Goljan & First Aid Path- Vascular Disorders And Cancers (Heme/onc)

Front 1

Which apolipoprotein is involved in Chylomicron synthesis?

Back 1

Apo-B48.

Front 2

How do Chylomicrons get broken down in the periphery?

Back 2

Via Capilary Lipoprotein Lipase.

Front 3

Which apolipoprotein is involved in VLDL synthesis?

Back 3

Apo-B100.

Front 4

When CPL breaks down a VLDL molecule what are the two products that it breaks into?

Back 4

VLDL gets broken down to IDL and LDL.

Front 5

Which of the lipoproteins is the main carry of cholesterol in your body?

Back 5

LDL is the main carrier of cholesterol in your body.

Front 6

What are the functions of cholesterol in your body?

Back 6

Some of the functions include being a component of cell membranes, synthesis of Vit D, adrenal cortex hormones, and making bile salts and bile acids.

Front 7

What are the functions of HDL?

Back 7

It returns cholesterol and lipids to the liver for other lipoprotein fractions and removes cholesterol from atherosclerotic plaques for disposal in the liver.

Front 8

What are the four lipoprotein disorders that you need to know about?

Back 8

Type II, III, and IV hyperlipoproteinemia, and Apolipoprotein B deficiency.

Front 9

What is the cause of Type II Hyperlipoproteinemia? What clinical findings does this produce? How is this disease inherited.

Back 9

The cause of Type II hyperlipoproteinemia is a deficiency in LDL receptors. This leads to an increase in serum LDL. This is an autosomal dominant disorder.

Front 10

Describe the physical findings associated with Type II Hyperlipoproteinemia.

Back 10

Tendon xanthomas (cholesterol deposit located over tendons (e.g. Achilles) and extensor surfaces of joints. Xanthelasmae are also found, which are yellow raised plaques on the eyelids.

Front 11

What is the etiology of Type III hyperlipoproteinemia (familial dysbetalipoproteinemia, or "remnant disease")? Describe the clinical findings that are associated.

Back 11

Type III hyperlipoproteinemia is due to a deficiency of the Apo E protein. This results in decreased liver uptake of Chylomicron remnants and IDL, which leads to increased serum cholesterol and triglyceride, aka increased "remnants".

Front 12

What are the physical findings associated with Type III Hyperlipoproteinemia?

Back 12

Palmar xanthomas in flexor creases.

Front 13

Which serum lipoprotein is primarily raised in Type IV Hyperlipoproteinemia? Describe the etiology of this disease.

Back 13

In Type IV, there is increased VLDL. This can be due to either increased synthesis or decreased metabolism.

Front 14

Primary hypothyroidism is associated with what type of Hyperlipoproteinemia?

Back 14

Type II hyperlipoprotinemia due to decreased synthesis of LDL receptors.

Front 15

What is the most common hyper-lipid disorder and what are the acquired causes of this disorder?

Back 15

Type IV lipoproteinemia. This disorder can be acquired through excess alcohol intake (most common), Progesterone in oral contraceptives, Diabetes mellitus.

Front 16

Describe the physical findings associated with Type IV hyperlipoproteinemia.

Back 16

Eruptive xanthomas, which are yellow papular lesions.

Front 17

Describe the etiology of Apolipoprotein B deficiency (Abetalipoproteinemia). Does this produce a hyper- or hypo- lipoproteinemia?

Back 17

This disease is due to a deficiency of apoB-48 and apoB-100. This leads to a deficiency of Chylomicrons, VLDL, and LDL. This produces a hypolipoproteinemia and results in decreased serum cholesterol and triglyceride.

Front 18

Describe the clinical findings associated with Apolipoprotein B deficiency (Abetalipoproteinemia).

Back 18

Malabsorbtion due to decreased apoB-48 and apoB-100, leads to chylomicron accumulation in villi and prevents reabsorption of micelles. This accumulation and decreased lipid absorption leads to a marked decrease in Vitamin E. From this we see ataxia, and hemolytic anemia with thorny RBCs (acanthocytes)

Front 19

What type of blood vessels do we see Dystrophic Calcification in?

