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Pathology > Chapter 5 ( RBC Disorder ) > Flashcards

Chapter 5 ( RBC Disorder ) Flashcards

1
Q

Mechanism of microcytosis ?

A

Due to extra division of RBC progenitor cells in the bone marrow which occurs to maintain hemoglobin concentration in cases of decreased hemoglobin

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2
Q

In which forms is iron consumed ?

A 
Heme form ( meat-derived ) 
Non-heme form ( vegetable-derived )
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3
Q

Absorption of iron in git ?

A

In the duodenum , enterocytes have heme and non heme transporters (DMT1)

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4
Q

Hook worms that cause iron deficiency anemia in elderly ?

A

Ancylostoma duodenale

Necator americanus

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5
Q

Lab findings of iron deficiency anemia ?

A 
Microcytic hypochromic RBCs
⬆️ RDW
⬇️ferritin
⬆️ TIBC
⬇️serum iron and saturation 
⬆️ FEP
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6
Q

Presentation of Plummer Vinson syndrome ?

A

Anemia
Dysphagia
Beefy red tongue

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7
Q

Most common type of anemia in hospitalized patients ?

A

Anemia of chronic disease

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8
Q

Mechanism of anemia of chronic disease ?

A

Chronic disease results in production of acute phase reactant from the liver including Hepcidin

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9
Q

Action of Hepcidin ?

A

Sequesters iron in storage sites by
1- limiting iron transfer from macrophages to erythroid precursors
2- suppressing erythropoietin production

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10
Q

Lab findings of anemia of chronic disease ?

A

⬆️ ferritin
⬇️ TIBC
⬇️ serum iron and saturation
⬆️ FEP

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11
Q

Why its called sideroblastic anemia ?

A

Because of the presence of ringed sideroblasts ( iron laden mitochondria forming a ring around the nucleus of erythroid precursors )

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12
Q

Etiology of sideroblastic anemia ?

A

Congenital : most commonly involving ALA synthase
Acquired :
1- alcoholism ( mitochondrial poison )
2- lead poisoning ( inhibits ALA dehydratase and ferrochelatase )
3- vitamin B6 deficiency

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13
Q

Lab findings of sideroblastic anemia ?

A

⬆️ ferritin
⬇️ TIBC
⬆️ serum iron and saturation ( iron overloaded state )

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14
Q

Against which disease is thalassemia carriers protected ?

A

Plasmodium Falciparum Malaria

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15
Q

HbA2 ?

A

Alpha2 delta2

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16
Q

Defect of alpha thalassemia ?

A

Gene deletion of alpha genes (4 can be deleted on chromosome 16 )

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17
Q

One gene deletion in alpha thalassemia ?

A

Asymptomatic

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18
Q

Two gene deletion in alpha thalassemia ?

A

Mild anemia with increased RBC count , cis deletion is associated with increased risk if severe anemia in offspring

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19
Q

Three gene deletion in alpha thalassemia

A

Sever anemia

Beta chains form tetramer HbH that damages RBCs

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20
Q

Four gene deletion in alpha thalassemia ?

A

Lethal in utero ( Hydrops Fetalis )

Gamma chains forms tetramer Hb Barts that damages RBCs

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21
Q

Beta thalassemia defect ?

A

Gene mutations ( point mutation in promotor regions or splicing sites )

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22
Q

Beta genes (2) present on chromosome ?

A

11

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23
Q

Lab findings in beta thalassemia minor ?

A 
⬆️ RBC count 
Target cells 
⬇️ HbA
⬆️ HbA2 ( 5% normal 2.5% ) 
⬆️ HbF ( 2% normal 1% )
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24
Q

In beta thalassemia major , risk of aplastic crisis occurs with which virus ?

A

Parvovirus B19 infection of erythroid precursors

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25
Q

Lab findings of beta thalassemia major ?

A

Increased HbA2 and HbF

No or little HbA

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26
Q

Cause of hypersegmented neutrophils in megaloblastic anemia ?

