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USMLE Step 3 MTB > Hematology > Flashcards

Hematology Flashcards

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USMLE® Step 1 flashcards
1
Q

all forms of anemia lead to

A

fatigue/loss of energy

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2
Q

pt with severe anemia will have

A

SOB, lightheadedness, or confusion

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3
Q

diseases with similar presentations to anemia:

A
  • hypoxia
  • CO poisoning
  • methemoglobinemia
  • ischemic heart disease
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4
Q

craving for ice or dirt, think

A

anemia, PICA

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5
Q

physical exam findings on anemic patient

A
  • pallor
  • flow murmur
  • pale conjunctiva
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6
Q

physical exam findings in hemolytic anemia

A
  • Jaundice

- scleral icterus

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7
Q

in severe anemia, what is needed to exclude ischemia?

A

EKG

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8
Q

best INITIAL test for anemia

A

CBC with peripheral smear

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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
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10
Q

categorization of anemia is based on

A

MCV (mean corpuscular volume)

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11
Q

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

A

MCHC (mean corpuscular hemoglobin concentration)

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12
Q

anemia categorized based on MCV is as follows:

A
  • microcytic
  • macrocytic
  • normocytic
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13
Q

based on MCHC anemia can be further categorized as follows:

A
  • hypochromic
  • hyperchromic
  • normochromic
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14
Q
  • blood loss
  • THROMBOCYTOSIS

what is the diagnosis?

A

Iron Deficiency Anemia

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15
Q
  • RA
  • ESRD
  • any chronic infectious, inflammatory, or connective tissue disease

what is the diagnosis?

A

Anemia of Chronic dz

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16
Q
  • very small MCV w/ few or no symptoms
  • TARGET cells; normal iron studies

what is the diagnosis?

A

thalassemia

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17
Q
  • alcoholic
  • INH
  • lead exposure

what is the diagnosis?

A

sideroblastic anemia

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18
Q

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

A

iron studies

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19
Q

results of iron studies in Iron def. anemia

A
  • low ferritin
  • high TIBC (total iron binding capacity)
  • low iron
  • low iron saturation
  • elevated RDW (red cell distribution width)
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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
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21
Q

results of iron studies in thalassemia

A

NORMAL

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22
Q

results of iron studies in sideroblastic anemia

A

HIGH IRON

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23
Q

MOST ACCURATE test for IDA

A

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

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24
Q

MOST ACCURATE test for thalassemia

A

Hb electrophoresis

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

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25
Q

MOST ACCURATE test for sideroblastic anemia

A

Prussian blue stain

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26
Q

best INITIAL treatment for IDA

A

ferrous sulfate PO

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27
Q

best INITIAL treatment for ACD

A

correct underlying cause

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28
Q

best INITIAL treatment for thalassemia

A

no treatment for trait

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29
Q

best INITIAL treatment for sideroblastic anemia

A

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

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30
Q

MOST ACCURATE test for ALPHA thalassemia

A

DNA sequencing and not electrophoresis

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31
Q

name the 4 types of microcytic anemia

A
  1. IDA
  2. ACD
  3. thalassemia
  4. sideroblastic anemia
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32
Q

causes of macrocytic anemia

A
  • vitamin B12 deficiency

- folate deficiency

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33
Q

which medication blocks B12 absorption?

A

metformin

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34
Q

B12 deficiency causes:

A
  • hypersegmented neutrophils
  • peripheral neuropathy (can cause any neuro sx)
  • glossitis (smooth tongue)
  • diarrhea
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35
Q

folate deficiency causes:

A
  • hypersegmented neutrophils only
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36
Q

labs seen in B12 defiicincy

A
  • DECREASED reticulocyte count
  • elevated bilirubin
  • elevated LDH
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37
Q

mechanism of elevated indirect bilirubin and LDH in B12 deficiency

A

“ineffective erythropoiesis”

RBCs are destroyed as they leave bone marrow

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38
Q

MOST ACCURATE tests for B12 and folate deficiencies

A

B12 and folate levels

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39
Q

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

A

methylmalonic acid level

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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 (more specific)

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41
Q

treatment for B12 and folate deficiencies

A

replace

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42
Q

first thing that improves after B12 replacement therapy

A

reticulocytes

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43
Q

last thing that improves after B12 replacement therapy

A

neurological abnormalities

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44
Q

name the hemolytic anemias (9)

A
  1. sickle cell anemia
  2. Hb sickle cell disease
  3. autoimmune hemolysis (Warm agglutinins, IgG)
  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)
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45
Q

all forms of hemolytic anemia present with

A

SUDDEN ONSET of weakness and fatigue

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46
Q

diagnostic testing for hemolysis shows the following

A
  • elevated indirect bilirubin
  • elevated reticulocyte count
  • elevated LDH
  • decreased haptoglobin
  • spherocytes on smear
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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
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48
Q

what electrolyte should be monitored after treating vitamin B12 deficiency?

A

K+ (can be low)

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49
Q
  • very severe chest, back, and thigh pain
A

sickle cell anemia

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50
Q

best INITIAL treatment for sickle cell anemia

A
  • OXYGEN
  • HYDRATION
  • PAIN MEDICATIONS
  • ABX (ceftriaxone, levofloxacin, or moxifloxacin)
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51
Q

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

A

NO SPLEEN

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52
Q

tests that should be ordered for pt with sickle anemia

A
  • blood cultures
  • UA
  • reticulocyte count
  • CBC
  • CXR
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53
Q

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

A

physical examination

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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)

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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
  • brain: stroke
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56
Q

what is the goal of exchange transfusion?

