Hematology/Oncology (RBC related) Flashcards

Question 1

Q

Expain iron absorption

Answer

A

Fe+2 ferrous
Fe+3 ferric

we consume Fe+3 iron. for absorption it should be converted to Fe+2 by a transported named vitamin C ferrireductase
after conversion the Fe+2 enters in the enterocytes via DMT1 channel

Now if this iron is stored in cell it will be converted back to ferric as in cells and body tissues and blood iron is stored as Fe+3 (knpwn as Ferretin)

for leaving cell Fe+2

for transportation Fe+3 (via transferrin protien)

Question 2

Q

What is Apo transferrin?

Answer

A

Transferrin protien when not bound to the ferric iron Fe+3 is called apotransferrin

Question 3

Q

What is the fate of iron after absorption and travel through blood?

Answer

A

upto 75% of the iron goes to the bone marrow for the synthesis of the RBC

the remaining 10-20% goes to the liver where the transferrin receptors allow the fe to pass through and the liver store this iron as Fe+3

Question 4

Q

What is Ferretin?

Answer

A

A storage protien that binds iron into the tissues .
ferretin is stored in macrophages in the bone marrow
Made in liver and bone marrow
synthesized in large amount during inflammation due to IL-6
degraded in lysosomes——–> hemosiderin

Question 5

Q

What is serum iron?

Answer

A

iron in the Plasma (bounded to transferrin)

Senecent (mature RBC)—> degraded in macrophages in splee—> attached to transferrin and travels in plasma——> goes to tissues to be stored as Feretin

Question 6

Q

why iron cannot be free in the human body?

Answer

A

free iron s toxic.

Question 7

Q

what kind of relation is between Transferrin (plasma iron bounded) and ferretin (tissue bounded iron)

Answer

A

inverse relation.

ferretin increase transferrin decrease and vice versa

transferrin can be equal to TIBC that is the total iron binding capacity. (how well iron binds to the transferrin)

ferretin high means iron is more attatches to tissue and blood is not able to bind it so automatically TIBC or transferrin in blood will become low)

Question 8

Q

Explain soluble Transferrin Receptor concentration (sTFR)

Answer

A

it increase in iron deficiency anaemia

normal in anaemia of chronic disease

Question 9

Q

What is fenton reaction?

Answer

A

when iron left free—-> formation of free hyroxyl radical (OH^) —–> destruction

can applied to copper to

Fe+3 + h2o2 —–> Fe+2 + H+ + HO^

Question 10

Q

Iron Deficiency anaemia? (microcytic)

Answer

A

most common cause of Anaemia in the world

causes - decreased supply (nutritional)
-malabsorption due to celiac disease, veganism, post gastric surgery)

              increased demand (in growing children)(pregnancy)

                  acute blood loss or intravascular hemolysis

Question 11

Q

mention the clinical signs related to iron deficiency anaemia

Answer

A

PICA
Glossitis (inflammation of tongue) Chellitis (inflammation of lips)
restless leg syndrome
koilonchia (U cuped nails)
beeturia

LAB -
transferrin (TIBC) increases
Ferretin decrese

Question 12

Q

Explain anaemia of chronic disease or Anaemia of infammation.

Answer

A

Anemia of chronic disease refers to having low levels of red blood cells as a result of autoimmune diseases (diseases in which the body’s immune system attacks joints and/or body organs) or other chronic illnesses. Chronic diseases are those that last longer than 3 monthsMC anaemia in chronic inflammations and alchololics
giving blood to patients with chronic diseases worsens the conditions

iron remains trapped in the ferretin and cannot be utilized (reason unknown)

Question 13

Q

What is hepcidin?

Answer

A

Hepcidin is an iron-regulating peptide hormone made in the liver. It controls the delivery of iron to blood plasma from intestinal cells absorbing iron, from erythrocyte-recycling macrophages, and from iron-storing hepatocytes.

