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Hematology Flashcards

1
Q

most common inherited bleeding disorder

A

Von Willebrand Disease

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2
Q 
What is the most likely diagnosis:
Epistaxis
Ecchymosis
Menorrhagia
Normal platelet count and PT/INR
History of bleeding after aspirin or NSAID use
A

Von Willebrand Disease

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3
Q

What is the best initial test

-Von Willebrand disease

A

Evaluate bleeding
-CBC, PT, PTT

Investigate Von Willebrand

  1. Von Willebrand factor ristocetin cofactor activity (vWF:RCo)
  2. Von Willebrand factor antigen (vWF:Ag)
  3. Factor VIII activity
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4
Q

What is the treatment of choice

-Von Willebrand Disease

A

Desmopressin if symptomatic

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5
Q

What is 2nd line tx

-Von Willebrand Disease

A

Factor VIII

Von Willebrand factor

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6
Q

Hemophilia most commonly affects (M or F)

and it is __-linked __

A

Males

x-linked recessive

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7
Q

What is the most likely diagnosis:
Male child
Joint bleeding
May give history of prolonged bleeding with circumcision, dental repair, or surgery
PT, platelets, and platelet function is typically normal
PTT is prolonged

A

Hemophilia

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8
Q

What is the most accurate test

Hemophilia

A

Specific assay for the disease (factor VIII, IX, or XI)

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9
Q

What is the treatment of choice
Hemophilia
Mild bleeding;
Moderate/severe bleeding

A

Mild bleeding: desmopressin

Moderate/severe bleeding: give specific factor missing

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10
Q

Secondary ITP most commonly from:

A
  1. Systemic disease: adult
    • ITP most commonly chronic in adults
  2. Viral: Children
    - ITP most commonly resolves in children
  3. Common medications to induce ITP: quinidine, gold, penicillin, procainamide, TMP-SMX
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11
Q

Common medications to induce ITP:

A 
quinidine
gold
 penicillin
 procainamide
 TMP-SMX
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12
Q 
What is the most likely diagnosis:
Young woman
Isolated low  platelets 
Normal sized spleen
Giant platelets
A

immune thrombocytopenic purpura

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13
Q

Next best step

Immune Thrombocytopenic Purpura

A

CBC

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14
Q

Best initial step

Immune Thrombocytopenic Purpura

A

CBC

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15
Q

What is the most accurate test

Immune Thrombocytopenic Purpura

A

Bone marrow biopsy

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16
Q

What is tx of choice

Immune thrombocytopenic purpura

A
  1. Platelets >30,000: observation
  2. Platelets <20,000: steroids (dexamethasone/prednisone)
  3. Platelets <10,000: Add IVIG for bump in platelets
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17
Q

What is 2nd line tx

immune thrombocytopenic purpura

A

1.IVIG if steroids fail or contraindicated
Also used if need rapid increase in platelets (works in a few days)
2.Splenectomy
3.Rituximab
4.Immune modulators (azathioprine or cyclosporine) or thrombopoietin receptor agonists.
5. Severe bleeding is treated with platelet transfusion, IVIG, and steroids

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18
Q

Most common cause of drug induced

Thrombotic Thrombocytopenic purpura

A

quinine

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19
Q

Thrombotic Thrombocytopenic purpura

is most common in which patients

A

women
young adult
black

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20
Q

What is the most likely diagnosis:
Hemolysis
Fragmented RBCs (schistocytes/helmet cells)
Renal dysfunction (increased creatinine/proteinuria)
Neuro and GI symptoms may be thrown in as well
Neurological dysfunction
low platelets

A

Thrombotic Thrombocytopenic Purpura

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21
Q

Most accurate test

Thrombotic Thrombocytopenic purpura

A

Reduced plasma ADAMTS13 activity

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22
Q

Treatment of choice

Thrombotic Thrombocytopenic purpura

A
  1. plasma exchange
  2. Steroids
  3. Rituximab
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23
Q

