Hematology Flashcards

1
Q

Abciximab, epifibatide, tirofiban

A

Inhibits GpIIb/IIIa directly

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2
Q

Ristocetin (function)

A

Activates vWF to bind GpIb.

Failure of aggregantion: vWF D and Bernard- Soulier synd

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3
Q

FFP and prothrombin complex concentrate

A

Reverse action of warfarin immediately. Can be given with Vit K in cases of severe bleeding

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4
Q

Factor Shortest half life

A

F (7)

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5
Q

Factor longest Half-life

A

F (2)

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6
Q

Antithrombin (MOA)

A

Inhibits thrombin (IIa)
Factors: 7a, 9a,10a,11a,12a

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7
Q

Heparin

A

Enhances the activity of antithrombin

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8
Q

Principal target of antithrombin

A

Thrombin (IIa) and factor Xa

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9
Q

tPA

A

Thrombolytic

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10
Q

Factor V Leiden mutation

A

Factor V resistant to inhibition by activated protein C
Recurrent pregnancy loss

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11
Q

Iron deficiency

A

Low iron
High TIBC
Low Ferritin
High RDW
Low RI
Spoon nails (Koilonychia)

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12
Q

Plummer Vinson Synd

A

Iron deficiency anemia
Esophageal webs
Dysphagia

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13
Q

alpha-thlassemia

A

a-globin gene deletion on chromosome 16.
Can be cis or trans

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14
Q

A thalasemia
Hemoglobin H disease (- -/ - a) ; excess B globin forms B4

A

Moderate to severe microcytic hypochromic anemia

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15
Q

A thalasemia
Hemoglobin Barts disease (- -/ - -) ; NO a globin. Excess gamma globin form

A

Hydrops fetalis; incompatible with life

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16
Q

B THALASEMMIA

A

Point mutation in splice sites and promoter sequences on chromosome 11. High prevalence in Mediterranean descent.

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17
Q

B thalassemia minor (heterozygote)

A

B underproduced
Usually asymptomatic
Dx high HbA2 (> 3.5%)

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18
Q

B thalassemia major (homozygote)

A

B chain absent
Target cells
Anisopoikilocytosis
Marrow expansion (crew cut on skull)
Chipmunk facies
Hepatosplenomegaly
High risk of parvo B19
High HbF and HbA2

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19
Q

Lead poisoning (enzymes inhibition)

A

Inhibits ferrochelatase and ALA dehydratase

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20
Q

Lead poisoning symptoms

A

Lead lines on gingivae (burton) and metaphyses
Encephalopathy
Erythrocytes basophilic stippling
Abdominal colic
Sideroblastic anemia
Drops (wrist and foot droop)

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21
Q

Lead poisoning tx

A

Chelation (succimer)
EDTA
Dimercaprol

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22
Q

Sideroblastic Anemia causes:

A

X-linked defect ALA synthase
Alcohol
Lead poisoning
Vitamin B6 deficiency
Copper deficiency
Drugs (isoniazid, linezolid)

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23
Q

Sideroblastic anemia findings

A

High iron
Normal or low TIBC
High ferritin
Ringed sideroblast
Basophilic stippling of RBC

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24
Q

Sideroblastic anemia Tx:

A

B6 pyridoxine (cofactor of ALA synthase)

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25
Q

Megaloblastic anemia causes:

A

Vitamin B12 defi
Folate defi
Meds:
- hydroxyurea
- phenytoin
- methotrexate
- sulfa drugs

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26
Q

High homocysteine, NORMAL methylmalonic acid

A

Folate deficiency

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27
Q

High homocysteine, high methylmalonic acid

A

Vitamin B12 deficiency

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28
Q

Orotic aciduria cause:

A

Defect in UMP synthase
Inability to convert OROTIC acid to UMP

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29
Q

Orotic aciduria symptoms

A

Present in children as:
Failure to thrive
Developmental delay
Megaloblastic anemia refractory to folate and B12
Orotic acid in urine

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30
Q

Hemolysis lead to:

A

Increased LDH
Low haptoglobin
Reticulocytes
Unconjugated bilirubin
Pigmented gallstones
Urobilinogen in urine

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31
Q

Sickle cell anemia

A

Point mutation in B-globin gene
Glutamic acid ——> valine

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32
Q

Sickle cell anemia (AR) : electrophoresis

A

Low HbA
High HbF
High High HbS

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33
Q

Sickle cell anemia Tx:

A

Hydroxyurea (aumenta la HbF)

34
Q

Sickle cell anemia complications:

A

Aplastic crisis (parvo B19)
Autosplenectomy (risk of infection w/ S. Pneumoniae)
Salmonella osteomyelitis
Painful vaso-occlusive crisis
Acute chest Syndrome/ respiratory distress (cause of death)

35
Q

AIHA (Warm)

A

IgG
Seen in SLE, CLL
With drugs: b-lactams, a-methyldopa

36
Q

AIHA (Cold)

A

IgM
CLL
Mycoplasma
Infectious mononucleosis
Painful blue fingers and toes

37
Q

Warm AIHA tx

A

Steroids
Rituximab
Splenectomy (if refractory)

38
Q

Cold AIHA Tx:

A

Cold Avoidance
Rituximab

39
Q

Acute intermittent porphyria (enzyme defect)

A

Porphobilinogen deaminase deficiency

40
Q

Acute intermittent porphyria symptoms :

A

5 P’s
Painful abdomen
Port wine Pee
Polyneuropathy
Psychological disturbances
Precipitated by alcohol, starvation

41
Q

Acute intermittent porphyria Tx:

