Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

USMLE Step 3 > Hematology > Flashcards

Hematology Flashcards

You may prefer our related Brainscape-certified flashcards:
USMLE® Step 1 flashcards
1
Q

all forms of anemia lead to

A

fatigue/loss of energy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

pt with severe anemia will have

A

SOB, lightheadedness, or confusion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

diseases with similar presentations to anemia:

A
  • hypoxia
  • CO poisoning
  • methemoglobinemia
  • ischemic heart disease
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

craving for ice or dirt, think

A

anemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

physical exam findings on anemic patient

A
  • pallor
  • flow murmur
  • pale conjunctiva
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

physical exam findings in hemolytic anemia

A
  • jaundice

- scleral icterus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

in severe anemia, what is needed to exclude ischemia?

A

EKG

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

best INITIAL test for anemia

A

CBC with peripheral smear

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

additional initial tests for anemia

A
  • reticulocyte count
  • haptoglobin
  • LDH
  • total and direct bilirubin
  • TSH and free T4
  • B12/folate levels
  • iron studies
  • UA w/ microanalysis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

categorization of anemia is based on

A

MCV (mean corpuscular volume)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

what value may indicate if there’s a problem with the synthesis of Hb?

A

MCHC (mean corpuscular hemoglobin concentration)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

anemia categorized based on MCV is as follows:

A
  • microcytic
  • macrocytic
  • normocytic
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

based on MCHC anemia can be further categorized as follows:

A
  • hypochromic
  • hyperchromic
  • normochromic
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q
  • blood loss
  • THROMBOCYTOSIS

what is the diagnosis?

A

IDA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q
  • RA
  • ESRD
  • any chronic infectious, inflammatory, or connective tissue disease

what is the diagnosis?

A

ACD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q
  • very small MCV w/ few or no symptoms
  • TARGET cells

what is the diagnosis?

A

thalassemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q
  • alcoholic
  • INH
  • lead exposure

what is the diagnosis?

A

sideroblastic anemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

best INITIAL test for IDA, ACD, thalassemia, or sideroblastic anemia

A

iron studies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

results of iron studies in IDA

A
  • low ferritin
  • high TIBC (total iron binding capacity)
  • low iron
  • low iron saturation
  • elevated RDW (red cell distribution width)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

results of iron studies in ACD

A
  • HIGH ferritin (acute phase reactant)
  • low TIBC (total iron binding capacity)
  • low iron
  • normal or low iron saturation
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

results of iron studies in thalassemia

A

NORMAL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

results of iron studies in sideroblastic anemia

A

HIGH IRON

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

MOST ACCURATE test for IDA

A

bone marrow biopsy (don’t do this on CCS exam)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

MOST ACCURATE test for thalassemia

A

Hb electrophoresis

beta: elevated HbA2, and HbF
(alpha: normal)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

MOST ACCURATE test for sideroblastic anemia

A

Prussian blue stain

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

best INITIAL treatment for IDA

A

ferrous sulfate PO

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

best INITIAL treatment for ACD

A

correct underlying cause

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

best INITIAL treatment for thalassemia

A

no treatment for trait

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

best INITIAL treatment for sideroblastic anemia

A

minor: pyridoxine (vitamin B6)
major: remove toxin exposure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

MOST ACCURATE test for ALPHA thalassemia

A

DNA sequencing

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

name the 4 types of microcytic anemia

A
  1. IDA
  2. ACD
  3. thalassemia
  4. sideroblastic anemia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

causes of macrocytic anemia

A
  • vitamin B12 deficiency

- folate deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

which medication blocks B12 absorption?

A

metformin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

B12 deficiency causes:

A
  • hypersegmented neutrophils
  • peripheral neuropathy (can cause any neuro sx)
  • glossitis (smooth tongue)
  • diarrhea
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

folate deficiency causes:

A
  • hypersegmented neutrophils only
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

labs seen in B12 defiicincy

A
  • DECREASED reticulocyte count
  • elevated bilirubin
  • elevated LDH
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

mechanism of elevated indirect bilirubin and LDH in B12 deficiency

A

“ineffective erythropoiesis”

RBCs are destroyed as they leave bone marrow

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

MOST ACCURATE tests for B12 and folate deficiencies

A

B12 and folate levels

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

next step if B12 deficiency is SUSPECTED, but B12 level is NORMAL

A

methylmalonic acid level

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

after finding low B12 level or elevated methylmalonic acid level, next best step to confirm etiology of B12 deficiency

