Critical Care Medicine-Hematologic and Oncologic Disorders>>>>>Oncological Syndromes
Question 5#

A 63-year-old man is admitted to the ICU with epistaxis, blurred vision, and altered mental status. His medical history includes hypertension, diabetes mellitus, and Waldenstrom macroglobulinemia. Vital signs are:

His physical examination is notable for bloody nostrils, and he is somnolent but responsive to verbal stimuli. Laboratory studies are notable for:

Which one of the following interventions is not indicated at this time?

A. Red blood cell (RBC) transfusion
B. Plasma exchange
C. Endotracheal intubation
D. IV fluid therapy

Correct Answer is A

Comment:

Correct Answer: A

Hyperviscosity syndrome (HVS) is an oncological emergency characterized by the triad of neurological symptoms, visual disturbances, and mucosal bleeding and is an oncological emergency. HVS is caused by a pathological increase in serum proteins, RBCs, white blood cells (WBCs), and/or platelets, or by deformed RBCs. Waldenstrom macroglobulinemia is the most common cause of HVS. Other causes include multiple myeloma, rheumatoid disease, polycythemia, sickle cell disease, leukemia, and spherocytosis. 

Diagnosis is established by clinical evidence of elevated serum viscosity. Additional laboratory tests to confirm the diagnosis include complete blood count (CBC), peripheral blood smear, and coagulation profile. Measurement of serum immunoglobulins is not necessary for establishing diagnosis of HVS.

Timely treatment can prevent catastrophic ischemic sequalae such as myocardial infarction, stroke, thromboembolic events, and multiorgan system dysfunction. Supportive care in addition to plasma exchange or plasmapheresis is the mainstay of managing HVS. In cases where emergent plasmapheresis cannot be arranged, phlebotomy can be performed (removing 1-2 units of the patient’s blood) as a temporizing measure. Dehydration increases plasma viscosity and should be treated with IV fluids. Chemotherapy for the underlying malignancy is the definitive therapy. RBC transfusion can increase blood viscosity and worsen the thrombotic/ischemic effects.

References:

  1. Stone MJ, Bogen SA. Evidence based focused review of management of hyperviscosity syndrome. Blood. 2012;119:2205-2208.
  2. Lewis MA, Hendrickson AW, Moynihan TJ. Oncologic emergencies: pathophysiology, presentation, diagnosis, and treatment. CA Cancer J Clin. 2011;61:287-314.
  3. Perez Rogers A, Estes M. Hyperviscosity Syndrome. StatPearls [Internet]. Treasure Island, FL: StatPearls Publishing; 2018.
  4. Gertz MA. Acute hyperviscosity: syndromes and management. Blood. 2018;132:1379-1385.