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Hematologic Disease in Pregnancy

Hematologic Disease in Pregnancy. Raymond Powrie, MD, FRCP(C) FACP Professor of Medicine and Obstetrics and Gynecology Alpert Medical School at Brown University Interim Chief of Medicine Women and Infants Hospital of Rhode Island Chief Medical Quality Officer

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Hematologic Disease in Pregnancy

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  1. Hematologic Disease in Pregnancy Raymond Powrie, MD, FRCP(C) FACP Professor of Medicine and Obstetrics and Gynecology Alpert Medical School at Brown University Interim Chief of Medicine Women and Infants Hospital of Rhode Island Chief Medical Quality Officer Care New England Healthcare System

  2. Incidence of Common Hematologic Problems in Pregnancy Iron Deficiency Anemia ~10% B12 Deficiency 5% Folate Deficiency 5% Hemoglobinopathies and thalassemias 5% Sickle Cell 4% Alpha thalassemia 0.8-5% Beta thalassemia 0.25-10% Thrombocytopenia <150 6.6% <100 1.2% % Von Willebrand’s 1% Hemophilia

  3. Anemia

  4. Anemia • Defined <10.5 g/dL • Hgb < 6 g/dL has clear fetal consequences

  5. Anemia

  6. Iron Deficiency Anemia • Evaluate for GI blood loss • Ferrous sulfate 325 mg two to three times a day • Supplement with vitamin C or red meat to increase absorption • Do not take with calcium, tea, coffee or wine • Expect a 0.8 g/dL/week with iron supplementation after a few weeks • Rarely a need for parenteral iron

  7. Thalassemia Hemoglobinopathies • 5% of the world’s population • Mediterranean, Africa, Indian Subcontinent, SE Asia, the Pacific

  8. Thalassemias • Hemoglobin chains NOT made so there is anemia • Hemoglobinapathies • Hemoglobin chains made with flaws so there is anemia

  9. Thalassemia

  10. Thalassemia/Hgb-opathy

  11. Hemoglobin Structure • Two Alpha globin chains from 4 genes • Two Beta globin chains from 2 genes • Thalassemia classified by • Phenotype: Clinical severity • Genotype: number of gene abnormalities • Fetuses have alternatives to Beta chains but not to alpha chain deletions

  12. Alpha thalassemia

  13. Beta Thalassemia

  14. Sickle Cell Disease

  15. Sickle Cell Trait/Disease • Increased pregnancy complications • Miscarriage: 36% versus 10% • IUFD: 6% versus 1% • Preeclampsia: 15-20% versus 5%

  16. Sickle Cell Disease • Prevention of Crises • Continuous good hydration • Folate 1 mg daily • No role for prophylactic blood transfusions

  17. Sickle Disease • Treatment of Crises • Aggressive pain control with narcotics • Hydration • Oxygen • Continued folate • Careful search for and treatment of any infection • Transfusion to achieve at least 50% normal hemoglobin if severe Chest crises have a significant mortality associated with them and should be taken very seriously

  18. Combinations • HbE 20-60% of Southeast Asians carry this gene • Hb C 3% of Africans • Hb S 12% of Africans • Hb Lepore • Significant when combined with thalassemia traits or found in homozygous states

  19. Thrombocytopenia

  20. Thrombocytopenia • <150 X 109 /L abnormal • < 80-100 X 109 /L epidural precluded • <50 X 109 /L Increased risk of surgical bleeding • <10-20 X 109 /L increased risk of spontaneous bleeding

  21. Etiology

  22. GestationalThrombocytopenia • The frequency in the largest series of consecutive women admitted for delivery is 5% (Burrows RF, Kelton JG, N Engl J med 1993; 329:1263) • Defined by the following five criteria: • Mild and asymptomatic thrombocytopenia, More than 70.000 • No past history of thrombocytopenia (except previous pregnancy) • Occurrence during late gestation • No association with fetal thrombocytopenia • Spontaneous resolution after delivery

  23. Immune (Idiopathic) Thrombocytopenia • Common acquired bleeding disorder • Two criteria required for the diagnosis: • 1. Isolated thrombocytopenia with other wise normal CBC • 2. Absence of clinically associated conditions ( e.g. SLE, APL, CLL) • Incidence is 50-60/million/year • Women of childbearing age accounts for the majority of the cases • It is not uncommon therefore for a woman to enter pregnancy with a known diagnosis of ITP or to develop de novo ITP during pregnancy itself

  24. ClinicalManifestations • Marked variability in the clinical presentation • Onset usually insidious but can be acute and abrupt • Usually asymptomatic • Bleeding usually muco-cutaneous • Petichiae, purpura, and easy bruising (Expected)) • Epistaxis, gingival bleeding , and menorrhagia Common) • GI bleeding and gross hematuria( Rare) • Intracranial hemorrhage ( Fatal, Very rare)

