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PA MS > Smartpance Hematology > Flashcards

Smartpance Hematology Flashcards

1
Q

What is autoimmune hemolytic anemia (AIHA)?

A

Autoimmune hemolytic anemia is an extrinsic
type of hemolytic anemia because the immune
system mistakenly believes our own red blood
cells are foreign, or non-self, structures, so it
secretes antibodies against proteins found on
the RBC membrane.

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2
Q

Based on the type of antibodies produced, autoimmune hemolytic anemia
can be divided into what
two classifications?

A

Based on the type of antibodies produced,
autoimmune hemolytic anemia can be divided
further into IgG, also called warm antibody,
hemolytic anemia, and IgM hemolytic anemia,
also called cold agglutinin disease.

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3
Q

Warm Autoimmune Hemolytic Anemia (WAIHA)?

A

Warm is the more common type and is almost
always due to IgG antibodies. It’s when hemolysis occurs at temperatures greater than
or equal to core human body temperature of
37º celsius. The main red blood cell antigen
that reacts with these IgG antibodies is the Rh
antigen.

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4
Q

Cold Autoimmune Hemolytic Anemia (CAIHA)?

A

Cold autoimmune hemolytic anemia is much
rarer, and it occurs when people’s blood is
exposed to cold temperatures, usually in the
range of 0º to 10º celsius - like when a person
goes out into cold weather during winter. With
cold autoimmune hemolytic anemia, the culprits are IgM-type antibodies, and the primary
red blood cell antigens are termed L, I, and P.

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5
Q

Is autoimmune hemolytic
anemia microcytic, normocytic, or macrocytic?

A

Since these red blood cells are normal in
size, approximately 80-100 fL, autoimmune
hemolytic anemia is considered normocytic.

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6
Q

How are the LDH levels
in someone with hemolytic
anemia?

A

With intravascular hemolysis, when RBCs are
broken down, an intracellular enzyme called
lactate dehydrogenase, or LDH, spills out directly into the plasma, so LDH plasma levels
increase

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7
Q

How are the Bilirubin levels
in someone with hemolytic
anemia?

A

Hemoglobin spills out of the RBC and some
of this hemoglobin breaks up into heme and
globin.
Heme is converted into unconjugated, or indirect, bilirubin which is then taken up by the
liver cells and eventually secreted out with bile.
Some of the bilirubin is converted to urobilin
which is what gives urine that yellow color, but
if there’s too much of it, the urine becomes a
much darker color, like black tea.

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8
Q

Causes of warm autoimmune hemolytic anemia?

A

In children, viral infections are a common precipitant. Other causes include systemic lupus
erythematosus, lymphomas, and leukemia, to
name a few, as well as some drugs such
as beta-lactam antibiotics like penicillin and
cephalosporins.

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9
Q

Causes of cold autoimmune hemolytic anemia?

A

Cold autoimmune hemolytic anemia, on the
other hand, has different causes depending
on presentation. With chronic disease, causes
also include leukemia and lymphomas, while
acute forms are more often caused by infections like viral pneumonia, mycoplasma, and
infectious mononucleosis.

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10
Q

Autoimmune hemolytic
anemia usually progresses
slowly, over days to weeks,
but sometimes hemolysis
occurs at a rapid rate, over
the course of hours, which is called ?

A

Hemolytic crisis

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11
Q

Treatment of warm autoimmune hemolytic anemia and cold autoimmune
hemolytic anemia?

A

corticosteroids for warm
autoimmune hemolytic anemia and plasmapheresis for cold autoimmune hemolytic anemia

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12
Q

A 37-year-old female presents with pale mucous
membranes, epistaxis, and
scattered petechiae on her
arms, legs, and torso that
do not blanch to palpation/pressure. Laboratory
findings note a hemoglobin, hematocrit, and white
count in the normal range.
Platelet count is <30,000.
What is the likely diagnosis?

A

Idiopathic thrombocytopenic purpura

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13
Q

What is idiopathic
thrombocytopenic purpura
(ITP)?

