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1 USMLE Step 3 Main one > 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

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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

IDA

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15
Q
  • RA - ESRD - any chronic infectious, inflammatory, or connective tissue disease what is the diagnosis?
A

ACD

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16
Q
  • very small MCV w/ few or no symptoms - TARGET cells 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 IDA

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

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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

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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 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 - brian: 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 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 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

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

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109
Q

possible drugs that can cause methemoglobinemia

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

in methemoglobinemia, what color is the blood?

A

BROWN

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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)

269
Q

Blood transfusion: Anaphylaxis occurs when?

A

In the first few seconds to minutes and occurs with kids who get blood tranfusion with IgA deficiency

270
Q

Blood transfusion:Acute hemolytic reaction occurs when?

A

When patient recieves a tranfusion which is not cross matched and occurs within an hour. you will often find coombs test positive and UA showing hemoglobinuria.

271
Q

Blood transfusion:Febrile nonhemolytic reaction occurs when?

A

When patient recieves blood that which is not Leukoreduced(WBC removed-which also removes cmv and reduces HLA alloimmunization) these reactions usually occur within 1 to 6 hours of tranfusion.

272
Q

Why do you never use whole blood?

A

Whole blood contains numerous leukocytes and increase the risk of febrile reactions.

273
Q

What are irradiated RBCS?

A

They are irradiated for bone marrow transplant recipients, Immuno deficient, or blood products from 1deg or 2nddeg relatives

274
Q

Whats are washed rbcs?

A

Washed For those with Iga deficiency and prevent autoimmunie hemolytic anemia

275
Q

What are leukoreduced RBCS?

A

For thos who are chronically transfused, CMV at risk patients, potential transplant patient and previously febrile nonhemolytic reaction in PMH patients

1 USMLE Step 3 Main one (37 decks)

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