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Step 1 High Yield > Hematology and Oncology > Flashcards

Hematology and Oncology Flashcards

1
Q

What component of the erythrocyte membrane allows transport of CO2 from the periphery to the lungs?

A

Chloride HCO3- antiporter - exports HCO3- and imports CO2

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2
Q

What are the dense granules in platelets? Alpha granules?

A

Dense: ADP, calcium

Alpha granules: vWF, fibrinogen

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3
Q

What is contained in the specific granules of neutrophils?Azurophilic granules?

A

Specific granules: ALP, collagenase, lysozyme, and lactoferrin
Azurophilic granules: Proteinases, acid phosphatase, myeloperoxidase, and Beta glucuronidase

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4
Q

Cell marker for macrophage?

A

CD14

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5
Q

What is the difference between a mast cell and a basophil?

A

Both release histamine and heparin and mediate allergic reaction
Mast cell: Binds Fc portion of IgE and releases eosinophil chemotactic factors
Basophil: Releases leukotrienes

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6
Q

What is the difference between a mast cell and a basophil?

A

Both release histamine and heparin and mediate allergic reaction
Mast cell: Binds Fc portion of IgE causing degranulation; releases eosinophil chemotactic factors
Basophil: Releases leukotrienes

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7
Q

What two factors are necessary to turn fibrin monomers into a fibrin mesh (one is activated by thrombin)?

A

Factor XIIIa (activated by thrombin) and calcium

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8
Q

What activates Protein C?

A

Thrombomodulin (and vitamin K allows precursor Protein C to become mature form beforehand)
Protein S allows protein C to cleave and inactivate Va and VIIIa

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9
Q

What is ESR?

A

Erythrocyte sedimentaiton rate - acute phase reactants (fibrinogen) can cause RBC aggregation

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10
Q

What increases ESR?

What decreases ESR?

A

Increase: Infections, autoimmune diseases (SLE, RA, temporal arteritis), malignant neoplasms, ulcerative colitis, pregnancy
Decrease: Polycythemia, sickle cell anemia, CHF, microcytosis, hypofibrinogenemia

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11
Q

What is an acanthocyte and when is it seen?

A

AKA a spur cell

Associated with liver disease; Abetalipoproteinemia (cholesterol dysregulation)

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12
Q

When do you see basophilic stippling?

A

Anemia of chronic disease, alcohol abuse, lead poisoning, thalassemia

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13
Q

What is it called when there is excess iron in mitochondria? What is the defining feature?

A

Sideroblastic anemia; Ringed sideroblasts (due to decreased protoporphyrin)

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14
Q

When do you see target cells?

A

HbC disease, Asplenia, Liver disease, Thalassemia

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15
Q

What are Howell Jolly bodies?

A

Basophilic nuclear remnants found in RBCs

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16
Q

What is the triad of Plummer Vinson syndrome?

A

Iron deficiency anemia
Esophageal webs
Atrophic glossitis

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17
Q

How do different numbers of deletions affect alpha thalassemia?
4 allele deletion:
3 allele deletion:
1-2 allele deletion:

A

4 allele deletion: Excess gamma globulin forms (gamma-4 = Hb Barts) - incompatible with life (hydrops fetalis)
3 allele deletion: HbH disease - very little alpha globin (excess beta globin forms Beta-4 (HbH)
1-2 allele deletion: asymptomatic

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18
Q

What is seen on electrophoresis in beta thalassemia minor (heterozygote)?

A

HbA2

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19
Q

What is the major type of hemoglobin found in beta thalassemia major?

A

HbF (protective in the infant)

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20
Q

What musculoskeletal abnormality is associated with lead poisoning?
What CNS effect?
What biochemical effect?

A

Wrist and foot drop
Encephalopathy
Inhibits rRNA degradation

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21
Q

What is used to treat lead poisoning?

A

Dimercaprol and EDTA (1st line)

Succimer used for chelation for kids

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22
Q

What deficiency is associated with sideroblastic anemia? What is the deficiency in the hereditary form?

A

Defect in heme synthesis

Hereditary: X-linked defect in ALA synthase gene

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23
Q

What labs are used to detect Hereditary spherocytosis?

A

Osmotic fragility test (+); Eosin-5-maleimide binding test

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24
Q

What mutation leads to HbC hemoglobin?

A

Glutamic acid to lysine mutation at residue 6 in beta globin

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25
Q

What is the membrane defect in paroxysmal nocturnal hemoglobinuria?

A

Impaired synthesis of GPI anchor for decay accelerating factor that protects RBC membrane from complement

26
Q

What is the treatment for PNH?