A

Autoantibodies are directed against platelet
surface antigens, leading to premature platelet
destruction.

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14
Q

What are the signs and
symptoms of idiopathic
thrombocytopenic purpura
(ITP)?

A

Symptoms of thrombocytopenia which include
may include easy bruising, mucosal bleeding,
purpura, and petechial rashes. Some patients
complain of a viral syndrome several weeks
before the onset.

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15
Q

What are purpura?

A

A rash of purple spots due to small blood
vessels leaking blood into the skin, joints, intestines, or organs.

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16
Q

Petechiae vs purpura?

A

The main difference in appearance between
petechiae and purpura is their size:
*Petechiae are very small, less than 4 millimeters (mm) in size.
*Purpura are larger areas of bleeding under the
skin, typically between 4 mm and 10 mm.
*Areas that are larger than 10 mm are referred
to as ecchymosis.

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17
Q

How is the diagnosis of idiopathic thrombocytopenic
purpura (ITP) made?

A

ITP is a diagnosis of exclusion, so no specific
test confirms the diagnosis. The CBC usually
shows isolated thrombocytopenia, with a normal hematocrit and leukocyte count, normal
kidney function, normal RBC morphology on
peripheral smear, and normal PT/aPTT

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18
Q

What would a bone marrow
aspirate or biopsy show in
an ITP patient?

A

Normal to increased megakaryocytes

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19
Q

What diseases are associated with ITP?

A

Chronic lymphocytic leukemia, Hodgkin disease, non-Hodgkin lymphoma, SLE, rheumatoid arthritis, and HIV infection

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20
Q

Should all patients with ITP
receive treatment?

A

No, only if there is significant bleeding or a
platelet count <30 × 109/L. Occasionally, the
disease spontaneously regresses, especially
in childhood.

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21
Q

What clinical intervention
would be the best option for a patient who is
diagnosed with ITP whose platelet count averages 55,000 over 3 to 4
months?

A

Observation in patients with no active or regular signs of bleeding and counts that remain above 50,000, observation is the best
approach.

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22
Q

Do platelet transfusions increase the platelet count in
ITP?

A

No, but if severe uncontrollable bleeding occurs, then platelet transfusion is indicated.

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23
Q

What is the mainstay of initial treatment of severe ITP
in adults without bleeding?

A

Corticosteroids

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24
Q

What are other treatment
options for ITP if corticosteroids fail?

A

IV Ig, anti-Rho(D) Ig (in Rh-positive patients only), thrombopoietin receptor agonists, and
rituximab

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25
Q

Cure of ITP?

A

Splenectomy

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26
Q

What is pernicious anemia?

A

Pernicious anemia is a decrease in red blood
cells that occurs when the intestines cannot
properly absorb vitamin B12.

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27
Q

What is the pathophysiology of pernicious anemia?

A

Pernicious anemia is synonymous with intrinsic factor deficiency, usually induced by antibodies against intrinsic factor or parietal cells,
leading to decreased absorption of B12. Intrinsic factor binds vitamin B12 in the stomach and
facilitates the absorption of vitamin B12 in the
ileum.

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28
Q

What physical findings
suggest the diagnosis of
vitamin B12 deficiency?

A

Vitamin B12 deficiency can cause physical, neurological and psychological symptoms.
Peripheral neuropathy, pallor, jaundice, and
splenomegaly.

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29
Q

What diagnostic tests are
used to aid in the diagnosis
of pernicious anemia?

A

Serum vitamin B12 level (low) and intrinsic
factor antibody—positive in 60% of patients.
Antiparietal cell antibody—found in 90% Ptients with pernicious anemia, but specificity is
low.

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30
Q

What is the second line
test, indicated if anti-Intrinsic factor antibodies are
negative?

A

Serum Gastrin (increased) - Second line test,
indicated if anti-Intrinsic Factor antibodies negative

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31
Q

Will MCV be increased or
decreased in pernicious
anemia?

A

Increased: Pernicious anemia causes a Megaloblastic Macrocytic Anemia

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32
Q

Treatment of pernicious
anemia?

