Prinzmetal's angina

- usually occurs at rest or while asleep

- relieved by vasodilator

What is a "fixed" stenosis?

What type of chest pain (Angina pectoris) is associated with a "fixed" stenosis?

A fixed plaque or fixed stenosis is indicated by 50% or greater coronary artery occlusion.

A stenosis becomes critical when 75% of the vessel lumen is occluded.

Stable (typical) angina is associated with fixed stenosis.

What type of chest pain (Anginia pectoris) is associated with acute plaque changes and non-occlusive thrombosis?

Unstable (crescendo or preinfarction) angina

- the most serious form of angina, bordering on infarction

Most myocardial infarcts:

A. are caused by occlusive thrombosis

B. involve the full thickness of the ventricular wall

C. Involve the left ventricle and a part of the interventricular septum

D. all of the above

D. all of the above

True or False:

Acute myocardial infarctions begin in the epicardium and progress toward the subendocardial zone.

False.

They begin in the subendocardial zone and progress towards the epicardium. The subendocardial zone is more poorly perfused to begin with.

True or False:

Cardiac injury associated with rheumatic fever occurs when a strep throat infection spreads to the heart.

False.

Cardiac injury is secondary to the strep throat infection, and is thought to be immune-mediated, not the result of direct infection.

How long after a strep throat infection could you expect to find "bread and butter" pericarditis?

10 days to 6 weeks after the sore throat

- pericarditis is part of the acute phase of rheumatic heart disease

- in this phase all 3 heart layers are involved in sterile pancarditis

What is the most common cause of mitral valve stenosis?

Chronic rheumatic valvulitis

What is the most common cause of isolated aortic stenosis in the US?

Calcific aortic stenosis

- dystrophic calcifications accumulate on valve tissue damaged from chronic wear and tear

- while valves are distorted from scarring and calcification, cusps are usually not fused

- similar changes may also affect the mitral valve

What is the most common cause of isolated mitral valve regurgitation in the US?

Mitral valve prolapse

- etiology unknown, though higher incidence with Marfan syndrome--> fibrillin gene mutation

- microscopically, valves show excessive mucopolysaccharides --> myxomatous degeneration of the mitral valve

sustained elevated blood pressure

normal < 120/80

stage 1 HTN = 140-159/90-99

stage 2 HTN >or= 160/100

Mottling with pallor

Gross findings 1-3 days post infarction

- light microscopic finding show complete coagulative necrosis and increased neutrophils

- mottling due to ruptured capillaries

What is an "onion skin" arteriole?

Is it more typical of benign or malignant hypertension?

Hyperplastic arteriosclerosis; wall of arteriole thick with reduplicated basement membrane and increased smooth muscle cells --> narrow lumen.

Onion skin/hyperplastic arteriosclerosis typical of malignant hypertension

What is hyperplastic arteriosclerosis in the kidney called?

What is hyaline arteriosclerosis in the kidney called?

hyperplastic - malignant nephrosclerosis

hyaline - benign nephrosclerosis

2-4 hours

 Where would you likely find "berry aneurisms"?

in the brain, especially at branch points in the Circle of Willis

aka Intracranial Saccular Aneurisms

Which type of lymphoma usually involves a t(8;14) translocation?

Which type usually involves a t(14;18) translocation?

t(8;14) - Burkitt lymphoma - activation of MYC oncogene

t(14;18) - Follicular lymphoma - overexpression of antiapoptosis gene BCL-2

Reed-Sternberg cell

 The malignant B cell in Hodgkin lymphoma

- This is a large cell (15-45μm in diameter) with an enlarged multilobated nucleus, exceptionally prominent nucleoli, and abundant, usually slightly eosinophilic, cytoplasm. Particularly characteristic are cells with two mirror-image nuclei or nuclear lobes, each containing a large (inclusion-like) acidophilic nucleolus surrounded by a distinctive clear zone; together they impart an owl-eye appearance. The nuclear membrane is distinct.

Which type of leukemia typically involves a t(15;17) translocation?

Which type typically involves a t(9;22)?

t(15;17) - Acute promyelocytic leukemia (FAB M3)

-PML/RARA fusion gene, DIC

t(9;22) - Chronic myelongenous leukemia (CML)

-BCR-ABL gene, massive splenomegaly

Intrinsic factor

- usual cause for B12 anemia, not nutritional deficiency

What is pica?

What disorder might it be a clinical symptom of?

Eating dirt or clay

- Iron deficiency anemia

What is pancytopenia?

In what disorder might you find it?

Pancytopenia includes anemia, thrombocytopenia (bleeding tendency), and granulocytopenia (infection, poor wound healing).

- Characteristic of marrow failure found in Aplastic anemia and leukemia

Neurologic abnormalities can occur in what type of deficiency?

A. Vitamin B12

B. Folate

C. Iron

D. All of the above

A. Vitamin B12

- due to demyelination of peripheral nerves and spinal cord

The bone marrow of someone with a megaloblastic anemia (folate or B12 deficiency) is loaded with megaloblasts.

