Heme Part 1 Flashcards

0
Q

Hemostasis

A

Arrest of bleeding

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1
Q

Hemarthrosis

A

Bleeding into joint

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2
Q

Virchows Triangle

A
  1. Vascular component
  2. Platelets
  3. Plasma Proteins
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3
Q

Purpura

A

Superficial dermatological bleeding due to functional platelet problem or vascular abnormality
>.5 cm

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4
Q

Petechiae

A

Usually from thrombocytopenia. Not a functional problem. <.5cm

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5
Q

Romiplostim

A

Med used during chemotherapy and for ITP when platelet counts are low. Attaches to thrombopoietin receptor and activates STAT5 for megakaryocyte proliferation and differentiation and JAK2 for protein production and shedding of platelets.

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6
Q

Thrombopoietin

A

Stimulus for megakaryocytes when there is a low platelet count causing megakaryocyte nuclei to divide

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7
Q

EDTA

A

Calcium chelator that makes it easier for antibodies to attach to platelets

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8
Q

Aggregation Dependent GPIIbIIIa

A

Epi, ADP, collagen

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9
Q

Adhesion dependent

A

Ristocetin

Dependent on presence of GPI and vWF

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10
Q

ASA

A

Irreversible COX inhibitor that blocks production of thromboxane (7 - 10 days)

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11
Q

NSAIDS

A

Reversible COX inhibitor that blocks production of thromboxane

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12
Q

Clopidogrel

A

Plavix. ADP receptor inhibitor block activation of platelets (GPIIbIIIa).

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13
Q

Prasugrel (Effient)

A

ADP receptor inhibitor that blocks activation of platelets (GPIIbIIIa)

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14
Q

Ticlopidine (Ticlid)

A

ADP receptor inhibitor that blocks activation of platelets (GPIIBIIIA)

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15
Q

Abciximab (ReoPro)

A

GPIIBIIIA inhibitor blocking platelet aggregation. Can still have adhesion.

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16
Q

Eptifibatid (Integrilin)

A

GPIIBIIIA inhibitor blocking platelet aggregation. Can still have adhesion.

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17
Q

Tirofiban (Aggrastat)

A

GPIIBIIIA inhibitor blocking platelet aggregation. Can still have adhesion.

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18
Q

Dipyridamole (Persantine)

A

Adenosine reuptake inhibitor increasing cAMP which potentiates PGI2causing vasodilation and thus decrease platelet aggregation

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19
Q

May-Hegglin Anomaly

A

Autosomal dominant thrombocytopenia - non-muscle myosin heavy chain 11a causing mild to mod bruising and large platelets

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20
Q

Wiskott-Aldrich Syndrome

A

X-linked thrombocytopenia. Defect in WASP protein - cannot make IgM antibodies leading to eczema, bloody diarrhea, and immunodeficiency

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21
Q

Congenital Amegakaryocytic Thrombocytopenia

A

Autosomal recessive thrombocytopenia caused by a platelet growth factor receptor mutation. No megakaryocytes so will need a bone marrow transplant.

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22
Q

Thiazides

A

First line HTN drug that can have a side effect of thrombocytopenia

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23
Q

Tolbutamide

A

DM type 2 medication to increase insulin production. May have a side effect of thrombocytopenia.

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24
Q

Evans Syndrome

A

An autoimmune hemolytic anemia with reticulocytosis and spherocytes that can occur with ITP

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25
Q

WinRho

A

Ab to Rh factor in Rh + patients

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26
Q

Dexamethazone

A

Suppress production. More effective with Rituximab for ITP.

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27
Q

Rituximab

A

Attaches to CD20 on B cells and will not be able to make plasma cell antibodies

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28
Q

Danazol

A

Attenuated androgen

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29
Q

Romiplostim and eltrombopag

A

Thrombopoietin receptor agonist

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30
Q

TTP Pentad

A
Fever
Thrombocytopenia 
Microangiopathic hemolytic anemia 
Transient neurological deficits
Renal failure
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31
Q

ADAMTS13

A

Enzyme that normally degrades very high molecular weight multimers of vWF that accumulate in plasma and promote platelet activation. Deficient in TTP

32
Q

Reticulocytosis schistovytes

A

Fragmented rbcs in anemia

33
Q

Protamine sulfate

A

Antidote to heparin

34
Q

Coombs test

A

Checks for antibodies to rbcs

35
Q

Enoxaparin

A

Lovenox. LMW heparin. Do need blood tests to monitor.

36
Q

Argatroban

A

Direct thrombin inhibitor. May be used in heparin induced thrombocytopenia.

37
Q

Lepirudin (Refludan)

A

Direct thrombin inhibitor. May be used in heparin induced thrombocytopenia

38
Q

HELLP syndrome

A

Hemolysis elevated liver enzymes and low platelet

Associated with thrombocytopenia in the 3rd trimester

39
Q

Bernard-Soulier Syndrome

A

Auto recessive - GPIb. Causes severe bleeding due to no adhesion.

