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FIRST aid 2015 - Sahaja > First Aid pg 397-401 Heme > Flashcards

First Aid pg 397-401 Heme Flashcards

1
Q

What causes cell death due to peroxidation of membrane lipids?

A

Fe poisoning

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2
Q

Symptoms of Fe poisoning?

A

n/v, gastric bleeding, lethargy, scarring leading to GI obstruction

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3
Q

Drugs used for Fe poisoning?

A

Chelation - IV deferoxamine, oral deferasirox

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4
Q

Which factors are tested in PT?

A

Factors I, II, V, VII, and X

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5
Q

Which factors are not tested in PTT?

A

All factors except VII and XIII

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6
Q

Inheritance patterns of all three hemophilias?

A

Hemophilia A and B - XLR, and C is AR

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7
Q

Tx for Hemophilia A?

A

Desmopressin + factor VIII concentrate

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8
Q

Vitamin K def will have an increase in what lab tests?

A

PT and PTT, Bleeding time is Normal

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9
Q

What parts of the coag pathway are affected by Vit K def?

A

F II, VII, IX, X, and Prot C and S

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10
Q

Which 3 hemat diseases have an issue with platelet plug formation?

A

Both Bernard Soulier, Glanzmann thrombasthenia, vW disease

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11
Q

Which platelet disorder has normal platelet count?

A

Glanzmann , vW disease

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12
Q

With which heme disorder will you potentially see enlarged platelets?

A

Bernard Soulier - release of relatively immature platelets to replace loss.

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13
Q

Which heme disorder is associated with inc BUN ?

A

TTP, DIC, Hemolytic Uremic Syndrome, PNH, Sickle Cell, porphyria, Multple Myeloma

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14
Q

What enzyme activates vit K?

A

vit K epoxide reductase

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15
Q

Normal bleeding time?

A

2-7 min

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16
Q

Normal PT time?

A

11-15 sec

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17
Q

Normal PTT time?

A

25-40 sec

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18
Q

Which two hematological disorders affect GpIIb/IIIa? How do they effect it?

A

Glanzmann - dec levels of GpIIb/IIIa

Immune Thrombocytopenia - anti GpIIb/IIIa antibodies

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19
Q

Which hemat disorder show inc megakaryocytes on bone marrow biopsy?

A

ITP

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20
Q

Which hemat disorder has a def of metalloprotease?

A

TTP - ADAMTS 13 = vWF metalloprotease

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21
Q

Schistocytes are seen in which platelet disorder? why?

A

TTP - due to microthrombi formation (inc vWF multimers –> Inc platelet adhesion –> Inc platelet aggregation and thrombosis) DIC, HUS

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22
Q

Which two hemat disorders have neurological symptoms?

A

TTP and HUS - pentad of neuro/renal symptoms, fever, thrombocytopenia, fever, microangiopathic hemolyic anemia

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23
Q

What is the difference between HUS in adults and children?

A

Children tend to have diarrhea, adults do not.

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24
Q

Most common cause of dec platelet #?

A

ITP

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25
Q

Which blood tests are elevated in vW disease?

A

BT inc, and PTT may be normal or Inc

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26
Q

Explain the Ristocetin test result of vW Disease?

A

No platelet aggregation, without vW.

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27
Q

Which two hemat diseases are treated with desmopressin?

A

Hemophilia A, vW disease (affiliated with FVIII)

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28
Q

Causes of DIC?

A

Sepsis, Trauma, Obstetric complications, acute Pancreatitis, Malignancy, Nephrotic syndrome, Transfusion, Rattlesnake venom , AML

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29
Q

Lab values of DIC?

A

Inc fibrin degradation, dec fibrinogen, Dec factors V and VIII

30
Q

How does Antithrombin deficiency affect PT, PTT, and thrombin time? It can affect which of those values following an admin of which drug?

A

No direct effect on PT, PTT, or thrombin time. However, it can decrease the level of PTT increase following Heparin admin. (PTT is normally used to follow Heparin admin)

31
Q

Nephrotic syndrome is associated with which two coagulation disorders?

A

DIC and Antithrombin deficiency

32
Q

What type of mutation in FV Leiden?

A

DNA point mutation, G –> Arg506Gln

33
Q

The mutation is FV Leiden def stops its degradation by what protein?

A

Prot C

34
Q

Most common cause of hypercoagulability in Caucasians?

A

F V Leiden

35
Q

What issue specific to women must one be concerned about in terms of FV Leiden def?

A

1) Inc risk of PE with OCP

2) Recurrent pregnancy loss

36
Q

Prot C and S def will lead inability to inhibit which factors?

A

Factor Va and VIIIa

37
Q

Be careful with prescribing which anti-coag when it comes to Prot C or S def?