Iron Deficiency anemia MC secondary to

A

blood loss

-GI or menses

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24
Q 
What is the most likely diagnosis
Will give blood loss in vignette
Heavy menses or GI bleed
Decreased MCV
Decreased iron/ferritin
Increased TIBC/RDW
A

Iron deficiency anemia

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25
Q

Next best step

Fe deficiency anemia

A

look for source of bleeding

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26
Q

Best initial test

Fe deficiency anemia

A

CBC

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27
Q

Most accurate test

Fe deficiency anemia

A

Bone marrow biopsy

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28
Q

tx of choice

Fe deficiency anemia

A

ferrous sulfate

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29
Q 
What is the most likely diagnosis
Will give chronic disease in vignette
Decreased or Normal MCV
Decreased iron
Normal ferritin
Decreased TIBC
A

Anemia of chronic disease

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30
Q

Best initial test

anemia of chronic disease

A

CBC

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31
Q

What is the most accurate test

anemia of chronic disease

A

bone marrow biopsy

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32
Q

what is the treatment of choice

anemia of chronic disease

A
  1. address underlying issue

2. erythropoietin in ESRD

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33
Q

Alpha thalassemia is common in ____ populations

A

Asian

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34
Q

Beta thalassemia is common in ___ populations

A

African descent

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35
Q

What is the most likely diagnosis

Elevated iron and ferritin
Decreased MCV
Target cells or tear drop cells on smear

A

thalassemia

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36
Q

how does minor thalassemia present

A

asymptomatic

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37
Q

how does Beta major thalassemia present

A
  • symptoms start 4-6mo
  • bone marrow deformity
  • severe anemia
  • pathologic fractures
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38
Q

how does alpha major thalassemia present

A

hydrops fetalis

-causes fetal death

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39
Q

Target cells or tear drops on smear

A

thalassemia

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40
Q

what is best initial test

-thalassemia

A

CBC

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41
Q

What is the most accurate test

-thalassemia

A

electrophoresis

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42
Q

what is the treatment of choice
thalassemia
-trait
-major

A

trait: observation
major: transfusion & Fe chelation w/ deferoxamine

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43
Q

what is second line tx for thalassemia major

A

splenectomy

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44
Q

What is the most likely diagnosis
Hypersegmented neutrophils
Increased MCV
Elevated homocysteine

A

folate/b12 deficiency

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45
Q

hypersegmented neutrophils

& elevated homocysteine

A

folate/b12 deficiency

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46
Q

peripheral neuropathy and neurologic symptoms associated with which anemia

A

B12

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47
Q

increased methylmalonic acid (MMA)

A

B12 deficiency/ anemia

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48
Q

medications that can cause B12 deficiency

A
  1. metformin
  2. PPI
  3. cimetidine
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49
Q

Medications that can cause folate deficiency

A
  1. methotrexate
  2. trimethoprim
  3. phenytoin
  4. Etoh
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50
Q

Best initial test for B12 anemia

A
  1. get B12 level

2. Get MME (methylmalonic acid)

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51
Q

treatment of choice

B12 deficiency

A
  1. B12 replacement PO or IM

- if severe symptoms of preg IM preferred

52
Q

treatment of choice

folate deficiency

A

folate replacement

53
Q

polycythemia vera

presentation

A
  • itching after hot showers
  • elevated hematocrit
  • Jak2 mutation
  • typically asymptomatic
  • hx of HTN or thrombotic event
54
Q

Best initial test

-polycythemia vera

55
Q

Most accurate test

-polycythemia vera

A

Jak2 mutation

56
Q

tx of choice

polycythemia vera

A

phlebotomy

ASA

57
Q

2nd line tx

polycythemia vera

A

high risk: hydroxyurea

-over 60 w/ thrombosis

58
Q

which gender is more commonly affected in thrombocytosis

59
Q

thrombocytosis

MC secondary causes

A
  1. chronic inflammation
  2. infection
  3. malignancy
60
Q

erythromelalgia: burning of hands &feet

A

thrombocytosis

61
Q

What is the most likely diagnosis
Look for symptoms of clotting and bleeding
Erythromelalgia: burning of hands and feet
Recurrent 1st trimester fetal loss in women
Elevated platelet count (can be up to 1,000,000 or more)

