Critical Care Medicine-Hematologic and Oncologic Disorders>>>>>Platelet Disorders
Question 4#

A 32-year-old male presents to the hospital complaining of progressive fatigue, productive cough, and intermittent epistaxis. His initial evaluation is significant for left lower lobe consolidation on chest X-ray and a temperature of 38.7°C. During attempts to obtain peripheral venous access, he bruises easily and missed attempts bleed for over 2 minutes. A CBC and coagulation studies are obtained and are as follows:

He is subsequently admitted to the ICU where broad spectrum antibiotics are initiated, and a bone marrow aspirate (BMA) is obtained, which shows finding consistent with acute myeloid leukemia.

Which of the following is the best initial treatment option for this patient?

A. Platelet transfusion
B. All-trans-retinoic acid (ATRA)
C. Packed RBC transfusion
D. Administer IV Vitamin K and Fresh Frozen Plasma

Correct Answer is B

Comment:

Correct Answer: B

This patient most likely has Acute promyelocytic leukemia (APL), a variant of Acute Myelogeneous Leukemia (AML) given his presentation with bleeding diathesis and the elevated PT and aPTT with BMA findings of AML. APL can cause acute Disseminated Intravascular Coagulopathy (DIC), which may be life-threatening. DIC is a consumptive coagulopathy; activation of the clotting cascade results in the development of thrombi, which in turn activate the fibrinolytic pathway resulting in accumulation of fibrin degredation products (FDP). FDP inhibits further fibrin clot formation and bleeding ensues. 

It is critical to start treatment with ATRA without delay as soon as the diagnosis is suspected, and before definitive confirmation of the diagnosis has been made. If the diagnosis is not confirmed, ATRA can be discontinued and treatment changed to that used for other types of AML. Transfusion of blood products, specifically platelets may be ineffective owing to the high rate of platelet consumption caused by DIC. Administration of red blood cells may be indicated for patients experiencing large volume blood loss, although this is rarely seen in the absence of trauma. Fresh Frozen Plasma may be given to replenish clotting factors and Vitamin K can cause an increase in Vitamin-K–dependent clotting factors that can further assist in reversing the coagulopathy. However, the initial therapy that can be lifesaving in these patients is administration of ATRA.

References:

  1. Tallman MS, Altman JK. How I treat acute promyelocytic leukemia. Blood. 2009;114(25):5126-5135.
  2. Park JH, Qiao B, Panageas KS, et al. Early death rate in acute promyelocytic leukemia remains high despite all-trans retinoic acid. Blood. 2011;118(5):1248-1254. doi:10.1182/blood-2011 to 04 to 346437. Epub 2011 Jun 8.
  3. Carlson KB. Acute leukemia. In: Hall JB, Schmidt GA, Kress JP, eds. Principles of Critical Care. 4th ed. New York, NY: McGraw-Hill; 2014:chap 29.