A

antiparietal cell Ab and anti-intrinsic factor Ab

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

treatment for B12 and folate deficiencies

A

replace

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

first thing that improves after B12 replacement therapy

A

reticulocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

last thing that improves after B12 replacement therapy

A

neurological abnormalities

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

name the hemolytic anemias (9)

A
  1. sickle cell anemia
  2. Hb sickle cell disease
  3. autoimmune hemolysis
  4. cold-induced hemolysis (cold agglutinins)
  5. glucose-6-phosphate dehydrogenase (G6PD) deficiency
  6. pyruvate kinase deficiency
  7. hereditary spherocytosis
  8. hemolytic uremic syndrome (HUS), and thrombotic thrombocytopenic purpura (TTP)
  9. paroxysmal nocturnal hemoglobinuria (PNH)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

all forms of hemolytic anemia present with

A

SUDDEN ONSET of weakness and fatigue

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

diagnostic testing for hemolysis shows the following

A
  • elevated indirect bilirubin
  • elevated reticulocyte count
  • elevated LDH
  • decreased haptoglobin
  • spherocytes on smear
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

in hemolysis, why is indirect bilirubin elevated, and haptoglobin decreased?

A
  • limited capacity of liver to glucuronidate indirect to DIRECT bilirubin
  • haptoglobin is a transport for indirect bilirubin which is quickly used up
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

what electrolyte should be monitored after treating vitamin B12 deficiency?

A

K+ (can be low)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q
  • very severe chest, back, and thigh pain
A

sickle cell anemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

best INITIAL treatment for sickle cell anemia

A
  • OXYGEN
  • HYDRATION
  • PAIN MEDICATIONS
  • ABX (ceftriaxone, levofloxacin, or moxifloxacin)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

why is FEVER an EMERGENCY in a pt w/ sickle cell anemia?

A

NO SPLEEN

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q

tests that should be ordered for pt with sickle anemia

A
  • blood cultures
  • UA
  • reticulocyte count
  • CBC
  • CXR
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
53
Q

if fever is present after treatment for sickle cell anemia, then?

A

physical examination

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
54
Q

possible physical exam findings in sickle cell anemia:

  • HEENT
  • CVS
  • respiratory
  • abdomen
  • extremities
  • neurological
A

HEENT- retinal infarction
CV- flow murmur from anemia
respiratory- rales, or consolidation from infection or infarction
abdomen- splenomegaly in children, absence of spleen in adults
extremities- skin ulcers, aseptic necrosis of hip (found on MRI)
neurological- stroke (current or previous)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
55
Q

when is exchange transfusion done in sickle cell anemia?

A
  • eye: visual disturbance from retinal infarction
  • lung: pulmonary infarction leading to pleuritic chest pain and abnormal CXR
  • penis: priapism from infarction of prostatic plexus of veins
  • brian: stroke
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
56
Q

what is the goal of exchange transfusion?

A

decrease Hb S to 30-40%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
57
Q
  • sudden drop in hematocrit in pt with hemoglobinopathy

- low reticulocyte count

A
  • parvovirus B19

- folate deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
58
Q

MOST ACCURATE test for parvovirus B19

A

PCR for parvovirus DNA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
59
Q

treatment for sickle cell crisis d/t parvovirus B19

A

transfusions and IVIG

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
60
Q

sickle cell anemia patients should be discharged on:

A
  1. folate
  2. Pneumococcal vaccine
  3. hydroxyurea, if crises occur > 4x/year
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
61
Q

MOA of hydroxyurea

A

increases % of Hb F

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
62
Q

sickle cell disease manifestations

A
  • visual disturbance (frequent)
  • hematuria
  • isosthenuria (inability to concentrate/dilute urine)
  • UTIs
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
63
Q

sickle cell trait manifestations

A
  • hematuria
  • isosthenuria
  • splenic vein thrombosis can occur with severe hypoxia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
64
Q

look for other autoimmune diseases in history, or medications

A

autoimmune hemolysis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
65
Q

diagnostic tests for autoimmune hemolysis

A
  • elevated LDH
  • elevated indirect bilirubin
  • elevated reticulocyte count
  • decreased haptoglobin
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
66
Q

peripheral smear will show what in autoimmune hemolysis

A

spherocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
67
Q

MOST ACCURATE diagnostic test for autoimmune hemolysis

A

Coombs test

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
68
Q

best INITIAL treatment for autoimmune hemolysis

A

steroids

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
69
Q

treatment if RECURRENT episodes of hemolysis

A

rituximab

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
70
Q

MOST EFFECTIVE treatment if RECURRENT episodes of hemolysis

A

splenectomy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
71
Q

mechanism of spherocytes in autoimmune hemolysis

A

Abs attack RBC membranes which are biconcave turning them into spheres

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
72
Q

in autoimmune hemolysis, steroids and splenectomy only work on?