A

Hemin and glucose

42
Q

Porphyria cutanea tarda: (enzyme)

A

Uroporphyrinogen decarboxylase deficiency

43
Q

Porphyria cutanea tarda symptoms:

A

Cutaneous photosensitivity, hyperpigmentation
Exacerbated by alcohol
Causes: familial, hepatitis C

44
Q

Chronic Iron poisoning symptoms

A

Arthropathy
Cirrhosis
Cardiomyopathy
Hypogonadism
Skin pigmentation

45
Q

PT test

A

Common (1,2,5,10) and Extrinsic pathway (7)

46
Q

INR (patient PT/ control PT): use

A

Most common test to follow pte on Warfarin, which prolongs INR

Normal =1
Prolonged > 1

47
Q

PTT test

A

Common an INTRINSIC pathway (all factor EXCEPT 7 y 13)

48
Q

Hemophilia A

A

Deficiency factor 8
X- recessive
Intrinsic coagulation defect (high PTT)

49
Q

Hemophilia B

A

Deficiency of factor 9
High PTT
X- Recessive

50
Q

Hemophilia C

A

Deficiency factor 11
AR

51
Q

Hemophilia symtoms

A

Hemarthroses (bleeding into joints)
Easy brusing
Bleeding after trauma (dental procedures)

52
Q

Vitamin K deficiency

A

High PT and PTT
Bleeding time normal
Decrease activity of 2 7 9 10 protein C and S

53
Q

Bernard soulier (AR)

A

Defect in adhesion
Decrease GpIb
Decrease platelet to vWF adhesion
Labs: abnormal ristocetin test
Large platelets

54
Q

Glanzmann thrombasthenia (AR)

A

Defect in aggregation
Low GpIIb/IIIa
Low platelet to platelet aggregation

Labs: no platelet clumping

55
Q

Immune thrombocytopenia

A

Low platelet count
Anti- GpIIb/IIIa antibodies
Splenic macrophages phagocytes platelets
Labs: high megakaryocytes on BM

56
Q

Von Williebrand disease

A

Intrinsic pathway defect: high PTT
vWF carries Factor 8
AD
High bleeding time
Platelet count: normal

57
Q

Disseminated intravascular coagulation (DIC)

A

Widespread clotting factor activation
Low PC
High BT
High PT
High PTT

Labs: high fibrin degradation products (D- dimers)
Low fibrinogen
Low factors V and factor VIII

58
Q

Heparin overdose

A

Protamine sulfate

59
Q

Burkitt

A

T(8;14) c-MYC
Starry sky
Asso EBV
Jaw lesion

60
Q

Diffuse large B cell lymphoma

A

BCL-2 , BCL-6
Mc type in adults

61
Q

Follicular lymphoma

A

t(14;18)
BCL2 (inhibits apoptosis)
Indolent
Waxing and waning lymphadenopathy

62
Q

Mantle cell lymphoma

A

t(11;14) translocation of cyclin D1
Very aggressive

63
Q

Marginal zone

A

t(11;18)
Asso w/ chronic inflammation (sjogren syndrome)
Chronic gastritis

64
Q

Primary Central nervous system lymphoma

A

EBV related
HIV/AIDS
CNS mass (ring enhancing lesion)

65
Q

What cell has the RANKL?

A

Osteoblast

66
Q

CO poisoning changes

A

Increase carboxyhemoglobin concentration
Does NOT affect the PaO2
Left shift of the hb-oxygen dissociation curve.

67
Q

G6PD deficiency

A

Decrease NADPH
Reducen glutathione
High RBC susceptible to oxidative stress
Heinz bodies and bite cells

68
Q

Heparin induced thrombocytopenia type 2 cause:

A

IgG formation to complexes of heparin and endogenous platelet factor 4.

69
Q

Major manifestation of HIT type 2

A

Thrombocytopenia (is due to de destruction of ab-coated platelets by splenic macrophages)

70
Q

Burkitt lymphoma asso w/ virus ?

A

Epstein- Barr virus

71
Q

Burkitt lymphoma
Myc (function?)

A

Function as a Transcription activator

72
Q

HbS (valine in place of glutamic) promotes:

A

Hydrophobic interaction among Hb molecules and results in HbS polymerization and erythrocyte sickling

73
Q

Döhle bodies

A

Blue cytoplasmatic inclusions of rough endoplasmic reticulum

74
Q

Leukocyte alkaline phosphatase is found in:

A

Maturing neutrophils
Normal or increased in (Leukemoid reaction)
Usually low in CML

75
Q

Basophilic stippling (made of ? And see in what disorders)

A

Blue granules (ribosomal precipitates) in the cytoplasm of RBC
See in thalassemia, alcohol use disorder, lead/heavy mental poisoning

76
Q

Acute hemolytic transfusion (ABO) example of:

A

Ab-mediated (type II) hypersensitivity
Leads to complement mediated cell lysis

77
Q

Acute intermittent porphyria tx is Hemin (MOA)

A

Hemin downregulates hepatic aminolevulinate (ALA) synthase

78
Q

Heinz bodies made of:

A

Denatured hemoglobin

79
Q

Reticulocytes appear as blue- gray on Wright- giemsa staining due to precesence of:

A

Ribosomal RNA
See in iron deficiency anemia

80
Q

Tumor lysis syndrome (levels of ions)

A

Hyperkalemia
Hyperphosphatemia
Hypocalcemia

81
Q

Tumor lysis syndrome tx:

A

Hydration
Allopurinol
Rasburicase