A

Impaired division of granulocytic precursors

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27
Q

Causes of macrocytic anemia without megaloblastic changes ?

A

Alcoholism
Liver cirrhosis
Drugs as 5-Flurouracile

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28
Q

Site of absorption of folate ?

A

Jejunum

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29
Q

Role of salivary gland in absorption of Vit B12 ?

A

Salivary gland enzymes as amylase librate vit B12 from animal derived proteins which is then bound to R-binder ( also from salivary gland ) that carries it through the stomach

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30
Q

Role of pancreatic enzymes in absorption of vit B12 ?

A

Detach vit B12 from R-binder

31
Q

Site of absorption of intrinsic factor-Vit B12 complex ?

A

Ileum

32
Q

Parasite that causes vit B12 deficiency ?

A

Diphyllobothrium latum ( Fish Tapeworm )

33
Q

Reticulocytes appearance on a blood film ?

A

Larger cells with bluish cytoplasm due to residual RNA

34
Q

Why reticulocyte count is falsely elevated in anemia ? How to correct it ?

A

Decrease in total RBCs falsely elevates percentage of reticulocytes
Corrected by multiplying reticulocyte count by ( Hct / 45 )

35
Q

Reticulocyte count

A

1 - 2 %

36
Q

Corrected reticulocyte count >3% indicates what ?

A

Good marrow response

And suggests peripheral destruction of RBCs ( cause of anemia )

37
Q

Corrected reticulocyte count < 3% indicates what ?

A

Poor marrow response

And suggests underproduction of RBCs ( cause of anemia )

38
Q

Site of extravascular hemolysis ?

A

Reticuloendothelial system as :
Macrophages of spleen
Liver
Lymph nodes

39
Q

Clinical and laboratory findings of extravascular hemolysis ?

A

1- anemia with splenomegaly
2- jaundice due to unconjugated bilirubin and increased risk for bilirubin gallstones
3- marrow hyperplasia with corrected reticulocyte count > 3%

40
Q

Clinical and laboratory findings of intravascular hemolysis ?

A

1- hemoglobinemia
2- hemoglobinuria
3- hemosiderinuria
4- decreased serum haptoglobin

41
Q

Defect of hereditary spherocytosis ?

A 
Inherited defect of RBC cytoskeleton-membrane tethering proteins 
Most commonly involves : 
1- Ankyrin 
2- spectrin 
3- band 3
42
Q

Lab findings of hereditary spherocytosis ?

A

1- ⬆️ RDW
2- ⬆️ MCHC
3- spherocytes with loss of central pallor

43
Q

Hereditary spherocytosis is associated woth increased risk pf what ?

A

Aplastic crisis with parvovirus B19 infection of erythroid precursors

44
Q

Anemia with increased MCHC ?

A

Hereditary spherocytsis

Autoimmune anemia

45
Q

Blood smear findings after splenectomy ?

A

Howell-jolly bodies ( fragments of nuclear material in RBCs )

46
Q

Defect of sickle cell anemia ?

A

Autosomal recessive mutation of Beta chain of hemoglobin , a single aa change replaces glutamic acid ( hydrophilic ) with valine ( hydrophobic )

47
Q

Mechanism of hydroxyurea in ttt of sickle cell anemia ?

A

Icreases level of HbS which protects against sickling

48
Q

Cause of Increased risk of sickling ?

A

Hypoxemia
Dehydration
Acidosis

49
Q

Complications of vaso-occlusion ?

A

1- Dactylitis ( common presenting sign in infants )
2- autosplenectomy
3- acute chest syndrome
4- pain crisis
5- renal papillary necrosis result in gross hematuria and proteinuria

50
Q

Results of autosplenectomy in sickle cell disease ?

A

1- increased risk of infection with encapsulated organisms such as streptococcus pneumoniae and hemophilus influenza ( most common cause of death in children )
2- increased risk if Salmonella paratyphi osteomyelitis
3- Howell-Jolly bodies on blood smear

51
Q

Most common cause of death of adult patients with sickle cell disease ?