A

IgG Abs “warm antibodies”

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
73
Q

if severe hemolysis does not respond to steroids or repeated blood transfusions use

A

IVIG

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
74
Q

a response to IVIG predicts a response to

A

splenectomy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
75
Q

cold-induced hemolysis (cold agglutinins); look for what in the history?

A
  • mycoplasma

- Epstein-Barr virus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
76
Q

in cold-induced hemolysis Coombs test will be?

A

NEGATIVE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
77
Q

in cold-induced hemolysis complement test will be?

A

POSITIVE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
78
Q

treatment for in cold-induced hemolysis

A

rituximab

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
79
Q

will steroids, splenectomy, or IVIG work for cold-induced hemolysis?

A

NO

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
80
Q

MOA of rituximab

A

MAB against CD20R on lymphocytes which make Abs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
81
Q

syphilis causes cold agglutinins with?

A

IVIG

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
82
Q
  • sudden onset hemolysis
  • X-linked
  • oxidant stress causes acute hemolysis
  • MC oxidant stress = infection
  • sulfa, primaquine, dapsone, fava beans
A

glucose-6-phosphate dehydrogenase (G6PD) deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
83
Q

best INITIAL test for glucose-6-phosphate dehydrogenase (G6PD) deficiency

A
  • Heinz body test

- bite cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
84
Q

Heinz bodies are

A

collection of oxidized, precipitated Hb embedded in RBC membrane

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
85
Q

bite cells are

A

pieces of RBC membrane removed by spleen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
86
Q

MOST ACCURATE test for glucose-6-phosphate dehydrogenase (G6PD) deficiency

A

G6PD LEVEL, BUT ONLY AFTER 2 MONTHS HAVE PASSED from acute episode (otherwise, may be falsely normal)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
87
Q

treatment for glucose-6-phosphate dehydrogenase (G6PD) deficiency

A

avoid oxidant stress

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
88
Q
  • sudden onset hemolysis

- NOT provoked (unclear cause)

A

pyruvate kinase deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
89
Q
  • recurrent episodes of hemolysis
  • splenomegaly
  • bilirubin gallstones
  • elevated mean corpuscular Hb concentration (MCHC)
A

hereditary spherocytosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
90
Q

MOST ACCURATE test for hereditary spherocytosis

A

osmotic fragility test

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
91
Q

treatment for hereditary spherocytosis

A

splenectomy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
92
Q

mechanism of hereditary spherocytosis

A

genetic loss of ankyrin and spectrin in RBC membrane

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
93
Q

hemolytic uremic syndrome (HUS) diagnosis is based on

IT’S IN THE NAME!!!

A
  • intravascular hemolysis (Autoimmune hemolysis)
  • elevated BUN and creatinine (Renal failure)
  • thrombocytopenia (Thrombocytopenia)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
94
Q

thrombotic thrombocytopenic purpura (TTP) diagnosis is based on

A
  • intravascular hemolysis
  • elevated BUN and creatinine
  • thrombocytopenia

AND

  • fever
  • neurological abnormalities
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
95
Q

never use what in HUS or TTP?

A

platelets

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
96
Q

what is LOW in TTP?

A

ADAMTS-13 level

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
97
Q

treatment for HUS/TTP

A
  • some cases resolve on their own

- severe cases: plasmapheresis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
98
Q

what makes HUS worse?

