testing at term, low-molecular-weight heparin (LMWH), heparin, and low-dose aspirin.
    • Inherited Thrombophilia Fetal Surveillance: Begin at 36 weeks gestation.
    • High Hemoglobin and Hematocrit: Values > 15 g/dL and > 45%, respectively. These indicate poor volume expansion and adverse pregnancy outcomes.
    • High Hemoglobin/Hematocrit Fetal Outcome: IUGR (Intrauterine Growth Restriction).
    • Anemia Consequences in Pregnancy: Fetal growth restriction, pregnancy loss, maternal death, increased preterm birth.
    • Glucose-6-Phosphate Deficiency Anemia: RBC enzyme deficiency causing episodic anemia triggered by certain drugs, infections, or surgery.
    • Glucose-6-Phosphate Deficiency Inheritance: X-linked (more common in males).
    • Inducing Drugs for Glucose-6-Phosphate Deficiency: Sulfonamides, nitrofurantoins, antipyretics, analgesics, antimalarial compounds.
    • Hereditary Spherocytosis Anemia: Genetic erythrocyte membrane defect leading to spherical RBCs.
    • Hereditary Spherocytosis Inheritance: Autosomal dominant (variable penetrance).
    • Hereditary Spherocytosis Feature: Not dependent on provoking agent/exposure.
    • Hereditary Elliptocytosis Anemia: Genetic defect in hemoglobin molecule polarization, resulting in rod-shaped RBCs.
    • Hereditary Elliptocytosis Inheritance: Autosomal dominant.
    • G6PD Screening Frequency: Monthly in pregnancy.
    • G6PD Common Presentation: UTIs (Urinary Tract Infections), in the third trimester.
    • G6PD Management: Discontinuation of suspected exposures, physician consultation, avoiding oxidant medications.
    • Hereditary Spherocytosis Clinical Presentation: Acute hemolytic crisis (precipitated by infections, trauma, pregnancy).
    • Hereditary Spherocytosis Diagnostics: Hyperproliferative anemia (CBC changes, reticulocytes), osmotic fragility test.
    • Hereditary Spherocytosis Prenatal Care: Hemolytic crisis monitoring, folate supplementation.
    • Hereditary Elliptocytosis Clinical Presentation: Mild hemolytic state (similar to spherocytosis).
    • Hereditary Elliptocytosis Diagnostics: Hyperproliferative anemia (CBC changes, blood smear), osmotic fragility test.
    • Thalassemia Syndromes: Hereditary hemoglobinopathies with quantitatively abnormal hemoglobin synthesis.
    • Structural Hemoglobinopathies: Hereditary hemoglobinopathies with qualitatively abnormal hemoglobin structure.
    • Embryonic Hemoglobin Variants: Hb Gower I, Hb Gower II.
    • Fetal Hemoglobin Variants: Hb Portland, HbF (most abundant).
    • Adult Hemoglobin Variants: HbA1 (most prevalent), HbA2, HbF (some remains).
    • Thalassemia Syndrome Disorder: Disordered synthesis of either alpha or beta peptide chains.
    • Alpha Thalassemia Major: Severe fetal outcome (hydrops, stillbirth), female sterility.
    • Alpha Thalassemia Minor: Minimal to moderate hypochromic microcytic anemia; fetal monitoring for hydrops.
    • Beta Thalassemia Major: Healthy neonate, decline after 6 months, female sterility.
    • Beta Thalassemia Minor: Common in practice; generally favorable maternal and fetal outcomes.
    • Beta Thalassemia Major (Clinical Presentation): Severe disease, fractures, bone distortion, hepatosplenomegaly.
    • Beta Thalassemia Minor (Clinical Presentation): Variable severity based on beta chain production, potentially asymptomatic.
    • Beta Thalassemia Minor Bloodwork: Hemoglobin 8–10 g/dL, low MCV (< 80%), unresponsive to iron supplementation.
    • Thalassemia Syndrome Management in Pregnancy: Antenatal fetal assessment, IUGR/oligohydramnios monitoring, prophylactic iron and folate.
    • Thalassemia Common Presentation: Low serum hemoglobin, low MCV.
    • Sickle Cell Disease Risks in Pregnancy: Preterm labor, IUGR (fetal inheritance a concern).
    • Sickle Cell Disease Symptoms: Hemolysis, vascular occlusion, increased susceptibility to infection.
    • Sickle Cell Disease in Pregnancy Supplementation: Folic acid supplementation.
    • Sickle Cell Trait Risks in Pregnancy: Asymptomatic bacteriuria, urinary tract infection (frequent screening), pyelonephritis.
    • Hemoglobin Electrophoresis: Determines genetic hemoglobin makeup, helps identify hemoglobinopathies.
    • Duffy Antibody implication: Death (hemolytic anemia)
    • Kell Antibody implication: Kills (hemolytic anemia)
    • Lewis Antibody implication: Lives
    • Rh(c) Antibody implication: Hemolytic disease of newborn

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    Explore the critical hematologic disorders that can occur during pregnancy, including thrombophilia, thrombocytopenia, and antiphospholipid syndrome. This quiz tests your knowledge on the causes, risks, and management strategies for these disorders. Perfect for medical students and healthcare professionals.

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