A

Eculizumab

27
Q

What immunoglobulin is associated with Warm agglutinin (autoimmune hemolytic anemia)? Cold agglutinin?

Bonus: What diseases are associated with each?

A

Warm agglutinin: IgG - triggered by warm weather (Chronic anemia associated with SLE, CLL or certain drugs)
Cold agglutinin: IgM - triggered by cold (Acute anemia seen in CLL, Mycoplasma pneumonia or infectious mononucleosis)

28
Q

What causes Eosinopenia? What is the mechanism?

A

Cushing syndrome, corticosteroids

Corticosteroids sequester eosinophils in lymph nodes and cause apoptosis of lymphocytes

29
Q

What is the mechanism of corticosteroid induced neutrophilia?

A

Corticosteroids decrease activation of neutrophil adhesion molecules, impairing migration out of the vasculature to sites of inflammation (so neutrophils accumulate in the blood)

30
Q

Which enzyme is associated with acute intermittent porphyria? Porphyria cutanea tarda?

A

AIP: Porphobillinogen deaminase
PCT: Uroporphyrinogen decarboxylase (UROD)

*Don’t “cutanea” UR ROD

31
Q

What are the 5 Ps of acute intermittent porphyria?

A 
Painful abdomen
Port wine-colored urine
Polyneuropathy
Psychological disturbances
Precipitated by drugs, alcohol, and starvation
32
Q

What are the causes of DIC?

A 
STOP Making New Thrombi
Sepsis (gram negative) 
Trauma
Obstetric complications
Acute Pancreatitis
Malignancy
Nephrotic syndrome
Transfusion
33
Q

What are the hereditary thrombosis syndromes that lead to hypercoagulability?

A

Factor V Leiden (resistant to degradation by protein C)
Prothrombin gene mutation (increased production)
Antithrombin deficiency (Diminishes the increase in PTT following heparin administration - only symptom)
Protein C or S deficiency (decreased ability to activate factors V and VIII)

34
Q

When do you give cryoprecipitate therapy and what does it contain?

A

Contains fibrinogen, factor VIII, factor XIII, vWF and fibronectin
Use: Treat coagulation factor deficiencies involving fibrinogen and factor VIII

35
Q

How is CML differentiated from a leukemoid reaction?

A

Both have increased WBC count but CML has a decreased leukocyte ALP whereas a leukemoid reaction has an increased leukocyte ALP

36
Q

What is the difference between Hodgkin and Non-Hodgkin lymphoma in terms of nodes, cell type, and peak incidence?

A

Hodgkin: Localized single group of nodes (extranodal rare); Reed Sternberg cells; Bimodal age distribution (young adulthood and >55)

Non-Hodgkin: Multiple peripheral nodes (extranodal involvement); Majority involve B cells; Peak incidence at 20-40 years old

37
Q

What predicts a better prognosis in ALL? What are the serum markers?

A

t(12:21) - better prognosis
TdT+ (marker of pre-T and pre-B cells)- it is a DNA polymerase
CD10+ (pre-B cells only)

38
Q

What is the difference between CLL and SLL?

A

CLL has increased peripheral blood lymphocytosis or bone marrow involvement (Smudge cells in both)

39
Q

What part of the spleen undergoes splenomegaly with hairy cell leukemia?
How is hairy cell leukemia distinguished from other B-cell lymphomas?
What is the treatment for hairy cell leukemia? (TRAP+)

A

Splenomegaly of the red pulp
Lyphoadenopathy is usually absent
Cladribine (an adenosine analog that inhibits adenosine deaminase)

40
Q

What is the significance of t(15;17) in acute myelogenous leukemia (AML)?
How does AML cause DIC?

A

t(15;17) is seen in the M3 type of AML - Responds to all-trans retinoic acid (vitamin A) inducing differentiation of myeloblasts

Auer rods (composed of myeloperoxidase) can induce coagulation cascade leading to DIC

41
Q 
Name the disease and gene associated with the following...
t(9:22):
t(8:14):
t(11:14):
t(14:18):
t(15:17):
A

t(9:22): CML (and some B-ALL) - bcr-abl hybrid (tyrosine kinase)
t(8:14): Burkitt lymphoma - c-myc activation
t(11:14): Mantle cell lymphoma - cyclin-D1 activation
t(14:18): Follicular lymphoma (or diffuse large B-cell lymphoma) - bcl2 activation (inhibits apoptosis)
t(15:17): M3 type of AML - responsive to all-trans retinoic acid

42
Q

Why is there low leukocyte alkaline phosphatase in CML?