A

antibiotics

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
99
Q

mechanism of HUS/TTP

A

ADAMTS-13 = metalloproteinase that breaks down VWF to RELEASE platelets from one another

if VWF is NOT dissolved, platelets form abnormally long strands and RBCs break down as they pass through

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
100
Q
  • pancytopenia
  • recurrent episodes of dark urine, especially in morning
  • MCC of death = large vessel venous thrombosis
A

paroxysmal nocturnal hemoglobinuria (PNH)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
101
Q

paroxysmal nocturnal hemoglobinuria (PNH) can transform into

A
  • aplastic anemia

- acute myelogenous leukemia (AML)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
102
Q

MOST ACCURATE test for paroxysmal nocturnal hemoglobinuria (PNH)

A

CD55 and CD59 Abs (aka, decay accelerating factor)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
103
Q

best INITIAL treatment for paroxysmal nocturnal hemoglobinuria (PNH)

A

glucocorticoids

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
104
Q

treatment for transfusion-dependent pts with severe paroxysmal nocturnal hemoglobinuria (PNH)

A

eculizumab

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
105
Q

MOA of eculizumab

A

inhibits C-5 and prevents complement activation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
106
Q

how can HELLP syndrome be distinguished from DIC?

A

HELLP has NORMAL coagulation studies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
107
Q
  • SOB for no clear reason
  • clear lungs on exam
  • normal CXR
A

methemoglobinemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
108
Q

methemoglobinemia is

A

Hb locked in OXIDIZED state that cannot pick up O2

109
Q

possible drugs that can cause methemoglobinemia

A
  • nitroglycerin
  • amyl nitrate
  • nitroprusside
  • dapsone
  • any anesthetic (ends in -CAINE; lidocaine/bupivacaine/tetracaine)
110
Q

in methemoglobinemia, what color is the blood?

A

BROWN

111
Q

treatment for methemoglobinemia

A

methylene blue

112
Q

transfusion reactions: case 1

  • 20 minutes after a pt receives a blood transfusion, the pt becomes SOB
  • there are transient infiltrates on CXR
  • all symptoms resolve spontaneously
A

transfusion-related acute lung injury (TRALI), or “leukoagglutination reaction”

113
Q

transfusion reactions: case 2

  • as soon as a pt receives a transfusion, he becomes HYPOTENSIVE, SOB, TACHYCARDIC
  • LDH, and bilirubin levels are NORMAL
A

IgA deficiency

114
Q

transfusion reactions: case 3

  • during a transfusion, a pt becomes hypotensive and tachycardic
  • has back and chest pain
  • has dark urine
  • LDH, and bilirubin are ELEVATED
  • haptoglobin is LOW
A

ABO incompatibility

115
Q

transfusion reactions: case 4

  • a few days after a transfusion, pt becomes jaundiced
  • hematocrit does NOT rise with transfusion
  • generally without symptoms
A

minor blood group incompatibility

116
Q

transfusion reactions: case 5

  • a few hours after a transfusion, pt becomes FEBRILE with a rise in temperature of about 1 degree
  • no evidence of hemolysis
A

febrile nonhemolytic reaction

117
Q
  • present w/ signs of pancytopenia:
    1. fatigue
    2. bleeding
    3. infections d/t functional immunodeficiency
A

acute leukemia

118
Q

best INITIAL test for acute leukemia

A

peripheral smear showing blasts

119
Q

Auer rods are associated with?

A

acute myeloid leukemia (AML)

120
Q

most important prognostic finding in acute leukemia

A

cytogenetic abnormalities (specific karyotype abnormalities)

121
Q

if pt is a high risk for relapse of acute leukemia after chemotherapy, pt should have what after chemotherapy induces remission?

A

bone marrow transplantation

122
Q

best INITIAL treatment for acute leukemia

A

chemotherapy

  • idarubicin, or daunorubicin

AND

  • cytosine arabinoside
123
Q

what treatment should be added for acute promyelocytic leukemia (M3 AML) to the initial treatment?

A

all trans retinoic acid (ATRA)

124
Q

what treatment should be added for acute lymphocytic leukemia (ALL) to the initial treatment?

A

intrathecal methotrexate

125
Q

M3, acute promyelocytic leukemia is associated with

A

disseminated intravascular coagulation (DIC)

126
Q
  • SOB, confusion, and blurry vision

- white cell count > 100,000

A

leukostasis = sludging of blood vessels of brain, eyes, and lungs

127
Q

treatment for leukostasis

A

leukapheresis

128
Q

what should be added in a pt with leukostasis to lower the white cell count?

