Hematological Disorders PPT -Josh Flashcards

1
Q

Hematological Disorders:

what are types of d/o involing RBCs

A
  • 2,3 DPG
  • Anemia
    • Aplastic
    • Meylodysplastic
    • Iron Deficiency
    • Pernicious
    • Autoimmune
    • SICKLE CELL
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2
Q

Hematological Disorders:

what are type of d/o involving WBCs

A
  • Lymphoma
  • LEUKEMIA
  • Multiple Myeloma
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3
Q

Hematological Disorders:

what are d/o of platelets

A
  • Thrombocytopenia
  • Idiopathic thrombocytopenic Purpura
  • HIT
  • Thrombotic thrombocytopenic purpura
  • Essential thrombocytosis
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4
Q

Hematological Disorders:

what are d/o of plasma

A
  • Hemophilia
  • DIC
  • FACTOR DEFICIENCIES
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5
Q

what is the most abundantncell in the body?

A

erythrocytes

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6
Q

Hematological Disorders:

what is Anemia?

A

a reduction in the total number of erythrocytes in the circulating blood or a decrease in the quality or quanity of hgb

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7
Q

Anemia:

is caused from what 4 causes

A
  1. Impaired erythrocyte production
  2. Blood loss
  3. Increased erythrocyte destruction
  4. any combination of the above 3
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8
Q

Hemoglobin:

types of Hemoglobin

A
  • Hgb A
  • Hgb A2
  • Hgb F
  • Hgb S
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9
Q

Anemia:

what is Hgb A, as in how many Beta and Alpha?

A
  • 2 alpha
  • 2 beta

(92% of adult hgb)

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10
Q

Hemoglobin:

what is the most common kind in adults?

A

Hgb A

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11
Q

Hemoglobin:

What is Hgb A2, as in how many alpha, beta, delta, kappa, etc? and when is this tye increased

A
  • 2 Alpha
  • 2 Delta
  • increased in Thalassemia

(2% of adult Hgb)

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12
Q

Hemoglobin:

Hgb F, how many Alpha, gamma, delta, beta, kappa, etc? and what is its major functions?

A
  • 2 Alpha
  • 2 Gamma
  • major feta Hgb from 3rd-9th month gestation
  • promotes O2 transfer across platelets
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13
Q

Hemoglobin:

Hgb S, how many alpha, beta, gamma, delta, kappam etc?

A
  • 2 Alpha
  • 2 mutated Beta
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14
Q

Hemoglobin:

how does one obtain a Hgb S?

A

inherited

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15
Q

Hemoglobin:

Hgb S results from the substitution of ______ ______ in the 6th position of the beta globin chain

A

Glutamic acid

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16
Q

Hemoglobin:

what is a popular d/o that people have with Hgb S d/o

A

Sickle cell

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17
Q

Hemoglobin:

what is a group of disorders characterized by the presence of Hgb S w/in the erythrocytes

A

Sickle cell

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18
Q

Sickle Cell:

One way Sickle Cell is formed by a genetic mutation on which one amino acid (valine) replaces another (glutamic acid), leading to 2 separate types, what are they?

A
  1. Sickle cell HEMOLYTIC anemia (homozygous form) and most severe
  2. Sickle Cell-thalassemia disease (heterozygous)
    3.
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19
Q

Sickle Cell:

Also Sickle Cell can come from Sickle Cell Hgb C disease (heterozygous) where as Hgb S reacts to __________ and __________ by solidifying and stretching the erythrocyte into an elongated sickle shape

A

Deoxygenation

and

Dehydration

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20
Q

Sickle Cell:

deoxygenation is the most important triggering varible, and will sickle Trait cells will sickle w/ a PaO2 of __-__ mmHg, and SIckle cell disease cells will begin to sickle w/ a PaO2 of ___-___mmHg

A

20-30mmHg

30-40mmHg

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21
Q

Sickle Cell:

what is the problem w/ sickled cells in the vasculature?

A

tend to “plug” the vessels causing vascular occlusion, pain, and organ infartion

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22
Q

Sickle Cell:

what is the complication with sickled cells in the spleen?

A

undergo hemolysis in teh spleen or become sequestered there causing pooling or onfarction of spleenic vessels

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23
Q

Sickle Cell:

A crisis may be caused but what?