Question 14

Q

Explain haemoglobin

Answer

A

Hb-- 4 subunits
protien part -- globin
non protien part---heme---iron anf porphyrin
 adult 2 alpha 2 beta subunit (95%)
foetal 2 alpha and 2 gamma (1 %)

Question 15

Q

what happens if Hb have Fe+3 instead of Fe+2?

Answer

A

its known as met-haemoglbin and that is not normal since Fe+3 cannot carry oxygen

Question 16

Q

Explain Thalasemia

RBC caoount is increases reason unknown

Answer

A

Problem with the globin chains of the Hb

Microcytic

Autosomal Recessive

Thalassemia is an inherited blood disorder that causes your body to have less hemoglobin than normal.

-Globin chain synthesis is unbalanced (alpha and beta0
-Hemotetramers are formed (incorrect protien chain ) that are insoluble and precipitated in RBC
unnatural RBC= destroyed= anaemia

or low Globin formn, low Hb and Low RBC

Ineffective Erythropoesis seen (BM will try to produce RBC but cannot)

Question 17

Q

Explain Alpha and beta thalassemia.

Answer

A

Alpha -
we have 4 genes on the 16th chromosome responsible for producing the alpha subunit of the Hb chain
here these genes are deleted
the more gene deleted the worse the problem becomes
1 gene (aa/a-) asymtomatic (lpha thalassemia trait)

2 gene (aa/–) (a-/a-) asymptomatic . maybe mild MCV

3 gene (–/a-) here now beta chains are unpaired
(HbH) and will now form
hemotetramers.
Moderately to severe hemolytic
anaemia
DO BLOOD TRANSFUSION

4 gene (–/–) death occurs in foetus itself
(Hb barts_ since foetus have t
wo gamma cahins heter forms

                                hemotremers formed. foetus dies.

                                 NO CURE

beta thalassemia chromosome 11

For dognosis use Electrophoresis

Alpha thalassemia trait- Normal
HBH nd HB barts seen in other variants

Question 18

Q

Explain sideroblastic anaemia? (sidero=constellation and iron)

Answer

A

Problem with Protophyrin synthesis.

Protoporphyrin+ fe+2=heme

without protophyrin availability Iron will have no use and will start to accumulate
no heme=anaemia

main cause = alcohol
B6 deficiency
lead poisioning
congeniatal diseases.

(see heme synthesis pathway)

the iron that is stored is stored in the RBC in form of a ring around the nucleus (constellation)

mcv low
serum iron high
ferretin high
TIBC low (transferrin) (total iron binding capacity)
% saturation= iron/TIBC = increases since iron high and TIBC low

Diagnosis- organ damage due to iron (fenton rxn)
hepatomegaly or splenomegaly

the RBC with ring sideroblastic cells will not exit the bone marrow so by doing the bone marrow biopsy we should see these cells by using Prussian blue stain (for iron)

Question 19

Q

Explain Macrocytic Anaemia. (MCV>100)

Answer

A

high RBC size or MCV more than 100

two types
1) Megaloblastic (hypersegmentet Nuterophils found) (upto 10 lobes rather than 2-3)

folate and B12 deficiency

2)Non Megaloblastic
no hypersegmented neutrophils in blood
liver disease,alcholoh and drugs

Question 20

Q

how Macrocytic anaemia takes place?

Answer

A

Normally durinf the synthesis of the mature RBC the starting cell is the proerythroblast from the myeloid lineage

now the Proerythroblasts divides forming smaller cells . Here the DNA synthesis plays an important role for the dicision of cells

in the case of macrocytic anaemia DNA synthesis is impaired so the cell are not dividing/getting smaller/maturing so the cell size remains.

the RNA synthesis and protien synthesis is taking place normally so these are synthesized causing the cytoplasm of the cell to increase causing more increase of size of the cell.

the problem with the dna synthesis will also affect the other cell lineages such as WBC and platelets.

these cells are not normal cells so they will be phagocytosed by the micropphages leading to Pancytopenia.