A

Treatment involves vitamin B-12 shots or pills.
More than 50% of patients with B12 deficiency
related symptoms will have incomplete resolution despite treatment

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33
Q

Coagulation disorder etiologies?

A

Coagulation Disorder etiologies:
Hypercoagulable State
Ï Protein C Deficiency
Ï Protein S Deficiency
Ï Antithrombin III deficiency
Ï Factor V Leiden
Bleeding Diathesis
Ï Factor VIII Deficiency (Hemophilia A)
Ï Factor IX Deficiency (Hemophilia B)
Ï Von Willebrand’s Disease
Medication Induced
Ï Warfarin (Coumadin)
Ï Heparin

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34
Q

What are Protein C and Protein S deficiencies?

A

Protein C and Protein S deficiency are autosomal dominant inherited disorders characterized by an inability to inactivate coagulation
factors Va and VIIIa. Because normal levels of protein C and S help to inhibit these clotting factors, those with a deficiency are in a hypercoagulable state

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35
Q

How do patients with Protein C and Protein S deficiency present?

A

Patients display recurrent DVTs, or DVTs that arise at a young age.

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36
Q

What is the most common
presentation of protein C
and protein S deficiency?

A

The most common presentation of protein C
and protein S deficiency is venous thromboembolism.

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37
Q

Protein C and S inactivates
which factors in the coagulation cascade?

A

Va and VIIIa

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38
Q

Protein C & Protein S deficiency can precipitate skin
necrosis following administration of what medication?

A

warfarin

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39
Q

What is the best initial step
for the management of warfarin-induced skin necrosis?

A

Stopping the drug

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40
Q

Protein C and S deficiency
are inherited in what manner?

A

Autosomal dominant.

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41
Q

Where is Protein C synthesized in the body?

A

Protein C is synthesized in the liver.

42
Q

Protein S is a cofactor for
what protein?

A

C

43
Q

What is measured to establish the diagnosis of protein C deficiency?

A

Protein C activity is measured to establish the
diagnosis of protein C deficiency.

44
Q

What is the best screening
tool for protein S deficiency?

A

The best screening tool for protein S deficiency
is measuring free protein S level.

45
Q

In patients with disseminated intravascular coagulation, how are protein C levels affected?

A

In patients with disseminated intravascular coagulation, protein C levels are decreased.

46
Q

For how long should patients with protein C or protein S deficiency with a history of a thrombotic event
receive prophylactic anticoagulation?

A

prophylactic anticoagulation for life.

47
Q

Can patients with protein S
deficiency take oral contraceptives?

A

No contraindicated

48
Q

What is the most common
inherited cause of DVT and
hypercoagulability in Caucasians?

A

Factor V Leiden is the most common inherited
cause of DVT and hypercoagulability in Caucasians.

49
Q

What is Factor V Leiden?

A

Factor V Leiden thrombophilia is an inherited disorder results from a
single point mutation in the factor V gene resulting in a mutant factor V, which is called factor V Leiden. This mutation causes factor V to be resistant to degradation by activated protein C leading to hypercoagulability.

50
Q

What is the most common presentation of factor
V Leiden?

A

The most common presentation of factor V
Leiden is venous thromboembolism.

51
Q

. Patients with what inheritance pattern for factor V Leiden trait have more chance of developing venous thromboembolism?

A

Patients who are homozygous for factor V Leiden

52
Q

Diagnosis of Factor V Leiden?

A

DNA testing and protein C resistance testing.
The gold standard for the diagnosis of factor V
Leiden is genetic testing for the mutation.

53
Q

What will be seen on coagulation studies for factor V
Leiden?

A

Coagulation studies can show a Partial Thromboplastin Time or PTT that is shortened or
normal.

54
Q

Tx of Factor V Leiden?

A

Treatment with heparin is done when a venous
thromboembolism develops, and studies have
shown no role for prophylaxis in Factor V Leiden.

55
Q

When should Factor V Leiden patients with no history of venous thromboembolism receive prolonged
anticoagulation prophylaxis?

