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STEP 1 > Hematology > Flashcards

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
Megaloblastic anemia causes:
Vitamin B12 defi Folate defi Meds: - hydroxyurea - phenytoin - methotrexate - sulfa drugs
26
High homocysteine, NORMAL methylmalonic acid
Folate deficiency
27
High homocysteine, high methylmalonic acid
Vitamin B12 deficiency
28
Orotic aciduria cause:
Defect in UMP synthase Inability to convert OROTIC acid to UMP
29
Orotic aciduria symptoms
Present in children as: Failure to thrive Developmental delay Megaloblastic anemia refractory to folate and B12 Orotic acid in urine
30
Hemolysis lead to:
Increased LDH Low haptoglobin Reticulocytes Unconjugated bilirubin Pigmented gallstones Urobilinogen in urine
31
Sickle cell anemia
Point mutation in B-globin gene Glutamic acid ——> valine
32
Sickle cell anemia (AR) : electrophoresis
Low HbA High HbF High High HbS
33
Sickle cell anemia Tx:
Hydroxyurea (aumenta la HbF)
34
Sickle cell anemia complications:
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
AIHA (Warm)
IgG Seen in SLE, CLL With drugs: b-lactams, a-methyldopa
36
AIHA (Cold)
IgM CLL Mycoplasma Infectious mononucleosis Painful blue fingers and toes
37
Warm AIHA tx
Steroids Rituximab Splenectomy (if refractory)
38
Cold AIHA Tx:
Cold Avoidance Rituximab
39
Acute intermittent porphyria (enzyme defect)
Porphobilinogen deaminase deficiency
40
Acute intermittent porphyria symptoms :
5 P’s Painful abdomen Port wine Pee Polyneuropathy Psychological disturbances Precipitated by alcohol, starvation
41
Acute intermittent porphyria Tx:
Hemin and glucose
42
Porphyria cutanea tarda: (enzyme)
Uroporphyrinogen decarboxylase deficiency
43
Porphyria cutanea tarda symptoms:
Cutaneous photosensitivity, hyperpigmentation Exacerbated by alcohol Causes: familial, hepatitis C
44
Chronic Iron poisoning symptoms
Arthropathy Cirrhosis Cardiomyopathy Hypogonadism Skin pigmentation
45
PT test
Common (1,2,5,10) and Extrinsic pathway (7)
46
INR (patient PT/ control PT): use
Most common test to follow pte on Warfarin, which prolongs INR Normal =1 Prolonged > 1
47
PTT test
Common an INTRINSIC pathway (all factor EXCEPT 7 y 13)
48
Hemophilia A
Deficiency factor 8 X- recessive Intrinsic coagulation defect (high PTT)
49
Hemophilia B
Deficiency of factor 9 High PTT X- Recessive
50
Hemophilia C
Deficiency factor 11 AR
51
Hemophilia symtoms
Hemarthroses (bleeding into joints) Easy brusing Bleeding after trauma (dental procedures)
52
Vitamin K deficiency
High PT and PTT Bleeding time normal Decrease activity of 2 7 9 10 protein C and S
53
Bernard soulier (AR)
Defect in adhesion Decrease GpIb Decrease platelet to vWF adhesion Labs: abnormal ristocetin test Large platelets
54
Glanzmann thrombasthenia (AR)
Defect in aggregation Low GpIIb/IIIa Low platelet to platelet aggregation Labs: no platelet clumping
55
Immune thrombocytopenia
Low platelet count Anti- GpIIb/IIIa antibodies Splenic macrophages phagocytes platelets Labs: high megakaryocytes on BM
56
Von Williebrand disease
Intrinsic pathway defect: high PTT vWF carries Factor 8 AD High bleeding time Platelet count: normal
57
Disseminated intravascular coagulation (DIC)
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
Heparin overdose
Protamine sulfate
59
Burkitt
T(8;14) c-MYC Starry sky Asso EBV Jaw lesion
60
Diffuse large B cell lymphoma
BCL-2 , BCL-6 Mc type in adults
61
Follicular lymphoma
t(14;18) BCL2 (inhibits apoptosis) Indolent Waxing and waning lymphadenopathy
62
Mantle cell lymphoma
t(11;14) translocation of cyclin D1 Very aggressive
63
Marginal zone
t(11;18) Asso w/ chronic inflammation (sjogren syndrome) Chronic gastritis
64
Primary Central nervous system lymphoma
EBV related HIV/AIDS CNS mass (ring enhancing lesion)
65
What cell has the RANKL?
Osteoblast
66
CO poisoning changes
Increase carboxyhemoglobin concentration Does NOT affect the PaO2 Left shift of the hb-oxygen dissociation curve.
67
G6PD deficiency
Decrease NADPH Reducen glutathione High RBC susceptible to oxidative stress Heinz bodies and bite cells
68
Heparin induced thrombocytopenia type 2 cause:
IgG formation to complexes of heparin and endogenous platelet factor 4.
69
Major manifestation of HIT type 2
Thrombocytopenia (is due to de destruction of ab-coated platelets by splenic macrophages)
70
Burkitt lymphoma asso w/ virus ?
Epstein- Barr virus
71
Burkitt lymphoma Myc (function?)
Function as a Transcription activator
72
HbS (valine in place of glutamic) promotes:
Hydrophobic interaction among Hb molecules and results in HbS polymerization and erythrocyte sickling
73
Döhle bodies
Blue cytoplasmatic inclusions of rough endoplasmic reticulum
74
Leukocyte alkaline phosphatase is found in:
Maturing neutrophils Normal or increased in (Leukemoid reaction) Usually low in CML
75
Basophilic stippling (made of ? And see in what disorders)
Blue granules (ribosomal precipitates) in the cytoplasm of RBC See in thalassemia, alcohol use disorder, lead/heavy mental poisoning
76
Acute hemolytic transfusion (ABO) example of:
Ab-mediated (type II) hypersensitivity Leads to complement mediated cell lysis
77
Acute intermittent porphyria tx is Hemin (MOA)
Hemin downregulates hepatic aminolevulinate (ALA) synthase
78
Heinz bodies made of:
Denatured hemoglobin
79
Reticulocytes appear as blue- gray on Wright- giemsa staining due to precesence of:
Ribosomal RNA See in iron deficiency anemia
80
Tumor lysis syndrome (levels of ions)
Hyperkalemia Hyperphosphatemia Hypocalcemia
81
Tumor lysis syndrome tx:
Hydration Allopurinol Rasburicase

STEP 1 (18 decks)

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