Question 21

Q

Why the deficiency of folate or B12 causes Megaloblastic anaemia?

Answer

A

folate and B12 both are important factors for the dna synthesis of RBC

folate is reduced to THF (tetrahydrofolic acid) in the intestine

this THF has a methyl group attached that is not neede
so THF-CH3 transfers methyl (CH3) to the B12

then the B12 transfers this to the Homocysteine which then converts to Methionine.

Now the B12 and THF can take part in synthesis of dna

Question 22

Q

where is iron, folate and B12 is absorbed in intestine?

Answer

A

duodenum- iron

jejunum - folate

illeum- B12

(Dude Is Just Felling Ill Bro)

Question 23

Q

explain Folate deficiency.

Answer

A

cause of megaloblastic anaemja and pancytopenia.

Folate is importannt for the DNA synthesis

any folate deficiency (decreased intake,malabsorption,increases demand pregnancy and lactation,alchoholism,or drug inhibition)

Question 24

Q

Explain how folate is used in Dna synthesis.

Answer

A

Folate is eaten by us as Polyglutamate.

this polyglutamante is coverted to monoglutamate via CONJUGASE enzyme.

Conjugase is inhibited by Phenytoin (antiepileptic drug)

monoglutamate is absorbed in the form of THF-CH3\

CH3 send to B12 (cobalamine to methylcobalamine)

further B12 sends the Ch3 group to the Homocysteine which then gets converted to methionine

Now THF goes and is then coverted to Methylene THF (not methyl,Methylene.)

Methylene THF—–> DHF——->THF (circle)

When methyleneTHf gets converted to DHF dTMP is formed which is used in dna synthesis

this is catalyzed by THYMEDYLATE SYNTHASE. (blocked by 5-FU)

Question 25

Q

Explain B12 deficiency.

Answer

A

Eat.

B12 is with animal protien.
Pepsin from stomach will break the animal protien.

the salivary gland ,the stomach and the billiary tract produces R protien that will attach to B12 to save it from the HCL in stomach.

goes to duodenum where the Pancreastic enzyme called Protease will break the R protien cause it is not needed.

Now Castle intrinsic factor (produced by stomach but travel through illeum) will join B12 and helps the absorption.

after absorption Transcobalmine will bind to B12 to travel through blood.

thats why pancreatic isufficiency, illeum destruction (chrons or tapeworm) , illeum resuction, stomch resection will cause deficiency of the B12
B12 either can go to liver to be store (for yearss) or to cells to be utilized in the dna synthesis

Question 26

Q

Explain how B12 deficiency will leas to demyelination of the CNS?

Answer

A

odd chain FA—>Propinyl CoA——–(B7 carbocylase)——> methylmalonyl CoA—-(B12 mutase)—–> succinyl coa

this succinyl coa will go to krebb cycle.

now if there is a deficiency of B12 mutase cannot work leading to increase concentration of methylmalonyl and propynyl Coa

these will replace acetyl Coa in neurons and myelin sheath leading to demyelination

Question 27

Q

Symptoms of B12 deficiency

Answer

A

pale
tired
Glossitis
Peripheral Neuritis
Lemon yellow skin
subacute combined degenration od spinal cord
Dementia

Question 28

Q

What is Schilling test? (used to diagnose B12 deficiency) (not used today)

Answer

A

radioactive B12 is given and then it is noticed in urine to see if it is absorbed or not in the GIT.

Question 29

Q

main diffrences between B12 and folic acid deficiency.

Answer

A

If homocysteine ans Mathomalonyl acid increase = B12

if only homocysteine = folic

see the pathways.

Question 30

Q

Explain non-megaloblastic anaemia.