A

hydroxyurea

129
Q
  • elderly pt w/ pancytopenia
  • elevated MCV
  • low reticulocyte count
  • macroovalocytes
  • “Pelger-Huet cell” (neutrophil w/ 2 lobes)
  • NORMAL B12 level
  • small # of blasts (not enough to be considered acute leukemia)
A

myelodysplasia

130
Q

mild, slow progressive preleukemia syndrome

A

myelodysplasia

131
Q

myelodysplasia may progress to

A

acute leukemia

132
Q

MCC of death in myelodysplasia

A

infection or bleeding

133
Q

main treatment for myelodysplasia

A

transfusions as needed

134
Q

only thing that increases survival in myelodysplasia

A

azacytidine

135
Q

decreases transfusion dependence in myelodysplasia

A

lenalidomide

136
Q

name the myeloproliferative disorders (6)

A
  1. chronic myelogenous leukemia (CML)
  2. chronic lymphocytic leukemia (CLL)
  3. hairy cell leukemia
  4. myelofibrosis
  5. polycythemia vera
  6. essential thrombocythemia
137
Q
  • elevated white cell count
  • predominantly NEUTROPHILS
  • splenomegaly (common)
A

chronic myelogenous leukemia (CML)

138
Q

of all forms of myeloproliferative disorders, which has the highest risk of transforming into acute leukemia?

A

untreated chronic myelogenous leukemia (CML)

139
Q

mechanism of early satiety in CML and CLL

A

d/t splenomegaly compressing stomach

140
Q

diagnostic test for chronic myelogenous leukemia (CML)

A

elevated neutrophil count with LOW LAP score

141
Q

MOST ACCURATE test for chronic myelogenous leukemia (CML)

A

Philadelphia chromosome by PCR,

or

BCR/ABL by fluorescence in situ hybridization (FISH)

142
Q

best INITIAL treatment for chronic myelogenous leukemia (CML)

A

imatinib

143
Q

curative treatment for chronic myelogenous leukemia (CML)

A

bone marrow transplantation

144
Q
  • pts > 50 y/o w/ elevated white cell count described as “normal appearing lymphocytes”
  • often asymptomatic
A

chronic lymphocytic leukemia (CLL)

145
Q

best INITIAL test for chronic lymphocytic leukemia (CLL)

A

peripheral blood smear shows “SMUDGE” cells

ruptured nuclei of lymphocytes

146
Q

stages for chronic lymphocytic leukemia (CLL)

A 
stage 0 = elevated white cell count alone
stage 1 = enlarged lymph nodes
stage 2 = spleen enlargement
stage 3 = anemia
stage 4 = low platelets
147
Q

mechanism of infection and hemolysis in chronic lymphocytic leukemia (CLL)

A
  • lymphocytes produce abnormal/insufficient immunoglobulins
  • insufficient immunoglobulins = infection
  • abnormal immunoglobulins = thrombocytopenia/hemolyis
148
Q

treatment for chronic lymphocytic leukemia (CLL)

A

based on disease stage

149
Q

treatment for chronic lymphocytic leukemia (CLL): stages 0 and 1

A

no treatment needed

150
Q

treatment for chronic lymphocytic leukemia (CLL): stages 2-4

A

fludarabine, and rituximab and cyclophosphamide

151
Q

which treatment is most likely to extend survival in treatment for chronic lymphocytic leukemia (CLL)?

A

fludarabine

152
Q
  • middle-aged pt (50’s)
  • pancytopenia
  • massive splenomegaly
A

hairy cell leukemia

153
Q

MOST ACCURATE test for hairy cell leukemia

A

smear showing HAIRY cells and immunophenotyping

154
Q

best INITIAL treatment for hairy cell leukemia

A

cladribine (2-CDA)

155
Q
  • pancytopenia
  • splenomegaly
  • NORMAL TRAP level
A

myelofibrosis

156
Q

key feature seen on PBS in myelofibrosis

A

teardrop-shaped cells

157
Q

finding on bone marrow biopsy in myelofibrosis

A

fibrosis and JAK2 mutation

158
Q

best INITIAL treatment for myelofibrosis if bone marrow transplant is NOT possible

A

lenalidomide/thalidomide

159
Q

curative treatment for myelofibrosis

A

bone marrow transplantation

160
Q

inhibits Janus kinase

A

ruxolitinib

161
Q
  • headache
  • blurred vision
  • dizziness
  • fatigue
  • pruritis, after hot bath/shower
  • splenomegaly
A

polycythemia vera

162
Q

key to diagnosis in polycythemia vera

A

MARKEDLY HIGH hematocrit in the ABSENCE of hypoxia with a LOW MCV

(erythropoietin level will be low)

