Heme Onc Flashcards

1
Q

Neutrophil chemotactic agents (5)

A
IL-8
C5a
LTB4
Kallikrein
Platelet activating factor
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2
Q

What activates macrophages

A

Interferon-gamma

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3
Q

What chemical process initiates septic shock

A

Lipid A from bacterial LPS binds CD14 on macrophages

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4
Q

What does cromolyn sodium do?

A

prevent mast cell degranulation (used in asthma prophylaxis)

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5
Q

What costimulatory signal is needed for T cell activation

A

CD28

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6
Q

T helpers recognize what MHC?

A

class II

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7
Q

T cytotoxic recognize what MHC?

A

Class I

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8
Q

What antibodies exist against blood type vs Rh factor?

A

IgM against blood type

IgG against Rh Factor (cause of hemolytic dz of newborn - IgG can cross placenta)

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9
Q

GpIIb/IIIa blockers (integrin)

A

abciximab, eptifibatide, tirofiban

These prevent the platelet receptor for fibrinogen from being able to bind, prevents platelet aggregation

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10
Q

Aspirin MOA

A

inhibits cyclooxyrgenase, which prevents thromboxane TXA2 synthesis, preventing platelet activation

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11
Q

Vorapaxar MOA

A

blocks Par1 receptor to prevent thrombin from activating platelets

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12
Q

Clopidogrel (and prasugrel, and ticlopidine)MOA

A

Blocks P2Y12 receptor, prevents ADP-platelet binding, which prevents platelet activation ()mnemonic: Plavix and P2Y12)

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13
Q

What is ristocetin?

A

activates vWF to bind GpIb. Failure of agglutination with ristocetin assay occurs in vW disease and Bernard-Soulier syndrome

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14
Q

Acanthocyte

A

spiky RBC; seen in liver disease and states of cholesterol dysregulation

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15
Q

Lead poisoning blood smear

A

Basophilic stippling: RBC appears blue spotted w/ white center

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16
Q

Degmacyte

A

Bite cell - seen in G6PD deficiency

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17
Q

Elliptocyte

A

seen in hereditary elliptocytosis

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18
Q

Macro-ovalocyte

A

big ass RBC that looks weird; seen in megaloblastic anemia (also hyperhsegmented PMNs) and marrow failure

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19
Q

Ringed sideroblast

A

are atypical, abnormal nucleated erythroblasts (precursors to mature red blood cells) with granules of iron accumulated in the mitochondria surrounding the nucleus

RBC that has blue stuff and a whiteish/bluey ring. Seen in sideroblastic anemia; excess iron in mitochondria

hereditary or not; can be seen with progression to AML
iron available but cannot incorporate it into hemoglobin

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20
Q

when do we see schistocytes

A

DIC, TTP/HUS, HELLP, mechanical hemolysis

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21
Q

Target Cell

A
"HALT" said the hunter to his target:
Hemoglobin C disease
Asplenia
Liver disease
Thallassemia
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22
Q

Heinz bodies

A

oxidation of Hb-SH groups to S-S leads to Hb precipitation. Subsequent phagocyte damage leads to bite cells

Seen in G6PD deficiency
Similar Heinz-like inclusions seen in alpha thalassemia

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23
Q

Howell Jolly bodies

A

RBC with little blue spot; basophilic nuclear remnant usually removed in spleen by macrophages
seen in asplenia and functional asplenia

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24
Q

Plummer Vinson syndrome

A

iron def anemia, esophageal webs, and atrophic glossitis

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25
Q

Alpha globin found on which chromosome?

A

16
think 4 copies of alpha chain. 4x4 = 16
alpha thalassemia results from gene deletion not mutation

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26
Q

Beta globin gene found on which chromosome?

