Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Mattias - Hemotology > Week 1 > Flashcards

Week 1 Flashcards

1
Q

Three types of blood cells

A

Red blood cells
White blood cells
Platelets

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Types of White blood cells

A 
Monocytes
Neutrophils
Basophils
Eosinophils
Lymphocytes
NK Cells
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Production of blood cells are called

A

Hemopoiesis OR Hematopoiesis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Site of Hematopoiesis in Embryo

A

Yolk sac then liver

3rd-7th month - Spleen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Site of Hematopoiesis at birth

A

Mostly bone marrow, liver and spleen when needed

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Site of Hematopoiesis in adult

A

Bone marrow of skull, ribs, sternum, pelvis, proximal ends of femur

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

How many cells are made per minute

A

100 million RBC/minute
60 million neutrophils/minute
150million platelets/minute

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is proliferation

A

Rapid increase in numbers

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is differentiation

A

Development of the features of the specialized end cell

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Initial three tiers of the the Hemopoietic tree

A

Long term Hemopoietic Stem Cell –> Short Term Hemopoietic Stem Cell –> Multipotent progenitors

LT-HSC –> ST-HSC –>MPP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Multipotent Progenitors develop into what in the hemopoietic tree

A

Common Myeloid Progenitor
OR
Common Lymphoid progenitor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Common Myeloid Progenitor is precursor to

A

Erythrocytes – Megakaryocytes (Platelets) – Granylocytes – Monoblast (Macrophages) – Dendritic cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Common Lymphoid progenitor is precursor to

A

T cells
B cells
NK cells
Dendrititc cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Progression of Neutrophils from Myeloblast

A

Myeloblast –> Promyelocyte –> Myelocyte –> Metamyelocyte –> Band form –> Neutrophils

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Progression to Erythrocyte from Common myeloid progenitor

A

CMP –> Pronormoblast –> Basophilic Erythroblast –> Polychromatophilic Erythroblast –> Orthochromatic erythroblast –> Erythroblast –> Reticulocyte –> Erythrocyte

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

How are platelets formed

A

Bud of Megakaryocyte

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Names of the Granulocytes

A

Eosinophils
Basophils
Neutrophils

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Structures of Neutrophils

A

Segmented nucleus

Neutral staining granules

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Structure of Eosinophils

A

Bi-lobed

Bright orange/red granules

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Function of Eosinophils

A

Fight parasitic infections
Involved in hypersensitivity
Often elevated in pt w/ allergic conditions

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Structure of Basophils

A

Infrequent in circulation
Large deep purple granules obscuring nucleus.
Dark spots on top of nucleus on image

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Basophils functions

A

Circulating version of tissue mast cells
Granules contain histamine
FcReceptors bind IgE, mediates hypersensitivity reactions

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Structure of Monocytes

A

Large single nucleus

Faintly staining granules, some vacuoles

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Function of Monocytes

A

Circulating version of Macrophages
Phagocyose invades, kill and present antigen to lymphocytes
Attract other cells
More long lived than neutrophils

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Structure of Mature Lymphocytes

A

Small w/ condensed nucleus and rim of cytoplasm

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Function of Neutrophils

A

Phagocytose invaders
Kill with granule contents and die in the process
Attract other cells
Short lived

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Structure of Atypical (activated) Lymphocytes

A

Large w/ plentiful cytoplasm extending around nearby RBC.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Atypical lymphocyte during viral infection (EBV) has what

A

A relatively open chromatin pattern of nucleus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Where is a common site for bone marrow aspiration

A

Posterior illiac crests

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

How is a core biopsy taken of the bone marrow

A

With a Jamshidi needle

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Properties of mature RBCs

A

Packed w/ hemoglobin
No nucleus or mitochondria
No DNA/RNA, no cell division
Life span 120 days

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Where is RBC produced and broken down

A

Produced in Red bone marrow

Removed by spleen and liver

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

What component of RBC is broken down to bilirubin

A

Heme group (minus iron)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

What protein regulate RBC production

A

Erythropoietin released by kidneys

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

When is the nucleus extruded during Erythropoesis

A

During erythroblast stage, right before Reticulocyte

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

Structure of Erythrocytes

A 
Biconcave disc (8um in diameter, 2um thick at edge, 1um in middle)
Maximized surface to volume ratio
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

What is Hematocrit

A

Volume of RBCs as % of total blood volume
Normal male 40-50%
Female 36-46%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

If you spin a test tube with blood you get what layers

A

Top layer - Plasma ~55%

Buffy coat - Platelets and WBC

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

How does RBCs get energy

A

Anaerobic glycolysis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

What is Methemoglobin

A

HbFe3+

HbFe2+ is normal

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

What is Glutathione and what is its role

A

Tripeptide (Glutamate, cysteine, glycine)
Reduced GSH combats oxidative stress
Maintains reduced state in a cell