Answer

A

big oval ceels
problem only with RBC . no other cells affected
sometimes anaemia is not there. only MCV

Question 31

Q

Explain normocytic anaemia. Mcv 80-100

Answer

A

size normal. low count rbc
1) acute blood loss (retculocytes < 2.5)

any abdominal puncture. 
losing plasma and RBc at the same rate (start of trauma)
plasma replaced after sometime so RBC count low as compared.

2) underproduction (retculocytes < 2.5)

     anaemia of chronic disease and iron deficiency anaemia (usually ealy because later it becomes microcytic) 
     renal failiure (EPO low) or aplastic (bone marrow problem)

3) increased destruction (reticulocytes>2.5) (for compensation high reticulocytes are produced)

intrinsic- Hb defect, membrane defect, G6PD deficiency
Ectrinsic- antibpdies

Question 32

Q

In acute blood loss (more than 20% blood lost) can we see reticulocytosis?

Answer

A

yes but after some time. first we will see depletion of both plasma and RBC (hypovolemic shock) then plasma is compensated and RBC gets diluted we will see anaemia (normocytic)

after that for compensation body will strat making more reticulocyte

Question 33

Q

Explain Aplastic Anaemia. ( failed bone marrow) (high MCV. no reason :/)

Answer

A

Even though it is named anaemia, all blood cells are low in number. correctly it should be named Pancytopenia)

Aplastic Anaemia= Pancytopenia + hypocellular BM

( MDS = Pancytopenia + Hypercellular BM )

reason -
Fanconi ANemia
Radiation,drugs,toxin,viruses
Intensive chemo therapy
Tcells-->cytokines---> cytokines will destroy myeloid stem cells.

signs and symtoms will be those related to pancytopenia that includes of general anaemia, of thrombocytopenia and of leukocytopenia (Infections and diseases)

Question 34

Q

What is the Difrence between the Aplatic anaemia and MDS (myelodysplasia syndrome)

Answer

A

Classically, MDS has a hypercellular marrow – too many cells. In contrast, aplastic anemia has a hypocellular marrow, showing a very low number of cells

Dx- hypocellularity (cells replaces by fat and Stroma)

Question 35

Q

Explain the Anaemia in Chronic Kidney Disease (CKD)

Renal failiure is today known as CKD.

Answer

A

Chronic kidney disease, also called chronic kidney failure, involves a gradual loss of kidney function.

EPO deficiency is the cause of Anaemia related to CKD

There are 5 stages on based on the GFR rate.

Normal GFR is above 60

1st (normal )  90-120
2nd (mild)      60-90
3rd  (moderate) 30-60
4th (severe)        15-30
5th (end stage)    0-15

the anaemia of CKD usually starts at Stage 3 and universally at stage 4

Question 36

Q

anaemia due to malignany/cancer?

Answer

A

Nutrietional deficiency in cancer
cachezia
bowel bleeding
due to treatment (radiation and chemo)

Question 37

Q

what is Pure Red cell Aplasia?

Answer

A

Pure absence/Low RBC

is it diffrent from pancutopenia or aplastic anaemia as in this WBC and platelets ae normal

problem only with RBC
only one cell line is compromised from top to bottom
(from proerythroblasts to Mature RBC)
unlikely to progress to any other blood disease

Main causes - Parvo 19 virus

in Bone marrow biopsy– Red cell Aplasia + giant pronormoblast (as they cannot progress to mature form)
\

reticulocyte indiex will also be low

Question 38

Q

Explain Extravascular Hemolysis.

Answer

A

Hemolysis happening inside the Spleen (monocytes), liver (macrophages), Lymph nodes (histiocytes)

too much destruction- High Unconjugated billirubin.

High LDH due to RBC destruction

high protophyrin

Question 39

Q

What should you see first about the anaemia?

Answer

A

wheather it is due to over under production or due to blood loss.

each type of anaemia is becaause of one of these causes

Question 40

Q

what is Haptoglobin?

