Platelet Disorders

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Platelet Essentials - Hemostasis Heroes

  • Small, anucleated cells derived from bone marrow megakaryocytes. Lifespan: 7-10 days. Normal count: 1.5 - 4.5 lakhs/µL.
  • Key players in primary hemostasis: forming the initial platelet plug.
  • Contain α-granules (vWF, fibrinogen) & dense granules (ADP, Ca²⁺, serotonin).
  • Key surface receptors: GPIb (binds vWF for adhesion), GPIIb/IIIa (binds fibrinogen for aggregation). Platelet structure, activation, and functions

⭐ Glanzmann thrombasthenia results from a deficiency or dysfunction of the GPIIb/IIIa receptor, leading to impaired platelet aggregation.

Thrombocytopenia: Causes & Approach - Count Catastrophe

  • Platelets < 150,000/μL. Severe: < 50,000/μL. Bleed risk ↑ if < 10,000-20,000/μL.
  • Always rule out 📌 Pseudothrombocytopenia (EDTA clumping) with citrate tube/smear.
  • Major Mechanisms & Causes:
    • ↓ Production: Marrow disorders (aplasia, leukemia, MDS), B12/folate def., drugs, viral (HIV).
    • ↑ Destruction:
      • Immune: ITP, HIT, drugs, SLE.
      • Non-immune: DIC, TTP-HUS, sepsis, HELLP.
    • Sequestration: Splenomegaly (e.g., cirrhosis).
    • Dilutional: Massive transfusion.
  • Approach Algorithm:

⭐ ITP is a diagnosis of exclusion: isolated thrombocytopenia, normal marrow (if done), no other cause. Often follows viral illness in children.

Immune Thrombocytopenias - Self Sabotage

FeatureITPHITDrug-Induced
MechanismAnti-GpIIb/IIIa autoAbsAbs vs PF4-heparinDrug-dependent Abs
OnsetInsidious/Acute5-10 days post-heparinAcute post-drug
Platelets<30k/µL often>50%; rarely <20k/µLVariable, severe
HallmarkIsolated thrombocytopeniaThrombosis risk; 📌 4Ts ScoreDrug link; resolves on stop
DiagnosisExclusion; Anti-platelet Abs4Ts; HIT Ab assayDrug Hx
TreatmentSteroids, IVIG, TPO-RAsStop heparin; DTI (e.g. Argatroban)Stop drug; supportive

⭐ HIT: prothrombotic despite thrombocytopenia due to Ab-mediated platelet activation.

MAHAs & Consumptive Coagulopathies - Fragmented Frenzy

RBC fragmentation (schistocytes) & thrombocytopenia define Microangiopathic Hemolytic Anemias (MAHAs). Schistocytes and platelets in MAHA peripheral blood smear

  • Comparison:
    FeatureTTPHUSDIC
    Cause↓ADAMTS13 (<10%)Shiga toxin (E.coli)Sepsis, trauma, malignancy
    Clinical📌 FAT RN Pentad (Fever, Anemia, Thrombocytopenia, Renal, Neuro)Triad (Anemia, Thrombocytopenia, AKI - esp. kids)Bleeding & thrombosis
    CoagsNormalNormal↑PT/PTT, ↑D-dimer, ↓Fibrinogen
    RxPlasma Exchange (PEX)SupportiveTreat cause, FFP, Cryo

⭐ In TTP, initiate plasma exchange emergently upon suspicion, pre-ADAMTS13 results.

Platelet Dysfunction & Thrombocytosis - Sticky & Swarming

  • Platelet Dysfunction (Qualitative)
    • Inherited:
      FeatureGlanzmann's (GT)Bernard-Soulier (BSS)
      DefectGpIIb/IIIa (Aggregation)GpIb (Adhesion)
      InheritanceARAR
      Ristocetin Agg.Normal↓ / Absent
      Platelet SizeNormalGiant (📌 Big Suckers)
    • Acquired: Uremia, drugs (Aspirin, Clopidogrel). Platelet aggregation curves: Glanzmann vs Bernard-Soulier
  • Thrombocytosis (Quantitative): Platelet count > 450,000/μL.
    FeatureReactiveClonal (ET)
    CauseInfection, inflammation, Fe defJAK2 mut. (MPN)
    Platelet Func.NormalOften Abnormal
    SplenomegalyNo (or from cause)Possible
    TreatmentTreat causeCytoreduction, Aspirin

⭐ BSS: Absent Ristocetin aggregation. Giant platelets. Defect in GpIb (vWF receptor).

High‑Yield Points - ⚡ Biggest Takeaways

  • ITP: Isolated thrombocytopenia from anti-GpIIb/IIIa antibodies. First-line: steroids.
  • TTP: ADAMTS13 deficiency; Pentad (Fever, MAHA, Thrombocytopenia, Renal, Neuro). Plasma exchange is crucial.
  • HUS: Often post-E. coli O157:H7; Triad (MAHA, Thrombocytopenia, ARF). Primarily supportive.
  • Bernard-Soulier Syndrome: GpIb defect (adhesion); Giant platelets, thrombocytopenia.
  • Glanzmann Thrombasthenia: GpIIb/IIIa defect (aggregation); Normal count, impaired aggregation.
  • von Willebrand Disease (vWD): Most common inherited bleeding disorder; ↓vWF, ↓FVIII. Desmopressin for Type 1.
  • DIC: Consumptive coagulopathy; ↓platelets, ↓fibrinogen, ↑PT/APTT, ↑D-dimer. Treat underlying cause.

Practice Questions: Platelet Disorders

Test your understanding with these related questions

Which of the following statements best describes the underlying mechanism of heparin-induced thrombocytopenia?

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Flashcards: Platelet Disorders

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The most common presentation of heparin-induced thrombocytopenia is _____.

TAP TO REVEAL ANSWER

The most common presentation of heparin-induced thrombocytopenia is _____.

venous thrombosis

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