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

What is needed for reduced Glutathione production

A

NADPH is needed

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

Glucose 6-phosphate dehydrogenase insufficiency leads to

A

NADPH deficiency
Reduced GSH insufficiency
Cell damage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

What are the forms of CO2 transport

A

10% Physically dissolved in solution
30% Bound to Hb
60% As bicarbonate ion - HCO3-

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

What enzyme facilitate Bicarbonate production, formula

A

CO2+H2O –> H2CO3 –> H+ +HCO3-

Carbonic anhydrase facilitate first part of reaction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

What part of the hemoglobin does oxygen bind to, Carbondioxide

A

O2 - Heme group

CO2 - Globin portion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

How is HCO3- transported through the cell membrane

A

Chloride/bicarbonate exhange. Chloride ions facilitates the diffusion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

Describe adult hemoglobin

A

4 protein subunits (globin) each containing a single heme group.
Each heme group has a single Fe2+ ion that can bind one O2 molecule.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

What is the structure of the heme group

A

Porphyrin ring with Fe2+ in the middle

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

HbF has what components

A

Alpha2-gamma2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

HbA has what components

A

Alpha2-Beta2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q

What is the affinity of HbF vs HbA of O2 and 2,3BPG

A

O2 - HbF has higher affinity

2,3HBG - HbA has higher affinity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
53
Q

Spectrophotmetric method for measuring [Hb]

A

Lyse cells
Stabilize Hb molecules (cyan-metHb)
Measure optical density
OD is proportional to conc (Beer’s Law)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
54
Q

Bodies response to Anemia

A

Increased red cell production

Peripheral blood reticulocytosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
55
Q

What are Reticulocytes

A

Immature RBCs
Larger than average RBCs
Still have RNA (purple/deep red stain)
Polychromatic on blood film

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
56
Q

Two main causes of Anemia

A 
Decreased production (low reticulocyte count)
Increased loss or destruction of red cells (High reticulocyte count)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
57
Q

Likely underlying defect in microcytic vs macrocytic anemia

A 
MCV low (microcytic) defect with hemoglobinisation
MCV high (macrocytic) defect with maturation
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
58
Q

Why is microcytic anemia linked to hemoglobin production

A

Hb is synthesized in cytoplasm. Problems with the production of heme such as shortage of components, results in a smaller cytoplasm and low Hb content. Also hypochromic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
59
Q

To make Hb you need

A

Globins

Heme (Porhyrin ring and Iron)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
60
Q

Hypochromic, microcytic anemia =

A

Defective hemoglobin synthesis: cytoplasmic defect

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
61
Q

Commonest causes of hypochromic microcytic anemias

A

Heme deficiency - Lack of iron

Globin deficiency - Thalassemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
62
Q

Problems with porphyrin synthesis is very very rare but can be due to

A

Lead posioning

Pyridoxine responsive anemias

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
63
Q

Congential Sideroblastic anemia causes what

A

Heme deficiency so Hypochromic microcytic anemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
64
Q

Where is most of the bodies iron

A

In red blood cells, 2500mg.

In comparison, we absorb only 1mg/day

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
65
Q

Iron in RBCs are in hemoglobin, in which form is iron in Liver and macrophages stores

A

Bound to Ferritin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
66
Q

What is the form of circulating iron

A

Bound to transferrin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
67
Q

Measure of Transferring indicates what

A

% saturation of transferring measures iron supply

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
68
Q

What is the best way to measure iron storage

A

Serum ferritin, tiny amount present but good representation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
69
Q

Iron deficiency can be confirmed by

A

Combination of microcytic hypochromic anemia and low serum ferritin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
70
Q

Causes of iron deficiency

A

Not eating sufficient
Loosing too much -blood loss
Not absorbing enough - malabsorption

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
71
Q

Iron deficiency due to not enough intake can be divided into two groups

A

Relative deficiency - women of child bearing age and children
Absolute deficiency - vegetarian diets

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
72
Q

Causes of chronic blood loss

A

Menorrhagia
GI (Tumors, ulcers, NSAIDs)
Hematuria

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
73
Q

What is koilonychia

A

Spoon shaped nails

Sign of iron deficiency anemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
74
Q

Skin changes seen in Iron deficient anemia

A

Angular Cheilitis
Pale skin
Smooth red tongue

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
75
Q

What is macrocytic anemia

A

Anemia in which the red cells have a larger than normal value

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
76
Q

What is normal cell volume

A

80-100femtoliters

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
77
Q

What are the main categories of causes of true macrocytosis

A

Megaloblastic

Non-Megaloblastic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
78
Q

What is a megaloblast

A

An abnormally large nucleated red cell precursor with an immature nucleus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
79
Q

What causes megaloblastic anemias

A

Predominant defects in DNA synthesis and nuclear maturation with relative preservation of RNA and hemoglobin synthesis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
80
Q