A
  • Decrease O2 Sat
  • Decreased temp
  • Infections
  • dehydration
  • stasis
  • acidosis
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24
Q

Sickle Cell:

what are the different typw of crisis’s

A
  1. Vasoocclusive
  2. Aplastic
  3. Sequestration
  4. Hyperhemolytic
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25
Q

Sickle Cell:

what type of crisis is- begining w/ a sickling in the microcirculation, as blood flow is obstructed thrombosis and infarction of local tissue can occur

A

Vasoocclusive

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26
Q

Sickle Cell:

what type of crisis is transient cessation in RBC production

A

Aplastic crisis

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27
Q

Sickle Cell:

what type of crisis is when large amounts of blood become acutely pooled in the liver and spleen

A

sequestration crisis

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28
Q

Sickle Cell:

what type of crisis is the accelerated rate of RBC destruction

A

hyperhemolytic crisis

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29
Q

Sickle Cell: Clinical Manifestations

Brain Acute

A
  • Thrombosis
  • Hemorrhage
  • paralysis
  • sensory deficits
  • death
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30
Q

Sickle Cell: Clinical Manifestations

Lungs Acute

A
  • Atelectasis
  • Infarction
  • Pneumonia
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31
Q

Sickle Cell: Clinical Manifestations

Abdominal organs Acute

A
  • Acute hepatomegaly
  • Gallstones
  • Splenic sequestration
  • Splenomegaly
  • Infarction
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32
Q

Sickle Cell: Clinical Manifestations

Bones and joints Acute

A
  • Hand - foot syndrome

(painful swelling of hands and feet)

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33
Q

Sickle Cell: Clinical Manifestations

Kidneys Acute

A
  • Hematuria
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34
Q

Sickle Cell: Clinical Manifestations

eye

A
  • Hemorrhage
  • Exudation
  • Blindness
  • Retinopathy
35
Q

Sickle Cell: Clinical Manifestations

Pulmonary

A
  • HTN
  • Tachypnea
36
Q

Sickle Cell: Clinical Manifestations

Heart Chronic

A

High output failure

37
Q

Sickle Cell: Clinical Manifestations

spleen chronic

A

splenic atrophy

38
Q

Sickle Cell: Clinical Manifestations

Kidney Chronic

A
  • Dilute urine
  • Diuresis
39
Q

Sickle Cell: Clinical Manifestations

Penis Chronic

A

Priapism

40
Q

Sickle Cell: Clinical Manifestations

Skin Chronic

A
  • Stasis ulcers

(hands, feet, and ankles)

41
Q

Managment of Sickle Cell:

  1. Is there evidence supporting preop transfusions?
  2. Do low risk sx’s need transfusions?
  3. What is teh target Hct% w/ moderate-high risk sx’s?
A
  1. Nope
  2. Rarely
  3. 30%
42
Q

Managment of Sickle Cell:

  1. will the different anesthestic tech affects complications?
  2. What do you want to avoid Intaop to prevent complications?
  3. Can you use tourniquets?
A
  1. Nope
  2. dehydration, acidosis, hypothermia, hypotension
  3. Yes, but but increases periop complications
43
Q

Managment of Sickle Cell:

how should you treat pain postop?

A

very aggresively

44
Q

What is an Organic Phasphate which is present in RBCs?

A

2,3 DPG

45
Q

2,3 DPG

what pathway is responsible for the production of 2,3 DPG

A

Luebering-Rapaport pathway

46
Q

2,3 DPG

how does 2,3 DPG work?

A

Binds to Hgb, diminishing the oxygen affinity of Hgb (thus off loading O2)

47
Q

2,3 DPG

the amount of 2,3 DPG in RBCs determines how readily Hgb gives up what?

A

O2

48
Q

2,3 DPG

Severe ______ depletion in pt’s w/ DKA or nutritional deficiency can lead to a reduced 2,3 DPG production

A

Phasphate

49
Q

2,3 DPG

2,3 DPG is a product of ________ that accumulates during anaerobic Metabolism

A

Glycolysis

50
Q

2,3 DPG

At higher altitudes the level of 2,3 DPG in the blood increases or decreases?

A

Increases

51
Q

Left or Right shift of ODC

Less release of O2 to tissue?