Back 19

Dystrophic calcification occurs in the walls of muscular arteries.

Front 20

Disrupted (inflammatory) fibrous plaques have a serum marker that is associated with them. What is this?

Back 20

C-Reactive protein.

Front 21

What are the most common sites for atherosclerosis in decreasing order?

Back 21

Abdominal aorta, Coronary artery, Popliteal artery, Internal carotid artery.

Front 22

What is the most common site for atherosclerosis in the body?`

Back 22

The Abdominal aorta

Front 23

Describe the clinical triad of a Ruptured Abdominal Aortic anuerysm.

Back 23

Left flank pain, hypotension, pulsatile mass.

Front 24

What is the most common cause of death related to Aortic dissection?

Back 24

Cardiac tamponade.

Front 25

Stasis dermatitis is sign of what?

Back 25

Deep venous thrombosis.

Front 26

What is Acute Lymphangitis usually due to?

Back 26

Acute Lymphangitis is usually due to cellulitis caused by Strep pyogenes.

Front 27

What is the webbed neck associated with Turner's syndrome due to?

Back 27

Turner's syndrome is associated with lymphatic problems. There is lymphedema of hands and feet in newborns caused by defective lymphatics. Dilated lymphatic channels in the neck (cystic hygroma) produce webbed neck.

Front 28

What types of cells and molecules do Chylous effusions contain?

Back 28

Chylomicrons with triglycerides, plus mature lymphocytes.

Front 29

What is an Angiomyolypoma and what disease is it associated with?

Back 29

This is a kidney hamartoma that consists of blood vessels, muscle tissue, and mature adipose tissue. It has an association with Tuberous Sclerosis.

Front 30

What are the three associated toxicities found to cause Liver Angiosarcoma?

Back 30

Polyvinyl chloride, Arsenic, Thorium dioxide.

Front 31

What is a benign capillary proliferation involving skin and visceral organs in AIDs pateints that simulates Kaposi sarcoma? What is the cause of this lesion?

Back 31

Bacillary angiomatosis is the condition that simulates Kaposi sarcoma, and it is caused by Bartonella Henselae (gram (-) bacili)

Front 32

What is the most common benign tumor of the liver and spleen?

Back 32

Cavernous hemangioma.

Front 33

What is a Glomus tumor and where are they found?

Back 33

This tumor is derived from arteriovenous shunts in glomus bodies, which present as painful, red subungual nodules is a digit.

Front 34

Lymphangiosarcoma arises out what setting?

Back 34

This is a malignancy of the lymphatic vessels that arises out of longstanding lymphedema such as that associated with radical mastectomy.

Front 35

What type anatomic malformation is a Spider Telangectasia?

Back 35

This is an arteriovenous fistula. It is assoicated with hyperestrenism.

Front 36

What are the neoplastic changes associated with Von Hippel-Lindau syndrome?

Back 36

Cavernous hemanigiomas of the cerebellum and retina, as well as increased incidence of pheochromocytoma and bilateral renal cell carcinoma.

Front 37

Henoch-Schonlein purpura and microscopic polyangitis are what type of vasculites?

Back 37

Small vessel vasculitis.

Front 38

Palpable purpura = what type vasculitis?

Back 38

Small vessel vasculitis.

Front 39

Polyarteritis nodosum and Kawasaki disease are examples of what types of vasculitis?

Back 39

These are Medium vessel vasculites.

Front 40

Thrombosis and infarction or aneurysm formation = what type of vasculitis?

Back 40

Medium vessel vasculitis.

Front 41

Takayasu arteritis and giant cell arteritis (temporal arteriis) = what type of vasculitis?

Back 41

Large vessel vasculitis.

Front 42

Absent pulse and stroke = what type of vasculitis?

Back 42

Large vessel arteritis.

Front 43

What is unique about the stage of vessel inflammation in Microscopic Polyangitis? What is the associated ANCA?

Back 43

All vessels are at the same stage of inflammation. This disease is associated with p-ANCA.

Front 44

What is unique about the stage of vessel inflammation in Polyarteritis Nodosum? What is the antigen that is commonly associated with this disease?

Back 44

The vessel inflammation associated with this disease is at all stages of acute and chronic inflammation. It is associated with HBsAg in 30% of cases.

Front 45

What type of cells make up the white pulp of the spleen?

Back 45

B cells.

Front 46

What are the CD markers that mark B cells?

Back 46

CD19 and CD20.

Front 47

What two organelles are particularly well developed in the plasma cell?

Back 47

RER and Golgi.

Front 48

Which MHC molecules do the CD4 and CD8 lymphocytes respond to respectively?

Back 48

MHC II and MHC I.

Front 49

What is the stimuli for EPO production?

Back 49

Stimuli for EPO production is hypoxemia, left shifted OBC and high altitude.

Front 50

Where is EPO synthesized in the kidney's?

Back 50

EPO is synthesized in the endothelial cells of the peritubular capillaries.

Front 51

Where does Extramedulllary hematopoeisis occur?

Back 51

This most commonly occurs in the liver and spleen.

Front 52

Describe the progression of hematopoesis in the fetus and the locations in the body where it occurs.

Back 52

In the fetus, hematopoesis begins in the yolk sac and subsequently moves to the liver and finally the bone marrow by the fifth to sixth months of gestation.

Front 53

What is testosterone's effect on erythropoeisis?

Back 53

It stimulates Erythropoeisis.

Front 54

If you find iron deficiency anemia along with eosinophilia what infection should you think of?

Back 54

You should think of hookworm infection: Necator americanus and Ancylostoma duodenale

Front 55

There is an acute phase reactant produced by the liver during inflammation that tells the Macrophage there, not to release iron stores into the blood. What is this molecule?

Back 55

Hepcidin.

Front 56

What are the most common causes of sideroblastic anemia in order of most to least common?

Back 56

1. Alcoholism
2. Pyridoxine (B6) deficiency
3. Lead (Pb) deficiency

Front 57

Describe the clinical findings associated with Fanconi's syndrome. What causes this?

Back 57

This syndrome includes proximal renal tubular acidosis (loss of bicarbonate in the urine), aminoaciduria, phosphaturia, and glucosuria. Pb poisoning is the cause of this.

Front 58

Why does phenytoin cause Folate deficiency, which can cause macrocytic anemia?

Back 58

Phenytoin inhibits intestinal conjugase, which is responsible for breaking down the ingested polyglutamate form of Folate to its monoglutamate form that is reabsorbed in the jejunum.

Front 59

What are 6 drugs that produce Folate deficiency?

Back 59

1) 5-Fluorouracil
2) Methotrexate
3) TMP/SMT
4) Phenytoin
5) Oral Contraceptives
6) Alcohol

Front 60

B12 is a cofactor in what reaction involved of beta-oxidation of odd chained fatty acids?

Back 60

Methylmalonyl CoA mutase.

Front 61

What region of the spinal cord is affected in B12 deficiency?

Back 61

Posterior columns- responsible for proprioception and vibratory sense

Lateral corticospinal tract- leads to dysfunction with spasticity.

Front 62

What is the most sensitive test for B12 deficiency?

Back 62

Methylmalonic acid test.

Front 63

What is the Schilling test used for?

Back 63

The Schilling test is used to localize the causes of Vitamin B12 Deficiency.

Front 64

What is the best test to use for folate deficiency screening?

Back 64

RBC folate is the best screening test.

Front 65

What types of reversible drug reactions are responsible for aplastic anemias? What about the irreversible reactions?

Back 65

Alkylating agents (reversible); Chloramphenicol (irreversible)

Front 66

Mutation in what cell structural proteins are associated with Hereditary Spherocytosis?