Why does DNA synthesis defect cause macrocytic cells

A

Cytoplasm has developed and is ready to divide but nucleus is running behind. As the nucleus matures to division stage the cytoplasm continues to grow. Cell is not large due to increase in production, it is big because it fails to divide itself

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
81
Q

Causes of megaloblastic anemia

A

B12 deficiency
Folate deficieny
Others - Drugs, rare inherited abnormalities

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
82
Q

Why does B12 and folate cause megaloblastic anemia

A

They are essential co-factors for nuclear maturation. Part of the cycles that produce nucleosides for DNA synthesis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
83
Q

What two cycles are B12 and folate involved in

A

Methionine cycle and Folate cycle (both inter-linked)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
84
Q

How is Vitamin B12 ingested

A

Together with foods, found in meat, eggs normally.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
85
Q

What happens to protein-VitaminB12 complex when it comes to the stomach

A

Pepsin and Low pH breaks down the protein and renders VitB12 free.
Haptocorrin is released from salivary glands and parietal cells in stomach and binds VitB12

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
86
Q

What happens to VitB12-Haptocorrin when it gets to the duodenum

A

Pancreatic proteases breaks down Haptocorrin and allows VitB12 to be bound to intrinsic factor produced by parietal cells.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
87
Q

What happens after duodenum to the VitB12-Intrinsic factor complex

A

In the mucosal cells of the distal ileum Cubulin receptors recognize the complex and absorbed VitB12

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
88
Q

What happens to VitB12 when it is absorbed in distal ileum

A

Binds to transcobalamin and enters the blood

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
89
Q

Causes of VitB12 deficiency

A

Vegans, atrophic gastritis, PPIs/H2 receptor antagonists, Gastrectomy/bypass. Jejunum (celiac, bacterial overgrowth) Duodenum (resection/Crohn’s disease) Cubulin receptor insufficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
90
Q

Where is Folate absorbed and in what form

A

Converted to monoglutamate and absorbed in Duodenum and Jejunum

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
91
Q

How long is the bodies store of B12 and Folate

A

B12 - 2-4years

Folate - 4 months

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
92
Q

Food source for B12 and Folate

A

B12 - Animal proteins

Folate - Leafy veg, yeast, destroyed by cooking

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
93
Q

Causes of folate deficiency

A

Inadequate intake
Malabsorption
Excess utilisation (hemolysis, exfoliating dermatitis, pregnancy, Malignancy)
Drugs (Anticonvulsants)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
94
Q

Which antipsychotic drug causes agranulocytosis

A

Clozapine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
95
Q

Clinical features of both B12 and folate deficiency

A

Anemia
Weight loss, diarrhea, infertility
Sore tongue, jaundice
Developmental problems

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
96
Q

Vitamin B12 deficiency only may cause __ before hematological findings occur

A

Neurological problems.

Dorsal column abnormalities, neuropathy, dementia or psychiatric manifestations

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
97
Q

What is pernicious anemia

A

Autoimmune condition with resulting destruction of gastric parietal cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
98
Q

What is pernicious anemia associated with apart from B12 deficiency related things

A

Atrophic gastritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
99
Q

Blood film results in pernicious anemia

A

Macrovalocytes and hypersegmented neutrophils

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
100
Q

FBC results in pernicious anemia

A

Macrocytic (megaloblastic) anemia

Pancytopenia in some pt

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
101
Q

How does hypersegmented neutrophils look

A

Like someone cut up the nucleus into tons of small pieces

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
102
Q

Auto-antibodies found in pernicious anemia

A

Anti Gastric-parietal cell (anti-GPC)

Anti-intrinsic factor (anti-IF)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
103
Q

Treatment for pernicious anemia

A

Vitamin B12 (Hydroxycobalamin) injections for life

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
104
Q

Treatment of megaloblastic anemia

A

Treat cause where possible.

VitB12 and Folic acid replacement

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
105
Q

Cause of non-megaloblastic macrocytosis

A

Alcohol – Liver disease – Hypothyroidism – Marrow failure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
106
Q

Types of Marrow failure that leads to non-megaloblastic macrocytosis

A

Myelodysplasia
Myeloma
Aplastic anemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
107
Q

Is non-megaloblastic macrocytosis caused by alcohol, liver disease or hypothyroidism associated with anemia

A

May not. The change in blood cells is due to red cell membrane changes

108
Q

What are some false causes of macrocytosis

A

Reticulocytosis

Cold agglutinins

109
Q

Why are Reticulocytosis and Cold agglutini-disease considered false macrocytosis

A

Machine reads reticulocytes (which are bigger than mature RBC) as RBC and therefore increase the mean.
In cold-agglutinin disease cells clump together and machine count them as one giant cell.

