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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Journal Amyloidosis

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