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How I treat thrombocytopenia in pregnancy

Terry Gernsheimer, Andra H. James and Roberto Stasi

Article Figures & Data

Figures

  • Figure 1

    Algorithm for workup of thrombocytopenia based on the observation of the peripheral blood film. The thrombocytopenia of ITP is by definition an isolated hematologic abnormality, although anemia of pregnancy or iron deficiency may also be present. Other than an occasional large form, platelets should appear normal. Consistently large and/or hypogranular platelets may suggest congenital thrombocytopenia. Uniformly small platelets are typically found in Wiskott-Aldrich syndrome. The presence of targeted red blood cells, schistocytes, macrocytosis, or spherocytes may be clues to liver disease, thrombotic microangiopathy, nutritional deficiencies, or autoimmune hemolysis. A direct antiglobulin test is necessary to rule out complicating autoimmune hemolysis (Evans syndrome). DITP indicates drug-induced thrombocytopenia; HIT, heparin-induced thrombocytopenia; and GT, gestational thrombocytopenia.

  • Figure 2

    Suggested approach to the management of patients with HELLP syndrome. EGA indicates estimated gestational age.

Tables

  • Table 1

    Causes and relative incidence of thrombocytopenia in pregnancy

    Pregnancy-specific
        Isolated thrombocytopenia
            Gestational thrombocytopenia (70%-80%)
        Thrombocytopenia associated with systemic disorders
            Preeclampsia (15%-20%)
            HELLP syndrome (< 1%)
            Acute fatty liver of pregnancy (< 1%)
    Not pregnancy-specific
        Isolated thrombocytopenia
            Primary immune thrombocytopenia–ITP (1%-4%)
            Secondary ITP (< 1%)*
            Drug-induced thrombocytopenia (< 1%)
            Type IIb VWD (< 1%)
            Congenital (< 1%)
        Thrombocytopenia associated with systemic disorders
            TTP/HUS (< 1%)
            SLE (< 1%)
            Antiphospholipid antibody syndrome (< 1%)
            Viral infections (< 1%)
            Bone marrow disorders (< 1%)
            Nutritional deficiency (< 1%)
            Splenic sequestration (liver diseases, portal vein thrombosis, storage disease, etc; < 1%)
    • * Secondary ITP includes isolated thrombocytopenia secondary to some infections (HIV, HCV, H pylori) and to other autoimmune disorders, such as SLE.

  • Table 2

    Differential diagnosis of gestational thrombocytopenia versus ITP

    CharacteristicGestational thrombocytopeniaITP
    Onset during pregnancyMid-late second trimester and third trimesterAnytime
    Frequency increases as term approaches
    Evidence for alternative etiologiesNoNo
    Platelet count, × 109/L> 50*Any < 100
    Progressively decreases as term approaches
    Thrombocytopenia outside of pregnancyNoPossible
    Neonatal thrombocytopeniaNoPossible
    Postpartum resolutionYesPossible
    • * Rare cases have been reported with platelet counts at term 40-50 × 109/L.

    • A total of 10% of infants have platelet counts < 50 × 109/L.

  • Table 3

    Basic laboratory evaluation of pregnant women with isolated thrombocytopenia

    Complete blood count and reticulocyte count
    Peripheral blood film
    Liver function tests
    Thyroid function tests
    Quantitative immunoglobulin level measurement
    Direct antiglobulin test
    Antiphospholipid antibodies
    ANA
    H pylori
    HIV
    HCV
    HBV
    VWD type IIB testing*
    • ANA indicates antinuclear antibody.

    • * Laboratory investigation of type IIB VWD may be indicated if there is a history of bleeding or family history of thrombocytopenia or if therapy for ITP is ineffective. First-tier tests will include VWF activity (VWF:RCo) and antigen, ristocetin-induced platelet aggregation, and multimeric analysis of VWF.

  • Table 4

    Therapeutic options for ITP in pregnancy

    First-line therapyIntravenous gammaglobulin (IVIg) oral corticosteroids
    Second line*Combination therapy with corticosteroids and IVIg
    Splenectomy (second trimester)
    Other therapeutic options
        Relatively contraindicatedAnti-D immunoglobulin [C]
    Azathioprine [D]
        Not recommended but use in pregnancy describedCyclosporine A [C]
    Dapsone [C]
    Thrombopoietin receptor agonists [C]
    Campath-1H [C]
    Rituximab [C]
        ContraindicatedMycophenolate mofetil [C]
    Cyclophosphamide [D]
    Vinca alkaloids [D]
    Danazol [X]
  • Table 5

    Clinical and laboratory features of microangiopathies of pregnancy

    FeaturePreeclampsiaHELLPAFLPaHUSTTPCAPSSLE
    Hypertension+++++++++++/−++
    Proteinuria++++++/−++++/−++++
    Nausea/vomiting+++++/−+/−+/−+/−
    Abdominal pain+/−+++++/−+/−+/−+/−
    Jaundice+/−+/−+++/−+/−+/−+/−
    Neurologic symptoms++++/−+++++
    Thrombocytopenia+++++++++++++
    Hemolysis+/−+++++++++++/−+
    Raised bilirubin+/−+++++++++++++/−+/−
    Renal impairment+/−+++++++++++
    DIC+/−++++++/−+/−+/−+/−
    Hypoglycemia+/−+/−++++/−+/−+/−+/−
    Elevated ammonia+/−+/−++/−+/−+/−+/−
    Elevated transaminases++++++++/−+/−+/−+
    Peak time of onsetThird trimesterThird trimesterThird trimesterPostpartumSecond or third trimesterAnytimeAnytime
    • CAPS indicates catastrophic antiphospholipid syndrome; +/−, rare or absent (0%-20% of cases); +, fairly common (20%-50% of cases); ++, common (50%-80% of cases); and +++, very common or constant feature (80%-100% of cases).