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Quick Notes for Step 1 > Heme/Onc > Flashcards

Heme/Onc Flashcards

1
Q

Pure- Red Cell Aplasia (PRCA)

A

Aw/ Thymic tumors (thymomas), lymphocytic leukemias, and parvovirus B19

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2
Q

CO Binding Heme

A

Competitively binds to heme (not covalent)

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3
Q

Hepcidin

A

Acute phase reactant made in liver that regulates iron

- Low levels stimulate iron absorption in intestine and iron release from macrophages

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4
Q

Iron Deficiency

A

May have glossal pain, dry mouth, loss of taste and alopecia in addition to the weakness, fatigue, headache, etc.

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5
Q

APL

A

(t15;17)

  • may pw/ hemorrhagic signs and symptoms and persistent infections
  • will have immature blast cells and Auer rods
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6
Q

Vitamin needed for Carboxylating Glutamate

A
  • VitK needed to carboxylate glutamate to make clotting factors
  • Biotin (B7): carboxylation
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7
Q

Diseases aw/ JAK mutation (constitutive):

A

Essential thrombocytopenia
Polycythemia vera
Primary myelofibrosis
(Not CML)

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8
Q

Citrate in Preserved Blood

A

Chelates Ca and Mg

May cause paresthesias

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9
Q

Late Defects in Heme Synthesis

A

(after PBG)

- photosensitivity

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10
Q

Early Defects in Heme Synthesis

A
  • neuropsych changes without photosensitivity
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11
Q

Bleeding Problems w/ Cystic Fibrosis Due to:

A

Impaired fat absorption so decreased Vit A, D, E, and K (bleeding problems due to low synthesis of coagulation factors)

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12
Q

PTT

A

Left side: PTT (intrinsic); affected by heparin

- Monitor with aPTT

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13
Q

PT

A

Right side: PT (extrinsic); affected by warfarin

- Monitor PT or INR

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14
Q

Glanzmann

A

gpIIb/IIIa problem (aggregation)

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15
Q

Bernard-Soulier

A

gpIb problem (binding to vwf)

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16
Q

Aminocaproic Acid

A

tPA or alteplase toxicity reversal

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17
Q

Warfarin Reversal

A

Fresh frozen plasma for fastest warfarin reversal, vit K can be used but not as fast

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18
Q

Protamine sulfate

A

Heparin reversal

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19
Q

Homan’s Sing

A

pain that increases with dorsiflexion → indicates a DVT

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20
Q

When Iron is Low, Serum Transferrin is _____

A

increased

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21
Q

Porphyrin Synthesis: Early vs Late Defects

A
  • Early steps: neuropsychiatric manifestations

- Later steps (after PBG): photosensitivity (eg. Acute cutanea tarda with UROD deficiency)

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22
Q

Rat Poison

A

will deplete all vitK dependent coagulation factors and patient should be immediately given fresh frozen plasma

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23
Q

Koilonychia

A

(spoon-shaped nails) with iron deficiency anemia

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24
Q

Leukemoid Reaction and CML Differentated By:

