MOFFITT INTERN REPORT PEARLS 6/9/16: HIT, APLS, and Evans Syndrome!

Thanks to Alayn for presenting the case of a young woman with lupus who developed anemia and thrombocytopenia after biopsy and cryoablation of a renal mass! Some diagnoses that we discussed were HIT, APLS, and Evans Syndrome!

HIT: Remember the 4T score!

  • Thrombocytopenia
    • Typical fall >50% of baseline value
    • BUT platelet level usually stays >20 (not super low)
  • Timing
    • Typical timing 5-10 days after heparin exposure
    • Exception: If previously exposed within 100 days, fall expected <24 hours after re-exposure
  • Thrombosis
    • Clinical/imaging evidence of thrombosis
      • Skin necrosis, stroke, etc
  • oTher
    • No other cause for platelet drop readily apparent

HIT management:

  • If suspect HIT, stop all heparin including heparin flushes!
  • Send PF4 antibody if intermediate or high probability by 4Ts score.
  • Controversial whether to send SRA or other functional assay.
  • Anticoagulate with argatroban or other agent since HIT is a prothrombotic disorder!
  • Recommended to continue anticoagulation x 2-3 months even if no thrombus!
  • Lifetime avoidance of heparin recommended.

APLS:

  • When to suspect APLS?
    • Unexplained venous or arterial thrombosis in young patient.
    • Unexplained adverse pregnancy outcomes.
    • Unexplained thrombocytopenia or prolonged PTT.
  • Antibody workup if suspected
    • Anti-cardiolipin ab
    • Anti-beta2-glycoprotein ab
    • Lupus anticoagulant (Russell viper venom and/or PTT)
  • Sapporo criteria: 1 clinical + 1 laboratory criteria for diagnosis
    • Clinical criteria
      • Thrombosis (arterial or venous)
      • Pregnancy morbidity
    • Laboratory criteria
      • Lupus anticoagulant on two or more occasions 12 wks apart
      • Anticardiolipin ab on two or more occasions 12 wks apart
      • Anti-beta2-glycoprotein ab on two more occasions 12 wks apart
  • Clinical manifestations
    • Thrombosis
    • Pregnancy complications
    • Thrombocytopenia
    • Pulmonary HTN
    • Valve thickening and Libman-Sacks endocarditis
    • Livedo reticularis
    • Catastrophic APLS = widespread thrombotic disease with multiorgan failure
  • Treatment
    • Anticoagulation (indefinite duration if APLS dx confirmed)
    • Hydroxychloroquine if underlying SLE
    • Catastrophic APLS: steroids, plasma exchange, IVIG

 

Evans Syndrome

  • Combination of AIHA + ITP
  • 15% of patients also have autoimmune neutropenia (pancytopenia)
  • Associated with infections (HCV, HIV), SLE, lymphoproliferative disorders
  • Often resistant to standard therapies (steroids, IVIG, splenectomy)
  • Rituximab may be helpful
  • Often chronic relapsing course

 

Evernote: https://www.evernote.com/shard/s272/sh/18330b80-3ca7-4862-a4d1-907a8a9aa440/f42845742ccf268f33657a9ea62386f5

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