Journal Amyloidosis
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Transcript of Journal Amyloidosis
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Journal Presentationcase records
of the
massachusetts general hospital
An 80-Year-Old Man with
Shortness of Breath, Edema, andProteinuria
Fathia Rachmatina
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Present History
An 80-year-old man
was admitted to the hospital because of
shortness of breath, pleuraleffusions, and
edema of the legs.
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Past History
Atrial fibrillation had developed seven
years earlier, with bradycardia and
syncope,
a pacemaker had been placed. Angina
developed two and a half years before
admission and was treated with three-
vessel coronary-artery bypass grafting.
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urinalysis revealed 3+ proteinuria, an
increase from 1+ one year earlier. Nine
months before admission, a subtotal
colectomy was performed because of
ischemic colitis with bleeding.
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Bilateral pleural effusions, pulmonary
edema, and cardiomegaly were noted on
chest radiography.
Five months before admission, an
increase in exertional fatigue and
shortness of breath developed. A chest
radiograph showed small pleural effusions,diffuse irregular opacities, cardiomegaly,
and pulmonary venous hypertension.
.
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The patient received treatment with
furosemide, and there was improvement in
his symptoms and radiographic findings
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A fine-needle aspiration biopsy of an
abdominal fat pad was performed; a
Congo red stain for amyloid was negative.
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Habitual History
He did not drink alcohol,smoke cigarettes,.
He had not traveled recently, was retired,
and lived with his wife
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Family and Medication History
Both parents and several uncles had had
coronary artery disease. His medications
were warfarin sodium and furosemide
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Physical Examination
He appeared fatigued, with increased
respiratory effort. The temperature was
36.1C, the pulse was irregular at 77 beats
per minute, the respiratory rate was 22breaths per minute. The blood pressure
was 80/40 mm Hg. The oxygen saturation
was 93 percent while the patient wasbreathing two liters of oxygen with the use
of a nasal cannula
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The tongue was enlarged and had a
whitish shallow ulceration on the right side.
The neck was supple with a jugular
venous pressure of 7 cm of water.
Therewere decreased breath sounds two
thirds of the way up the posterior chest on
the right side and halfway up on the leftside, with dullness to percussion
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There were irregular first and second heart
sounds. The abdomen was normal, and
there was pitting edema (+++) of the legs
from the feet to the knees
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Laboratory FindingsTable 1. Laboratory Data.*
Variable On AdmissionGlucose (mg/dl) 119
Sodium (mmol/liter) 130
Potassium (mmol/liter) 3.8
Chloride (mmol/liter) 93
Carbon dioxide (mmol/liter) 32
Urea nitrogen (mg/dl) 29
Creatinine (mg/dl) 0.9
Calcium (mg/dl) 8.4
Phosphorus (mg/dl) 3.5
Magnesium (meq/liter) 1.8
Protein (g/dl) 6.8Albumin 3.0
Globulin 4.3
Bilirubin
(mg/dl)
Direct 0.2
Total 0.5
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Chest X-Ray
Figure 1. Chest Radiograph.
Two weeks before admission, a
posterioranterior chest
radiograph revealed a large right-sided
pleural effusion
extending to the level of the right hilum, a
small left-sided
pleural effusion, pulmonary venous
hypertension,
and persistent diffuse irregular
parenchymal opacities.
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Transthoracic
Echocardiogram.
RV
RV
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Figure 2. Transthoracic Echocardiogram.
A four-chamber view shows mild symmetric left ventricular
hypertrophy, left atrial enlargement, thickening of the
mitral and tricuspid valves, and a small pericardial effusion
(Panel A) (RV right ventricle, LV left ventricle, LA left
atrium, RA right atrium). A four-chamber view shows
moderate mitral regurgitation (MR) (Panel B). Pulsedwave
Doppler evaluation of the mitral inflow gives information
about the diastolic filling properties of the left
ventricle and is expressed by two waves (Panel C).The E
wave corresponds to rapid early diastolic filling of the left
ventricle, whereas the A wave corresponds to atrial contraction.
(The A wave is not present in this patient because
of the presence of atrial fibrillation.) When leftventricular diastolic pressure is elevated, the equalization
of pressures between the ventricular and atrial
chambers is rapid, and the deceleration time (dec time)
of early transmitral filling is shortened. A deceleration
time of less than 150 msec indicates a restrictive filling
pattern. In this patient, the deceleration time was 120 msec
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Diagnosis
Amyloidosis, AL type.
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Anatomical Diagnosis
Systemic amyloidosis involving the heart
and colon,in the setting of a monoclonal
gammopathy; probably AL amyloidosis
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Organ Dysfunction in Systemic
Amyloidosis The kidney is the most frequent site of
amyloid fibril deposition in both
immunoglobulin light chain (AL)
amyloidosis and serum amyloid A (AA)
amyloidosis,and this condition is typically
manifested as the nephrotic syndrome, aswe see in this patient.
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The proteinuria can be massive, and the
accompanying edema can be resistant to
diuretics, as in this case. The glomerular
filtration rate may be normal, butprogressive renal impairment typically
follows unless new amyloid production can
be reduced or eliminated
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Table 3. Clinical or Laboratory Findings in This PatientThat Might Be Due to Amyloidosis.
Kidney
Nephrotic syndrome
Heart
Restrictive cardiomyopathy
Valve thickening
Conduction-system abnormalities
Gastrointestinal tract
Colonic bleeding or ischemia
Liver
Elevation of alkaline phosphatase and aminotransferase
levels
Lung
Refractory pleural effusions
Parenchymal opacifications
Autonomic nervous system
Hypotension
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Pathological Discussion
The presence of amyloid in tissue is not
always readily apparent, and pathologists
frequently do not recognize it on
examination of sections routinely stainedwith hematoxylin and eosin. Examination
of slides from the resected colon revealed
the presence of amyloid in the walls ofblood vessels in the submucosa and
serosa
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Figure 3. Right Ventricular Endomyocardial-Biopsy Specimen.
There is amorphous extracellular material present in a vascular
distribution (Panel A, hematoxylin and eosin). With Congo
red staining (Panel B), the deposits have a pinkorange color; under
plane-polarized light (Panel C), the deposits display
apple-green birefringence, which is indicative of the presence of amyloid.
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