Hematology Flashcards

1
Q

megaloblastic anemias

A

Macrocytic-Normochromic Anemias
Pernicious anemia
folate deficiency anemia

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2
Q

Methylmalonic acid and homocysteine levels are elevated early in the disease

A

pernicious anemia evaluation

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3
Q

Folate (folic acid) deficiency anemia s/s

A

Severe cheilosis: Scales and fissures of the lips and corners of the mouth
Stomatitis: Mouth inflammation
Painful ulcerations of the buccal mucosa and tongue: Characteristic of burning mouth syndrome
Dysphagia (difficulty swallowing), flatulence, and watery diarrhea

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4
Q

Folate (folic acid) deficiency anemia and CNS s/s

A

usually not seen

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5
Q

Pernicious anemia s/s

A

Weakness, fatigue
Paresthesias of the feet and fingers, difficulty walking
Loss of appetite, abdominal pains, weight loss
Sore tongue that is smooth and beefy red, secondary to atrophic glossitis
“Lemon yellow” (sallow) skin as a result of a combination of pallor and icterus
Neurologic symptoms from nerve demyelination - Not reversible

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6
Q
  • Most common type of anemia worldwide
A

Iron-deficiency anemia

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7
Q

Sideroblastic anemia cause

A

defect in mitochondrial heme synthesis. Altered mitochondrial metabolism causes ineffective iron uptake and results in dysfunctional hemoglobin synthesis

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8
Q

o Sideroblasts

A

Erythroblasts contain iron granules that have not been synthesized into hemoglobin.

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9
Q
  • Reversible sideroblastic anemia a/w
A

Associated with alcoholism

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10
Q

Sideroblastic anemia s/s

A

o Iron overload (hemochromatosis)
o Enlarged spleen (splenomegaly) and liver (hepatomegaly)

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11
Q

Sideroblastic anemia diagnostic

A

o Dimorphism

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12
Q

o Dimorphism

A

Normocytic and normochromic cells concomitantly observed with microcytic-hypochromic cells

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13
Q

Sideroblastic anemia tx (hereditary)

A

Pyridoxine (B6) therapy; life-long maintenance therapy at a lowered dose

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14
Q

Sideroblastic anemia tx (congenital)

A

Stem cell transplantation

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15
Q

Sideroblastic anemia tx (myelodysplastic synd (decreased RBC production))

A

Recombinant human erythropoietin

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16
Q
  • Pancytopenia
A

Reduction or absence of all three types of blood cells

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17
Q
  • Most aplastic anemias are
A

autoimmune disorders

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18
Q

Aplastic anemia patho

A

Hypocellular bone marrow that has been replaced with fat

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19
Q
  • Paroxysmal nocturnal hemoglobinuria: deficiency in
A

CD55 and CD59: Cause complement-mediated intravascular lysis and release of hemoglobin

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20
Q

hapten model

A

Drug-induced hemolytic anemia

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21
Q

Drug-induced hemolytic anemia most common cause

A

cephalosporins. others also PCN and hydrocortisone

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22
Q

hemolytic anemia s/s

A

o May be asymptomatic
o Jaundice (icterus)
o Splenomegaly

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23
Q

hemolytic anemia tx first line

A

Corticosteroids

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24
Q

hemolytic anemia tx 2nd line

A

Splenectomy and Rituximab (Rituxan) (monoclonal antibody)

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25
Q

o Paroxysmal nocturnal hemoglobinuria tx

A

Eculizumab (Solaris)

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26
Q
  • Polycythemia vera: acquired mutation in
A

Janus kinase 2 (JAK2) - * Negates the self-regulatory activity of JAK2 that allows the erythropoietin receptor to be constitutively active, regardless of the level of erythropoietin.