163
Q

diagnostic tests for polycythemia vera

A
  • CBC
  • ABG to r/o hypoxia
  • erythropoietin level (low)
  • JAK2 mutation (97% sensitive)
164
Q

best INITIAL treatment for polycythemia vera

A

phlebotomy

165
Q

used to lower the cell count in polycythemia vera

A

hydroxyurea

166
Q

treatment for thrombocytopenia in polycythemia vera

A

anagrelide

167
Q

other treatment that should be given for polycythemia vera

A

aspirin

168
Q
  • markedly elevated platelet count

- presents w/ headache, visual disturbance, and pain in hands

A

essential thrombocythemia

169
Q

MCC of death in essential thrombocythemia

A

thrombosis and bleeding

170
Q

treatment for essential thrombocythemia

A

hydroxyurea

171
Q

treatment for essential thrombocythemia if pt is thrombosing

A

aspirin

172
Q

what other lab values are elevated in polycythemia vera?

A
  • B12

- LAP (leukocyte alkaline phosphatase) score

173
Q

name the plasma cell disorders (4)

A
  1. multiple myeloma
  2. monoclonal gammopathy of unknown significance (MGUS)
  3. Waldenstrom’s macrogammaglobulinemia
  4. aplastic anemia
174
Q
  • bone pain caused by fracture occurring under normal use (most frequent presentation)
A

multiple myeloma

175
Q

MCC of death from MM

A
  • infection: pts effectively immunodeficient

- renal failure

176
Q

initial tests for MM

A
  • skeletal survey
  • serum protein electrophoresis (SPEP)
  • urine protein electrophoresis (UPEP)
  • peripheral smear
  • elevated Ca++ level
  • B2 microglobulin level
  • elevated BUN/creatinine
177
Q

skeletal survey findings in MM

A

punched out osteolytic lesions

osteoblastic lesions suggest metastatic prostate cancer

178
Q

serum protein electrophoresis (SPEP) findings in MM

A

elevated monoclonal Ab (usually IgG) levels

20% are IgA

179
Q

urine protein electrophoresis (UPEP) findings in MM

A

Bence-Jones protein

180
Q

peripheral smear findings in MM

A

“rouleaux” formation of blood cells

mean platelet volume (MPV) is elevated bc cells stick together

181
Q

cause of elevated Ca++ level in MM

A

d/t osteoLYTIC lesions

182
Q

prognostic indicator in MM

A

B2 microglobulin level

183
Q

mechanism of renal failure in MM

A
  • hypercalcemia = nephrocalcinosis
  • hyperuricemia = directly toxic to kidney tubules
  • Bence-Jones protein clog up glomeruli and are also toxic to kidney tubules
  • amyloid occurs in myeloma
184
Q

reverses renal dysfunction in MM

A

bortezomib

185
Q

single MOST SPECIFIC test in MM

A

bone marrow biopsy (detects high numbers of plasma cells (10%))

186
Q

treatment for MM

A

melphalan and steroids

187
Q

most effective treatment for MM

A

autologous stem cell bone marrow transplantation

(pts

188
Q

other treatment considerations for MM:

  • hypercalcemia
  • bone fractures
  • renal failure
  • anemia
  • infection
A
  • hydration/diuresis
  • bisphosphonates
  • hydration
  • erythropoietin
  • vaccinations
189
Q

MGUS presents w/

A

ASYMPTOMATIC elevation of IgG on SPEP

190
Q

treatment for MGUS

A

none

191
Q

Waldenstrom’s macrogammaglobulinemia presents w/

A

hyperviscosity from IgM overproduction

192
Q

how does Waldenstrom’s macrogammaglobulinemia present?

A
  • blurred vision
  • confusion
  • headache
  • enlarged LN’s/splenomegaly
193
Q

best INITIAL test for Waldenstrom’s macrogammaglobulinemia

A
  • serum viscosity level (will be markedly increased)

AND

  • SPEP (elevated IgM level)
194
Q

best INITIAL treatment for Waldenstrom’s macrogammaglobulinemia, if SYMPTOMATIC

A

plasmapheresis

195
Q

further treatment for Waldenstrom’s macrogammaglobulinemia

A
  • rituximab
  • fludarabine
  • chlorambucil
196
Q

hemolytic uremic syndrome (HUS) is associated w/

A

E. coli 0157:H7

197
Q
  • ticlopidine predisposes to this
A

thrombotic thrombocytopenic purpura (TTP)