Answer

A

It is a Plasma protien produced by Liver

When RBC’s are destroyes EXtravascularly (by the rectoendohelial system) the Hb comes out.

Haptoglobin is the protien that binds the Hb to form the Hapto-Hb complex

In the case of hemolytic anaemias this complex is also phagotised by Macrophages leading to the decreased serum level of Haptoglobin.

Question 41

Q

what is the main diffrence between Macrophage, monocyte and histiocytes?

Answer

A

deally, the macrophage is the umbrella term for any phagocytic agranulocyte. This can be divided into two major categories:

1) The histotiocyte is the macrophage of solid tissue.
2) The monocyte is the macrophage of blood.

Question 42

Q

Explain Intravascular Hemolysis.

Answer

A

hemolysis inside blood vessels.

causes can be Macro or Microangiopathic Hemolytic Anaemia

high unconjugates billirubin present
high protophyrin
since the destruction is taking place in the vessels some Hb will go as it is to the kidney which will give us hemoglobinuria /hemosudineria (only seen in intravascular)

increase LDH
less haptoglobin

Hepatomegaly and splenomegaly both are seen in both intra and extravascular hemolysis

Question 43

Q

Explain hereditary spherecytosis.

Answer

A

Hemolytic anaemia
normal RBC= Biconcave, can expand through osmosis, canmaneuver/pass through sinusoidal spaces in spleen

herediatry spherocytes- defect in the membrane (spectrin, ankyrin band3.1)

microvesicles formed as membrane is not solid, h2o lost and cells become circular

spherocytes can be seen with all types of hemolytic anaemia

Hereditary spherocytosis
can be autosomal recessive or dominant

dark brown stone is seen as extravascular hemolysis taking place in spleen (cll stuck outside sinusioid due to their size and macrophage eats them) as too much CB is formed, goes to bile and with calcium forms stone)

Question 44

Q

Explain Hereditary elliptocytosis. (elongated RBC)

Answer

A

Defect in spectrin band 4.1
Autosomal dominant
many genes
Family history

this condition either can lead to hemolysis or not at all.
this depends
some person can live normally without any symtoms at all or
some can have all the symtoms related to extravascular hemolysis

Question 45

Q

Explain Sickle cell anaemia. (HbS)

Answer

A

Autosomal Recssive
subsaharan African Population

Defect in Beta globin chain in the Hb (instead of Glutamine (hydrophillic), Valine (hydrophobic) is there)
(point mutation) (non-conservative)

This valine cause the RBC to change to Sickle shape when there is a deficiency of O2

no O2= sickle shape = destroyed
present O2 = no sickle

It is not the same as Beta thalessemia as in this no globin chain is formed. in sickle cell Beta chain is converted to a diffrent chain because a whole protien is replaced by another)

Right oxygen curve is seen (oxygen not coming to cells/blood)

In foetus cells will not sickle (upto 6 months) because they will have HbF (high affinity to O2) not HbS (no beta chain until 6 months)

Question 46

Q

Explain clinical signs of sickle cell disease.

Answer

A

all signs and symptoms of extravascular hemolysisi

also signs and symtoms of vaso-occlusive crisis.

Nephropathy

Question 47

Q

what is vaso occlusive crisis?

Answer

A

This occurs when the flow of blood is blocked to an area because the sickle-shaped cells have become stuck in the blood vessel. Pain can occur anywhere but most often occurs in the bones of the arms, legs, chest, and spine.

Question 48

Q

what is acute chest syndrome? (due to sickle cell anaemia)

Answer

A

Acute chest syndrome is a term used to cover conditions characterized by chest pain, cough, fever, hypoxia (low oxygen level) and lung infiltrates. Acute chest syndrome may be the result of sickling in the small blood vessels in the lungs causing a pulmonary infarction/emboli or viral or bacterial pneumonia.

Question 49

Q

How can sickle cell anaemia affect the spleen?