A

Acute chest syndrome

52
Q

Where does RBCs of sickle cell trait sickle ?

A

Renal medulla
Extreme hypoxia and hypertonicity of the medulla causes sickling resulting in microinfartions leading to microscopic hematuria and eventually decreased ability to concentrate urine

53
Q

Electrophoresis of sickle cell disease and trait ?

A 
Disease : 
90% HbS 
8% HbF
2% HbA2
No HbA

Trait :
55% HbA
43% HbS
2% HbA2

54
Q

Defect of hemoglobin C ?

A

Autosomal recessive mutation of Beta chain of hemoglobin in which glutamic acid is replaced with Lysine

55
Q

Blood smear findings of hemoglobin C disease ?

A

HbC crystals are seen in RBCs

56
Q

Defect of paroxysmal nocturnal hemoglobinuria ?

A

Acquired defect in myeloid stem cells resulting in absent glycosylphosphatidylinositol (GPI) renders cells susceptible to destruction by complement

57
Q

Function of GPI ?

A

Secure the Decay accelerating factor (DAF) to the cell membrane of RBCs

58
Q

Function of DAF ?

A

Protects the RBCs against complement mediated damage by inhibiting C3 convertase

59
Q

Why intravascular hemolysis occurs during sleep in PNH ?

A

As mild respiratory acidosis develops with shallow breathing during sleep and activates complement

60
Q

Screening test and confirmatory tests for PNH ?

A

Screen : sucrose test

Confirmation : acidified serum test and flow cytometry ( detects lack of CD55 (DAF) on blood cells

61
Q

Complications of PNH ?

A

Iron deficiency anemia

AML

62
Q

Main cause of death in PNH ?

A

Thrombosis of hepatic , portal , cerebral veins , because destroyed platelet release cytoplasmic contents into circulation inducing thrombosis

63
Q

G6PD variants ?

A

African variant : mildly reduced half life of G6PD

Mediterranean variant : markedly reduced half life of G6PD

64
Q

Drugs that cause oxidative stress in G6PD deficiency ?

A

Primaquine
Sulfa drugs
Dapsone

65
Q

Screen and confirmatory test for G6PD deficiency ?

A

Screen : Heinz preparation

Confirmatory : enzyme studies ( performed weeks after hemolytic episodes resolves )

66
Q

Warm agglutinin mechanism ? Causes ? Ttt ?

A

Mechanism : IgG mediated disease usually involves extravascular hemolysis , IgG binds RBCs in relatively warm temperature of the central body , membrane of the antibody coated RBCs is consumed by splenic macrophages resulting in spherocytes

Causes : SLE , CLL , drugs as penicillin and cephalosporins

Ttt : stop the drug , sterods , IVIG , splenectomy

67
Q

Cold agglutinin mechanism ? Causes ?

A

Mechanism : IgM mediated disease usually involves intravascular hemolysis , IgM binds RBCs and fix complement in relatively cold temperature of the extremities

Causes : Mycoplasma pneumoniae , infectious mononucleosis

68
Q

Causes of microangiopathic hemolytic anemia ?

A 
TTP
HUC
DIC 
HELLP ( Hemolysis Elevated Liver enzymes and Low Platelets ) 
Prosthetic heart valves 
Aortic stenosis
69
Q

How often fever episodes occurs in malaria ?

A

P falciparum : daily fever

P vivax and P ovale : fever every other day

70
Q

Mechanism of Parvovirus B19 anemia ?

A

Infects red cells progenitor cells and temporarily halts erythropoiesis , leading to significant anemia in the setting of preexisting marrow stress

71
Q

Marrow stimulating factors ?

A

Erythropoietin
GM-CSF
G-CSF

72
Q

Why immunosuppression may be helpful in aplastic anemia ?

A

As idiopathic cases are due to abnormal T-cell activation with release of cytokines

73
Q

Myelophthisic process ?

A

Pathologic process that replaces bone marrow resulting in impairment of hematopoiesis and pancytopenia

Pathology (18 decks)

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