40
Q

Glandzmann Thrombasthenia

A

Autosomal recessive - GPIIbIIIa.

41
Q

vonWillebrand Disease

A

Autosomal dominant - reduced activity or abnormal structure of vWF

42
Q

Grey platelet syndrome

A

Absence of alpha granules. Life long mild bleeding.

43
Q

Dense granule deficiency

A

ADP and serotonin deficiency, mild to mod bleeding

44
Q

Epoxide reductase

A

Enzyme that reduces Vitamin K for the production of clotting factors 2, 7,9, and 10. Warfarin blocks this enzyme.

45
Q

Alpha 2 antiplasmin

A

Inhibitor of non-fibrin bound clot (free floating) associated plasmin. Does not affect plasmin already attached to a clot.

46
Q

Hemophilia A

A

X-linked factor VIII deficiency

47
Q

Christmas disease

A

Hemophilia B. X linked abnormally functioning or deficient factor IX

48
Q

Hemophilia C

A

Autosomal recessive - factor XI deficiency

49
Q

What ate the primary target organs of acute GVHD?

A

Skin, gut epithelium (distal ileum is most severe) bile ducts, and lymphoid tissue

50
Q

What are the primary determinants of successful bone marrow transplant?

A

1) closeness of HLA types 2) severity of the GVHD 3) ability of the host to withstand the conditioning regimen

51
Q

Most common infection post- transplant

A

Viral infections - CMV. Shows up as a general interstitial thickening by a mononuclear infiltrate and scattered cells with enlarged nuclei.

52
Q

CMV pneumonitis

A

Infected resp. epithelial cells 2-4x larger than normal and intranuclear inclusion that is eccentrically placed and surrounded by a clear halo (owls eye appearance)

53
Q

Satellitosis

A

Individual apoptotic keratinocytes seen in association with lymphocytes

54
Q

Spongiosis

A

Loosening of intercellular junctions creating increased spaces between keratinocytes

55
Q

Within the skin, what are the actual initial target cells?

A

Cells in the basal layer - these are the stem cells of the skin

56
Q

Graft vs Host disease

A

Lookup

57
Q

Graft vs. leukemia effect

A

Lookup

58
Q

Cyclophosphamide

A

Lookup

59
Q

DVT

A

Lookup

60
Q

Three natural anticoagulants

A

Antithrombin, protein C, protein S

61
Q

Factor V Leiden

A

Point mutation in FV position 506 causing Gln to replace Arg this leads to a continued generation of thrombin

62
Q

Hyperhomocysteinemia

A

Associated with thrombophilia especially arterial thrombosis. Acquired due to vitamin folate/B12 deficiency or the result of inherited methylene tetrahydrofolate reductase deficiency

63
Q

Why is Coumadin contraindicated in pregnancy?

A

It is associated with dysmorphic fetus especially in first trimester

64
Q

Iron deficiency anemia

A

Rbcs decreased in number and smaller than normal due to ineffective erythropoiesis. The central area of pallor is increased (hypochromia) - due to decreased hemoglobin

65
Q

Hemosiderin

A

Storage form of iron

66
Q

Prussian blue

A

Special stain that colors hemosiderin. Cytoplasm stains deep blue when there is a heavy conc. of storage iron (hemosiderin-laden MOs)

67
Q

Hemosiderin laden MO

A

Iron accumulates within MO as a byproduct of the breakdown of senescent red cells

68
Q

Transferrin

A

Delivers iron recovered from MOs to normoblasts to be used in the synthesis of hemoglobin

69
Q

Sideroblasts

A

Nucleated red cells containing tiny iron granules

70
Q

Most common cause of iron deficiency anemia in US

A

Chronic blood loss

71
Q

Major causes of megaloblastic anemia

A

Folate and B12 deficiency

72
Q

Tetrahydrofolate

A

Biologically active form of folic acid

73
Q

Folate vs. Vitamin B12 deficiency

A

B12 is failure to absorb and leads to neuro changes. Folate is inadequate intake and no neuro changes.

74
Q

Heme

A

Protoporphyrin + iron

75
Q

Primary hemostasis

A

Formation of a platelet plug at the site of vascular injury. Typically manifests as mucocutaneous bleeding.

76
Q

Secondary hemostasis

A

Activation of the clotting cascade and formation of a fibrin clot. Typically manifest as skin, muscle, or joint bleeding and severe postoperative bleeding

77
Q

Factor VIII

A

Gene is located on X chromosome. Factor synthesized by endothelial cells lining the sinusoids of liver. Short half life of 12 hrs so carried on vWF to protect from proteolysis

78
Q

Desmopressin (DDAVP)

A

Tx for vWF disease which acts on endothelial cells to release stored vWF and then also increase factor 8 in circulation