Answer

A

1) Autosplenectomy- Autosplenectomy can occur in cases of sickle-cell disease where the misshapen cells block blood flow to the spleen, causing scarring and eventual atrophy of the organ. (occlusion=ischemia=infarction)
useless spleen= more prone to infection by encapsulated organisms like salmonella

2) sequestration = Sickle cell gets trapped in spleen=spleen enlarged and painful
Hyposlenism can occur

Question 50

Q

Name all the sickle cell crisis.

Answer

A

1) Vaso-Occlusive crisis (pain,ischemia and infarction)
2) Aplastic crisis (when the body does not make enough new red blood cells to replace the ones that are already in the blood)
3) splenic sequestration crisis
4) Hemolytic crisis

Question 51

Q

what is sickle cell trait?

Answer

A

having homozygous sickle cell trait. (being a carrier)
(HbAHbS)
sicle cell disease= HbSHbS

Question 52

Q

What is howell Jolly Bodies?

Answer

A

A Howell–Jolly body is a cytopathological finding of basophilic nuclear remnants (clusters of DNA) in circulating erythrocytes. During maturation in the bone marrow, late erythroblasts normally expel their nuclei; but, in some cases, a small portion of DNA remains.

Jolly-Happy that the nuclear remains were somehow manage to survive :)

usually these cells are destroyed by spleen but in case of splenectomy or Functional asplenia(seen in sickle cell vaso-occlusive crisis) these cells somehow manage to survive.

Question 53

Q

Explain why we need G6PD in oour body (biochem)

Answer

A

Free radicals like hydrogen peroxide (H2O2) damages cell by destroying DNA,protiens and cell membranes.

Glutathione is the natural antioxidant that helps get rid of the free radicals

When reacting with the H2O2 the glutathione becomes oxidised (inactive form) and h2o2 is converted to water and oxygen.

H2O2——> H20 + O2

now NADPH comes and becomes NADP+ by reducing the glutathione and getting it back to it original form

so now it is important that we have a surplus of NADPH so we can always get back the glutathione in its working state and to neutralize the free radicals.

thats where G6Pd comes in place.

G6PD reduces the NADP+ into NADPH.

It does this by oxidising glucose-6-phosphate (already available through glucolysis) to 6 phosphogluconate.

so heres the simple explanation.

g6pd helps form NADPH that is needed to active the glutathione which will further help to neutralize free radical.

Red blood cell is protected :)

these free radicals converts the Fe+2 to Fe+3(ferric) state that causes the formation of met Hb

Question 54

Q

Explain G6PD deficiency.

Answer

A

Most common enzymopathy worldwide. (10 percent of population)

2 variants- african and medeterranian

X linked recessive. (affects male more)
rare in females.
males are either normal or affected (never carriers)
females are either normal,carriers(heterozygous).affected(homozygous)

signs syptoms- Related to intravascular haemolysis (RBC are damaged from inside in the vessels)
Jaundice
haemoglobinuria

clinical signs- acute hemolytic anaemia not related to pherocyotis
hemolytic episodes

when stressors are removed full recovery can happen (fava beans or oxidative stresses)

Question 55

Q

What is favism?

Answer

A

Favism refers to the clinical syndrome of acute hemolytic anemia from the ingestion of fava beans as an oxidative challenge in patients with G6PD deficiency

Question 56

Q

Name some oxidative agents or stressors

Answer

A

Antimalarials
Sulfa  drugs
ANtibiotics
Analgesics
Infections (No nadph) (WBC uses these h2o2 to fight infections)

Heinz bodies is seen (a signature of the cell being exposed to oxidative stress)

Question 57

Q

Why should we never test for the G6PD deficiency during an acute episode?