198
Q
  • pancytopenia w/ no identified etiology
A

aplastic anemia

199
Q

best possible treatment for aplastic anemia

A

bone marrow transplantation

200
Q

if bone marrow transplantation is not possible, treatment for aplastic anemia

A

antithymocyte globulin and cyclosporine

201
Q
  • enlarged LNs (most commonly cervical area)

- +/- “B” symptoms (fever, weight loss, night sweats)

A

lymphoma

202
Q

lymphoma: starts at the neck and spreads centrifugally away from center

A

Hodgkin’s lymphoma (HD)

203
Q

lymphoma: widespread disease

A

non-Hodgkin’s lymphoma (NHL)

204
Q

best INITIAL test for HD and NHL

A

excisional LN biopsy

205
Q

the major difference between HD and NHL, is HD has

A

Reed-Sternberg cells

206
Q

tests needed for staging HD and NHL

A
  • CXR
  • CT scans w/ contrast of chest, abdomen, pelvis, and head
  • bone marrow biopsy
207
Q

staging of HD and NHL

A

stage 1: single LN group
stage 2: 2 LN groups on ONE side of diaphragm
stage 3: LNs on BOTH sides of diaphragm
stage 4: widespread disease

208
Q

HD presents in what stages?

A

80-90% in stages 1 or 2

209
Q

NHL presents in what stages?

A

80-90% in stages 3 or 4

210
Q

lymphoma treatment: localized disease (stages 1 and 2) WITHOUT “B” symptoms

A

radiation and lower-dose chemotherapy

211
Q

lymphoma treatment: advanced disease (stages 3 and 4)

A

chemotherapy ONLY

212
Q

chemotherapy treatment for HD

A

ABVD

  • Adriamycin (doxorubicin)
  • bleomycin
  • vinblastine
  • dacarbazine
213
Q

chemotherapy treatment for NHL

A

CHOP

  • cyclophosphamide
  • hydroxyadriamycin
  • Oncovin (vincristine)
  • prednisone
214
Q

if anti-CD20 Ag is present in NHL, what should be added?

A

rituximab (adds efficacy to CHOP)

215
Q

name the coagulation disorders (4ish)

A
  1. von Willebrand’s disease (VWD)
  2. idiopathic thrombocytopenic purpura (ITP)
  3. uremia-induced platelet dysfunction
  4. clotting factor deficiencies (factors 8, 9, 11, 12)
216
Q
  • bleeding from platelet dysfunction (superficial bleeding: skin and mucosal surfaces)
  • epistaxis
  • bleeding worse w/ aspirin use
  • NORMAL platelet count
  • elevated aPTT in up to 50% of pts
A

von Willebrand’s disease (VWD)

217
Q

MOST ACCURATE tests for von Willebrand’s disease (VWD)

A
  • ristocetin cofactor assay

- von Willebrand’s factor (VWF) level

218
Q

first-line treatment for von Willebrand’s disease (VWD)

A

desmopressin

219
Q

MOA of desmopressin

A

releases subendothelial stores of VWF and factor 8

220
Q

treatment for von Willebrand’s disease (VWD) if desmopressin is ineffective

A

factor 8 replacement

221
Q
  • platelet-type bleeding

- platelet count

A

idiopathic thrombocytopenic purpura (ITP)

222
Q

diagnostic tests for idiopathic thrombocytopenic purpura (ITP)

A
  • peripheral smear
  • sonogram
  • bone marrow
  • Abs to glycoprotein 2b/3a receptor
223
Q

finding of peripheral smear in idiopathic thrombocytopenic purpura (ITP)

A

large platelets

224
Q

finding of sonogram in idiopathic thrombocytopenic purpura (ITP)

A

normal spleen size

225
Q

finding of bone marrow biopsy in idiopathic thrombocytopenic purpura (ITP)

A

increased # of megakaryocytes

226
Q

most important step in idiopathic thrombocytopenic purpura (ITP)

A

initiating treatment

227
Q
  • platelet count of 5,000
  • epistaxis and petechiae
  • intracranial hemorrhage and melena

what is the best initial step?

