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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
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
Vasoocclusive
26
Sickle Cell: what type of crisis is transient cessation in RBC production
Aplastic crisis
27
Sickle Cell: what type of crisis is when large amounts of blood become acutely pooled in the liver and spleen
sequestration crisis
28
Sickle Cell: what type of crisis is the accelerated rate of RBC destruction
hyperhemolytic crisis
29
Sickle Cell: Clinical Manifestations Brain Acute
* Thrombosis * Hemorrhage * paralysis * sensory deficits * death
30
Sickle Cell: Clinical Manifestations Lungs Acute
* Atelectasis * Infarction * Pneumonia
31
Sickle Cell: Clinical Manifestations Abdominal organs Acute
* Acute hepatomegaly * Gallstones * Splenic sequestration * Splenomegaly * Infarction
32
Sickle Cell: Clinical Manifestations Bones and joints Acute
* Hand - foot syndrome (painful swelling of hands and feet)
33
Sickle Cell: Clinical Manifestations Kidneys Acute
* Hematuria
34
Sickle Cell: Clinical Manifestations eye
* Hemorrhage * Exudation * Blindness * Retinopathy
35
Sickle Cell: Clinical Manifestations Pulmonary
* HTN * Tachypnea
36
Sickle Cell: Clinical Manifestations Heart Chronic
High output failure
37
Sickle Cell: Clinical Manifestations spleen chronic
splenic atrophy
38
Sickle Cell: Clinical Manifestations Kidney Chronic
* Dilute urine * Diuresis
39
Sickle Cell: Clinical Manifestations Penis Chronic
Priapism
40
Sickle Cell: Clinical Manifestations Skin Chronic
* Stasis ulcers (hands, feet, and ankles)
41
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?
1. Nope 2. Rarely 3. 30%
42
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?
1. Nope 2. dehydration, acidosis, hypothermia, hypotension 3. Yes, but but increases periop complications
43
Managment of Sickle Cell: how should you treat pain postop?
very aggresively
44
What is an Organic Phasphate which is present in RBCs?
2,3 DPG
45
2,3 DPG what pathway is responsible for the production of 2,3 DPG
Luebering-Rapaport pathway
46
2,3 DPG how does 2,3 DPG work?
Binds to Hgb, diminishing the oxygen affinity of Hgb (thus off loading O2)
47
2,3 DPG the amount of 2,3 DPG in RBCs determines how readily Hgb gives up what?
O2
48
2,3 DPG Severe ______ depletion in pt's w/ DKA or nutritional deficiency can lead to a reduced 2,3 DPG production
Phasphate
49
2,3 DPG 2,3 DPG is a product of ________ that accumulates during anaerobic Metabolism
Glycolysis
50
2,3 DPG At higher altitudes the level of 2,3 DPG in the blood increases or decreases?
Increases
51
Left or Right shift of ODC Less release of O2 to tissue?
Left
52
Left or Right shift of ODC More release of O2 to tissue
Right (thus increased 2,3 DPG)
53
Left or Right shift of ODC P50 \< 26 mmHg
Left
54
Left or Right shift of ODC P50 \> 26 mmHg
Right
55
Left or Right shift of ODC Acidosis?
right
56
Left or Right shift of ODC Alkolosis?
Left
57
Left or Right shift of ODC hypothermia
Left
58
Left or Right shift of ODC Hyperthermia
Right
59
Left or Right shift of ODC Increased 2,3,DPG
Right
60
Left or Right shift of ODC Decreased 2,3DPG
left
61
Leukocytes (WBC): main function
* defend body against organisms that cause infection * Remove debris including dead or injured host cells
62
Leukocytes (WBC): Act primarily where?
tissues
63
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?
Granulocytes
64
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?
Leukemia
65
Leukemia: Excessive accumulation of leukemic cells results in an overcrowding of bone marrow which causes a decreased production and function of normal ______ cells?
Hematopoietic cells
66
Leukemia: what are the 2 types
Acute Chronic
67
Leukemia: name the 2 types of Acute?
* Acute Lymphocytic Leukemia (ALL) * Acute myelogenous Leukemia (AML)
68
Leukemia: Name the 2 types of chronic
* Myelogenous (CML) * Lymphocytic (CLL)
69
Leukemia: in recap regardless of acute or chronic there is only 2 tyoes what are they?
Myelogenous and Lymphocytic
70
Leukemia: What type is the most common in children?
ALL
71
Leukemia:ALL The \_\_\_\_\_\_\_\_chromosome positive ALL carries the worste prognosis?
Philadelphia
72
Leukemia:CML what is the unique distisguishing marker ?
philadelphia chromosome
73
Leukemia: What are the clinical manifestations?
* Anemia * Bleeding * DIC * Infection * Weight loss * Bone pain * Elevated Uric Acid * Liver, spleen, and lymph node enlargement
74
Leukemia: Anesthesia management?
* Asses Labs * Aseptic tech at all times * Chemotherapy? * Neutropenia may affect abx choice * severe pancytopenia may require correction prior to sx
75
Chemotherapy agent: What is the SE of Methotrexate?
pulmonary toxixity CNS toxicity Hepatc Toxicity Renal toxicity
76
Chemotherapy agent: SE of L-asparginase?
* renal toxicity * Hepatic toxicity
77
Chemotherapy agent: SE of vincristine?
* CNS toxicity * SIADH
78
Chemotherapy agent: SE of Danunorubucin
Cardiac toxicity
79
Chemotherapy agent: SE of Cyclophoshamide
SIADH Pulmonry Toxicity
80
Clotting Factors: name them all?
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
What is teh product of choice for Hemophilia VIII?
Cryoprecipitate
82
what product do you give for Von Willebrand disease
croprecipitate
83
What is a drug you can use for both VIII deficiences?
Desmopressin (DDAVP)
84
There is more in her slides about factor deficiency but not much that i found to be inportant?
if you wish refer to her slides if needed?

BioScience II (39 decks)

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