Answer

A

during acute episode the RBC with low G6PD are beinf hemolysized so the BM produces reticukocytes in high number (reticulocytosis) . now these reticulocytes wll have sufficient G6PD and will alter the test results.
we should wait atleast 2-3 months before doing the test.

management- blood transfusion
folic acid supplements
iron chelations

Question 58

Q

What are heinz bodies an Bite cells?

Answer

A

heinz bodies are the denatured or damaged inclusions of Hb in the cells (Heinz=Hb) ‘
they are the SIgnature that the rBC are exposed to Oxidative stress

this part is usually removed by splenic macrophages leading to a half bitten cell called bite cell

ramanovsky stain- hemighost
supravital stain- blue damaged Hb
 seen in - NADPH deficiency
G6PD deficiency
chronic liver disease
asplenia/hyposplenia

Question 59

Q

what is paroxysmal nocturmal hemoglobinuria?

Answer

A

Acquired
Chronic hemolytic aaemia
Defect in myeloid stem cells (PIGA gene)

PIGA encodes the formation of GPI (anchor protiens for the inhibitory protiens of the myeloud lineage)

No complementary inhibitory protiens - CD55 and CD59

RBC and other mylod lineage cell cannot protect themselves from the compement system (alternative pathway)

they gets Intravascular hemolysis (inside vessels)

So overall PNH is the Intrinsic, intravascular and acquired hemolytic anaemia

hemolysis occurs throughout the day, in the night the urine have Hb (hemoglobinuria)

most common death in PNH= Thrombosis. why? no one knows. maybe due to plastelets destruction (myelooid lineage)

second cause is infection

Question 60

Q

Explain complemet system.

Answer

A

group of 20 plasma protiens
synthesized in liver
mostly 11 protien involved

three pathway
1) antigen antibody
20 bacterial endotoxin (alternative) (for pnh)
3)lectin pathway

Question 61

Q

How does complement diffrentiate between non self (bacterial) and self cells

Answer

A

Via regulatory protiens
CD55 (DAF) and CD59 (MIRL)

the rBC, Platetlets and WBC (only myeloid lineage) have anchor protiens GPI that have these inhibitory regulatory protien that prevents formation of convertase C3 and blocks binding of C9 and doesnt let form MAC.

thus they prevent the complement to attack themselves.

Question 62

Q

what are the comlications od PNH?

Answer

A

Iron deficiency anaemmia and acute myeloid leukemia

Question 63

Q

What is the triad for the PNH?

Answer

A

Hemolytic anaemia
Pancytopenia
Thrombosis

Question 64

Q

can reticulocytosis increase MCV

Answer

A

yes. Reticukocytes are larger than RBC because they are one step immature. as they mature they become smaller in size

Answer 65

A

The Coombs’ test is used to detect antibodies that act against the surface of your red blood cells. The presence of these antibodies indicates a condition known as hemolytic anemia, in which your blood does not contain enough red blood cells because they are destroyed prematurely.

Answer 66

A

eculizamab – antibody against C5 to stop the formation of MAC

Answer 67

A

HAM test (ham=pig=piga gene)

Answer 68

A

In hemolytic anemia, red blood cells in the blood are destroyed earlier than normal. Immune hemolytic anemia occurs when antibodies form against the body’s own red blood cells and destroy them. This happens because the immune system mistakenly recognizes these blood cells as foreign.

if Mac formation- intravascular
if IgG etc- intravascular as engulfed by macrophages in spleen

Answer 69

A

1) autoimmune
2) drug induced
3) alloimmune (non self such as transplant)

Answer 70

A

IgG is warm

IgM is cold

Answer 71

A

It is organ specific (RBC)
can be warm (80% of the case) and cold (20%) dpending on th AB

Usually all type of autoimmune diseases can be of two types- 1) organ specific (affecting only one type of organ)
2)Multisystem (Immune response against widespread target antigens)

Warm - IgG - it is usually against the rhesus antigen - autoimmune hemolysis of RBC

Cold- IgM - pentamer structure- binding of the antigen to the complement- cold Agglutination Disease.

