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USMLE Hematology Leukemmia and Lymphoma > High yield intro > Flashcards

High yield intro Flashcards

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USMLE® Step 1 flashcards
1
Q

What amino acid is a precursor to heme?

A

Glycine

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2
Q

What is not contained in serum?

A

Clotting factors

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3
Q

What kind of infection may be suspected (other than viral) when lymphocytes are raised?

A
  • Fungal

- Bordetella pertusis infection

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4
Q

What does iron incorporate into to make heme?

A

Protoporphyrin ring

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5
Q

Where does heme synthesis occur?

A

Cytoplasm and mitochondria

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6
Q

Glycine and succinyl coA make what molecule in the heme synthesis pathway?

A

Delta ALA

  • enzyme: Delta ALA synthase
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7
Q

What enzyme is inhibited in Sideroblastic anemia? (X-linked)

A

Delta ALA synthase

Reaction:
- glycine + succinyl coA -> Delta ALA

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8
Q

What are the signs of sideroblastic anemia?

A
  • Anemia
  • Basophilic remnants of rRNA
  • Male - X-linked
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9
Q

Porphobilinogen is converted to what in the heme synthesis pathway?

A

Hydroxymethylbilane

  • Enzyme: PGD De-Aminase (PROD)
  • Affected in acute intermittent porphyria
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10
Q

What enzyme is affected by Acute Intermittent Porphyria?

A

PBG De-Aminase (PROD)

Reaction:
- Porphorobilinogen -> Hydroxymethylbilane

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11
Q

What are the symptoms of acute intermittent porphyria?

A

4 Ps

  • Intermittent abdo pain
  • Port wine urine
  • Polyneuropathy
  • Psychosis
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12
Q

What are the symptoms of Porphyria Cutanea Tarda?

A
  • Painful blisters post sun exposure
  • Red urine

URO-Decarboxylase

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13
Q

What reaction does Porphoryia Cutanea Tarda affect?

A

Uroporphyrinogen -> Corporophyrinogen

  • Enzyme: URO-Decarboxylase
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14
Q

What kind of drugs may induce acute intermittent porphyria?

A
  • CYP Inducing drugs
  • Anti-convulsants
  • Alcohol
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15
Q

What are the CYP inducing drugs?

- May induce acute intermittent porphyria

A
  • Griseofulvin
  • Carbamazepine
  • Phenytoin
  • Barbiturates
  • Rifampin
  • St Johns Wort
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16
Q

What is the function of Griseofulvin? (MOA and used to treat)

A
  • Tinea Capidis, unguium (head, toe fungal infections)

- Inhibits MTs and thus fungal mitosis

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17
Q

What is the function of Carbamazepine? (MOA and used to treat)

A
  • Anti-seizure, trigeminal neuralgia

- Inactivates Na+ channels

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18
Q

What is a possible side-effect of Carbamezepine?

A

SIADH

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19
Q

What is the function of Barbiturates?

A

Increase the duration of GABA Cl- channel opening

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20
Q

What is the function of Rifampin?

A
  • Blocks DNA dependent RNA polymerase -> decreases bacterial protein synthesis
  • Used for TB
  • Meningococcal or Hib prophylaxis
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21
Q

WHat is St Johns Wort used for?

A

Depression (increases serotonin)

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22
Q

What does lead inhibit?

A
  • Ferrochelatase (iron into protoporphyrin ring)

- ALAD (d-aminolevulinic acid dehydratase)

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23
Q

Where is Ferrochelatase located and what is its function?

A
  • Mitochondria

- Catalyses reaction of iron and protoporphyrin IX into heme (heme synthesis)

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24
Q

What will a blood smear show in a patient with lead poisoning?

A

Basophilic stippling

- Represents remnants of rRNA in RBCs

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25
Q

What non-specific symptoms may a patient with lead poisoning present with?

A
  • Headaches
  • Constipation
  • Developmental delay

Other family members will have symptoms

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26
Q

What are the causes of Target cells?

GET A PICTURE

A

HALT

  • Hemoglobin C disease (patient with hemolytic anemia, splenomegaly, and Glu-> Lysine)
  • Asplenia
  • Liver disease (alcoholism, obstructive)
  • Thalassemia (chipmunk facies, hairon-end appearance, chronic transfusions)
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27
Q

What are the 2 mechanisms behind the making of target cells?

A
  • Low RBC mass (thalassemia)
  • Redundant membrane (liver disease, splenectomy)

(Increased surface area to volume ratio)

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28
Q

What is basophilic stippling due to?

GET A PIC

A

Denatured ribosomes

  • Alcoholic or patient on isoniazid (thru B6 deficiency) for TB
  • Sideroblastic anemias, thalssemias
  • Lead poisoning
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29
Q

What are Howell-Jolly bodies?

GET A PIC

A

DNA remnants

- Functional (sickle) or anatomic asplenia

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30
Q

What are Heinz bodies caused by?

A
  • Drug trigger + normal MCV anemia + jaundice
  • Patient with drug trigger and hemolytic anemia
  • G6P deficiency (bite cells)
31
Q

What do heinz body cells become in the spleen?

A

Bite cells

32
Q

What do heinz bodies require to be visualised?

A

Crystal violet

33
Q

What are general symptoms indicative of anemia?

A
  • Fatigue
  • SOB
  • Pallor
  • Angina
  • Tachycardia
34
Q

What are reticulocytes and why are the pathologically important?

A
  • Immature RBCs

- Indicate whether bone marrow is responding (in hemolytic anemia)

35
Q

What is the opposite of ferritin?

A
  • Ferritin is the storage form of iron

TIBC (Total iron binding capacity)

36
Q

What is the opposite of ferritin?

