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

First Aid pg 397-401 Heme Flashcards

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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
Which blood tests are elevated in vW disease?
BT inc, and PTT may be normal or Inc
26
Explain the Ristocetin test result of vW Disease?
No platelet aggregation, without vW.
27
Which two hemat diseases are treated with desmopressin?
Hemophilia A, vW disease (affiliated with FVIII)
28
Causes of DIC?
Sepsis, Trauma, Obstetric complications, acute Pancreatitis, Malignancy, Nephrotic syndrome, Transfusion, Rattlesnake venom , AML
29
Lab values of DIC?
Inc fibrin degradation, dec fibrinogen, Dec factors V and VIII
30
How does Antithrombin deficiency affect PT, PTT, and thrombin time? It can affect which of those values following an admin of which drug?
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
Nephrotic syndrome is associated with which two coagulation disorders?
DIC and Antithrombin deficiency
32
What type of mutation in FV Leiden?
DNA point mutation, G --> Arg506Gln
33
The mutation is FV Leiden def stops its degradation by what protein?
Prot C
34
Most common cause of hypercoagulability in Caucasians?
F V Leiden
35
What issue specific to women must one be concerned about in terms of FV Leiden def?
1) Inc risk of PE with OCP | 2) Recurrent pregnancy loss
36
Prot C and S def will lead inability to inhibit which factors?
Factor Va and VIIIa
37
Be careful with prescribing which anti-coag when it comes to Prot C or S def?
Warfarin - inc risk of skin necrosis
38
location of Gene mutation of Prothrombin?
Mutation in 3' UTR
39
Risk associated with blood transfusion?
transfusion reactions, Fe overload (could lead to 2ndary hemochromatosis) hypocalcemia, hyperkalemia
40
Viruses associated with Hodgkins and Non Hodgkins lymphoma?
EBV and HIV
41
Which type of lymphoma is assoc with B symptoms? What are those symptoms?
Hodgkin's, low grade fever, night sweats, weight loss
42
CD # of Reed-Sternberg cells?
CD15, CD30
43
What are the two genes affected in Burkitts? (not the translocation itself, but the products of them)
Chr 8 - c -myc | Chr 14 - Ig heavy chain
44
Tingible body macrophages associated with?
Burkitts
45
What type of lesion is seen in the sporadic form of Burkitts?
Pelvis and abdomen lesions
46
Most common type of Non Hodgkin's in adults?
Diffuse large B cell lymphoma
47
Why can blood transfusions lead to tingling sensations and paresthesias?
hypocalcemia
48
Gene products of the genes translocated in Mantle cell lymphoma?
Chr 11 - cyclin D1 (G1--> S) | Chr 14 - Ig heavy chain
49
What are the gene products of the gene translocation of Follicular lymphoma?
Chr 14 - Ig heavy chain | Chr 18 - bcl2
50
Which lymphoma presents with intermittent pain of lymph nodes?
Follicular lymphoma
51
What are the cell types in grades 1-3 of Follicular lymphoma?
Grade 1 - small cleaved cells Grade 2 - Mixed Grade 3 - large cells
52
Name an AIDS defining lymphoma?
Primary Central Nervous System Lymphoma
53
Presentation of PCNSL? Assoc with which virus?
Confusion, memory loss, seizures - assoc with EBV
54
3 diseases with mass lesions in CNS?
Toxoplasmosis, PCNL, GBM
55
Which lymphoma is associated with cutaneous lesion and tremor?
ATCL (Adult T cell ), tremor due to hypercalcemia, also associated with lytic bone lesions
56
Which condition is associated with lymphocytes with cerebriform nuclei? What CD #?
Mycosis Fungoides, CD 4+
57
Ig associated with M spike in Mult myeloma and in Waldenstrom macroglobulinemia?
MM - IgG, IgA | WM - IgM
58
What does the M in M spike mean?
M is not for IgM, but for Monoclonal - one clonal type of Ig
59
What type of amyloidosis seen in MM?
Primary (AL)
60
What are Bence Jones proteins?
Ig light chains
61
Name two signs of MM you'd see in a blood smear?
Stacked RBC (rouleaux) and plasma cells with "clock face" chromatin
62
The intracytoplasmic inculsions of plasma cells in MM contain what?
Ig
63
Clinical diff between MGUS and MM?
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
What hematological issue has visual defect, bleeds, strokes, Raynauds?
Waldenstrom Macroglobulinemia - Sx from hyperviscosity
65
Neutrophils with bilobed nuclei seen after chemo?
Pseudo Pelger Huet anomaly
66
Causes of MDS?
de novo mutations, environmental (radiation, benzene, chemo)
67
MDS can lead to what type of leukemia?
AML
68
What is the Warburg effect, esp in relation to Burkitt's
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
Desmopressin also used in what neuro issue?
Enuresis
70
What disease looks very similar clinically to FV Leiden def?
Anti phospholipid syndrome
71
Deferoxamine is also used in what chemo drug tox?
Doxorubicin
72
MDS is most common acquired cause of heme issues?
Sideroblastic anemia

FIRST aid 2015 - Sahaja (31 decks)

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