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
Sickle Cell: Clinical Manifestations
eye
- Hemorrhage
- Exudation
- Blindness
- Retinopathy
Sickle Cell: Clinical Manifestations
Pulmonary
- HTN
- Tachypnea
Sickle Cell: Clinical Manifestations
Heart Chronic
High output failure
Sickle Cell: Clinical Manifestations
spleen chronic
splenic atrophy
Sickle Cell: Clinical Manifestations
Kidney Chronic
- Dilute urine
- Diuresis
Sickle Cell: Clinical Manifestations
Penis Chronic
Priapism
Sickle Cell: Clinical Manifestations
Skin Chronic
- Stasis ulcers
(hands, feet, and ankles)
Managment of Sickle Cell:
- Is there evidence supporting preop transfusions?
- Do low risk sx’s need transfusions?
- What is teh target Hct% w/ moderate-high risk sx’s?
- Nope
- Rarely
- 30%
Managment of Sickle Cell:
- will the different anesthestic tech affects complications?
- What do you want to avoid Intaop to prevent complications?
- Can you use tourniquets?
- Nope
- dehydration, acidosis, hypothermia, hypotension
- Yes, but but increases periop complications
Managment of Sickle Cell:
how should you treat pain postop?
very aggresively
What is an Organic Phasphate which is present in RBCs?
2,3 DPG
2,3 DPG
what pathway is responsible for the production of 2,3 DPG
Luebering-Rapaport pathway
2,3 DPG
how does 2,3 DPG work?
Binds to Hgb, diminishing the oxygen affinity of Hgb (thus off loading O2)
2,3 DPG
the amount of 2,3 DPG in RBCs determines how readily Hgb gives up what?
O2
2,3 DPG
Severe ______ depletion in pt’s w/ DKA or nutritional deficiency can lead to a reduced 2,3 DPG production
Phasphate
2,3 DPG
2,3 DPG is a product of ________ that accumulates during anaerobic Metabolism
Glycolysis
2,3 DPG
At higher altitudes the level of 2,3 DPG in the blood increases or decreases?
Increases
Left or Right shift of ODC
Less release of O2 to tissue?
Left
Left or Right shift of ODC
More release of O2 to tissue
Right (thus increased 2,3 DPG)
Left or Right shift of ODC
P50 < 26 mmHg
Left
Left or Right shift of ODC
P50 > 26 mmHg
Right
Left or Right shift of ODC
Acidosis?
right
Left or Right shift of ODC
Alkolosis?
Left
Left or Right shift of ODC
hypothermia
Left
Left or Right shift of ODC
Hyperthermia
Right
Left or Right shift of ODC
Increased 2,3,DPG
Right
Left or Right shift of ODC
Decreased 2,3DPG
left
Leukocytes (WBC):
main function
- defend body against organisms that cause infection
- Remove debris including dead or injured host cells
Leukocytes (WBC):
Act primarily where?
tissues
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
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
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
Leukemia:
what are the 2 types
Acute
Chronic
Leukemia:
name the 2 types of Acute?
- Acute Lymphocytic Leukemia (ALL)
- Acute myelogenous Leukemia (AML)
Leukemia:
Name the 2 types of chronic
- Myelogenous (CML)
- Lymphocytic (CLL)
Leukemia:
in recap regardless of acute or chronic there is only 2 tyoes what are they?
Myelogenous
and
Lymphocytic
Leukemia:
What type is the most common in children?
ALL
Leukemia:ALL
The ________chromosome positive ALL carries the worste prognosis?
Philadelphia
Leukemia:CML
what is the unique distisguishing marker ?
philadelphia chromosome
Leukemia:
What are the clinical manifestations?
- Anemia
- Bleeding
- DIC
- Infection
- Weight loss
- Bone pain
- Elevated Uric Acid
- Liver, spleen, and lymph node enlargement
Leukemia:
Anesthesia management?
- Asses Labs
- Aseptic tech at all times
- Chemotherapy?
- Neutropenia may affect abx choice
- severe pancytopenia may require correction prior to sx
Chemotherapy agent:
What is the SE of Methotrexate?
pulmonary toxixity
CNS toxicity
Hepatc Toxicity
Renal toxicity
Chemotherapy agent:
SE of L-asparginase?
- renal toxicity
- Hepatic toxicity
Chemotherapy agent:
SE of vincristine?
- CNS toxicity
- SIADH
Chemotherapy agent:
SE of Danunorubucin
Cardiac toxicity
Chemotherapy agent:
SE of Cyclophoshamide
SIADH
Pulmonry Toxicity
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
What is teh product of choice for Hemophilia VIII?
Cryoprecipitate
what product do you give for Von Willebrand disease
croprecipitate
What is a drug you can use for both VIII deficiences?
Desmopressin (DDAVP)
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?