A

11

beta thalassemia results from gene mutation not deletion

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27
Q

Beta thalassemia finding on electrophoresis

A

Increased HbA2

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28
Q

Hemoglobin C smear finding

A

HbC crystals

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29
Q

Complications of paroxysmal nocturnal hemoglobinuria

A

AML
Iron deficiency anemia
Main cause of death is thrombosis 2/2 released platelet stuff when they get destroyed–> activate coagulation cascade

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30
Q

Treatment for paroxysmal nocturnal hemoglobinuria

A

Eculizumab - inhibits terminal complement

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31
Q

What do GM-CSF and G-CSF do?

A

drive production of granulocytes in BM

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32
Q

Pyruvate kinase deficiency

A

extravascular hemolysis, due to AR defect in pyruvate kinase which decreases ATP resulting in rigid RBCs. Findings - hemolytic anemia in the newborn

33
Q

Drug causes of aplastic anemia

A

benzene, chloramphenicol, alkylating agents, antimetabolites

34
Q

Fanconi anemia

A

DNA repair affect, can cause aplastic anemia

35
Q

Bands have low numbers of what receptor?

A
Fc receptor (CD16) 
Happens during left shift
36
Q

hodgkin lymphoma increases Eos count by which molecule?

A

IL-5

37
Q

Why does pertussis cause increased lymphocytes?

A

Lymphocytosis promoting factor - blocks Lymphocytes from going into LN - stuck in blood

38
Q

Pseudo-Pelger-Huet anomaly

A

neutrophils with bilobed nuclei; typically seen after chemotherapy

39
Q

Treatment for hairy cell leuykemia

A

Cladribine (2-CDA) - excellent response (adenosine deaminase inhibitor)
Pentostatin

40
Q

What is Pautrier Microabscess?

A

Aggregates of neoplastic T cells in epidermis found in mycosis fungoides

41
Q

high basophils makes you think what?

A

CML

42
Q

what tumor exogenously makes EPO?

A

Renal Cell Carcinoma – can lead to reactive polycythemia and resemble PV (except that in PV, EPO is low)

43
Q

What are the three types of blood cancers associated with Jak2 mutation

A

PV
Essential thrombocytosis
Myelofibrosis

NOTE: CML is NOT; it’s associated with t(9;22) and BCR-ABL

44
Q

elevated interleukin in Multiple Myeloma

A

IL-6 - growth factor for plasma cells

45
Q

What is IL-6 significant for?

A

Multiple myeloma

growth factor for plasma cells

46
Q

What do you see on EM with langerhans cell histiocytosis

A

tennis racket! aka Birbeck granules

47
Q

Drainage of superficial inguinal LNs

A

Most cutaneous lymph from umbilicus down, including external genitalia and anus (below the dentate line)
EXCEPTIONS: Glans penis and skin of posterior calf (popliteal LNs) which bypass the superficial inguinal nodes to drain into the deep inguinal nodes

48
Q

Drainage from lymph to prostate

A

internal iliac nodes

49
Q

Lymph drainage from testes

A

parallels the blood supply, going to the retroperitoneal LNs

50
Q

Lymph drainage from upper third of rectum

A

inferior mesenteric lymph nodes

51
Q

Bilirubin metabolism

A

Heme broken down by heme oxygenase into BILIVERDIN (green color) broken down by biliverdin reductase to unconjugated bilirubin. This then gets conjugated by glucuronyl transferase

In the GI: gets turned to urobilinogen –> stercobilin –> brown color to feces

52
Q

EBV binds to what receptor on B cells?

A

CD21 (aka CR2)

also the CD21 are present on nasopharyngeal epithelial cells

53
Q

best meds for chemo-induced N/V

A

5-HT3 receptor antagonists (ondansetron, granisetron, dolasetron)

Also NK1 receptor antagonists (aprepitant), inhibits substance P

54
Q

What part of the spleen is responsible for removing damaged RBCs?

A

Reticuloendothelial cells in splenic red pulp

55
Q

Drugs that increase warfarin activity (CYP450 inhibitors)

A
Acetaminophen, NSAIDs
Abx/antifungal (metronidazole)
Amiodarone
Cimetidine
Cranberry juice/ginko bilboa/ vitamin E
Omeprazole
Thyr hormone
SSRIs
56
Q

Drugs that decrease warfarin activity (cyp450 inducers)

A
carbamazepine, phenytoin
Ginseng and St. Johns Wort
OCPs
Phenobarbital
Rifampin
57
Q

What is DiGeorge syndrome?

