Thread by @RosenelliEM

Time for some #SpacedRepetition: @CPSolvers @DxRxEdu @rabihmgeha
Chat recap of the #ClinicalPearls #VirtualMorningReport

August 12th Episode 115: clinicalproblemsolving.com/morning-report…
w / Sonia Silinsky Krupnikova @Flower_freeland @pri_athavale
Teaching points @sukritibanthiya

Let’s start with an initial problem representation:

A 50 y/o F w/ a PMH of remote gastric bypass, anemia, MGUS & hereditary elliptocytosis p/w worsening fatigue, dyspnea on exertion, chronic chest discomfort and trouble sleeping at night

Looks like CHF

Also wondering if difficulty sleeping at night is orthopnea.

As well as if the difficulty doing tasks is due to SOB, extreme fatigue., or muscle weakness

Let's activate the dyspnea schema and connect some dots

Fatigue is a very non-specific symptom, considering a lot of things with her presentation, cardiac/resp/endocrine/MSK causes.

Let's take a look at this schema and see if we can find some overlap

What about possible ILD with how progressive the symptoms have been.

On exam, we should look for Raynaud's, dry hands (antisynthetase syndrome), JVP, edema, pallor, rashes, thyroid gland, proptosis, muscle strength.

She has a gastric bypass, I wonder if vitamin levels have been checked recently, worry about possible copper deficiency, thiamine, b12, Vit E etc.

Thiamine can cause a high output heart failure.

"Beriberi heart disease" Due to severe long-term (>3 mon) def of thiamine....

....it is more common in areas of dietary deficiency with high carbohydrate intake (such as the Far East).

In the developed world it is most frequently observed in chronic alcoholics due to poor dietary intake of thiamine, impaired thiamine absorption, metabolism and storage.

Beriberi heart disease is a cause of CHF w/ assoc elevated cardiac output, edema, fatigue, and general malaise (‘wet’ beriberi).

High output heart failure is possibly due to arteriolar &cutaneous vasodilatation leading to a🔽systemic vascular resistance. academic.oup.com/qjmed/article/…

Could also wonder about the transformation of MGUS or amyloidosis although the age would be on the younger side.

People with chronic hemolysis can also get a secondary folic acid deficiency. ncbi.nlm.nih.gov/books/NBK53537…

Now a physical exam showing her to be fatigued, pale conjunctiva, no JVD, normal heart sounds, normal lung exam, 2+ pedal edema

Neuro exam shows +tinels sign on the right

+ weakness in prox muscles 3-4/5 in UE and LE

Unable to get achilles reflexes

Let's look at conjunctival pallor and anemia

To rule in anemia with confidence, the presence of conjunctival pallor should have a likelihood ratio that is greater than 10 for predicting anemia.

ncbi.nlm.nih.gov/pmc/articles/P…

For ex typical angina has a likelihood ratio of 115 for predicting greater than 75% coronary artery stenosis
ncbi.nlm.nih.gov/pubmed/440357/

Right-lower-quadrant pain has a likelihood ratio of 3 for predicting appendicitis. ncbi.nlm.nih.gov/pubmed/6671182/

In the article cited above, the presence of pallor had a likelihood ratio of only 4.49 for predicting anemia.

W/ this likelihood ratio, if pretest probability of anemia is 50% and then finds pale conjunctivae on physical examination, the probability of anemia increases to 82%.

Now looking at her neuro and MSK exam

I wonder if she had any prev neuropathy from her MGUS that could explain the achilles réflex.

Proximal weakness makes me think thyroid, MGS, myositis. Also want to look at thiamine def, Vit E def, thyroid (would expect non-pitting edema)

Labs show nml WBC, anemia at 8.4, high plts 600, otherwise nml BMP. Kappa=14, Lambda=229

Kappa/Lamba ration of 0.06 (An abnormal FLC ratio (kappa-lambda ratio < 0.26 or > 1.65)

And an M spike in IgG

Could Waldenstrom macroglobulinemia cause the IgM spike?

Need a refresher on serum light chain ratio, "Serum free light chain ratio is an independent risk factor for progression in MGUS" ncbi.nlm.nih.gov/pmc/articles/P…

Does My Patient with a Serum Monoclonal Spike have Multiple Myeloma? ncbi.nlm.nih.gov/pmc/articles/P…

TTE: EF 35% w/ global hypokinesis, concentric LVH w/ significant diastolic dysfunction

Smells like amyloidosis. Would get urine studies and possible fat pad biopsy Or maybe a cardiac MRI

BM bx: 10-15% plasma cells, 10% Lambda secreting

And the final dx is....

Final dx: AL Amyloidosis

The term "amyloidosis" refers not to a single disease but to a collection of diseases in which a protein-based infiltrate deposits in tissues as beta-pleated sheets.

The proper nomenclature uses the letter "A" for amyloid, followed by the letter(s)..

referring to the main protein being deposited. For example, light-chain amyloidosis is "AL" ("A" for amyloid and "L" for light chain).

Transthyretin amyloidosis is "ATTR"

The most commonly diagnosed form of systemic amyloidosis is AL. acc.org/latest-in-card…

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