110
Q

Why would a patient with pernicious anemia present mildly jaundice

A

Ineffective erythropoiesis leads to death of red cells in marrow. Hemoglobin is broken down to bilirubin causing jaundince

111
Q

Can pancytopenia occur due to B12/folate deficiency

A

Yes, needed for DNA reproduction

112
Q

What are blood products

A

Purified constituents of the blood plasma. Ie. Human albumin, Iv Ig, Specific Ig, Anti-D Ig, Prothrombin complex conentrates

113
Q

What is Fresh frozen plasma

A

Plasma that has been frozen within 8h of collection

114
Q

To identify a blood component from a bag, what two things do you need

A 
Component label (ie RBC)
Donation number (long number with a letter at the end)
115
Q

One donation gives how many units of platelets

A

1/4

116
Q

Human blood products, one unit is derived from how many patients

A

Many, 100s

117
Q

Quick description in how blood donations are taken and seperated

A

Taken into the primary bag
Spun to seperate layers
Attached bags’ valves are opened and Plasma goes up, RBC goes down and Buffy coat stays in primary bag.

118
Q

What is RBC stored in and for how long

A

35days in 4C fridge
Nutrient solution with saline-Adenine-Glucose-Mannitol SAG-M
Must be transfused within 4h of leaving controlled storage.
May only leave controlled storage for less than 30 minutes if it is put back in controlled storage.

119
Q

How ara platelets stored and for how long

A

22C w/ continual agitation
Shelf life of 5 days
Transfuse within 1h

120
Q

How is fresh frozen plasma stored and for how long

A

-30C for up to 2years

Thawed prior to transfusion - transfuse within 4h

121
Q

What are the types of blood groups that is tested for

A

ABO
Rh(D)
Others (currently 31 systems)

122
Q

Most common types of ABO Group

A

O - 47%

A - 42%

123
Q

If you are blood group B, which donor blood can you accept

A

From a B donor or a O donor

124
Q

What antibodies does a paitent with blood group A

A

Anti-B antibodies

125
Q

What types of antibodies are ABO-antibodies

A

Primarly IgM with a small proportion IgG

126
Q

Where is ABO blood group genes located

A

Chromosome 9

127
Q

What is the genetic of ABO blood group

A 
A and B are dominant over O.
A and B are co-dominant
Genotype AB =Phenotype AB
Gene AO or AA = Pheno A
Gene OO = Pheno O
Gene BB or BO = Pheno B
128
Q

Where is RhD gene located

A

Chromosome 1

129
Q

Describe genetic or RhD

A

2 alleles - D and d
d is silent = no protein
Gene dd = Phen RhD neg
Gene DD or D/d = Phen RhD pos

130
Q

Most common type of RhD status

A

RhD positive = 83%

RhD negative = 17%

131
Q

Name some Other blood groups

A 
Kell group (K,k)
Duffy group(Fy^a, Fy^b)
Kidd group (Jk^a, Jk^b)
132
Q

When are other blood groups taken into consideration

A

When the patient is known to have an antibody towards one or more of them

133
Q

What type of anti bodies are irregular antibodies in the blood

A

IgG

134
Q

How is irregular antibodies tested for

A

Donor O-blood with know irregular proteins on it is mixed with patient’s plasma. Left to intubate
Add Anti-Human IgG antibody who creates agglutination

135
Q

What is the test for irregular antibodies called

A

Indirect Antiglobulin Test

136
Q

How quick can blood components be transfused

A

As quick as patient tolerates them

2-3h per unit is average for non-urgent transfusion

137
Q

How to give a blood transufsion

A 
Check patient is correct
Establish IV access
Check bag and compatability label is correct
Pre-transfusion obs (HR, BP, T)
Check patient and bag correct
138
Q

At what percent blood loss does a patient start to be symtpomatic and how

A

20%

Tachycardia, postural hypotension, weak thirst

139
Q

How much blood loss is tolerated in a fit patient

A

30%

140
Q

> 30% blood loss gives what symptoms

A

Confusion, restless, oliguria, air hunger, coma

141
Q

> 50% blood loss indicate

A

Prompt resuscitation mandatory

142
Q

Aim at acute blood loss and blood transfusion

A

Maintain normal pulse rate, BP consciousness, urine output >30ml/hr, Hb >100g/L

143
Q

How long does an ABO and RH,D Ab screeen and cross-match take

A

1 hour

144
Q

Where should platelets never be stored and why

A

Fridge

Cold activates the platelets

145
Q

Commonest indication for fresh frozen plasma transfusion

A

Patients with liver disease who are bleeding

146
Q

Indications for fresh frozen plasma transfusion other than bleeding pt w/ liver disease

A

Oral anticogulant overdose
Coagulopathy following massive transfusion
Disseminated intravascular coagulation (DIC)

147
Q

What type of plasma can be given to anybody

A

AB donor plasma

No antibodies in the plasma

148
Q

What happens if A blood is given to a O recipient, initially

A

Immediate hemolytic transfusion reaction

IgM binds the donor cells and the complement cascade is activated

149
Q

Complement cascade is activated after a transfusion, what happens

A

Release of C3a and C5a - Powerful anaphylotoxins – increased vascular permeability (leaky) – dilated blood vessels – Serotonin and histamin release cause fever, chills, hypotension and shock

150
Q

During Hemolytic Transfusion reaction, activated Factor XII activates the kinin system, what does that do

A

Formation of bradykinin - Arteriolar dilation and increased vascular permeability. Leads to hypotension which leads to release of catecholamines causing vasoconstriction within kidneys and other organs.

