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Board exam 2015 > Pathology-rbc And Bleeding Disorders > Flashcards

Pathology-rbc And Bleeding Disorders Flashcards

1
Q

Red cell indeces:

Average content/mass of hemoglobin per red cell

A

Mean cell hemoglobin

Mean cell volume- ave volume of rbc
Mean cell hemoglobin conc- average hgb in given volume of rbc
Red cell variation of red cell volume- coeeficient variation of rbc volume

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2
Q

average hgb in given volume of rbc

A

Mean cell hemoglobin conc

Mean cell hemoglobin- ave volume if red cell expressed in femto litters
Mean cell volume- ave volume of rbc
Red cell variation of red cell volume- coeeficient variation of rbc volume

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3
Q

Site of hemolysis with:
Anemia
Jaundice
Splenomegally

A

Extravascular. Main feature is splenomegally bec if sequestration

Intravascular main feature is increased direct bilirubin manifested as jaundice. Other presentations: hemoglobinemia, hemoglubinuria amd hemosiderinuria

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4
Q

What is the expected peripheral smear in hemolytic anemias

A

Normocytic normochromic

Primary myelofibrosis also presents with normocytic normochro,ic anemia. It’ fibrotic obliteration of bone marrow spaces

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5
Q

Types of hemolytic anemia

A 
Hereditary spherocytosis
G6pd deficiency
Sickle cell
Thalasemia
Autoimmune ha
Microangiopatjic ha
Macroangiopathic ha
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6
Q

Autosomal dominant
Ankyrin and spectrin mutation causing structural changes of rbc
Rbc lacks central pallor
Splenomegally

A

Hereditary spherocytosis

Spherocytes- rbc lacks pallor

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7
Q

Aplastic crisis is associated with what virus?

A

Parvo virus

Hemolytic crisis is assoc with EBV mononucleosis

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8
Q

Small dark nuclear remnants in rbc in ASPLENIC patients

A

Howell-jolly bodies seen in heredetary spherocytosis

Other assoc sx: aplastic crisis (parvo virusb19) and hemolytic crisis (EBV)

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9
Q

X linked recessive
EPISODIC hemolysis triggered usually by infection
Other triggers (antimalarials, sulfonamides. Fava beans)
Protective against what disease?

A

Malaria

Dx at point: g6pd deficinecy
Reduces nadph to nadph which acts as reducing agents for oxidized rbcs

Recovery phase for hemolysis is heralded by reticulocytosis

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10
Q

Membrane bound precipitates on denatured globin chains. These can be phagocytized by macrophages leaving a distinct bite in rbc (bite cells)

A

Heinz bodies seen in g6pd deficiency

Bite cells not appreciated in asplenic patients

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11
Q

Point mutation in 6th codon affecting the BETA GLOBIN
Replacement of glutamate with valine
Autosomal recessive
Autosplenectomy

A

Sickle cell anemia

target cells, reticulocytosis, sickle cell, howell jolly bodies (seen also in spherocytosis)

Target cells or codocytes are dehydrated rbc with bulls eye appearance

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12
Q

Why is sickle cell in infants asymptomatic until 2-4months of age?

A

Due to high amount of fetal hbg (2alpha, 2gamma)

Adult hbg is 2alpha:2beta

Sickle cell affects only beta globins

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13
Q

Sickle cell crisis (three tyoes)

A

Vasoocclusive: dactylitis
Sequestration: splenic enlargement
Aplastic crisis: parvo virus b19 (seen also in spherocytosis)

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14
Q

Aplastic crisis caused by parvovirus b19 is seen in which condition

A

Heredetary spherocytosis

Sicle cell anemia

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15
Q

What is the drug of choice fo sickle cell anemia? And moa?

A

Hydroxyurea. It increases fetal hemoglobin

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16
Q

Tyoes of alpha thalasemia

A

Silent carrier state: deletion of ONE alpha globin chain
Alpha thalasemia trait: TWO globin chains
Hemoglobin h bonding disease- THREe
hydrops fetalis-all four alpha globin

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17
Q

Tetramers of gamma-globin chains sen in hydrops fetalis thalasemia

A

Hb barts

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18
Q

Types of beta thalasemia

A

Cooley’s anemia or Beta thalasemia major
BT untermediate
BT minor

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19
Q

Dehydrated rbc with bulls eye appearance is seen in what conditions

A

Thalasemia
Sickle cell anemia

These are called codocytes ir target cells

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20
Q 
Codocytes
basophilic stripling
Anisocytosis
Poikilocytosis
Microcytic, hypochromic anemia
Chipmunk facies
Crew cut appearance if skull
A

Thalasemia

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21
Q

Small peripheral dots in rbc with microcytic hypochromic anemia is seen these conditions:

A

TAIL: thalasemia, anemia of chronic cause, ida, lead poisoning

The dots are called basophilic stippling

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22
Q

Treatment of hemochromatosis? If unresponsive, what pharmacologic agent, what moa?