A

Alkaline phosphatase level

  • Normal or elevated in leukemoid reaction
  • Low in CML
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25
Acute Intermittent Porphyria (AIP)
- Urine turns darker (port-wine) with exposure to air/light due to excess PBG - No photosensitivity, just abdominal pain - HMB synthase deficiency (aka PBG deaminase) - Can treat by downregulating ALAS activity with glucose or heme infusion
26
JAK2
Cytoplasmic tyrosine kinase aw/ polycythemia vera
27
Severe anemia (erythroblastosis fetalis) → ___________
release of nucleated RBCs and extramedullary hematopoiesis
28
Migratory Thrombophlebitis
- should always consider cancer | - eg. Pancreatic cancer
29
Prostacyclin (PGI2)
- from endothelial cells | - inhibits platelet aggregation
30
Orotic Aciduria
- Megaloblastic anemia - treat with uridine to bypass the mutated step - UMP Synthase defect (build up orotic acid)
31
Angiogenesis Stimulated by:
VEGF and FGF
32
Follicular Lymphoma: Progression
indolent, wax and wane
33
Conversion of Heme to Biliverdin by:
Heme oxygenase
34
HbA2 elevated in:
Beta Thalassemia Minor
35
ALL
MC in children; 70-80% is B-ALL, the rest is T-ALL (mediastinal mass, may have dysphagia, respiratory sx, or SVC syndrome)
36
Burkitt Lymphoma
(EBV): jaw (African variant) or abdominal (endemic) - High mitotic index; round blue small cells - Starry sky - C-myc
37
GPIIb/IIIa Direct Inhibitors
Abciximab, Eptifibatide | - blocks a surface receptor that belongs to the integrin family
38
Inhibitors of ADP induced expression of GpIIb/IIIa
Irreversible: Clopidogrel, Prasugrel, Ticlopidine(neutropenia and mouth ulcers) Reversible: Ticagrelor
39
ESR decreased with:
sickle cell, polycythemia, and CHF
40
Heinz bodies
- Hemoglobin precipitation | - G6PD and a-thalassemia
41
Howell-Jolly Bodies
- nuclear remnants | - asplenia/hyposplenia or megaloblastic anemia
42
Pappenheimer Bodies
Iron aggregates/inclusions
43
Burr Cells
Seen in microangiopathic hemolytic anemia, mechanical damage or Pyruvate Kinase Deficiency (rigid RBCs)
44
Speed of Hb on Electrophoresis
HbA > HbS > HbC
45
HbA2 elevated in:
B-Thalassemia
46
Drugs that cause AIP (acute intermittent porphyria)
Phenobarbital, sulfonamides, steroid hormones (esp. progresterone
47
_____ & ______ inhibit ALAS (rate-limiting)
Glucose and Heme
48
Effects on Bleeding due to ESRD
Platelet aggregation is inhibited by uremic toxins --> increased bleeding time
49
Acquired Antithrombin Deficiency
Loss of antithrombin in urine with nephrotic syndrome or renal failure
50
Tx for CML
Imatinib (Gleevec) Tyrosine Kinase inhibitor
51
Tx for Hairy Cell
Cladribine (adenosine analog)
52
Lytic Bone Lesions seen in:
Multiple Myeloma, ATLL & Langerhan's Histiocytosis
53
Direct Thrombin Inhibitors (4)
Dabigatran, argatroban, bivalirudin, lepirudin
54
Heparin Induced Thrombocytopenia (HIT)
developing IgG against heparing bound to PF4 (platelet factor 4)
55
Cilostazol, Dipyridamole
PDE III inhibitor (increases camp) and inhibits platelet aggregation
56
Etoposide, Teniposide
Inhibits topo II - used for solid tumors (small cell and testicular), leukemia/lymphoma - myelosuppression
57
Irinotecan, Topotecan
Inhibits topo I - colon cancer (irino) - ovarian and small cell (topotecan)
58
Trastuzumab: Side effect
Cardiotoxicity
59
Drugs that increase risk of PML
Natalizumab and Rituximab
60
CO poisoning
PaO2 is 95 (dissolved not affected) Carboxyhemoglobin is increased Methemoglobin is decreased
61
Sickle Cell Vitamin Deficiencies
Predisposed to folic acid deficiency due to high cell turnover
62
Competitive Athlete w/ erythrocytosis, abnormal testicle size, and acne
Steroid abuse
63
Acute Hemolytic Transfusion Reaction
Antibody mediated (Type II hypersensitivity) - complement mediated lysis (different from IgE mediated Type I which would be an allergic transfusion reaction w/ sx of anaphylaxis and urticaria)
64
TdT+
ALL (B-ALL and T-ALL)
65
Transferrin in Iron Deficiency
Transferrin synthesis increases in states of low iron and TIBC increases
66
Biochemical Pathway that uses: Glycine and Succinyl-CoA
Part of the Heme Synthesis Pathway
67
ITP Immune Thrombocytopenic Purpura
immune-mediated consumption of platelets - low platelet count and petechiae - bone marrow biopsy results demonstrate that the body has appropriately increased platelet production
68
TTP Thrombotic Thrombocytopenic Purpura
``` thrombocytopenia (low platelet count) microangiopathic hemolytic anemia altered mental status renal failure fever ```
69
Petechiae vs Purpura vs Ecchymoses
Petechiae (less than 5 mm) Purpura (5mm to 1cm) Ecchymoses (greater than 1 cm)
70
PO2 normal, O2 Saturation decreased, O2 content decreased
CO Poisoning
71
PO2 normal, O2 Saturation normal, O2 content increased
Polycythemia
72
PO2 normal, O2 saturation normal, O2 content decreased
Anemia/Chronic blood loss
73
DIC aw/ which Leukemia
APL
74
MC type of Hodgkins Lymphoma
Nodular sclerosis

Quick Notes for Step 1 (18 decks)

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