Intern report pearls 5/5: myeloproliferative disorders and acute liver failure

When you see elevations in all cell lines of your CBC, think about a primary bone marrow process (myeloproliferative disorder) or an extra-medullary process!

  • For the primary myeloproliferative disorders:
    • Polycythemia vera = elevated RBCs (Hgb > 18.5 in men, >16.5 in women) (these patients can also have thrombocytosis and leukocytosis, which can have a worse prognosis!)
    • Essential thrombocytosis = clonal elevation of platelets (>450K and reactive thrombocytosis r/o)
    • Other things to think about: Myelofibrosis, CML, MDS
  • Often JAK2 positive
  • For both, at risk for bleeding and clotting!! Clots can be arterial or venous!! Also at risk of ischemia (eg strokes) from hyperviscosity
  • Treatment for PCV:
    • Phlebotomy to maintain Hgb<15 + low dose ASA
    • If symptomatic, high risk of thrombosis or progressive myeloproliferation (splenomegaly, leukocytosis, thrombocytosis) à cytoreductive therapy (hydroxyurea as first line, interferon if contraindications to hydroxyurea)
    • If thrombosis à anticoagulate! (UNLESS they have mesenteric ischemia from thrombosis – want to r/ GIB before starting anticoagulation if worried about this!_

 

When you see polycythemia, check JAK2 and EPO levels. High JAK2 suggests a primary myeloproliferative process; high epo suggests a secondary process. See the diagram below!

 

pcv

Thanks to Izzy for sharing 5 malignancies associated with high epo levels:

  1. HCC
  2. RCC
  3. Uterine fibroids
  4. Hemangioblastomas
  5. Thymoma

 

Acute liver failure

  • See this blog post for old M&M pearls on this!
  • 3 criteria for diagnosis: 1) no pre-existing liver disease, 2) INR>1.5, 3) encephalopathy
    • Remember there are 4 grades of hepatic encephalopathy (that you can look up!):
      • Grade I – change in behavior, sleep cycle reversal, hyperreflexia
      • Grade II – disorientation, lethargy, asterixis
      • Grade III – marked confusion, obtundation
      • Grade IV – comatose, unresponsive, loss of reflexes
    • Ammonia levels can be helpful in prognosis!
    • Most common cause of mortality is cerebral edema leading to herniation!! Minimize this with elevating head of bed, hypertonic saline, mannitol.
      • Other things to watch out for: high risk of infection, risk of hypoglycemia (from impaired gluconeogenesis), electrolytes, renal dysfunction, bleeding

 

Indications to TIPs – typically, used to treat major consequences of portal HTN (variceal hemorrhage, ascites); also can consider for Budd Chiari (though indication is less clear). Remember that this increases the risk of hepatic encephalopathy!!

 

Evernote: https://www.evernote.com/shard/s34/sh/138b708b-373c-4c97-b233-cb6d42510858/377e510ac9cd602229ee560e83127a05

 

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