Case of the Month 11: Answer

This month’s winner is ablumbenberg with a very thorough answer. This case was a doozy and involved an extremely rare Emergency Department diagnosis.

To Recap: We have a 64 lady with a history of problems with her blood’s “globes” who presents with AMS, new onset CHF, and neurologic deficits.

The differential diagnosis here is quite long and well described by ablumenberg. I agree that B12 deficiency with high output cardiac failure, endocarditis, and anticholinergic toxidrome should be on the differential.

Here, I must apologize a little for making the answer so difficult, but I didn’t want to make it too easy. Would it help if I told you her blood problem was Waldenstrom’s Macroglobulinemia? Or if she had a protein gap (total protein – albutmin is > 4), or “sausage veins” on the ophthalmologic exam that we always do in the ER?

If that did help, then you have probably already determined the correct answer

– Hyperviscosity Syndrome –

 

How does this disease commonly present?

Hyperviscosity syndrome develops from increased serum proteins increasing the blood’s viscosity. Most common in Waldenstrom’s Macroglobulinemia or Multiple Myeloma, it can also happen in any syndrome with hyper-production of a blood cell lines. This increased viscosity changes blood fluid mechanics leading oftentimes to decreased blood flow to certain areas.

Unfortunately, part of the problem with both this case of the month and actual cases is that this disease presents with remarkably vague complaints. Most common is bleeding secondary to abnormal platelet function (gum bleeding/ epistaxis) or thrombosis (retinal vein or digits). Patients can also have neurologic deficits such as seizures, coma, paresthesias, vision changes, ataxia, or headache, or other manifestations such as CHF (increased plasma volume) and unexplained SOB. Vague symptoms such as abdominal pain, fatigue, and weakness can also occur. Keep hyperviscosity syndrome high on the differential for patients with unexplained SOB or AMS, especially if they have a history of diseases with elevated immunoglobulins or cell line production.

 

Diagnosis?

This is a tough one! Clinical signs such as sausage veins are helpful. True diagnosis involves testing the viscosity of the blood.Other signs include: abnormal protein electrophoresis (IgM > 4g/dL), rouleaux formation on CBC, polycythemia (Hct >60%), leukemia (WBC > 100,000/mm^3) or leukocyte count > 10%

 

Treatment?

Stat Hematology consult, IV fluids, diuresis, and plasma exchange (plasmapheresis, leukapharesis, plateletpheresis, or phlebotomy depending)

 

 

 

 

 

By Dr. Andrew Grock

 

References

emedicine

Tintinalli’s 7th ed

The MD Anderson Manual of Medical Oncology

Harrison’s Principles of Internal Medicine

 

 

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