A

thrombocytosis

62
Q

Best initial test

thrombocytosis

63
Q

Most accurate test

thrombocytosis

A

Bone Marrow Biopsy

64
Q

Tx of choice

-thrombocytosis

A

hydroxyurea

65
Q

Hemochromatosis

-MC affects which population

A

50-60

male

66
Q 
What is the most likely diagnosis
Male in 50s/60s
Elevated LFTs
Bronzing skin (hyperpigmentation)
Diabetes
Cirrhosis
A

Hemochromatosis

67
Q

hemochromatosis

-presentation

A
  • 50-60yo
  • elevated LFT
  • Bronzing sign (hyperpigmentation)
  • DM
  • Cirrhosis
68
Q

Best initial test

-hemochromatosis

A

-ferritin & iron

69
Q

most accurate test

-hemochromatosis

A

-liver biopsy

70
Q

tx of choice

hemochromatosis

A

-phlebotomy

71
Q

2nd line tx

-hemochromatosis

A

-deferoxamine (Fe chelation)

72
Q

Sickle cell

-most commonly affected pop

A

African americans

73
Q 
What is the most likely diagnosis
African American patient
Acute chest, back, and/or extremity pain
Lung infiltrates might be shown
Fever 
Hemolysis on lab work
A

sickle cell

74
Q

helmet cell & schistocytes

A

Thrombotic Thrombopcytopenia purpura

75
Q

next best step

-sickle cell anemia

A

-if pt presents w/ fever, start antibiotics

76
Q

Best initial test

-sickle cell anemia

A

peripheral smear:

  • sickled RBC (NOT seen with trait)
  • Howell-Jolly bodies
77
Q

Howell-Jolly bodies

A

sickle cell anemia

78
Q

Most accurate test

-sickle cell

A

HgB electrophoresis

79
Q

Tx of choice

-sickle cell anemia

A
  1. folic acid supplements
  2. hydroxyurea (prevents acute syndrome)
  3. Acute pain crisis: hydration, O2, analgesia (may require opiods)
  4. IF fever >101 give ceftriaxone
80
Q

Most Common leukemia in adults

81
Q 
What is the most likely diagnosis
Patient over 50 complaining of fatigue
Elevated WBC
Cervical lymphadenopathy
Peripheral smear shows smudge cells and small mature lymphocytes
Anemia and thrombocytopenia
82
Q

smudge cells & immature lymphocytes

83
Q

best initial test for CLL

84
Q

Most accurate test for CLL

A
  1. flow cytometry

2. bone marrow biopsy

85
Q

tx of choice for

CLL

A

symptomatic & active:

  1. fludarabine
  2. cyclophosphamide
  3. rituximab
86
Q 
What is the most likely diagnosis
Patient over 50 complaining of pruritis after hot showers
Fatigue
Weight loss
Abdominal fullness
Splenomegaly
Extremely high WBC >100,000
Philadelphia chromosome
translocation mutation
87
Q

Phildelphia chromosome is what kind of mutation and between which chromosomes

A

translocation

BCR (chromosome 22) and ABL (Chromosome 9)

88
Q

Best initial test for

CML

89
Q

Most accurate test for

CML

A

BCR-ABL1 fusion gene

Also known as philadelphia chromosome

90
Q

Initial tx of choice

CML

A

Tyrosine Kinase inhibitors

  1. imatinib
  2. ibrutinib
91
Q

2nd line tx

CML

A

bone marrow transplant

92
Q

AML is more common in which pts

A
  1. Down Syndrome
  2. Li-Fraumeni
  3. Fanconi
93
Q

Patient over 60 presenting with pancytopenia
-Low hgb: fatigue
-Low wbc: infection
-Low platelets: bleeding
> 20% blasts on peripheral smear
Auer Rods and Phi bodies on bone marrow biopsy