A

IVIG

228
Q

idiopathic thrombocytopenic purpura (ITP) treatment:

platelet count > 50,000

A

NO treatment

229
Q

idiopathic thrombocytopenic purpura (ITP) treatment:

platelet count

A

prednisone

230
Q

idiopathic thrombocytopenic purpura (ITP) treatment:

platelet count

A

IVIG aka Rhogam

231
Q

idiopathic thrombocytopenic purpura (ITP) treatment:

RECURRENT episodes

A

splenectomy

232
Q

idiopathic thrombocytopenic purpura (ITP) treatment:

no response to splenectomy

A
  • romiplostim

- eltrombopag

233
Q

uremia prevents platelets from working properly by

A

preventing degranulation

234
Q
  • renal failure

- normal platelet count w/ platelet-type bleeding

A

uremia-induced platelet dysfunction

235
Q

the ristocetin test and VWF level will be what in uremia-induced platelet dysfunction

A

NORMAL

236
Q

best INITIAL treatment for uremia-induced platelet dysfunction

A

desmopressin, dialysis, and estrogen

237
Q

the first test to determine difference between clotting factor deficiency and a factor inhibitor Ab

A

mixing study

aPTT will correct to normal w/ clotting factor deficiency

238
Q
  • woman present w/ bleeding into her thigh after minor trauma
  • aPTT is prolonged, and PT is normal
  • mixing study does NOT correct the aPTT

what is the diagnosis?

A

factor 8 Ab

239
Q

clotting factor deficiencies:

joint bleeding or hematoma in a MALE child

A

factor 8 deficiency

240
Q

treatment for factor 8 deficiency:

minor and major (

A
  • desmopressin

- factor 8 replacement

241
Q

clotting factor deficiencies:

  • joint bleeding or hematoma
  • less common than factor 8 deficiency
A

factor 9 deficiency

242
Q

treatment for factor 9 deficiency

A

factor 9 replacement

243
Q

clotting factor deficiencies:

rare bleeding w/ trauma or surgery

A

factor 11 deficiency

244
Q

factor 11 deficiency treatment

A

fresh frozen plasma w/ bleeding episodes

245
Q

clotting factor deficiencies:

no bleeding

A

factor 12 deficiency

246
Q

treatment for factor 12 deficiency

A

no treatment needed

247
Q

best INITIAL test for all the clotting factor deficiencies

A

mixing study

248
Q

test after mixing study for all the clotting factor deficiencies

A

check the specific factor

249
Q

how does heparin-induced thrombocytopenia present?

A

drop in platelets of at least 50%

250
Q

when does heparin-induced thrombocytopenia occur?

A

a few days AFTER start of heparin

251
Q

is HIT exclusive to heparin only?

A

NO!! any form of heparin; this is an allergic reaction

252
Q

MC clinical manifestation of HIT

A

THROMBOSIS

253
Q

best INITIAL tests for HIT

A
  • platelet factor 4 Abs

- heparin-induced antiplatelet Abs

254
Q

best INITIAL therapy for HIT

A
  • STOP THE HEPARIN

- use a direct thrombin inhibitor (argatroban, lepirudin, fondaparinux)

255
Q

thrombophilia/hypercoagulable states:

  • venous or arterial thrombosis
  • elevated aPTT w/ normal PT
  • spontaneous abortion
  • false positive VDRL
A

lupus antiphospholipid syndrome

  • lupus anticoagulant
  • anticardiolipin Abs
256
Q

best INITIAL test for lupus antiphospholipid syndrome

A

mixing study

257
Q

MOST ACCURATE test for lupus anticoagulant

A

Russel viper venom test

258
Q

treatment for lupus antiphospholipid syndrome

A

heparin followed by warfarin

259
Q

thrombophilia/hypercoagulable states:

  • skin necrosis w/ warfarin use
  • venous thrombosis
A

protein C deficiency

260
Q

test for protein C deficiency

A

protein C level

261
Q

treatment for protein C deficiency

A

heparin followed by warfarin

262
Q

thrombophilia/hypercoagulable states:

  • MCC of thrombophilia
  • venous thrombosis
A

factor V Leiden thrombophilia

263
Q

test for factor V Leiden thrombophilia

A

factor V Leiden mutation test

264
Q

treatment for factor V Leiden thrombophilia

A

heparin followed by warfarin

265
Q

thrombophilia/hypercoagulable states:

  • no change in aPTT with IV heparin bolus
  • venous thrombosis
A

antithrombin deficiency

266
Q

test for antithrombin deficiency

A

antithrombin 3 level

267
Q

treatment for antithrombin 3 deficiency

A

large amounts of heparin or direct thrombin inhibitor followed by warfarin

268
Q

mechanism of factor 5 thrombophilia

A

mutated factor 5 cannot be inactivated by protein C

factor 5 mutation functions like protein C deficiency

USMLE Step 3 (20 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions