USMLE Step 2: HEME/ONC Flashcards

1
Q

Effects of Heparain

A

Increases PTT, activates antithrombin III and affects the INTRINSIC pathway, and decreases fibrinogen.

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2
Q

Antidote for heparin

A

Protamine Sulfate

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3
Q

Effects of Warfarin

A

Increases PT, extrinsic pathway

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4
Q

How is the intrinsic pathway measured?

A

PTT

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5
Q

How is intrinsic pathway initiated?

A

By exposure of collagen following vascular trauma

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6
Q

how is extrinsic pathway measured?

A

by PT

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7
Q

How is extrinsic pathway initiated?

A

By endothelium-produced tissue factor

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8
Q

intrinsic pathway

A

12, 11, 9, 8

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9
Q

Intrinsic pathway

A

7 (3 is cofactor)

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10
Q

Common pathway

A

10 (activated by 8 and 7), 5, prothrombin, thrombin

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11
Q

What does thrombin activate?

A

Fibrinogen & 13 (13 does cross linking of fibrin)

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12
Q

Labs in hemophilia

A

PT, thrombin time, fibrinogen, and bleeding time are NORMAL.

aPTT is PROLONGED!!

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13
Q

What is the inheritance of hemophilia?

A

X-linked

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14
Q

What is the inheritance of von Willebrand’s Disease?

A

Autosomal dominant

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15
Q

Labs in vWD

A

Platlet count and PT are NORMAL

aPTT and Bleeding time are PROLONGED

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16
Q

What is DIC?

A

Deposition of fibrin in small blood vessels leading to thrombosis and end-organ damage. Depletion of clotting factors and platlets leads to a bleeding diathesis

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17
Q

What is thrombotic thrombocytopenic purpura?

A

A bleeding disorder due to platelet microthrombi that block off small blood vessels, leading to end-organ ischemia and dysfunction
**similar to DIC

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18
Q

What happens to RBCs in TTP?

A

They become fragmented by contact with the microthrombi leading to hemolysis (microangiopathic hemolytic anemia)

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19
Q

What are the 5 s/sx of TTP?

A
  1. Low platlet count
  2. Microangiopathic hemolytic anemia
  3. Neurologic Changes
  4. Impaired Renal Function
  5. Fever
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20
Q

How is TTP diagnosed?

A

3/5 of the s/sx + schistocytes, low platlets, and rising creatinine

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21
Q

What else is on the same spectrum of disease as TTP?

A

HUS (which has more severe elevations of creatinine) & HELLP

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22
Q

What are the 3 causes of microangiopathic hemolytic anemia?

A

HUS, TTP, DIC

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23
Q

What is idiopathic thrombocytopenic Purpura?

A

IgG antibodies formed against the patient’s platelets

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24
Q

What do lymphoblasts differentiate into?

A

B & T Cells

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25
Q

What do myeloblasts differenciate into?

A

Neutrophils, Eosinophils, and Basophils

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26
Q

What is the value of looking at the retic?

A

2.5 hemolysis/hemorrhage

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27
Q

Causes of microcytic anemia?

A

TICS: thalassemia, iron deficiency, chronic disease, sideroblastic anemia

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28
Q

What is the Schilling test?

A

The patient is first given unlabeled B12 IM to saturate B12 receptors in the liver, followed by an oral challenge of radiolabeled B12. The radiolabeled B12 will pass into the urine if it is properly absorbed, as the liver’s B12 receptors will be saturated from the IM dose

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29
Q

What does it mean is radiolabeled B12 is in the urine for the schilling test?

A

It means there is a dietary B12 deficiency

30
Q

What doe sit mean if there is no radiolabeled B12 in the urine for the schilling test?

A

Consider pernicious anemia, bacterial overgrowth, or pancreatic enzyme deficienc

31
Q

What happens to MMA and homocysteine in B12 deficiency?

A

Both are elevated

32
Q

What happens to MMA and homocysteine in folate deficiency

A

MMA is normal & homocysteine is elevated

33
Q

What is PNH?

A

A disorder in which blood cell sensitivity to complement activation is increased; patients are prone to thrombotic events

34
Q

LDH, indirect bilirubin, and haptoglobin in hemolytic anemia

A

LDH: elevated
Indirect bilirubin: elevated
Haptoglobin: decreased

35
Q

how do patients with aplastic anemia present?