A

Never.

56
Q

What is Antiphospholipid
Syndrome?

A

Autoimmune disorder, often associated with
systemic lupus erythematosus (SLE), characterized by thromboses and recurrent spontaneous abortions.

57
Q

What are the 3 autoantibodies seen in antiphospholipid syndrome?

A

Anticardiolipin, anti-beta2-glycoprotein, and
lupus anticoagulant are the 3 autoantibodies

58
Q

Autoantibody seen in antiphospholipid syndrome that yields a falsely-elevated prothrombin time ?

A

Lupus anticoagulant is an autoantibody seen in antiphospholipid syndrome that yields a falsely-elevated prothrombin time.

59
Q

Where does venous thrombosis in antiphospholipid
syndrome most commonly
occur?

A

In the deep
veins of the lower limb.

60
Q

Arterial thrombosis in antiphospholipid syndrome
most commonly occurs
where?

A

Cerebral arteries.

61
Q

Which of the three autoantibodies can cause a false
positive venereal disease
research laboratory/rapid
plasma reagin test?

A

Anticardiolipin antibodies and lupus anticoagulant.

62
Q

How is the platelet count affected in patients with antiphospholipid syndrome?

A

low platelet count.

63
Q

What is the preferred
oral anticoagulant for secondary prophylaxis against
thrombosis in antiphospholipid syndrome?

A

warfarin

64
Q

Should patients with antiphospholipid syndrome
with no history of thrombosis receive routine anticoagulation?

A

No

65
Q

In antiphospholipid syndrome patients who are
on warfarin prophylaxis against thrombosis, what is the INR goal?

A

2 to 3

66
Q

What is the preferred anticoagulant prophylaxis in
pregnant women with antiphospholipid syndrome?

A

Low molecular weight heparin

67
Q

What are the 4 broad categories of bleeding disorders?

A
  1. Disorders arising from platelet dysfunction
  2. Clotting factor deficiency or dysfunction
  3. Vascular dysfunction (e.g., connective tissue
    diseases)
  4. Hyperfibrinolysis
68
Q

What kinds of bleeding occur with clotting factor deficiencies?

A

Hemarthroses, large bruises or hematomas,
delayed bleeding after trauma or surgery.

69
Q

What types of bleeding occur with the loss of platelet
function?

A

At mucocutaneous sites, small bruises, petechiae, and immediate bleeding after trauma
or surgery

70
Q

What are the causes of clotting factor-type bleeding?

A
  1. Hemophilia A (FVIII deficiency)
  2. Hemophilia B (FIX deficiency)
  3. Hemophilia C (FXI deficiency)
    Other factor deficiencies are rarer, but known
71
Q

What abnormalities of connective tissue are associated with abnormal bleeding?

A

Amyloidosis, hereditary hemorrhagic telangiectasia (HHT), ataxia telangiectasia, scurvy,
Ehlers-Danlos syndrome, Cushing syndrome,
and vasculitides

72
Q

What historical factors
point to a bleeding diathesis?

A

Frequent and severe epistaxis requiring packing or transfusion; hemarthroses; menorrhagia; excessive bleeding after tooth extraction,
surgical procedures, or childbirth; easy bruisability; and excessive gingival bleeding

73
Q

What screening tests are
most useful in the initial
evaluation of patients with
suspected bleeding disorders?

A

PT, aPTT, and platelet count

74
Q

Can patients have a significant bleeding diathesis
with a normal bleeding
time, normal platelet count,
and normal PT and aPTT?

A

Yes. The most common bleeding disorder,
vWD, may present in this fashion. Other causes include FXIII deficiency, hyperfibrinolysis,
mild deficiencies of multiple factors, and connective tissue disorders.

75
Q

What is hemophilia A?

A

An X-linked inherited deficiency of FVIII

76
Q

What is hemophilia B?

A

An X-linked inherited deficiency of FIX

77
Q

What is hemophilia C?

A

An autosomal recessive, variably penetrant,
inherited deficiency of FXI

78
Q

How is severe bleeding into
a joint of a patient with hemophilia A stopped?

