Hyperviscosity Syndrome

Hyperviscosity Syndrome: EM-focused highlights

By Cpt Joshua Kessler DO
(EM Resident Physician, Madigan Army Medical Center, @Kess0425) and Christopher Kang MD
(EM Attending Physician, Madigan Army Medical Center)

Edited by Alex Koyfman MD (@EMHighAK) and Stephen Alerhand MD (@SAlerhand)

 viscosity

Scenario #1

A 72 year-old male recently diagnosed with multiple myeloma presents to your Emergency Department complaining of abrupt onset of ataxia, blurry vision, and epistaxis 1 hour prior to arrival. Vital signs on initial presentation: BP 134/76, HR 84, RR 18, Temp 98.6, O2 sat 97% on RA. On physical exam he is alert and oriented x3. Visual acuities are OS 20/40 OD 20/30 and OU 20/30. He is ataxic when you ask him to ambulate. CNs II-XII are grossly intact and no horizontal or vertical nystagmus is appreciated on observation, no dysmetria on finger-to-nose, normal heel-to-shin, and negative Romberg. No other abnormalities are noted on his physical exam.

His initial laboratory work-up includes a coagulopathy study (Coags), complete blood count (CBC), complete metabolic profile (CMP), blood alcohol level (BAC), urinalysis (UA), and urinary drug screen (UDS). His CBC demonstrates a significant white blood cell count at 123,157, his CMP demonstrates a hypercalcemia at 11.0, a hypophosphatemia at 2.0, and a hyperkalemia at 5.7—otherwise his lab values are unremarkable. What diagnosis do you suspect? What additional diagnostic modalities do you want to order? How do you want to proceed with his treatment?

Scenario #2

A 65 year-old female recently diagnosed with Waldenstrom’s macroglobulinemia presents to your Emergency Department complaining of spontaneous gum bleeding, sudden vision loss, and vertigo x 1 day. Vital signs on initial presentation: BP 128/68, HR 76, RR 16, Temp 99, O2 sat 98% on RA. Her physical exam reveals blood oozing from both her lower and upper gums. Visual acuities are: OS 20/30, OD able to perceive motion, and OU: 20/30. Dix-Hallpike and HINTS exams are negative, CNs II-XII are grossly intact, and no horizontal or vertical nystagmus is appreciated on observation, no dysmetria on finger-to-nose, normal heel-to-shin, and negative Romberg. Otherwise, her physical is unremarkable.

Her initial laboratory work-up includes Coags, CBC, CMP, BAC, UA, and UDS. Her CBC demonstrates a significant white blood cell count at 101,234, her CMP demonstrates mild hypercalcemia at 12.1, hypophosphatemia at 2.3 and hyperkalemia at 5.9. What diagnosis do you suspect? What additional diagnostic modalities do you want to order to confirm this suspicion? How do you want to proceed with her treatment?

Unsurprisingly, these patients have a similar diagnosis and treatment: Hyperviscosity Syndrome (HVS).

Background

Viscosity is a property of a given liquid that describes the resistance to flow of one layer over another—how “fluid” a liquid is, how capable it is of flowing. HVS refers to the clinical sequelae that occur secondary to increased blood viscosity. This increase is usually the result of increased circulating serum immunoglobulins seen in plasma cell dyscrasias such as multiple myeloma and Waldenstrom’s macroglobulinemia, with a much more common occurrence in the latter (2-6 % vs 10-30%) [1]. However, hyperproliferative bone marrow conditions that increase circulating cellular components such as leukemias, polycythemia, and myeloproliferative conditions have also been implicated as causes of HVS. [2]

Clinical presentation

The syndrome typically presents as a triad of oro-nasal bleeding, visual changes, and neurological symptoms. Additionally, cardiovascular and pulmonary symptoms may occur. These symptoms have been noted to occur when a patient’s blood viscosity, measured in centipoise, increases from a normal range of 1.4-1.8 pg to 4-5 pg. The increase in viscosity has been correlated to several corresponding serum levels including immunoglobulin M (IgM) level >3 g/dL, IgG level >4 g/dL, and IgA level >6 g/dL. [1]

Diagnostic work-up

As an Emergency Medicine provider, you may consider several diagnostic tests if suspecting HVS. A CBC with a peripheral smear may demonstrate a rouleax formation. A CMP may demonstrate hypercalcemia, hypophosphatemia, and hyperkalemia. A total protein and albumin study may be abnormal. A coagulopathy study may reveal abnormalities. Imaging studies should include a CT and/or MRI of the brain if the patient presents with significant neurological abnormalities and/or a chest x-ray if the patient presents with additional symptoms of congestive heart failure (CHF). [2]

Treatment

Immediate therapy of symptomatic hyperviscosity is directed at reduction of blood viscosity. Plasmapheresis is the initial treatment of choice for the management and stabilization of HVS, as it is usually safe for and well tolerated by the patient. By removing the circulating excess paraproteins located in the intravascular space, serum viscosity decreases and the patient’s symptoms improve. Leukapheresis, plateletpheresis, and phlebotomy are indicated for HVS from leukostasis, thrombocytosis, and polycythemia, respectively. However, plasmapheresis and the aforementioned blood therapies are not the definitive treatment, as they do not address the underlying dysproteinemia and/or blood cell dyscrasia. Long-term management is directed at control of the underlying disease to prevent production of the monoclonal protein. A consult to a hematologist/oncologist is required. [3, 4]

 

 

References / Further Reading
  1. Bekelman J, Jackson N, Donehower R. Oncologic emergencies. 2nd ed. Philadelphia: Saunders Elsevier; 2006.
  2. Rahemtulla, A. and Chakrabartty, J. (2014) Multiple Myeloma and Hyperviscosity Syndrome, in Haematology in Critical Care: A Practical Handbook (eds J. Thachil and Q. A. Hill), John Wiley & Sons, Ltd, Oxford. doi: 10.1002/9781118869147.ch22
  3. Hemingway, T., Alexander, S., & Kupas, D. (2014, August 9). Hyperviscosity Syndrome. Retrieved May 21, 2015, from http://emedicine.medscape.com/article/780258-overview
  4. Kahn, Umar A., Carl B. Shanholtz, and Michael T. McCurdy. “Oncological Medical Emergencies.” Emergency Medicine Clinics of North America 32.3 (2014): 495-508. Print.
  5. http://www.ncbi.nlm.nih.gov/pubmed/16182994

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