A

maldevelopment of third and fourth pharyngeal pouches. Thymic aplasia –> extreme deficiency in mature T lymphocytes. Thus you see decreased paracortex region in LN and no thyme shadow. Predisposed to recurrent infx by viral, fungal, protozoa, and intracellular bacterial pathogens

cardiac abnormalities, parathyroid defects

58
Q

What’s in medullary cords of Lymph nodes?

A

B cells, plasma cells, macrophages

59
Q

What’s in medullary sinuses of lymph nodes

A

reticular cells and macrophages

60
Q

IL-2 use in cancer tx?

A

regulates activation/differentiation of T cells; aids in tumor cell destruction. FDA approved for RCC and melanoma

61
Q

What does ristocetin do?

A

Activates GPIb receptors on platelets to make them available for vWF binding. If adding this doesn’t help them agglutinate, then it’s characteristic of vW disease

If vWF is low, there is poor platelet aggregation in presence of ristocetin.

62
Q

Dermatomyositis is a paraneoplastic syndrome of which neoplasms?

A

ovarian, lung, colorectal, non-Hodgkin lymphoma

Look for heliotrope rash, gottron’s papule (raised erythematous plaques on joints of hand), and muscle biopsy showing perimysial infiltrates. Proximal weakness.

63
Q

What is 2,3-BPG?

A

Molecule in glycolysis specifically in RBCs, bypasses the ATP-creating step; it’s useful because it decreases hemoglobin’s affinity for oxygen, and gets increased in hypoxia so the RBC can more easily hand off O2 to the hypoxic tissues.

64
Q

What part of antibody chain determines the 5 isotopes?

A

Heavy chain constant regions; they are isotype specific. Class switching between the different Ig types happens in the germinal centers of the LN

65
Q

Initial isotype switch (between different Ig molecules) requires what?

A

CD40 receptor on activated B-cells w/ CD40 ligand (CD154) on activated T cells.
Then can occur through genetic rearrangement of heavy chain constant regions.

66
Q

Deficiencies in C5-9?

A

These are components of membrane attack complex. w/out them (made by liver) you can get recurrent infx with neisseria meningitides or N. Gonorrhoeae

67
Q

What enzyme is responsible for reducing methemoglobin back to hemoglobin?

A

NADH methemoglobin reductase

68
Q

Electrolyte side effect of blood transfusion?

A

hypocalcemia and/or hypomagnesemia, due to chelation by infused citrate (which is an anticoagulant stored in the PRBCs)

69
Q

Where is factor 8 synthesized?

A

endothelial cells

others are synthesized in the liver

70
Q

t(8;14)

A

burrito lymphoma (c-myc activation)

71
Q

t(9;22)

A

philadelphia chromosome BCR-ABL hybrid

“Philadelphia CreaML cheese” (CML)

72
Q

t(11;14)

A

Mantle cell lymphoma (Cyclin-D1 activation)

73
Q

t(14;18)

A

Follicular lymphoma (BCL-2 activation)

74
Q

t(15;17)

A

M3 type of AML - responds to all-trans retinoic acid

75
Q

Causes of sideroblastic anemia?

A

Isoniazid use (low b6), x linked ALAS mutation, myelodysplastic syndrome, EtOH abuse, copper deficiency, and other meds (chloramphenicol, linezolid_

76
Q

Pappenheimer body

A

iron inclusions in phagosomes in RBCs during sideroblastic anemia

77
Q

what bacteria should we be aware of for chronic granulomatous disease?

A

S. aureus, Burkholderia cepacia (Wtf), serratia, nocardia, and aspergillus
(catalase positive)

78
Q

CD14

A

surface marker for monocyte-macrophage cell lineage

79
Q

tx for primary myelofibrosis

A

ruxolitinib