A

Pancytopenic- lack of RBCs, WBCs, and platelets

36
Q

What does the indirect Coombs’ Test detect?

A

Antibodies to RBCs in the patients serum

37
Q

What does the direct Coombs test detect?

A

Sensitized erythrocytes

38
Q

What is the #1 and #2 cause of osteomyelitis in patients with sickle cel disease?

A
#1: S. aureus
#2: Salmonella
39
Q

Health maintenance in patients with sickle cell disease?

A

Treating cholelithiasis with lap chole, chronic folate supplementation, pneumococcal vaccination

40
Q

Thalassemia: 0/4 alpha-chain

A

hydrops fetalis- patients die in utero

41
Q

Thalassemia: 1/4 alpha-chain

A

Hemoglobin H disease (severe hypochromic, microcyticc anemia with chronic hemolysis, splenomegaly, jaundice, and cholelithiasis; skeletal changes

42
Q

Thalassemia: 2/4 alpha

A

Alpha-thal trait; patients have low MCV but are usually asymptomatic

43
Q

Thalassemia: 3/4 alpha

A

Silent carrier patients have no s/sx of disease

44
Q

What is polycythemia vera from?

A

Clonal proliferation of a pluripotent marrow stem cell due to a JAK2 mutation

45
Q

What cell line is affected in polycythemia vera?

A

ALL cell lines are affected- RBCs are most affected

46
Q

What are the types of porphyrias?

A
  1. Acute Intermittent
  2. Porphyria cutanea tarda
    3 Erythropoietic porphyria
47
Q

Leukocyte Alkaline Phosphatase (LAP)

A

Elevated from a leukemoid reaction and decreased in hematologic malignancy

48
Q

Effective of glucose on porphyria

A

Glucose provides negative feedback to the heme synthetic pathway, so high doses may be administered to decrease heme synthesis during an attack

49
Q

AML and ALL

A

Marrow that is infiltrated wtih blast cells (>20-30%); in AML the leukemic cells are myeloblasts; in ALL they are lymphoblasts

50
Q

What is the treatment for M3 AML?

A

all-trans-retinoic acid

51
Q

Genetic mutation in M3 AML?

A

Translocation involving chromosomes 15 & 17

52
Q

What do myeloblasts look like?

A

Large (2-4x RBC), lots of cytoplasm, conspicuous nucleoli, fine granules, +myeloperoxidase

53
Q

What do lymphoblasts look like?

A

Smaller (1.5-3x RBC), less cytoplasm, inconspicuous nucleoli, =myeloperoxidase

54
Q

Age groups for leukemia’s

A

ALL: 60

55
Q

Triad for multiple myeloma

A

Anemia, renal failure, and bone pain

56
Q

What is neutropenia?

A

Absolute neutrophil count <1500

57
Q

How is ANC calculated?

A

(WBC) X (%bands + % segmented neutrophils)

58
Q

What bacterial infections are associated with acute neutropenia?

A

S. aureus, Pseudomonas, E coli, Proteus, Klebsiella

59
Q

How is fever in a patient with neuropenia treated?

A

Broad-spectrum antibiotic (cefepime)

60
Q

Pathomechanism of hyperacute rejection

A

Preformed antibodies

61
Q

Pathomechanism of acute rejection

A

T-cell mediated

62
Q

Pathomechanism of chronic rejection

A

Chronic immune reaction causing fibrosis

63
Q

Neoplasm associated with down syndrome

A

ALL (we ALL go down)

64
Q

Neoplasm associated with xeroderma pigmentosum

A

SCC and BCC carcinomas of the skin

65
Q

Neoplasm associated with pernicious anemia

A

gastric adenocarcinoma

66
Q

Neoplasm associated with tuberous sclerosis

A

astrocytoma and cardiac rhabdomyoma

67
Q

Neoplasm associated with actinic keratosis

A

SCC of skin

68
Q

Neoplasm associated with Plummer-vinson syndrome

A

SCC of esophagus

69
Q

Neoplasm associated with Paget’s disease of bone

A

secondary osteosarcoma and fibrosarcoma

70
Q

Neoplasm associated with AIDS

A

Aggressive malignant NHLs and Kaposi’s sarcoma

71
Q

Neoplasm associated with acanthosis nigricans

A

Visceral malignancy (stomach, lung, breast, uterus)