A

Phlebotomy

Deferoxamine via chelation

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23
Q

Increcreased compliment mediated rbc lysis c5b-ca
Decreased cd59 expression
!Hemoglobinuria upon waking up
Thrombosis (mcc of fatality)

A

Paroxysmal nocturnal hemoglobinuria

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24
Q

Warm agglutinin is mediated by what immuno globulins?

A

IgG in warm aglutinin autohemolyric anemia
Also seen in methyldopa, sle and cll hemolysis

Another kind is cold mediated autohemolytic anemia, igM mediated seen in Mycoplasma and Mononucleosis
“Cold ice cream is mmm”

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25
Q

Coobs tests that checks patient’s serum for anti rbc surface iG

A

Indirect coombs test

Direct coombs test checks if patients rbs is coated with igG

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26
Q

Damage if rbc thriugh narrowed or obstructed lumina

Usually with cardiac valve prostheiss

A

Microangiopathic hemolytic anemia

27
Q

Schistocytes are fragmented rbc, called helmet cells if cut in half
Burr cells or echinocytes are rbcs with spikes

What condition?

A

Rbc trauma

28
Q

Hepatosplenomegally is seen in which conditions?

A

These are the extravascular site of hemolysis:
Heredetary sphenocytosis
Sickle cell anemia
Thalasemia

G6pd is an exemption bec site depends on presence of assaulting factor

29
Q

Conditions with hemolytic anemia caused by external factors ex drugs, temp

A

Autoimmune hemolytic anemia
Microangiopathic hemolysis
Macroangiopathic hemolysis

Intrinsic hemolysis- Conditions with hemolytic anemia caused by inherent structural defect

30
Q

Anemias from diminished erythropoeisis

A 
Megaloblastic anemia
Pernicious anemia
Iron defeciency anemia
Anemia of chronic disease
Aplastic anemia
Pure cell aplasia
31
Q

Macroovacytes (big oval rbcs)
Hypersegmented neutrophils
Has neurologic symptom

Causes are:
Impairment if dna synthesis leading to ineeficient vit b12 deficiency
Deficiency of nutrients

A

Megaloblastic anemia sec to vit b12 deficiency

Folate acid defficiency has no neurologic symptoms

32
Q

What parassite is assoc with vit b12 deficiency? Treatment.

A

Diphyllobotrium latum from raw fish (flatworm)

Treat with praziquantel

“PRESSiquantel- flats the worm”

33
Q 
Megaloblastic Anemia
Atrophy of the fundic glands of stomach
Intestinalization (with goblet cell formation)
Atrophic glossitis (glazed beefy tongue)
Autoimmune attacking the parietal cells
A

Pernicious anemia

Parietal cells produces intrinsic factor for vit b12 absorption
Chief cell- zymigen cells- producing pepsiongen

34
Q

Test for differentiating megaloblastic anemia with vit b12 deficiency?

A

Schiling test

Stage 1: oral or im vit b12
Stage 2: intrinsic factor

Normal 1 normal 2- deficiency
Low 1 normal 2- pernicious anemia
Low 1 and 2 malabsorption

35
Q 
Most common nutritional disorder in the world?
Microcytic hypochromic anemia
Basophilic stripping
Spoon nails (koilonychia)
Pica
A

Iron defficiency syndrome

Other basophilic stripping and microcytic and hypochromic conditions: “TAIL”

36
Q

What parasitic infection causes ida? Treatment

.

A

Hookworms (necator, ancylostoma) -blood suckers

Albendazole

37
Q

Plummer vinson syndrome. What maliganancy should be ruled out?

A

Ida
Esophageal webs
Atrophic glossitis

Inc risk of developing colorectal scca in the elderly
Ida in the elderly should be checked for colorectal ca.