signs and symptoms are related to anaemia and extravascular hemolysis in case of warm. massive splenomegaly and jaundc is also seen

It can be intravascular if the complemnt fixes itself completely

if the complemmnt activation is weak its an extravascular hemolysis

Answer 72

A

Always lookout for other autoimmune diseases as one autoimmune disease is almost always acompanied by another one.
sucl as SLE,CLL etc

Answer 73

A

Pyruvate kinase is the important enzyme in the last step of glycolysis.
In glycolysis 4 ATP molecule are generated in which 2 is used by the pathway itself, the other two is used by the cell for energy

after pyruvate is made it froms acetyl CoA and goes to mitochndria to participate in ETC however in RBC the glycolysis pathway ends at pyruvate (since RBC have no mitochondria) and this is the only way for the RBC to produce energy (2 ATP)

PhosphoenolPyruvate——(pyruvate kinase)—–>pyruvate
2ADP–>2ATP

Answer 74

A

Pyruvate kinase deficiency is a genetic blood disorder characterized by low levels of an enzyme called pyruvate kinase, which is used by red blood cells . Without pyruvate kinase, red blood cells break down too easily, resulting in low levels of these cells ( hemolytic anemia ).

Autosomal recessive
Intracorpuscular defect (intrinsic)---extravascular hemolysis

No PK= No ATP = RBC dehydrated= Burr cells (echinocytes) (echino=sea urchin shaped)

Answer 75

A

The glycolysis will not complete. In theis pathway when 1,3 Bis-phosphoglutarate converts to 3 phosphoglutarate, this step will not occur and instead 2,3 bisphosphglutarte is formed

2,3BPG increase= right shift of curve= RBC gives O2 to tissues
= symtoms of anaemia subsides :)

Answer 76

A

Drug-induced immune hemolytic anemia is a blood disorder that occurs when a medicine triggers the body’s defense (immune) system to attack its own red blood cells. This causes red blood cells to break down earlier than normal, a process called hemolysis.

Answer 77

A

Cephalosporins
Penicillin (BPO group of this drug adsorp on RBC and makes RBC susceptible to the macrophages)

antimalarial drug like Quinine

MethylDopa (change the structure of the Rh antigen on rbc)

Nitrofurantoin

Fludarapine

Answer 78

A

Patient takes penicillin—–> rash and hypotension —-> (this is allergic reaction type 1)
Patient takes penicillin——> Jaundice and hemolysis—->DIIHA—-> type 2 hypersen. Rxn (cytotoxic)

patient takes pen.—>1-2 weeks later fever,urticaria arthalgia,——> serum sickness like rxn

Answer 79

A

Echinocytes (also called burr cells) have serrated edges over the entire surface of the cell and often appear crenated in a blood smear (small projections more number)
These are also seen in Renal Failiure/uremia

Spur cells appear to be the extreme form of acanthocytes and are seen in patients with severe liver disease (Large projections, less numbers)

Answer 80

A

Acantha == thorn

Acanthocytes are found in 50-90% of cells on peripheral blood smear findings in abetalipoproteinemia, which is a rare autosomal recessive disorder with only about 100 cases described worldwide.
Acanthocytes are also relatively common in severe liver dysfunction and malnutrition.

Answer 81

A

Any abnormally shaped cells are called poikilocytes

phenomena of different size od RBC’s are called anisocytosis

Codocytes, also known as target cells, are red blood cells that have the appearance of a shooting target with a bullseye (from top view) and a mexican hat shape from side (basically a cup shape)

High surface/volume ratio

Cause- Liver disease, thalessemia, hemoglobinopathies

Answer 82

A

Except for acute blood loss and sequestraton the main causes of anaemia are hypoproliferaive or survavial causes

Brainscape's Knowledge GenomeTM

Hematology/Oncology (RBC related) Flashcards

Brainscape's Knowledge Genome^TM