Hematological Disorders PPT -Josh Flashcards Preview

BioScience II > Hematological Disorders PPT -Josh > Flashcards

Flashcards in Hematological Disorders PPT -Josh Deck (84):
1

Hematological Disorders:

what are types of d/o involing RBCs

 

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

2


Hematological Disorders:

what are type of d/o involving WBCs

 

  • Lymphoma
  • LEUKEMIA
  • Multiple Myeloma

3


Hematological Disorders:

what are d/o of platelets

 

  • Thrombocytopenia
  • Idiopathic thrombocytopenic Purpura
  • HIT
  • Thrombotic thrombocytopenic purpura
  • Essential thrombocytosis

4


Hematological Disorders:

what are d/o of plasma

  • Hemophilia
  • DIC
  • FACTOR DEFICIENCIES

5


what is the most abundantncell in the body?


erythrocytes

 

6


Hematological Disorders:

what is Anemia?


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

7


Anemia:

is caused from what 4 causes

 

 

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

8


Hemoglobin:

types of Hemoglobin

  • Hgb A
  • Hgb A2
  • Hgb F
  • Hgb S

9


Anemia:

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

 

  • 2 alpha
  • 2 beta

(92% of adult hgb)

10


Hemoglobin:

what is the most common kind in adults?

Hgb A

11


Hemoglobin:

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

 

  • 2 Alpha
  • 2 Delta
  • increased in Thalassemia

(2% of adult Hgb)

12

Hemoglobin:

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

 

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

13


Hemoglobin:

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

 

  • 2 Alpha
  • 2 mutated Beta

14


Hemoglobin:

how does one obtain a Hgb S?


inherited

15


Hemoglobin:

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


Glutamic acid

16


Hemoglobin:

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


Sickle cell

17


Hemoglobin:

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


Sickle cell

18


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?

 

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

19


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


Deoxygenation

and

Dehydration

20


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


20-30mmHg

30-40mmHg

21


Sickle Cell:

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

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

22


Sickle Cell:

what is the complication with sickled cells in the spleen?


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

23

Sickle Cell:

A crisis may be caused but what?

 

  • Decrease O2 Sat
  • Decreased temp
  • Infections
  • dehydration
  • stasis
  • acidosis

24


Sickle Cell:

what are the different typw of crisis's

 

  1. Vasoocclusive
  2. Aplastic
  3. Sequestration
  4. Hyperhemolytic

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?