A

Left

52
Q

Left or Right shift of ODC

More release of O2 to tissue

A

Right (thus increased 2,3 DPG)

53
Q

Left or Right shift of ODC

P50 < 26 mmHg

A

Left

54
Q

Left or Right shift of ODC

P50 > 26 mmHg

A

Right

55
Q

Left or Right shift of ODC

Acidosis?

A

right

56
Q

Left or Right shift of ODC

Alkolosis?

A

Left

57
Q

Left or Right shift of ODC

hypothermia

A

Left

58
Q

Left or Right shift of ODC

Hyperthermia

A

Right

59
Q

Left or Right shift of ODC

Increased 2,3,DPG

A

Right

60
Q

Left or Right shift of ODC

Decreased 2,3DPG

A

left

61
Q

Leukocytes (WBC):

main function

A
  • defend body against organisms that cause infection
  • Remove debris including dead or injured host cells
62
Q

Leukocytes (WBC):

Act primarily where?

A

tissues

63
Q

Leukocytes (WBC):

are classified as granulocytes or agranulocytes and phagocytes or immunocytes, which class contain enzymes capable of killing microorganisms and catabolizing debris by phagocytosis?

A

Granulocytes

64
Q

What is a malignant d/o of the blood and blood-forming organs causing an accumulation of dysfunctional cells and loss of cell division regulation?

A

Leukemia

65
Q

Leukemia:

Excessive accumulation of leukemic cells results in an overcrowding of bone marrow which causes a decreased production and function of normal ______ cells?

A

Hematopoietic cells

66
Q

Leukemia:

what are the 2 types

A

Acute

Chronic

67
Q

Leukemia:

name the 2 types of Acute?

A
  • Acute Lymphocytic Leukemia (ALL)
  • Acute myelogenous Leukemia (AML)
68
Q

Leukemia:

Name the 2 types of chronic

A
  • Myelogenous (CML)
  • Lymphocytic (CLL)
69
Q

Leukemia:

in recap regardless of acute or chronic there is only 2 tyoes what are they?

A

Myelogenous

and

Lymphocytic

70
Q

Leukemia:

What type is the most common in children?

A

ALL

71
Q

Leukemia:ALL

The ________chromosome positive ALL carries the worste prognosis?

A

Philadelphia

72
Q

Leukemia:CML

what is the unique distisguishing marker ?

A

philadelphia chromosome

73
Q

Leukemia:

What are the clinical manifestations?

A
  • Anemia
  • Bleeding
  • DIC
  • Infection
  • Weight loss
  • Bone pain
  • Elevated Uric Acid
  • Liver, spleen, and lymph node enlargement
74
Q

Leukemia:

Anesthesia management?

A
  • Asses Labs
  • Aseptic tech at all times
  • Chemotherapy?
  • Neutropenia may affect abx choice
  • severe pancytopenia may require correction prior to sx
75
Q

Chemotherapy agent:

What is the SE of Methotrexate?

A

pulmonary toxixity

CNS toxicity

Hepatc Toxicity

Renal toxicity

76
Q

Chemotherapy agent:

SE of L-asparginase?

A
  • renal toxicity
  • Hepatic toxicity
77
Q

Chemotherapy agent:

SE of vincristine?

A
  • CNS toxicity
  • SIADH
78
Q

Chemotherapy agent:

SE of Danunorubucin

A

Cardiac toxicity

79
Q

Chemotherapy agent:

SE of Cyclophoshamide

A

SIADH

Pulmonry Toxicity

80
Q

Clotting Factors:

name them all?

A

I-Fibrinogen

II-prothrombin

III-thromboplastin

IV- Ca++

V-Proaccelerin

VII- proconvertin

VIII:C- antihempphiliac factor

VIII:vWF-Von Willebrands Factor

IX-Christmas factor

X- stuart-power factor

XI-Plasma thromboplastin

XII- hageman Factor

XII- Fibrin stabalizing Factor

protein C and S

81
Q

What is teh product of choice for Hemophilia VIII?

A

Cryoprecipitate

82
Q

what product do you give for Von Willebrand disease

A

croprecipitate

83
Q

What is a drug you can use for both VIII deficiences?

A

Desmopressin (DDAVP)

84
Q

There is more in her slides about factor deficiency but not much that i found to be inportant?

A

if you wish refer to her slides if needed?