All that glitters is not gold

William Shakespeare, The Merchant of

Venice
Act II - Scene VII - Prince of Morocco
• All that glisters is not gold;
Often have you heard that told:
Many a man his life hath sold
But my outside to behold:
Gilded tombs do worms enfold.
Had you been as wise as bold,
Young in limbs, in judgement old
Your answer had not been inscroll'd
Fare you well, your suit is cold.

• The first record of this phrase is from French

theologian Alain de Lille who said "Do not hold
everything gold that shines like gold."
 CC/History of present illness

• 50 yo Caucasian female with vertigo

• Sudden onset after lunch, never happened before,
still symptomatic
• Worse with movement, associated with nausea
• “room spinning”, no tinnitus or hearing loss
• Better lying still
• Can not walk or change positions because of
symptoms
 Recent History
• Three separate clinic visits in last 9 months
for shortness of breath, cough, wheezing
attributed to allergies from cats, mold and
improved after prednisone. Has established
care in last 2 months with pulmonology and
cardiology with diagnosis of asthma, chf,
and obstructive sleep apnea
 Past history
• Medical: heart failure, asthma, sleep apnea
• Surgical: hysterectomy performed 4 days ago and
discharged home 2 days ago for dysfunctional
uterine bleed, fibroids, prior right oophorectomy
for cyst and laparotomy for adhesion and bowel
obstruction
• Social: no tobacco, alcohol or illicit drugs, works
as housewife, former furniture salesperson and
secretary, husband is trucker, 2 healthy children
• Family: mother – liver cancer, father – Alzheimer,
brother – diabetes mellitus
 Meds/allergies
• Coreg 3.125 mg bid
• Lasix 40 mg daily
• Spironolactone 25 mg daily
• Advair 250/50 1 puff bid
• Singulair 10 mg daily