A

Administration of FVIII concentrate, preferably recombinant, but plasma-derived concentrates may also be used. Other measures include rest, ice, and elevation.

79
Q

Prior to 1990s, which viruses did patients receiving
factor concentrates often
acquire?

A

HIV, hepatitis B, and hepatitis C

80
Q

What is the treatment for
patients with FXII deficiency?

A

None. FXII deficiency does not cause bleeding.

81
Q

What is the treatment for
severe joint bleeding in a
patient with hemophilia B?

A

Factor IX concentrate, preferably recombinant

82
Q

What is the most common inherited bleeding disorder?

A

vWD

83
Q

What is the function of
vWF?

A

vWF mediates the adhesion of platelets to the
vessel wall basement membrane after vascular injury

84
Q

Where is vWF synthesized?

A

Endothelial cells and megakaryocytes

85
Q

Can a patient with mild
vWD have a normal amount
and function of vWF?

A

Yes. vWF behaves as an acute-phase reactant;
thus, the levels may be increased into the normal range in times of stress, pregnancy, and
with estrogen replacement.

86
Q

What is the treatment of
bleeding in patients with
vWD?

A

ddAVP or clotting factor concentrates of
vWF-FVIII

87
Q

What are the two common
clinical manifestations a
patient may bring up in
their history in a patient
with von Willebrand disease?

A

Epistaxis and excessive bleeding secondary to
razor nicks. Patients will also present with easy
bruising, and menorrhagia.

88
Q

Normal or “ MCV, “ TIBC, ‘
Ferritin (high iron stores), “
serum erythropoietin

A

Anemia of chronic disease

89
Q

The only anemia where all
three cell lines are decreased (pancytopenia) “
WBC, “ RBC, “ Platelets - will
have normal MCV and “ Retic count

A

Aplastic anemia

90
Q

Folate, ‘ MCV (macrocytic
anemia) looks like B12 deficienxy but no neurologic symptoms. Common in alcoholics

A

Folate deficiency

91
Q

After infection or medication in an African American
male + Heinz Bodies and
Bite Cells on smear. Flare
triggers: Fava beans, antimalarials, sulfonamides

A

G6PD deficiency

92
Q

History of jaundice, transfusion, new medication
or infection (+) direct
coombs test

A

Autoimmune Hemolytic anemia

93
Q

“ MCV (microcytic), ‘ TIBC,
“ Ferritin (low iron stores) “
MCH (hypochromic). Associated with pica and nail
spooning, target cells

A

Iron deficiency

94
Q

African American, pain,
family history of blood disorder. Hemoglobin electrophoresis: Hemoglobin
S, Blood smear: Sickled
RBCs, howell-Jolly bodies,
target cells

A

Sickle cell anemia

95
Q

Microcytic hypochromic,
elevated iron. Most severe, Mediterranean descent, failure to thrive. Hemoglobin electrophoresis:
Hemoglobin A2 and F.

A

Beta thalassemia major

96
Q

Microcytic hypochromic,
elevated iron. Mild anemia,
often misdiagnosed as iron deficient. Hemoglobin electrophoresis: Hemoglobin
A2

A

Beta thalassemia trait

97
Q

Chinese and southeast
Asians. Hemoglobin electrophoresis: Hemoglobin H
(H disease), Hemoglobin
Bart’s (hydrops fetalis), Hemoglobin A (trait)

A

Alpha thalassemia

98
Q

‘ MCV (macrocytic anemia), Hypersegmented
Neutrophils and normal
folate, decreased vibratory and position sense

A

Vitamin B12 deficiency

99
Q

Less than 10% radiolabeled vitamin B12 in urine.
Normal results when repeated with administration
of intrinsic factor

A

Schilling test* for the diagnosis of *pernicious
anemia (antibody to intrinsic factor)

100
Q

What type of anemia
are cephalosporins, PCN,
NSAIDs, and methyldopa
associated with?

A

Immune hemolytic anemia

PA MS (44 decks)

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