Back 66

Ankryn and Spectrin.

Front 67

What virus induces aplastic anemia when you have hereditary spherocytosis?

Back 67

Parvovirus B19.

Front 68

What is the treatment for Hereditary Spherocytosis?

Back 68

Splenectomy.

Front 69

What is the genetic defect in Hereditary Elliptocytosis?

Back 69

Defective Spectin band 4.1.

Front 70

What is the most common hemolytic anemia due to enzyme deficiency?

Back 70

G6PD deficiency hemolytic anemia.

Front 71

What is the most common immune hemolytic anemia?

Back 71

Autoimmune warm type hemolytic anemia is the most common type.

Front 72

What is the most common type of autoimmune hemolytic anemia?

Back 72

SLE.

Front 73

What are the most common underlying causes of secondary Cold Antibody Hemolytic Anemia?

Back 73

Mycoplasma pneumoniae, and infectious mononucleosis.

Front 74

Explain the mechanism of how Penicillin causes a hemolytic anemia.

Back 74

Drug adsorption: IgG antibodies directed against the drug attached to the RBC membrane.

Front 75

Explain the mechanism of how Quinidine causes a hemolytic anemia.

Back 75

Immunocomplex: drug-IgM immunocomplex deposits on the RBC causes intravascular hemolysis.

Front 76

Explain the mechanism of how Methyldopa causes a hemolytic anemia.

Back 76

Autoantibody induction: drug alters Rh antigens on RBCs causeing synthesis of autoantibodies against Rh antigens.

Front 77

What antibodies does an indirect Coomb's test detect?

Back 77

This detects Anti-D-antibodies.

Front 78

What is a leukoerythroblastic reaction in a woman over 50 years of age usually due to?

Back 78

This is usually due to breast cancer.

Front 79

What is a Leukoerythroblastic reaction?

Back 79

This is when immature bone marrow cells enter the blood.

Front 80

Corticosteroids can cause what type of leukocytosis?

Back 80

They can cause an Neutrophilic leukocytosis, which is defined as an absolute neutrophil count above 7000/uL.

Front 81

What are two examples of helminthic infections that produce eosinophilia? What are two examples of helminthic infections that DO NOT produce eosinophilia?

Back 81

Examples of worms that cause eosinophilia are Strongyloides and Hookworms.

Examples of worms that DO NOT cause eosinophilia are Pinworms and ADULT ascariasis, because they are not invasive.

Front 82

What are three diseases that we see eosinophilia in that are not related to hypersensitivity reactions or infections?

Back 82

Polyarteritis nodosa, Addison's disease, and Asthma.

Front 83

Why do we see eosinophilia in Hypocortisol disorders? What do you expect to see in Cushing's syndrome?

Back 83

This is because there is no sequestering of eosinophils in the lymphnodes with this condition. Cushing's syndrome (hypercortisolism) we see Eosinopenia due to corticosteroids sequestering eosinophils in the lymphnodes.

Front 84

What bacteria produces lymphocytosis promoting factor?

Back 84

Bordetella pertussis.

Front 85

What is the most sensitive test for testing for EBV? What type of antibodies are detected in the heterophile antibody screening?

Back 85

The most sensitive test is positive Anti-viral-capsid-antigen testing. The heterophile antibody test tests for IgM antibodies.

Front 86

What are two key findings that separate a chronic leukemia from an acute leukemia?

Back 86

Hepatosplenomegaly and generalized lymphadenopathy.

Front 87

Which acute leukemia is more likely to have nervous system involvement: ALL or AML?

Back 87

ALL.

Front 88

What is the most important test in diagnosing leukemia?

Back 88

Bone marrow examination.

Front 89

What differentiates acute vs. chronic leukemia?

Back 89

The bone marrow aspirate with blast count. Acute = hypercellular with >20%; Chronic = hypercellular with <10%

Front 90

Which leukemia can actually present with Thrombocytosis?

Back 90

CML.