38
Q

Most common cause if anemia among hospitalized patients

Increase in il-6 –>hepcidin

A

Anemia of chronic disease

39
Q

Inhibits ferriporin function of macrophage reducing transfer of iron frim storage to bone for erythropoeisis

A

Hepcidin

Stimulated by il-6 in anemia of chronic disease

40
Q

PANCYTOPENIA
Hypocellular bone marrow with fat cells
Absent splenomegaly
Reticulocytopenia

A

Aplastic anemia

Vs. myelodysplastic syndrome–> the bine marrow in mds is hypercellular

41
Q

Drug known to cause aplastic anemia? Chemical to cause aplastic anemia?

A

Chloramphenicol

Benzene

42
Q

Selective hypoplasm of erythroid elements?
Thymoma with leukemia (paraneoplastic syndrome)

Differentiate with aplAstic anemia

A

Pure red cell aplasia

Anaplastic anemia is PANCYTOPENIA

43
Q

Causes of Bleeding disorders

A

Increased vessel fragility
Platelet deficiency
Platelet dysfunction
Derangement of coagulation

44
Q

NORMAL bleeding parameters
Non thrombocytic purpura
Seen in scurvy

What caused the bleeding disorder?

A

Increased fragility of vessels

45
Q

Spontaneous bleeding happen in what platelet level?

A

20,000/ml

Platelet transfusioon threshold

46
Q

Thrombocytopenia is defined as?

A
47
Q

Antiplatelet antibodies
Megathrombocytes
Spleen congestion

What treatment?

A

Case point: immune thrombocytopenic purpura

Most feared complication: ich
Tx with glucocorticoids, splenectomy, immunoglobulin or
rituximab (anti cd20 antibody)- if there is relapse s/p splenectomy

48
Q

Thrombotic Thrombocytopenic purpura pentad vs hemolytic uremic syndrome triad?

A

Fever and neurologic deficits in TTP

Common sdx: thrombocytopenia, microangiopathic hemolytic anemia, renal failure

Ttp caused by deficiency of ADAMTS13 vWf metalloprotease
HUS by shiga like toxin EHEC 157:7 from burger

49
Q

Increased bleeding time
Decreased GP1b leading to defective adhesion
Decreased platelet count

A

Bernard soulier syndrome of platelet dysfunction

50
Q

Increased bleeding time
Decreased GpIIb-IIIa
Defective platelet to platelet aggregation
NORMAL platelet count

A

Glanzmann’s thrombasthenia

51
Q

Three Types of coagulation disorders

A

Von willebramd disease
Hemophilia
Disseminated intravascular coagulation

52
Q

Most common inherited bleeding disorder
Differentiated from bernard soulier sundrome for having normal platelet count

What is the treatment?

A

Desmopressin and comc factor 8 and vWf

Dx at point: von willebrand factor disease. VWf is associated with factor 8. Without it f8 decreases

53
Q

Most common heredetary disease associated with life-threatening bleeding
What is the treatment?

A

Factor 8 and cryoprecipitate

Case: hemophilia A
“A, eight”

54
Q

Also known as christmas factor. Deficient in what coagulation disorder?

A

Hemophilia B, factor 9

“9 benign (less life threatening bleeding)

55
Q 
Consumptive coagulopathy
Thrombohemorrhagic disorder (coexistent state)
A

Dissiminated intravascular dissemination

56
Q

Common factors with ultimate goal to activate f2

A

10, 2, 5 fibrin

57
Q

Extrinsic factors

A

7

With common factors 10 2 5 fibrin

58
Q

Prothrombine time tests for

A

Extrinsic + common factor

Bleeding time- speed platelet plug is formed.
Partial thromboplastic time- intrinsic+common factor

59
Q

Causes of DIC

STOP Making New Thrombi

A 
Sepsis (gram neg)
Trauma
Obstetric complications (abruptio placenta)
Pancreatitis
Malignancy
Nephrotic syndrome
Transfusion
60
Q

Important in diagnosing dic?

A

D dimer most specific

Other ddx: 
Fibrinogen
Platelet count
Pt/ptt
Fibrin degredAtion products
61
Q

DIC
Fibrin thrombi leading to massive adrenal hemorrhage.
Renal Complication of meningococcemia and N. meningitidis

A

Waterhouse-friderichsen syndrome

62
Q

Dic special situation

A

Sheehan syndrome: post partum pituitary necrosis
Waterhouse friderichsen sdx
Toxemiaof pregnancy

63
Q

Overall most common cause of anemia?

A

Bleeding

Ida is most common anemia of defeciency

Board exam 2015 (2 decks)

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