Aplastic anemia

- No splenomegaly in this anemia

- marrow is hypocellular with increase in fat cells

Myelophthisic anemia

 - Associated with metastatic breast, lung, prostate cancers

- Teardrop-shaped red cells on a blood smear

I have: Giant metamyelocytes

                  hypersegmented macrocytes

                   hypersegmented neutrophils

                     hypersegmented granulocytes

What disease am I?

Megaloblastic anemia

(abnormally large erythroid and myeloid precursors due to folate /cobalamin deficiency)

slightly more women die from it than men, though slightly more men are diagnosed with it than women

Diffuse large cell NHL

-origin from B or T cell

I have:        B cell origin

bone marrow involvement

painless lymphadenopathy

indolent clinical course

What disease am I?

Follicular Non-Hodgkin Lymphoma

- hard to treat b/c low growth fraction

- 40% progress to diffuse B cell lymphoma

What type of lymphoma often involves MALT, such as oropharyngeal tissues?

What specific oral tissues are most often involved?

Diffuse large cell NHL

55% tonsil

30% palate

10% buccal mucosa

If bone marrow cells and circulating cells predominate or if they constitute the initial recognized manifestation of the disease, the pathologic process is termed _________.

leukemia

lymphoma

_________ lymphomas account for ~50% of all adult NHLs in the U.S.

Diffuse Large B cell

Which type(s) of Burkitt lymphoma (African/endemic or American/nonendemic) have:

a. high growth fraction

b. malignant B cells that grow in a "starry sky" pattern

both types of Burkitt lymphoma share those characteristics

differences:

- African (endemic) assoc. w/ EBV, tumor in mandible or maxilla

- American (nonendemic) not assoc. w/ EBV, tumor in abdomen

Accounts for ~80% of all childhood acute leukemias

Acute Lymphoblastic Leukemia (ALL)

Which subtype of AML is classified as FAB M5 and typically shows gingival enlargement from infiltration of leukemic cells?

Acute monocytic leukemia

The lacunar cell signals what disorder?

Nodular Sclerosis Hodgkin Lymphoma

-  lacunar cell is large, w/ single multilobate nucleus with multiple small nucleoli, abundant pale-staining cytoplasm. In formalin-fixed tissue, the cytoplasm often retracts -->appearance of cells lying in empty spaces, or lacunae.

- collagen bands divide tumor cells into nodules.

What 4 clinical syndromes are described by IHD?

angina pectoris

acute myocardial infarction

chronic ischemic heart disease

sudden cardiac death

Which coronary artery is most commonly involved in infarcts?

The least involved?

LAD > RCA > LCX > LCA

Aschoff bodies

found in Rheumatic fever

-involve all 3 layers of the heart in sterile pancarditis

-made up of Anitschkow cells

"fish mouth" or "button hole" stenosis

chronic rheumatic heart disease

- repeated inflammation and healing of valve cusps by fibrous scarring, caused by additional Strep throat infection in the adult

What is the usual cause of acute cor pulmonale?

What is the usual cause of chronic cor pulmonale?

What does the right ventricle look like in each?

acute - pulmonary embolism - right ventricle dilated

chronic - emphysema/COPD - right ventricle hypertrophied and eventually dilated

Small-Vessel Vasculitis (Arterioles, Venules, Capillaries, and Occasionally Small Arteries

-

Granulomatous inflammation involving the respiratory tract and necrotizing vasculitis affecting small vessels, including glomerulonephritis. Associated with c-ANCAs.

Giant-cell (temporal) arteritis

Large-Vessel Vasculitis (Aorta and Large Branches to Extremities, Head, and Neck)

-

Granulomatous inflammation; also frequently involves the temporal artery. Usually occurs in patients older than age 50 and is associated with polymyalgia rheumatica.

2 conditions that can result in a right to left shunt

Tetralogy of Fallot

Transposition of the great arteries

Truncus arteriosus

Hereditary spherocytosis

-disfunctional cytoskeleton proteins lead to small, round, apparently hyperchromic RBCs

-Splenectomy cures splenomegaly (and helps the anemia) but does not cure the disease

Match the disease(s) with the RBC morphology:

microcytic, hypocromic

microcytic, hyperchromic

normocytic, normochromic

macrocytic

microcytic, hypochromic: chronic blood loss anemia, thalassemias, Fe deficiency anemia

microcytic, hyperchromic:

Hereditary spherocytosis

normocytic, normochromic:

acute blood loss anemia, aplastic anemia

macrocytic: megaloblastic anemia

multiple myeloma

- renal insufficiency and recurrent infections leading causes of death

A positive immune peroxidase stain for S-100 and CD1 would indicate which white cell disease?

What marker of this disease could you see using electron microscopy?

Langerhans cell disease

- Birbeck granules