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27
Q

Polycythemia vera can convert to

A

acute myeloid leukemia (AML)

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28
Q

Polycythemia vera s/s

A

splenomegaly. painful itching by heat or water

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29
Q

Polycythemia vera tx

A

phlebotomy )300-500cc). spirin, interferon-a, hydroxyurea, radioactive phosphorus

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30
Q
  • Hereditary hemochromatosis: autosomal…
A

recessive

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31
Q

Hereditary hemochromatosis

A

increased gastrointestinal iron absorption with subsequent tissue iron deposition

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32
Q

Hereditary hemochromatosis s/s

A

 Hepatomegaly, abnormal liver enzymes, bronzed skin, diabetes, and cardiomegaly

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33
Q

Hereditary hemochromatosis tx

A

Phlebotomy; refrain from taking iron and vitamin C supplements and consuming raw shellfish; alcohol use in moderation

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34
Q

AML mutation

A

tyrosine kinase FLT3

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35
Q

ALL genetic anomoly

A

Philadelphia chromosome on chromosome 9and 22

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36
Q

CML genetics

A

Philly chromosome present and BCR-ABL1 causes intiaiton

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37
Q

CLL most common s/s

A

lymphadenopathy

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38
Q

CML cure

A

no cure

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39
Q

non hodgkins lymphoma cells

A

B cells, T cells, and NK cells

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40
Q

more localized: hodgkins or non?

A

hodgkins

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41
Q

Burkitt lymphoma

A

highly aggressive B-cell non-hodgkin lymphoma of jaw. EBV 90%. abd swelling. Rituximab tx

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42
Q

lymphoblastic lymphoma

A

rare non-hodgkin type.

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43
Q

lymphoblastic lymphoma population

A

1/3 in male children and adolescents

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44
Q

lymphoblastic lymphoma origin

A

T-cell in thymus 85%

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45
Q

lymphoblastic lymphoma first s/s

A

painless lymphadenopathy in neck

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46
Q

multiple myeloma translocation

A

imglobulin heavy chain on chromo 14

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47
Q

multiple myeloma patho

A

production of too much M protein (abnorm antibody). Light chains (Bence Jones proteins) pass through glomerulus and cause kidney damage

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48
Q

multiple myeloma s/s

A

hypercalc, renal failure, anemia, lytic lesions, infections

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49
Q

Occurs before multiple myeloma

A

Monoclonal Gammopathy of Undetermined Significance

50
Q

Monoclonal Gammopathy of Undetermined Significance diagnosis

A

presence of an M protein in the blood or urine without additional evidence of multiple myeloma

51
Q

Waldenström Macroglobulinemia

A

lymphoplasmacytic lymphoma. rare type of slow-growing plasma cell tumor that secretes a monoclonal immunoglobulin M (IgM) molecule. Arises from plasma cells that have genetic rearrangement of region genes (V, D, J)

52
Q

Thrombocytosis

A

Plt >400k

53
Q

Immune thrombocytopenic purpura: Ig_ antibody targets platelet _.

A

IgG, glycoprotein

54
Q

procoagulant factors

A

7,9,10

55
Q

vitamin K makes

A

prothrombin, procoag factors 79,10), and protein c and s (anticoags)

56
Q

DIC patho

A

release of tissue factor leading to increased fibrin and thrombin activity. and increased fibrinolysis. overconsumption of platelets and clotting factors

57
Q

primary thrombophilia

A

hereditary. defect in proteins for hemostasis

58
Q

secondary thrombophilia

A

acquired. decreased anticoag proteins

59
Q

hereditary thrombophilia (primary) are mostly autosomal…

A

dominant

60
Q

antiphospholipid syndrome

A

autoimmune antibodies against plasma membrane phospholipids. tx: heparin with aspirin

61
Q

normal rbc lifespan for full term infants

A

60-80 days

62
Q

rbc lifespan for preemies

A

20-30 days

63
Q

when are normal adult hgb % established

A

6-12 M

64
Q

when do erythrocyte production begin in bone marrow

A

5 M gestation

65
Q

hemolytic disease of newborn

A

erythroblastosis fetalis. alloimmune disease. Maternal blood and fetal blood incompatibility.

66
Q

hemolytic disease of newborn test and prevention

A

Coombs test. Rhogam

67
Q

glucose 6 phosphate dehydrogenase deficiency: genetics

A

x-linked, recessive

68
Q

glucose 6 phosphate dehydrogenase deficiency

A

G6PD maintains RBC metabolic processes. Deficiency shortens lifespan. Only happens when stressors are present

69
Q

Heinz bodies

A

denatures hgb in RBCs. seen in glucose 6 phosphate dehydrogenase deficiency.

70
Q

Hereditary spherocytosis

A

congenital hemolytic anemia causing abn proteins and increased intracellular Na+. no hemoglobin abnormality exists

71
Q

Hereditary spherocytosis is autosomal…

A

dominant

72
Q

Hereditary spherocytosis s/s and tx

A

anemia, jaundice, splenomegaly. Folic acid and partial splenectomy.