151
Q

What is the net effect of Immediate hemolytic transfusion reaction

A 
Systemic hypotension
DIC
Renal vasoconstriction
Formation of renal intravascular thrombi
Renal failure
Shock
OFTEN FATAL
152
Q

Early symptoms of Immediate hemolytic transfusion reaction

A 
Pyrexia/rigors/Pallor/Sweating
Faintness/dizziness
Tachycardia, tachypnea, hypotension
Pain at infusion site
Cyanosis/ Headaches
Patient express that something is wrong
153
Q

Actions in Immediate hemolytic transfusion reaction

A

Stop transfusion, DO NOT REMOVA CANULA
Start IV fluids to maintain BP and urine output
Obtain blood samples for another cross match

154
Q

When does Delayed hemolytic transfusion reaction occur

A

5-10 days post transfusion

155
Q

Features of Delayed hemolytic transfusion reaction

A

Similar to immediate but less acute
Unexplained fall in Hb
Jaundice, renal failure
Caused by irregular antibodies being produced
Positive Direct Antiglobulin test post transfusion

156
Q

What causes Febrile Non-hemolytic transfusion reactions

A

Recipient have antibodies against contaminating white cells in transfusion OR
Release of cytokines and vasoactive substance from white cells during storage

157
Q

Treatment to Febrile non-hemolytic transfusion reaction

A

Stop transfusion and make sure it’s not a more severe cause of symptoms.
Give anti-pyretic if it is FNHT

158
Q

What is Urticarial reactions, symptoms

A

Mast cells have an IgE respone towards infused plasma proteins
Rash/weals within few minutes of starting transfusion

159
Q

Treatement of Urticarial reactions

A

Slow the transfusion

Consider anti-histamines

160
Q

Circulatory overload caused by transfusion leads to what, in who

A

Pulmonary edema

Elderly and those with Congestive Cardiac failure

161
Q

Symptoms exactly as a mmediate hemolytic transfusion reaction but laboratory test come back saying it is not ABO incompatibility. Most likely cause

A

Bacterial infection

Start broad spectrum IV pending identification of the bacteria

162
Q

Commonest bacterial infections carried in Red cells transfusion

A

Pseudomonas

Yersinia

163
Q

Commonest bacterial infections carried in Platelet transfusion

A

Staph, Strep, Serratia, Salmonella

164
Q

How common is Viral spread from blood transfusions of HIV, HBV, HCV

A

HIV - 1/7million
HBV - 1/1.2million
HCV - 1/29million

165
Q

Parts of HbA, HbA2 and HbF

A

HbA - 2 alpha 2 beta chains
HbA2 - 2alpha 2delta chains
HbF - 2alpha 2gamma chains

166
Q

On which chromosomes are Alpha and Beta protein genes

A

Alpha - chromosome 16 (2 genes/chromosome - 4 total)

Beta - Chromosome 11 (1 gene/chromosome - 2 total)

167
Q

What are hemoblobinopathies

A

Hereditary conditions affecting globin chain synthesis

168
Q

Two main groups of Hemoglobinopathies

A

Thalassemias (decreased rate of globin production)

Structural hemoglobin variants (normal production but abnormal globin chains)

169
Q

Two types of Thalassemias

A 
Alpha thalassemia (affect alpha chains)
Beta thalassemia (beta chains affected)
170
Q

Thalassemia leads to what features on blood film

A

Microcytic hypochromic anemia

171
Q

Reduced and Absent synthesis is denoted how in relation to alpha thalassemia

A

Reduced a^+ (-a)

Absent a^0 (–)

172
Q

Classifications of Alpha thalassemia

A 
Unaffected -4 normal a genes (aa/aa)
Silent a thal trait (-a/aa)
a thal trait (--/aa OR -a/-a)
HbH disease (--/-a)
Hb Barts hydrops fetalis (--/--)
173
Q

Features of Alpha thalassemia trait

A

Clinically asymptomatic, no Rx needed.

