In clinic, you meet a new patient who just transferred clinics due to insurance. You are struck by how tan his skin seems to be after noticing the difference between the younger picture of himself in his chart as a younger man. To your surprise, he mentions he has not been able to see the Phlebotomist as often as he usually does.
a. Based on the following liver pathology sample stained in Prussian Blue, what genetic condition does this patient likely have? Name the clinical finding you noticed on his skin. Hereditary Hemochromatosis/Bronze Diabetes
b. Looking through his chart, you find there is also a significant piece of family history. You notice previous work ups and note the findings of a cardiac biopsy report, show below. Name the disease process and one major cardiac clinical finding. Familial Amyloidosis + Arrythmia/Heart Failure
This section of myocardium demonstrates amorphous deposits of pale pink material between myocardial fibers. This is characteristic for amyloid.
c. You also see this second pathology report. What type of staining did they do and summarize the findings.
A Congo red stain has been performed on the myocardium in a case of amyloidosis. The amyloid stains orange-red, but with polarized light, the amyloid has an "apple-green" birefringence as seen here.
d. What disease process do these two conditions have in common? Restrictive Cardiomyopathy
e. What the pathophysiology behind this disease process? Name one of two.
-Decreased ventricular compliance
-Diastolic dysfunction type of LHF
f. Name two other etiologies of this disease process
Most Common:
-Amyloidosis,
-Radiation
-Myocardial fibrosis after open heart surgery
Infiltrative
-Hemochromatosis
-Pompe's glycogenosis
Endocardial Fibroelastosis in a child
-thick fibroelastic tissue in the endocardium
Sarcoidosis
Systemic Sclerosis
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