How do you treat CML?

A

Result of low activity in mature granulocytes

Treat with Imatinib (inhibitor of bcr-abl tyrosine kinase)

43
Q

What is the presentation of langerhans cell histiocytosis?

A

Presents in a child as lytic bone lesions and skin rash or recurrent otitis media with a mass involving the mastoid bone

44
Q

What are the characteristic findings in Langerhans cell histiocytosis?

A

Cells express S-100 (mesodermal); and CD1a. Birbeck granules are characterisitic

45
Q

_____ is involved in hematopoietic growth factor signaling

A

JAK2

46
Q

What happens to EPO levels in polycythemia vera?

What is the main presentation?

A

Decreased due to negative feedback

Intense itching after a hot shower

47
Q

What three things are needed for activation of coagulation factors?

A
  1. Exposure to activating substance (Subendothelial collagen for intrinsic pathway and tissue thromboplastin for extrinsic)
  2. Phospholipid surface of platelets
  3. Calcium (derived from platelet dense granules)
48
Q

Heparin induced thrombocytopenia is due to heparin forming complexes with…
How does this result in thrombocytopenia?

A

Platelet factor 4 on the surface of platelets

IgG antibodies are formed against these complexes - results in consumption of these platelets by the spleen

49
Q

What inactivates plasmin?
How does this relate to a DIC-like condition?
What is the treatment?

A

Alpha-2 antiplasmin
Cirrhosis of liver causes decreased production of alpha2 antiplasmin leading to increased bleeding that resembles DIC
Treated with aminocaproic acid

50
Q

Explain warfarin skin necrosis and Protein C or S deficiency

A

Normally warfarin is initially given with heparin because blockade of K epoxide reductase affects C and S cells before it activates 2,7,9 and 10 leading to increased risk of thrombosis; If the patient has C or S deficiency they are more likely to have thrombosis which is called warfarin skin necrosis

51
Q

In paroxysmal nocturnal hemoglobinuria, what is the main cause of death? Why is AML a complication of this disease?

A

The main cause of death is thrombosis of the hepatic, portal, or cerebral veins (platelets also lack DAF and their destruction leads to the release of cytoplasmic contents which can induce thrombosis)

PNH is due to a mutation in the myeloid stem cell (no GPI) and so another mutation in the myeloid stem cell is not uncommon which could produce AML

52
Q

What is acute monocytic leukemia (what proliferates? what is lacking? what is the presentation?)
What is acute megaloblastic leukemia? (same as above)

A

Acute monocytic leukemia = proliferation of monoblasts; usually lack MPO; infiltration of gums

Acute megaloblastic leukemia = proliferation of megakaryoblasts; lack MPO; Associated with down syndrome

53
Q

In orotic aciduria, what enzyme is deficient? What type of anemia does this cause?

A 
Defect in UMP synthase
Megaloblastic anemia (no hyperammonemia)
54
Q

How do you differentiate between intravascular hemolysis and extravascular hemolysis?

A

Intravascular: Decreased haptoglobin, increased reticulocytes, urobilinogen in urine
Extravascular: Increased unconjugated bilirubin which causes jaundice

55
Q

What is deficient in Thrombotic thrombocytopenic purpura (TTP)?
What is the defect in ITP?

A

TTP: ADAMTS13 (vWF metalloprotease)
ITP: Anti-GpIIb/IIIa antibodies

56
Q

What are the CD markers on Reed Sternberg cells?

Lymphocyte (rich/poor) form has the best prognosis?

A

CD15+ and CD30+

Lymphocyte rich form has the best prognosis

57
Q

How is mantle cell lymphoma distinguished (aside from t(11:14)) from other non-hodgkin lymphomas

A

only CD5+

58
Q

Which T-cell neoplasm presents with cutaneous patches/plaques/tumors

A

Mycosis fungoides/sezary syndrome

59
Q

How does CRAB related to multiple myeloma?

What interleukin is elevated?

A 
Hypercalcemia
Renal insufficiency
Anemia
Bone lytic lesions/back pain
*elevated IL-6
60
Q

How is Waldenstrom macroglobulinemia different than multiple myeloma?

A

M spike in IgM instead of IgG/IgA

61
Q

Describe a pseudo-pelger-huet anomaly

What syndrome is this associated with?

A

PPH anomaly: neutrophils with bilobed nuclei (connected with a thin filament of chromatin) typically seen after chemotherapy
Associated with myelodysplastic syndromes

Step 1 High Yield (21 decks)

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