Microcytic, hypochromic red cells with mild anemia

174
Q

How is Alpha thalassemia trait distinguished from iron deficiency since they have very similar blood films and anemia

A

Normal ferritin in Alpha thalassemia trait

175
Q

What is HbH

A

Hemoglobin tetramer of four beta chains. Cannot carry oxygen.
Formed due to excess beta chains

176
Q

Features of HbH disease

A

HbH is present (1-40%)
Anemia with very low MCV and MCH.
Severe form of thalassemia

177
Q

Clinical features of HbH disease

A

Wide variation: nearly asymptomatic to transfusion dependent)
Splenomegaly due to extramedullary hematopoiesis
Jaundice due to hemolysis and ineffective erythropoiesis
Growth retardation, gall stones and iron overload may occur

178
Q

Where is HbH disease seen

A

South east asia
Middle east
Mediterranean

179
Q

Management of HbH disease

A

Mild - transfusion only needed at times of intercurrent illness
Severe - transfusion dependent
Folic acid supplementation
Splenectomy may reduce need of transfusions

180
Q

Hemoglobin seen in Hb Bart’s hydrops fetalis syndrome

A

Hb Barts - tetramer of gamma

HbH - tetramer of beta

181
Q

Clinical features of Hb Bart’s hydrops fetalis syndrome

A

Pallor, edema – cardiac failure – growth retardation – Severe hapatosplenomegaly – Skeleteal and cardiovascular abnormalities
Most die in utero
Few survive to birth but die shortly after

182
Q

How is Alpha thalassemia diagnosed on blood film

A

Target cells

Anisopoikilocytosis

183
Q

What is Beta Thalassemia

A

Disorder of beta chain synthesis
Reduced (b^+) or absent (b^0) chain produciton
Usually caused by point mutation

184
Q

Classifications of Beta thalassemia

A

B thal trait - B+/B OR B0/B
B thal intermedia - B+/B+ OR B0/B+
B thal major - B0/B0

185
Q

Symptoms of Beta thalassemia trait

A

Asymptomatic
Low MCV/MCH
Raised HbA2 is diagnostic

186
Q

Laboratory features of Beta thalassemia major

A 
Moderate to severe anemia
Very low MCV/MCH
Reticulocytosis
Target cells and Anisopoikilocytosis
Mainly HbF present, HbA2 is elevated
187
Q

Clinical features of Beta thalassemia major

A

Present aged 6-24 months
Failure to thrive
Pallor
Extramedullary hematopoiesis (causing hepatosplenomegaly, skeletal changes, organ damage)

188
Q

Management of Beta thalassemia major

A

Regular transfusions
Iron restricted diet
Bone marrow transplant may be an option

189
Q

What is a Codocyte

A

also called target cells.

Disproportional increase of surface to volume ratio

190
Q

Drug therapy against Iron overload

A

Iron Chelating drugs - Desferrioxamine

Ocular/ototoxicity in high doses

191
Q

Genetics of Sickle cell anemia

A

Autosomal recessive disorder
Point mutation in the Beta globin gene
(Codon 6 - Glutamine –>Valine)

192
Q

Pathophysiology of Sickle cell anemia

A

Genetic mutation causes altered structure to beta chain. Resulting Hb is HbS. HbS polymerize if exposed to low oxygen for prolonged period. Distorts the red cell and damage the membrane,

193
Q

What is Sicle Cell vaso-occlusion

A

Deformed RBCs cause occlusions, leading to tissue ischemia and severe pain

194
Q

What is Sickle Cell trait

A

One abnormal beta gene
Assymptomatic carrier state
May sickle in severe hypoxia, high altitude or under anaesthesia.

195
Q

Features of Sickle cell anemia

A

Tissue infarction due to vascular occlusion.
Digits (dactylitis) Bone marrow, spleen, CNS, lung, renal. Leg ulcers. Pain may be extremely severe.
Sequestrian of Sickled RBCs in liver and spleen

196
Q

Precipitants of Sickle Crisis

A 
Hypoxia
Dehydration
Infection
Cold exposure
Stress/fatigue
197
Q

Treatment of Painful Sickle Crisis

A

Opiate analgesia – Hydration – Rest – Oxygen – Antibiotics to treat underlying infection
Severe cases Exchange transfusion (Venesect – Transfuse – Venesect – Transfuse)

198
Q

Longterm effect of Sickle cell anemia

A

Poor growth – Infections (hyposlenism due to repeated infarcts)
Organ damage (pulmonary hypertension, renal disease, avascular necrosis, CVA, Imptence etc)
Psychosocial problems

199
Q

Long term treatment of Sickle cell anemia

A

Avoid precipitants
Reduce risk of infection (prophylactic penicillin, vaccinations)
Folic acid supplementation
Hydroxycarbamide (induces HbF production)