A

decrease Hb S to 30-40%

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57
Q
  • sudden drop in hematocrit in pt with hemoglobinopathy

- low reticulocyte count

A
  • parvovirus B19

- folate deficiency

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58
Q

MOST ACCURATE test for parvovirus B19

A

PCR for parvovirus DNA

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59
Q

treatment for sickle cell crisis d/t parvovirus B19

A

transfusions and IVIG

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60
Q

sickle cell anemia patients should be discharged on:

A
  1. folate
  2. Pneumococcal vaccine
  3. hydroxyurea, if crises occur > 4x/year
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61
Q

MOA of hydroxyurea

A

increases % of Hb F

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62
Q

sickle cell disease manifestations

A
  • visual disturbance (frequent)
  • hematuria
  • isosthenuria (inability to concentrate/dilute urine)
  • UTIs
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63
Q

sickle cell trait manifestations

A
  • hematuria
  • isosthenuria
  • splenic vein thrombosis can occur with severe hypoxia
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64
Q

look for other autoimmune diseases in history, or medications

A

autoimmune hemolysis

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65
Q

diagnostic tests for autoimmune hemolysis

A
  • elevated LDH
  • elevated indirect bilirubin
  • elevated reticulocyte count
  • decreased haptoglobin
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66
Q

peripheral smear will show what in autoimmune hemolysis

A

spherocytes

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67
Q

MOST ACCURATE diagnostic test for autoimmune hemolysis

A

Coombs test

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68
Q

best INITIAL treatment for autoimmune hemolysis

A

steroids

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69
Q

treatment if RECURRENT episodes of autoimmune hemolysis

A

rituximab

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70
Q

MOST EFFECTIVE treatment if RECURRENT episodes of hemolysis

A

splenectomy

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71
Q

mechanism of spherocytes in autoimmune hemolysis

A

Abs attack RBC membranes which are biconcave turning them into spheres

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72
Q

in autoimmune hemolysis, steroids and splenectomy only work on?

A

IgG Abs “warm antibodies”

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73
Q

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

A

IVIG

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74
Q

a response to IVIG predicts a response to

A

splenectomy

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75
Q

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

A
  • mycoplasma

- Epstein-Barr virus

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76
Q

in cold-induced hemolysis Coombs test will be?

A

NEGATIVE

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77
Q

in cold-induced hemolysis complement test will be?

A

POSITIVE

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78
Q

treatment for in cold-induced hemolysis

A

Rituximab

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79
Q

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

A

NO

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80
Q

MOA of rituximab

A

MAB against CD20R on lymphocytes which make Abs

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81
Q

syphilis causes cold agglutinins with?

A

IVIG

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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

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83
Q

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

A
  • Heinz body test

- bite cells

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84
Q

Heinz bodies are

A

collection of oxidized, precipitated Hb embedded in RBC membrane

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85
Q

bite cells are

A

pieces of RBC membrane removed by spleen

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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)

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87
Q

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

A

avoid oxidant stress

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88
Q
  • sudden onset hemolysis

- NOT provoked (unclear cause)

A

pyruvate kinase deficiency

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89
Q
  • recurrent episodes of hemolysis
  • splenomegaly
  • bilirubin gallstones
  • elevated mean corpuscular Hb concentration (MCHC)
A

hereditary spherocytosis

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90
Q

MOST ACCURATE test for hereditary spherocytosis

A

Eosin-5-maleimide (more accurate than osmotic fragility test)

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91
Q

treatment for hereditary spherocytosis

A

splenectomy

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92
Q

mechanism of hereditary spherocytosis

A

genetic loss of ankyrin and spectrin in RBC membrane

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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)
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94
Q

thrombotic thrombocytopenic purpura (TTP) diagnosis is based on

A
  • intravascular hemolysis
  • elevated BUN and creatinine
  • thrombocytopenia

AND

  • fever
  • neurological abnormalities
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95
Q

never use what in HUS or TTP?

A

platelets

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96
Q

what is LOW in TTP?

A

ADAMTS-13 level

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97
Q

treatment for HUS/TTP

A
  • some cases resolve on their own

- severe cases: plasmapheresis

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98
Q

what makes HUS worse?

A

antibiotics

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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

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100
Q
  • pancytopenia
  • recurrent episodes of dark urine, especially in morning
  • MCC of death = large vessel VENous thrombosis
A

paroxysmal nocturnal hemoglobinuria (PNH)

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101
Q

paroxysmal nocturnal hemoglobinuria (PNH) can transform into

A
  • aplastic anemia

- acute myelogenous leukemia (AML)

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102
Q

MOST ACCURATE test for paroxysmal nocturnal hemoglobinuria (PNH)

A

CD55 and CD59 Abs (aka, decay accelerating factor)

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103
Q

best INITIAL treatment for paroxysmal nocturnal hemoglobinuria (PNH)

A

glucocorticoids

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104
Q

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

A

eculizumab

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105
Q

MOA of eculizumab

A

inhibits C-5 and prevents complement activation

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106
Q

how can HELLP syndrome be distinguished from DIC?

A

HELLP has NORMAL coagulation studies

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107
Q
  • SOB for no clear reason
  • clear lungs on exam
  • normal CXR
A

methemoglobinemia

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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 MTB (20 decks)

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