73
Q

Sickle cell genetics

A

valine is replaced with glutamic acid. Autosomal recessive

74
Q

aplastic crisis

A

occurs d/t a viral infxn in sickle cell disease

75
Q

Thalassemias are autosomal…

A

recessive

76
Q

beta-thalassemia minor

A

mild-mod microcytic-hypochromic anemia.

77
Q

alpha-thalassemia minor

A

2 genes

78
Q

alpha-thalassemia major

A

4 genes, fatal

79
Q

hgb H disease

A

3 genes

80
Q

Hemophilia A

A

Factor 8 deficiency. X-linked

81
Q

Hemophilia B

A

Xmas disease. Factor 9 deficiency

82
Q

Hemophilia C

A

Factor 11 deficiency

83
Q

von wilibrand disease

A

autosomal dominant. factor 8 defiency

84
Q

Primary use for cryo

A

Hemophilia A. Blood factor 8

85
Q

What increases factor 8 activity

A

Desmopressin (ADH)

86
Q

Heparin requires what for action

A

antithrombin 3

87
Q

Heparin inhibits what factors

A

9a, 10a, 11a, 12a, and thrombin (2)

88
Q

t1/2 of heparin increases with

A

liver or renal disease

89
Q

heparin is found in

A

mast cell and nerve terminal vesicles

90
Q

heparin structure

A

glycosaminoglycan

91
Q

dextran -_ is fromed from dextran -_

A

40,70

92
Q

dextran-40 is removed in

A

the glomerulus

93
Q

dextran-70 is converted to

A

glucose

94
Q

which blood sub is actual O2 carrying

A

perfluorocarbons. not in market d/t surfactant destruction

95
Q

the platelet plug is strengthened by the addition of

A

fibrin via coag cascade

96
Q

PG-I2 produced by and action

A

COX-2. inhibits plt aggreg and vasodilates

97
Q

TX-A2 produced by and action

A

COX1. plt aggreg and vasoconstricts

98
Q

apsirin is covalently bonded to

A

COX-1> COX-2

99
Q

high CAMP levels _ aggregation

A

inhibits

100
Q

CAMP is controlled by

A

TX-A2 and others

101
Q

Diprydamole

A

PDE-5 inhibitor. weaky anitplt. given with warfarin. can induce angina

102
Q

ADP inhibitor MOA and examples

A

blocks decrease in CAMP by P2Y12 proteins
- clopidogrel: prodrug
- ticlopidine: prodrug
-Cangrelor
-Ticagrelor
-Prasugrel

103
Q

ADP inhibitor who cant tolerate aspirin

A

ticlopidine

104
Q

irreversible oral ADP Inhibitor

A

prasugrel

105
Q

reversible oral ADP Inhibitor

A

ticagrelor

106
Q

glycoprotein 2b/3a receptors. Inhibitors ex:

A

where fibrinogen binds on plts
-abciximab
-eptifibatide: peptide
-tirofiban: non peptide tyrosine

107
Q

Hirudin

A

thrombin inhibitor. peptide. doesnt need AT3 like heparin and can replace heparin

108
Q

bivalirudin

A

similar to hirudin

109
Q

Argotraban. What does it trigger?

A

thrombin inhibitor. arginine derivative. Triggers nitric oxide release

110
Q

does heparin cross placenta?

A

no

111
Q

LMWH

A

Lovenox. requires PENTAsacchiride like heparin

112
Q

fondaparinux inhibits?

A

LMWH. acts with AT3 to inhibit 10a, not thrombin

113
Q

warfarin reversal

A

vit K or FFP to provide prothrombin

114
Q

warfarin clearance rated decreased by

A

amio, cimetadine, omeprazole

115
Q

does warfarin cross the placenta?

A

yes

116
Q

Dabigatran

A

esterase prodrug activated by by plasma and liver esterases

117
Q

-XABANs MOA

A

inhibitors of factor 10a

118
Q

apixaban also inhibits

A

prothrombinase which converts prothrombin (factor 2) to thrombin (2a)

119
Q

Adexanet alfa

A

reverses factor 10a inhibitors

120
Q

Thrombolytic agents

A

streptokinase, alteplase

121
Q

streptokinase isoalted from

A

beta-hemolytic streptococci. possible allergic reaction

122
Q

alteplase

A

Tissue plasminogen activator (TPA)