94
Q

Auer Rods & phi bodies

95
Q

Best initial test

AML

A

CBC peripheral smear

96
Q

most accurate test

AML

A

flow cytometry

97
Q

treatment of choice AML

A
  1. chemo
  2. If patient has acute promyelocytic leukemia (APL) subtype, then add all-trans retinoic acid (ATRA) aka Vit A
    Used to be called M3 subtype
98
Q

2nd line tx

AML

A

bone marrow transplant

99
Q

MC pediatric anemia

100
Q 
What is the most likely diagnosis
5 year old patient presenting with a limp
Pancytopenia
Low hgb: fatigue
Low wbc: infection
Low platelets: bleeding
>20% blasts
101
Q

Best initial test ALL

A

CBC peripheral smear

102
Q

most accurate test

ALL

A

flow cytometry

103
Q

tx of choice

ALL

104
Q

2nd line tx

ALL

A

bone marrow transplant

105
Q

is non-hodgkin or hodgkin more common

A

non-hodgkin

106
Q

What is the most likely diagnosis
Older patient presenting with fever, night sweats, and weight loss
Painless lymphadenopathy
Normal CBC

107
Q

reed-sternberg cells

A

hodgkin lymphoma

108
Q

pain with alcohol ingestion

A

hodgkin lymphoma

109
Q

Best test lymphoma

A

excisional biopsy of lymph nodes

110
Q

What is the treatment of choice

Non-Hodgkin Stage 1, 2, 3 and 4

A

R-CHOP (gold standard chemotherapy)
Rituximab, cyclophosphamide, doxorubicin
vincristine
prednisone

111
Q

tx Hodgkin Stage 1 and 2:

Favorable disease:

A

ABVD (gold standard chemotherapy)
doxorubicin, bleomycin, vinblastine, dacarbazine
Unfavorable disease: ABVD + local radiation

112
Q

tx Hodgkin Stage 3 and 4

A

ABVD plus radiation

113
Q

multiple myeloma is MC in which population

A

African Americans

>70

114
Q

What is the most likely diagnosis
70 year old African American patient complaining of bone pain
Pathological fractures
Renal failure
Peripheral smear shows rouleaux formation
Hypercalcemia
Anemia

A

multiple myeloma

115
Q

Peripheral smear shows rouleaux formation

A

multiple myeloma

116
Q

multiple myeloma
What is the best initial test
if sure:
if unsure:

A

If unsure: X-Ray of the area with bone pain to look for lytic lesions
If sure about MM: CT, MRI, or PET as they are better than x-ray

117
Q

Most accurate test

multiple myeloma

A

bone marrow biopsy

118
Q

treatment of choice

multiple myeloma

A

Chemotherapy and immunomodulators

Bortezomib, lenalidomide or cyclophosphamide, and dexamethasone

119
Q

2nd line tx

multiple myeloma

A

Autologous hematopoietic cell transplantation

120
Q

G6PD MC in which pop

A

American black males and some Mediterranean populations.

121
Q

G6PD commonly exacerbated by

A
  1. Medications
    - Aspirin
    - Primaquine
    - Dapsone
    - Quinidine
    - Nitrofurantoin
    - Sulfonamides
  2. Fava bean ingestion
  3. infection
122
Q

G6PD presentation

A

Intermittent episodes of hemolysis

  • Elevated reticulocyte count
  • Elevated Indirect bilirubin
  • Elevated LDH
  • Decreased haptoglobin

Jaundice and darkening of the urine
2dary to increased bilirubin
G6PD levels will be low

Asymptomatic in between episodes

123
Q

Peripheral smear may reveal bite cells and Heinz bodies

124
Q

repeat testing of G6PD in ___ mo to confirm event

125
Q

G6PD initial test

A

peripheral smear

126
Q

most accurate test

G6PD

A

G6PD activity levels

127
Q

treatment of choice

G6PD

A

avoid oxidative stress/triggers

PANCE (5 decks)

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