  25. Diagnosis • Diagnostic approach to ITP in a is the same as in non-pregnant patients. • No “gold standard” test to diagnose ITP. • Diagnosis is reached after exclusion of other causes using clinical history and exam, CBC and peripheral blood smear. • Additional lab tests to consider include testing for autoimmune disorders, and exclusion of HIV Antiplatelet • Antibody testing is not recommended by American Society of Hematology

  26. Effect Of Pregnancy On ITP • ITP accounts for 3-4% of the cases of thrombocytopenia detected in pregnancy • Pregnancy does not per sealter the natural course of ITP or increase the risk of relapse in women with previously diagnosed ITP

  27. Effect Of ITPOn Pregnancy • Thrombocytopenia in the infant can occur due to passive diffusion of autoimmune antibodies across the placenta • In one review of 10 studies of 600 pregnancies in 469 women with known ITP reported neonatal thrombocytopenia: • < 150.000 in 28% • < 50.000 in 11% • ICH in 1.2% • Maternal platelet count at delivery is not predictive of neonatal platelet • The risk of spontaneous bleeding in pregnant women is low

  28. Treatment of ITP • The goal for treatment of ITP is to provide a safe platelet count to prevent major bleeding, rather than returning the platelet count to normal • Treatment is considered if the platelet count is <30-50,000. • Some will treat for platelet counts <80,000 to allow regional anesthesia

  29. Treatment in pregnancy Prednisone is First line • 1 mg/kg/day for 7-10 days and then gradually taper taper to keep platelet above the desired threshold • The lowest possible dose should be aimed for < 10 mg daily, most likely throughout the pregnancy • Can increase the risk of GDM, HTN, maternal infection and preterm delivery but no fetal effect

  30. Treatment(Continued) IVIG (gamma globulin) • May be considered first line in third trimester • 1 g/kg ( pre pregnancy wt) daily for 2 days • Provides effective ( 80% )but temporary improvement in platelet count (usually last for 2-4 weeks) • Indicated in women with moderate or severe bleeding symptoms or whose platelet < 10,000 • Can be repeated but expensive and time consuming to administer • No effect on fetal platelet count

  31. Treatment(Continued) • Azathioprine • Is a steroid-sparing agent • used in pregnancy in conjunction with corticosteroids in women with refractory ITP Splenectomy • Laparoscopic splenectomy has been safely carried out mainly in second trimester

  32. Treatment(Continued) • High doses Methylpredisone 1 gm IV • Dexamethasone 40 g daily for 4 days • can cross the placenta with fetal effect • Rituximab Monoclonal antibody therapy • used with caution in pregnancy as it crosses the placenta and can cause temporary suppression of fetal B lymphocytes and unclear long term effect on development of fetal immune system • Platelet transfusion • for severe, symptomatic thrombocytopenia

  33. Delivery • Vaginal delivery preferable but mode of delivery should be determined by obstetric indications • Neuroaxial anesthesia: • Although the precise platelet count needed to safely perform neuroaxial analgesia is unknown, a platelet count variously given as >50.000 or > 80.000 is considered safe for epidural/spinal anesthesia/analgesia if coagulation is otherwise normal.

  34. von Willebrand’s DiseaseVWD • Most common inherited bleeding disorder: 1% of population • Quantitative or qualitative deficiency in von Willebrand factor (VWF) • VWF has two roles • Helps platelets stick to each other, to damaged tissue and to fibrinogen • Carrier protein for FACTOR VIII that keeps FACTOR VIII from being broken down prematurely

  35. VWD

  36. Bleeding Disorders History • Easy bruising especially without injury • Bleeding following dental workor surgery • Gingival bleeding • Menorrhagia Lab • CBC • aPTT, INR • PFA-100 • Bleeding time

  37. Von Willebrand’s Disease

  38. Testing for VWD

  39. Pregnancy • VWF and factor VIII levels go up in pregnancy • Patients generally fine if Ristocetin cofactor activity levels >50 IU/dL • Patients with type 2 VWD may have worsened thrombocytopenia in pregnancy

  40. Treatment in Pregnancy Review prior tests and confirm diagnosis VWD testing at booking and at 34 weeks • If >50 IU/L: • no treatment • If <50 IU/L • If Type 1 or 2A • PRN DDAVP 0.3 mcg/kg IV/SQ postpartum for vaginal delivery • Preventive DDAVP 0.3 mcg/kg IV/SQ prior to CS • If Type 2B 2N 2 M or type 3… give intermediate purity FVIII concentrate 20-40 IU/kg

  41. Treatment in Pregnancy • Inherited illness but generally high levels of FVIII and VWF in newborn

  42. Hemophilia

  43. Hemophilia Treatment • Hemophilia A

  44. Hemophilias

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