• Allergy: PCN
 Exam
• 5’2” 155#, bp 120/80, hr 85, resp 16, oxygen
saturation 93% on 2L NC, temp 96.7
• Neuro: Finger to nose, dysmetric left hand, any
head movement reproduces vertigo and nausea
• HEENT: Nystagmus with leftward gaze
• Cardiac: S1S2 normal
• Pulm: clear but diminished on bases
• Extremities: trace pedal edema.
 Labs
• Wbc 15, hemoglobin 15, platelet 270 with 90%
neutrophils, 5% lymph, 5% monocytes, no bands.
• Sodium 140, potassium 2.8, chloride 98,
bicarbonate 26, BUN 7, creatinine 1, glucose 124,
calcium 8.2, magnesium 2, total protein 5.1,
albumin 2.5, total bilirubin 0.8, AST 34, ALT 50,
pro BNP 20443, troponin ranged from 0.39—
0.67—1.06—0.73, TSH 3.3, UA – 1+ protein
• Uterine pathology – cervix had some nabothian
cysts, endometrium was weakly proliferative, and
myometrium showed intravascular
leiomyomatosis, adenomyosis and adenomatoid
tumor
cxr
EKG
 Old labs
• Bnp 256
• Echo – abnormal LV systolic function with LVEF 40%,
mild mitral and aortic regurgitation, concentric LVH, mild
elevation of right ventricle systolic pressure
• Bedside spirometry – FVC 2.23 L or 73% predicted, FEV1
1.68 L or 67% predicted, FEF 25-75% is 1.28 L/s or 46%
predicted, FEV1/FVC 75% predicted, no change with
bronchodilator
• Spirometry a month ago – FVC 2.53 L or 87% predicted,
FEV1 2.12 L or 87% predicted with ratio 84% and FEF
25-75 79% predicted.
• Cardiolite – diminished LVEF 35% with global
hypokinesis and akinesis of intraventricular septum, no
wall motion abnormalities, no reversible defects to suggest
myocardial ischemia
• Aggregate apnea/hypopnea index 5.1 but during REM
sleep 19.8
 Differential diagnoses vertigo
• Peripheral: vestibular neuronitis, BPPV,
Meniere, autoimmune inner ear disease
• Central: migraine, TIA, CVA, acoustic
neuroma
 • MRI brain –
showed acute
Central left superior
cerebellar
distribution
infarct.
• MRA brain –
negative
 Other investigations
• Carotid doppler – normal
• Echo – left ventricular ejection fraction 35-40%,
moderate concentric left ventricular hypertrophy,
3+ MR, 1+ TR, grade 3 diastolic dysfunction,
echogenic speckling of ventricular myocardium,
moderate right and left atrial dilation, restrictive
filling of LV
• CT thorax – no PE, moderate bilateral pleural
effusion, right greater than left, heart enlarged, no
mediastinal adenopathy, wedge shaped
enhancement pattern on spleen, anasarca
echo
CT thorax
 Labs
• Thoracentesis – transudate, 700 mL
removed with cytology negative for
malignancy
• Negative hypercoagulability screen
• TEE showed thrombus in left atrial
appendage 1 X 3 cm.
 Differential diagnoses
cardiomyopathy
• Restrictive
• Hypertrophic
• Dilated
 Restrictive Cardiomyopathy
• Restricted LV filling with rigid LV wall
• amyloidosis, endomyocardial fibrosis
(equatorial Africa or less common in Asia
and S America), eosinophilic or Loeffler
endomyocarditis, hemochromatosis,
glycogen storage disease, treatment from
heart transplant, radiation fibrosis
 Dilated Cardiomyopathy
• LV dilation diffusely
• From alcohol, peripartum, neuromuscular
dystrophies, doxorubicin, cocaine,
Takotsubo, arrhythmogenic right ventricuar
cardiomyopathy/dysplasia, LV
noncompaction, other drugs (trastuzumab,
cyclophosphamide, imatinib)
 Hypertrophic cardiomyopathy
• Asymmetric LV hypertrophy of
interventricular septum with increased
outflow tract pressure gradient
• Risk for SCD in young athletes
• Ekg shows LVH with widespread deep
broad Q waves
Congo red staining of uterine
tissue
Congo red staining of stomach
tissue
Congo red stain of bone marrow
 Further labs
• IgA normal, IgG 269 (nl 681-1648), IgM normal
• Serum protein electrophoresis shows
hypogammaglobulinemia with immunofixation showing
small monoclonal lambda
• Urine protein electrophoresis shows monoclonal band of
free lamba light chains
• Beta 2 microglobulin 2.46 (nl 0.7-1.8)
• Kappa light chain normal, Lambda light chain 13.7 (nl
0.57-2.63)
• 24 hr urine protein 876
• Bone marrow aspirate with 12% plasma cells and biopsy
showed 10% CD38/CD138 cytoplasmic lambda
monoclonal plasma cells identified, normal cytogenetics,
multiple myeloma FISH negative for del chromosomes
13q and 17p and FGFR3/IgH and BCL-1/IgH
translocations
• LC MS on peptides from uterine tissue - AL amyloid
 Amyloidosis – deposition of
amyloid protein fibril
• AA – serum amyloid A from inflammatory
disorders, infections, occasionally neoplasms
• AL – light chain, monoclonal plasma cell disorder
similar to multiple myeloma
• AH – heavy chain
• ATTR – transthyretin, senile cardiac amyloidosis
• Cryopyrin associated periodic syndrome
• Others – renal, CNS, localized
• Diagnosis – organ biopsy, subcutaneous fat pad
biopsy, rectal mucosa biopsy
• Labs – Congo red, H and E, kappa/lambda light
chain, LC MS, B2M
 AL amyloidosis
• Renal - proteinuria
• Cardiac - diastolic/systolic dysfunction
• Nerve - Peripheral neuropathy
• GI - nausea, vomit, diarrhea, early satiety,
macroglossia, splenic involvement
• Heme - easy bruising, may develop
periorbital ecchymosis
• Skin - nail dystrophy
 Treatment
• Prognosis is worse with multisystem involvement
• Not candidate for cardiac transplant due to renal
and gastrointestinal involvement
• Not candidate for stem cell transplant due to
elevated troponin
• Melphalan and dexamethasone is good choice but
it is toxic to stem cells
• Recommendation -- bortezomib and
dexamethasone for 6 cycles at 3 wk intervals.
 References
• http://ci.yuma.az.us
• http://www.amyloidosis.org
• http://emedicine.medscape.com/
• Miller AL, Falk RH, Levy BD, Loscalzo J. A
Heavy Heart. NEJM. 2010;363:1464-9.
• Fauci AS, Braunwald E, Kasper DL, et al.
Harrison’s Principles of Internal Medicine. New
York: McGraw-Hill; 2008.
• http://en.wikipedia.org/wiki/All_that_glitters_is_n
ot_gold
ACP, Dr. Yturri
Thank you
Pathology: Drs. Sloop, Ausmus
Cardiology: Dr. Galeo
Neurology: Dr. Culcea
Office: Ms. Hansen