200
Q

Other sickling disorders are caused by

A

Heterozygosity for HbS and another beta chain mutation

201
Q

Heme to Bilirubin breakdown

A

Heme –> Biliverdin –> Bilirubin

202
Q

Definition of Hemolysis

A

Premature red cell destruction

203
Q

Bone marrow response to hemolysis

A

Reticulocytosis

Erythroid hyperplasia

204
Q

Special stain of Reticulocytes are called and stains what

A

New methylene blue

Stains ribosomal RNA

205
Q

Classifications of Hemolysis

A

Extravascular

Intravascular

206
Q

Features of Extravascular hemolysis

A

Commoner
Hyperplasia at site of destruction (Splenomegaly +/- hepatomeglay
Release of protoporhyrin
Unconjugated birubinemia (jaundice, gall stones)
Urobilinogenuria

207
Q

Intravascular hemolysis abnormal products

A

Hemoglobinemia
Methemalbuminemia
Hemoglobinuria
Hemosiduria

208
Q

Signs of Hemogblobinuria

A

Pink urine that turns black on standing

209
Q

Causes of Intravascular hemolysis

A

ABO incompatible transfusion
G6PD deficiency
Severe falciparum malaria (Blackwater fever)
Paroxyxmal Cold/Nocturnal hemoglobinuria PCH/PNH (very rare)

210
Q

What is haptoglobin

A

Protein in the blood that binds and removes free hemoglobin

211
Q

What are Heinz bodies on blood film a sign of

A

RBC oxidative damage

Dark lumps in RBC due to precipitated Hb

212
Q

Autoimmune hemolysis can be caused by two types of antiboides

A

Warm (IgG)

Cold (IgM)

213
Q

Causes of Warm autoantibody mediated hemolysis

A 
Idiopathic (commonest)
Autoimmune disorder (SLE)
Lymphoproliferaticve disorder (CLL)
Drugs
Infections
214
Q

Causes of Cold Autoantibody mediated hemolysis

A

Idiopathic
Infections (EBV, Mycoplasma)
Lymphoproliferative disorders

215
Q

What test is done for Autoimmune hemolysis

A

Direct Coomb’s test

216
Q

Causes of mechanical red cell destruction

A 
Disseminated intravascular coagulation
Hemolytic uraemic syndrome (E. Coli O157)
Thrombotic thrombocytopenic purpura
Leaking heart valve
Infections (malaria)
217
Q

Which anemia is related to mechanical valves

A

Microangiopathic hemolytic anemia (MAHA)

218
Q

What is seen on blood films of Burns related hemolysis

A

Microspherocytes

219
Q

What is Zieve’s syndrome

A

Hemolysis, Alcoholic liver disease, hyperlipidemia.
Presents w/ anemia, polychromatic macrocytes, irregular contracted cells.
Common in chronic alochol withdrawal

220
Q

Causes of membrane defect hemolysis

A

Zieve’s syndrome
Vit E deficiency
Paroxsmal Nocturnal Hemoglobinuria

221
Q

Genetic cause of red cell membrane abnormalities causing hemolysis

A

Hereditary Spherocytosis

Splenomegaly, jaundice and anemia

222
Q

G6PD causes what

A

Failure to cope with oxidant stress. RBC mostly affected. Cells are damaged and go through hemolysis

223
Q

What signs are seen in Beta thalassemia. General and on x-ray

A

Bone deformities - face widening

X-ray - hair on end appearance, rib within a rib

224
Q

22y female, splenomegaly, intermittent mild jauncice. Gallstones, father had splenectomy in young adulthood for gallstones. Diagnosis…

A

Hereditary spherocytosis

Familial connection, older than congenital cause

225
Q

Blood film with several red blood cells have ring stages inside them. possible diagnosis

A

Falciparum malaria.

Ring stages are the parasites

226
Q

Differene between Ferric and Ferrous

A

Ferric - Fe3+
Ferrous - Fe2+
There’s an o in 2(two)

227
Q

When and where would you take a Tissue biopsy to assess iron status

A

Bone marrow for Fe deficiency

Liver for Iron overload

228
Q

What does % saturation of transferring measure

A

Iron supply

229
Q

What is Hepcidin

A

Negative regulator of iron uptake
Produced by liver in response to iron overload
Down-regulates ferroportin (iron transporter in GI tract)

230
Q

Where is Iron absorption in the GI tract, which transporters

A

Duodenum
DMT-1 (into duodenal cell)
Ferroportin (out of duodenal cell)

231
Q

Hypochromic, microcytic anemia is caused by

A

Deficient hemoglobin synthesis. Cytoplasmic defect

232
Q

How is iron deficiency confirmed

A

Combination of anemia and reduced storage iron (low serum ferritin)

233
Q

How can you loose iron without noticing it?