A

Warfarin - inc risk of skin necrosis

38
Q

location of Gene mutation of Prothrombin?

A

Mutation in 3’ UTR

39
Q

Risk associated with blood transfusion?

A

transfusion reactions, Fe overload (could lead to 2ndary hemochromatosis) hypocalcemia, hyperkalemia

40
Q

Viruses associated with Hodgkins and Non Hodgkins lymphoma?

A

EBV and HIV

41
Q

Which type of lymphoma is assoc with B symptoms? What are those symptoms?

A

Hodgkin’s, low grade fever, night sweats, weight loss

42
Q

CD # of Reed-Sternberg cells?

A

CD15, CD30

43
Q

What are the two genes affected in Burkitts? (not the translocation itself, but the products of them)

A

Chr 8 - c -myc

Chr 14 - Ig heavy chain

44
Q

Tingible body macrophages associated with?

A

Burkitts

45
Q

What type of lesion is seen in the sporadic form of Burkitts?

A

Pelvis and abdomen lesions

46
Q

Most common type of Non Hodgkin’s in adults?

A

Diffuse large B cell lymphoma

47
Q

Why can blood transfusions lead to tingling sensations and paresthesias?

A

hypocalcemia

48
Q

Gene products of the genes translocated in Mantle cell lymphoma?

A

Chr 11 - cyclin D1 (G1–> S)

Chr 14 - Ig heavy chain

49
Q

What are the gene products of the gene translocation of Follicular lymphoma?

A

Chr 14 - Ig heavy chain

Chr 18 - bcl2

50
Q

Which lymphoma presents with intermittent pain of lymph nodes?

A

Follicular lymphoma

51
Q

What are the cell types in grades 1-3 of Follicular lymphoma?

A

Grade 1 - small cleaved cells
Grade 2 - Mixed
Grade 3 - large cells

52
Q

Name an AIDS defining lymphoma?

A

Primary Central Nervous System Lymphoma

53
Q

Presentation of PCNSL? Assoc with which virus?

A

Confusion, memory loss, seizures - assoc with EBV

54
Q

3 diseases with mass lesions in CNS?

A

Toxoplasmosis, PCNL, GBM

55
Q

Which lymphoma is associated with cutaneous lesion and tremor?

A

ATCL (Adult T cell ), tremor due to hypercalcemia, also associated with lytic bone lesions

56
Q

Which condition is associated with lymphocytes with cerebriform nuclei? What CD #?

A

Mycosis Fungoides, CD 4+

57
Q

Ig associated with M spike in Mult myeloma and in Waldenstrom macroglobulinemia?

A

MM - IgG, IgA

WM - IgM

58
Q

What does the M in M spike mean?

A

M is not for IgM, but for Monoclonal - one clonal type of Ig

59
Q

What type of amyloidosis seen in MM?

A

Primary (AL)

60
Q

What are Bence Jones proteins?

A

Ig light chains

61
Q

Name two signs of MM you’d see in a blood smear?

A

Stacked RBC (rouleaux) and plasma cells with “clock face” chromatin

62
Q

The intracytoplasmic inculsions of plasma cells in MM contain what?

A

Ig

63
Q

Clinical diff between MGUS and MM?

A

There are no Crab findings -
C - HyperCalcemia
R - Renal issues (due to BJ prot blocking tubules)
A - Amyloid, Anemia (plasma cells taking BM)
B - Bone lytic lesions, Back pain, Bence- Jones

64
Q

What hematological issue has visual defect, bleeds, strokes, Raynauds?

A

Waldenstrom Macroglobulinemia - Sx from hyperviscosity

65
Q

Neutrophils with bilobed nuclei seen after chemo?

A

Pseudo Pelger Huet anomaly

66
Q

Causes of MDS?

A

de novo mutations, environmental (radiation, benzene, chemo)

67
Q

MDS can lead to what type of leukemia?

A

AML

68
Q

What is the Warburg effect, esp in relation to Burkitt’s

A

Cmyc - nucl phosphated prot –> transcription of activator that controls cell proliferation, differentiation, and apoptosis –> leads to upreg of anaerobic gycolysis = Warburg Effect

Warburg Effect - is the observation that most cancer cells predominantly produce energy by a high rate of glycolysis followed by lactic acid fermentation in the cytosol, rather than by a comparatively low rate of glycolysis followed by oxidation of pyruvate in mitochondria as in most normal cells.

69
Q

Desmopressin also used in what neuro issue?

A

Enuresis

70
Q

What disease looks very similar clinically to FV Leiden def?

A

Anti phospholipid syndrome

71
Q

Deferoxamine is also used in what chemo drug tox?

A

Doxorubicin

72
Q

MDS is most common acquired cause of heme issues?

A

Sideroblastic anemia

FIRST aid 2015 - Sahaja (31 decks)

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