Hematological Disorders PPT -Josh Flashcards
Hematological Disorders:
what are types of d/o involing RBCs
- 2,3 DPG
- Anemia
- Aplastic
- Meylodysplastic
- Iron Deficiency
- Pernicious
- Autoimmune
- SICKLE CELL
Hematological Disorders:
what are type of d/o involving WBCs
- Lymphoma
- LEUKEMIA
- Multiple Myeloma
Hematological Disorders:
what are d/o of platelets
- Thrombocytopenia
- Idiopathic thrombocytopenic Purpura
- HIT
- Thrombotic thrombocytopenic purpura
- Essential thrombocytosis
Hematological Disorders:
what are d/o of plasma
- Hemophilia
- DIC
- FACTOR DEFICIENCIES
what is the most abundantncell in the body?
erythrocytes
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
Anemia:
is caused from what 4 causes
- Impaired erythrocyte production
- Blood loss
- Increased erythrocyte destruction
- any combination of the above 3
Hemoglobin:
types of Hemoglobin
- Hgb A
- Hgb A2
- Hgb F
- Hgb S
Anemia:
what is Hgb A, as in how many Beta and Alpha?
- 2 alpha
- 2 beta
(92% of adult hgb)
Hemoglobin:
what is the most common kind in adults?
Hgb A
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)
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
Hemoglobin:
Hgb S, how many alpha, beta, gamma, delta, kappam etc?
- 2 Alpha
- 2 mutated Beta
Hemoglobin:
how does one obtain a Hgb S?
inherited
Hemoglobin:
Hgb S results from the substitution of ______ ______ in the 6th position of the beta globin chain
Glutamic acid
Hemoglobin:
what is a popular d/o that people have with Hgb S d/o
Sickle cell
Hemoglobin:
what is a group of disorders characterized by the presence of Hgb S w/in the erythrocytes
Sickle cell
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?
- Sickle cell HEMOLYTIC anemia (homozygous form) and most severe
- Sickle Cell-thalassemia disease (heterozygous)
3.
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
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
Sickle Cell:
what is the problem w/ sickled cells in the vasculature?
tend to “plug” the vessels causing vascular occlusion, pain, and organ infartion
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
Sickle Cell:
A crisis may be caused but what?
- Decrease O2 Sat
- Decreased temp
- Infections
- dehydration
- stasis
- acidosis
Sickle Cell:
what are the different typw of crisis’s
- Vasoocclusive
- Aplastic
- Sequestration
- Hyperhemolytic
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
Sickle Cell:
what type of crisis is transient cessation in RBC production
Aplastic crisis
Sickle Cell:
what type of crisis is when large amounts of blood become acutely pooled in the liver and spleen
sequestration crisis
Sickle Cell:
what type of crisis is the accelerated rate of RBC destruction
hyperhemolytic crisis
Sickle Cell: Clinical Manifestations
Brain Acute
- Thrombosis
- Hemorrhage
- paralysis
- sensory deficits
- death
Sickle Cell: Clinical Manifestations
Lungs Acute
- Atelectasis
- Infarction
- Pneumonia
Sickle Cell: Clinical Manifestations
Abdominal organs Acute
- Acute hepatomegaly
- Gallstones
- Splenic sequestration
- Splenomegaly
- Infarction
Sickle Cell: Clinical Manifestations
Bones and joints Acute
- Hand - foot syndrome
(painful swelling of hands and feet)
Sickle Cell: Clinical Manifestations
Kidneys Acute
- Hematuria