A

Occult blood loss of 8-10ml per day can occur w/o signs or symptoms. That is 4-5mg iron lost which is the maximum dietary intake. Negative net iron occur

234
Q

What is primary iron overload

A

Long-term excess iron absorption with parenchymal rather than macrophage iron loading, and eventual organ damage. Due to low levels of Hepcidin due to mutation

235
Q

Clinical features of hereditary hemochromatosis

A

Weakness/fatigue – Joint pains – impotence – Arthritis – Cirrhosis – Diabetes – Cardiomyopathy

236
Q

Most common type of mutation in Hereditary Hemochromatosis

A

HFE gene mutation (C282Y mutation or H63D mutation)

Caused reduced hepcidin synthesis

237
Q

Diagnosis of Risk of iron loading and Iron load

A

Risk of iron loading - Transferrin saturation >50%

Iron load - Serum ferritin >300ug/L in men or >200ug/L

238
Q

Treatment of Hereditary hemochromatosis

A

Weekly venesection
Reduces the iron.
Aim at Feritin

239
Q

What is the cause of death in Hereditary hemochromatosis

A

Increased risk of Hepatocellular carcinoma

240
Q

When does hereditary hemochromatosis present

A

Middle age or later.

May be asymptomatic until irreversible end organ damage has occured

241
Q

Genetic inheritance pattern of Hereditary hemochromatosis

A

Autosomal recessive

242
Q

What is the cause of Iron-loading anemias

A

Repeated RBC transfusions

Excessive iron absorption related to over-active erythropoiesis

243
Q

What disorders cause Iron-loading anemias

A

Thalassemia disorders
Sideroblastic anemias
Red cell aplasia
Myelodysplasia

244
Q

What is considered Iron overload

A

Total >5g
OR
Liver >15mg/g dry weight

245
Q

How is secondary iron overload treated in an anemic person receiving regular blood transfusions

A 
Iron chelating agents
-Desferrioxamine (SC or IV)
-Deferiprone (Oral)
-Defereasirox (oral)
Oral drugs not used as much
246
Q

Two definitions of anemia

A

Reduction of Hb below optimum for that indiviudal

Reduction of Hb below 95%range for the population

247
Q

What is the content of a reticulocyte

A

No nucleus
rRNA still present
Larger than a mature red cell

248
Q

Signs and symptoms of Hemolytic anemia

A

Increased unconjugated serum bilirubin
Increased urinary urobilinogen
Anemia+Jauncide +/-splenomegaly

249
Q

Describe breakdown of Heme

A

Heme –>porphyrin –>Bilirubin (All in macrophage)
Bilirubin enters plasma as unconjugated. Taken up by hepatocytes and becomes conjugated. Enters gut in bile.
Taken up as Urobilinogen and secreted through kidneys.
Stercobilinogen stays in gut an excreted through feces.

250
Q

What does Mean Corpuscular Hemoglobin (MCH) measure

A

Hb/red cell count

251
Q

What does Mean Corpuscular Hemoglobin Concentration measure

A

Hb/Hct

252
Q

Iron deficiency causes what types of red cells

A

Microcytic and Hypochromic

253
Q

VitB12 (Cobalamin) or Folate deficiency causes what type of red cells

A

Macrocytic - Megaloblastic anemia

254
Q

Aplastic anemia, myeloma, myelodysplasia causes microcytic or macrocytic anemia

A

Macrocytic anemia

255
Q

Causes of Normochromic normocytic anemia

A 
Hypoproliferative stage
Chronic inflammatory, infective or malignant disorder
Renal failure
Hypometabolic states (Hypothyroidsim)
Marrow failure
256
Q

How does Inflammation cause anemia

A

Inhibits Erythropoietin release throught IL-1beta and TNF-alpha
Inhibits erythroid proliferation
Augments hemophagocytosis
Increase hepatic release of Hepicidin through IL-6

257
Q

What releases IL-6 and what does it do

A

Machrophages in response to inflammation. Acts on Hepatocyte to increase Hepcidin release

258
Q

Anemia of chronic disease findings: Serum Iron – Transferrin – %transferrin saturation – Ferritin – MCV

A 
Serum iron - Reduced
Tranferrin - normal or reduced
% transfer - reduced
Ferritin - Normal or increased
MCV - Normal (can be reduced)
259
Q

Why does renal failure cause anemia

A

Because kidneys release Erythropoeitin. In renal failure the release is decreased in most cases

260
Q

-penia means

A

Low

Ie. Neutropenia, thrombocytopenia

261
Q

-cytosis menas

A

High

Monocytosis

262
Q

-philia menas

A

High

Neutrophilia

263
Q

What shifts the Oxygen dissociation curve to the left

A

Decreased acidity
Decreased DPG
Decreased Temp
(Note: Decreased acidity=Increase pH)

264
Q

What shifts the Oxygen dissociation curve to the right

A

Increased acidity
Increased DPG
Increased Temp
(Note: Increased acidity=decreased pH)

265
Q

What keeps Fe2+ in it’s reduced form in the RBC

A

NADH generated by Ebden-Myerhoff pathway

266
Q

What does the Rapapoport-Lubering Shunt do

A

Generates 2,3 DPG which shifts the oxygen disassociation curve to the right

267
Q

Lecture week 1 done

A

Yes

Mattias - Hemotology (3 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions