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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