A
  • Ferritin is the storage form of iron

TIBC (Total iron binding capacity)

37
Q

What will the level of ferritin be like in iron deficiency anemia?

A

Decreased

38
Q

What kind of patients are characteristically have iron deficiency anemia?

A
  • Teenage female
  • Colon cancer (old, weight loss)
  • Pregnancy causing a dilutional anemia
  • 3rd world child
39
Q

What type of anemia is anemia of chronic disease typically?

A
  • Microcytic (normocytic sometimes in early stages)
40
Q

What causes anemia in conditions like RA and SLE? (anemia of chronic disease), may also be due to chronic infection

A
  • Hepcidin increases - decreasing iron absorption

- Increases trapping of iron in bone (ferritin) from IL-1

41
Q

What are the ferritin and TIBC levels like in anemia of chronic disease?

A
  • Ferritin high

- TIBC low

42
Q

What are the positive acute pahse reactants?

A
  • CRP
  • Ferritin
  • Fibrinogen
  • Hepcidin
  • Serum amyloid A
43
Q

What are the negative acute phase reactants?

A
  • Albumin

- Transferrin

44
Q

What does amyloid look like and on what stain?

A

Stained by Congo red

- Apple-green biferingence in polarised light

45
Q

What kind of meglaoblastic anemia gives neurological deficits?

A

B12 (due to increased MMA)

- Folate does not

46
Q

What do megaloblastic anemias show on blood smear?

A

Hypersegmented neutrophils (more than 5)

47
Q

How do B12 and folate create hypersegmented neutrophils?

A
  • Unable to undergo cytokinesis (high segmentation)
48
Q

What is a type 2 hypersensitivity reaction due to?

A

Auto-antibody to antigen

49
Q

What kind of anemia are vegans most likely to suffer from?

A

Macrocytic

- Not enough dietry B12

50
Q

WHat fish tapeworm may cause malabsorption and therefore B12 deficient macrocytic anemia?

A

Diphyllobothrium latum

51
Q

What enzyme does not function when there is no B12?

A

Methyl malonyl coA mutase

increased MMA

52
Q

How can normocytic anemias be divided?

A
  • Intravascular vs Extravascular

- Intrinsic vs Extrinsic

53
Q

What are the signs of an intravascular normocytic anemia?

A
  • Low haptoglobin (haptoglobin binds to heme from destroyed RBCs)
  • Hemoglobinuria
  • Increased LDH
  • High indirect BR
54
Q

What are the signs of an extravascular normocytic anemia?

A
  • Splenomegaly

- Normal haptoglobin

55
Q

What do reticulocyte levels represent?

A

Bone-marrows response to anemia

56
Q

WHat are the causes of extra-vascular hemolytic anemia?

A
  • Autoimmune Hemolytic Anemia
  • Heredetary spherocytosis
  • G6P deficiency
  • Thalassemia
57
Q

WHat are the causes of intra-vascular hemolytic anemia?

A
  • Microangiopasthic hemolytic anemia
  • Prosthetic heart valves
  • Paroxysmal noctural hemoglobinuria
58
Q

What gene is defective in PNH?

A
  • PIGA gene

Low decay accelerating factor making it more susceptible to complement

59
Q

What are the intrinsic hemolytic anemias

A
  • Hereditary spherocytosis: (membrane defects)
  • G6PD, pyruvate kinase (enzyme defects)

Hemoglobin defects:

  • Sickle cell anemia
  • Paroxysmal nocturnal hemoglobinuria
60
Q

HUS (Hemolytic uremic syndrome) and TTP (Thrombotic thrombocytopenic purpura) common symptoms/signs

A
  • Infectious diarrhoea
  • acute renal failure
  • Shishtocytes
  • Endothelial injury (microthrombi, reduced platelets)
  • Low platelets
61
Q

What is the triad for HUS?

A
  • Anemia
  • Thrombocytopenia
  • Acute renal failure
62
Q

What is the pentad for diagnosis of TTP?

A
  • Anemia
  • Thrombocytopenia
  • Acute renal failure
  • Fever (>38)
  • Altered mental status
63
Q

What are the different causes of polycythemia?

A

JAK-2 positive - polycythemias Vera (itchy female)

JAK-2 Negative *Reactive)

  • Cyanotic heart disease
  • Sleep apnea / COPD
  • EPO tumor RCC
64
Q

What does high hematocrit mean

A

Polycythemia

65
Q

What does DDAVP do for clotting?

A
  • Increases vWF

- Increases factor 8

66
Q

What does the vignette for vWF Disease usually include?

A

Mucocutaneous bleeding after trigger (i.e. dental) or aspirin use

67
Q

What is vWF treated with?

A

DDAVP

68
Q

What kind of hemostasis is affected in DIC?

A
  • Primary and Secondary hemostasis (low platelets and PT, PTT etc.)
  • Big trigger!
69
Q

What does heparin increase the activity of?

A

AT3

70
Q

What are the different types of heparin?

A
  • UFH (Unfractionated Heparin) - PTT
  • LMWH (-parin) - Xa specific
  • Fondaparinux - Xa specific
71
Q

What can reverse the effects of heparin?

A

Protamine

- Also can use prothrombin complex precipitate

72
Q

What does vit K epoxide reductase do?

A

Gamma-carboxylation of 2, 7, 9, 10, C, S

- Warfarin prevents this

73
Q

What ca be used to reverse warfarin?

A
  • Acutely: fresh frozen plasma

- Vit K supplements long term

74
Q

What are some signs of multiple myeloma?

A
  • Back pain
  • Hypercalcemia
  • Reccurrent infection
  • Abnormal blood smear

USMLE Hematology Leukemmia and Lymphoma (14 decks)

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