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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
Lab findings of beta thalassemia major ?
Increased HbA2 and HbF | No or little HbA
26
Cause of hypersegmented neutrophils in megaloblastic anemia ?
Impaired division of granulocytic precursors
27
Causes of macrocytic anemia without megaloblastic changes ?
Alcoholism Liver cirrhosis Drugs as 5-Flurouracile
28
Site of absorption of folate ?
Jejunum
29
Role of salivary gland in absorption of Vit B12 ?
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
30
Role of pancreatic enzymes in absorption of vit B12 ?
Detach vit B12 from R-binder
31
Site of absorption of intrinsic factor-Vit B12 complex ?
Ileum
32
Parasite that causes vit B12 deficiency ?
Diphyllobothrium latum ( Fish Tapeworm )
33
Reticulocytes appearance on a blood film ?
Larger cells with bluish cytoplasm due to residual RNA
34
Why reticulocyte count is falsely elevated in anemia ? How to correct it ?
Decrease in total RBCs falsely elevates percentage of reticulocytes Corrected by multiplying reticulocyte count by ( Hct / 45 )
35
Reticulocyte count
1 - 2 %
36
Corrected reticulocyte count >3% indicates what ?
Good marrow response | And suggests peripheral destruction of RBCs ( cause of anemia )
37
Corrected reticulocyte count < 3% indicates what ?
Poor marrow response | And suggests underproduction of RBCs ( cause of anemia )
38
Site of extravascular hemolysis ?
Reticuloendothelial system as : Macrophages of spleen Liver Lymph nodes
39
Clinical and laboratory findings of extravascular hemolysis ?
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
Clinical and laboratory findings of intravascular hemolysis ?
1- hemoglobinemia 2- hemoglobinuria 3- hemosiderinuria 4- decreased serum haptoglobin
41
Defect of hereditary spherocytosis ?
``` Inherited defect of RBC cytoskeleton-membrane tethering proteins Most commonly involves : 1- Ankyrin 2- spectrin 3- band 3 ```
42
Lab findings of hereditary spherocytosis ?
1- ⬆️ RDW 2- ⬆️ MCHC 3- spherocytes with loss of central pallor
43
Hereditary spherocytosis is associated woth increased risk pf what ?
Aplastic crisis with parvovirus B19 infection of erythroid precursors
44
Anemia with increased MCHC ?
Hereditary spherocytsis | Autoimmune anemia
45
Blood smear findings after splenectomy ?
Howell-jolly bodies ( fragments of nuclear material in RBCs )
46
Defect of sickle cell anemia ?
Autosomal recessive mutation of Beta chain of hemoglobin , a single aa change replaces glutamic acid ( hydrophilic ) with valine ( hydrophobic )
47
Mechanism of hydroxyurea in ttt of sickle cell anemia ?
Icreases level of HbS which protects against sickling
48
Cause of Increased risk of sickling ?
Hypoxemia Dehydration Acidosis
49
Complications of vaso-occlusion ?
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
Results of autosplenectomy in sickle cell disease ?
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
Most common cause of death of adult patients with sickle cell disease ?
Acute chest syndrome
52
Where does RBCs of sickle cell trait sickle ?
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
Electrophoresis of sickle cell disease and trait ?
``` Disease : 90% HbS 8% HbF 2% HbA2 No HbA ``` Trait : 55% HbA 43% HbS 2% HbA2
54
Defect of hemoglobin C ?
Autosomal recessive mutation of Beta chain of hemoglobin in which glutamic acid is replaced with Lysine
55
Blood smear findings of hemoglobin C disease ?
HbC crystals are seen in RBCs
56
Defect of paroxysmal nocturnal hemoglobinuria ?
Acquired defect in myeloid stem cells resulting in absent glycosylphosphatidylinositol (GPI) renders cells susceptible to destruction by complement
57
Function of GPI ?
Secure the Decay accelerating factor (DAF) to the cell membrane of RBCs
58
Function of DAF ?
Protects the RBCs against complement mediated damage by inhibiting C3 convertase
59
Why intravascular hemolysis occurs during sleep in PNH ?
As mild respiratory acidosis develops with shallow breathing during sleep and activates complement
60
Screening test and confirmatory tests for PNH ?
Screen : sucrose test | Confirmation : acidified serum test and flow cytometry ( detects lack of CD55 (DAF) on blood cells
61
Complications of PNH ?
Iron deficiency anemia | AML
62
Main cause of death in PNH ?
Thrombosis of hepatic , portal , cerebral veins , because destroyed platelet release cytoplasmic contents into circulation inducing thrombosis
63
G6PD variants ?
African variant : mildly reduced half life of G6PD | Mediterranean variant : markedly reduced half life of G6PD
64
Drugs that cause oxidative stress in G6PD deficiency ?
Primaquine Sulfa drugs Dapsone
65
Screen and confirmatory test for G6PD deficiency ?
Screen : Heinz preparation | Confirmatory : enzyme studies ( performed weeks after hemolytic episodes resolves )
66
Warm agglutinin mechanism ? Causes ? Ttt ?
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
Cold agglutinin mechanism ? Causes ?
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
Causes of microangiopathic hemolytic anemia ?
``` TTP HUC DIC HELLP ( Hemolysis Elevated Liver enzymes and Low Platelets ) Prosthetic heart valves Aortic stenosis ```
69
How often fever episodes occurs in malaria ?
P falciparum : daily fever | P vivax and P ovale : fever every other day
70
Mechanism of Parvovirus B19 anemia ?
Infects red cells progenitor cells and temporarily halts erythropoiesis , leading to significant anemia in the setting of preexisting marrow stress
71
Marrow stimulating factors ?
Erythropoietin GM-CSF G-CSF
72
Why immunosuppression may be helpful in aplastic anemia ?
As idiopathic cases are due to abnormal T-cell activation with release of cytokines
73
Myelophthisic process ?
Pathologic process that replaces bone marrow resulting in impairment of hematopoiesis and pancytopenia

Pathology (18 decks)

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