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COMLEX Level 3 > Hematology > Flashcards

Hematology Flashcards

1
Q

generalized anemia symptoms

A

fatigue, malaise, loss of energy

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2
Q

severe anemia symptoms

A

short of breath, lightheadedness, confusion

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3
Q

anemia symptoms

A

pallor
flow murmur (I/VI or II/VI systolic murmur)
pale conjuctiva

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4
Q

for anemia, there are no uneque physical findings to specify diagnosis, so what do you do?

A

order CBC

general appearance, CV, chest, extremities, HEENT

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5
Q

diagnostic tests for anemia

A

CBC with peripheral smear (paying mind to MCV and MCHC)
reticulocyte count, haptobloin, LDH, total and direct bilirubin, TSH with T4, B12/folate, iron
Urinalysis with microscopic analysis
chest exam (if shortness of breath)
EKG (for severe anemia to exclude eschemia)

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6
Q

best initial test for anemia

A

CBC with peripheral smear

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7
Q

MCV

A

mean corpuscular volume

may clarify whether anemia is microcytic, macrocytic, or normocytic

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8
Q

MCHC

A

mean corpuscular hemoglobin concentration
may reveal whether there is a problem with the synthesis of hemoglobin
hypochromic, hyperchromic, or normochromic

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9
Q

what has similar presentations to anemia?

A

hypoxia
carbon monoxide poisoning
methemoglobinemia
ischemic heart disease

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10
Q

microcytic anemia with: blood loss, elevated platelet count - diagnosis

A

iron deficiency

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11
Q

microcytic anemia with: blood loss, elevated platelet count - best initial diagnostic test?

A

iron studies:

  • low ferritin
  • high TIBC
  • low Fe
  • low Fe sat
  • elevated RDW
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12
Q

microcytic anemia with: blood loss, elevated platelet count - most accurate diagnostic test?

A

bone marrow biopsy - but do not do

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13
Q

microcytic anemia with: blood loss, elevated platelet count - best initial therapy?

A

prescribe ferrous sulfate orally

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14
Q

microcytic anemia with: rheumatoid arthritis, ESRD, or any chronic infectious, inflammatory, or connective tissue disease - diagnosis

A

anemia of chronic disease

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15
Q

microcytic anemia with: rheumatoid arthritis, ESRD, or any chronic infectious, inflammatory, or connective tissue disease - best initial diagnostic test?

A

iron studies:

  • high ferritin
  • low TIBC
  • low Fe
  • normal or low Fe sat
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16
Q

microcytic anemia with: rheumatoid arthritis, ESRD, or any chronic infectious, inflammatory, or connective tissue disease - most accurate diagnostic test?

A

no specific diagnostic test

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17
Q

microcytic anemia with: rheumatoid arthritis, ESRD, or any chronic infectious, inflammatory, or connective tissue disease - best initial therapy?

A

correct underlying disease

EPO only with renal failure

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18
Q

microcytic anemia with: very small MCV with few or no symptoms, target cells - diagnosis?

A

thalassemia

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19
Q

microcytic anemia with: very small MCV with few or no symptoms, target cells - best initial diagnostic test?

A

iron studies:

- normal

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20
Q

microcytic anemia with: very small MCV with few or no symptoms, target cells - most accurate diagnostic test?

A

hemoglobin
electrophoresis
beta: elevated HgA2, HgF
alpha: normal

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21
Q

microcytic anemia with: very small MCV with few or no symptoms, target cells - what is the best initial therapy?

A

no treatment for trait

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22
Q

microcytic anemia: alcoholic isoniazid, lead exposure - diagnosis?

A

sideroblastic anemia

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23
Q

microcytic anemia: alcoholic isoniazid, lead exposure - best initial diagnostic test?

A

iron studies:

- high Fe

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24
Q

microcytic anemia: alcoholic isoniazid, lead exposure - most accurate diagnostic test?

A

prussian blue stain of marrow (shows ringed sideroblasts)

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25
Q

microcytic anemia: alcoholic isoniazid, lead exposure - best initial therapy?

A

major: remove the toxin exposure
minor: prescribe pyridoxine replacement

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26
Q

most accurate diagnosis of alpha thalassemia

A

DNA sequencing

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27
Q

diagnostic tests for anemia:

A

iron studies/profile (Fe, Fe sat, ferritin, TIBC)

bone marrow biopsy

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28
Q

macrocytic anemia causes

A

B12 or folate deficiency

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29
Q

B12 deficiency findings:

A 
peripheral neuropathy
least common: dementia
resolve with treatment if present for a short time
glossitis
diarrhea
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30
Q

Folate deficiency findings

A

no neurologic problems

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31
Q

diagnostic testing of macrocytic anemia

A 
CBC with peripheral blood smear (hypersegmented neutrophils and oval cells)
bilirubin, LDH (commonly elevated)
decreased reticulocyte
oval cells on peripheral smear
B12, folate (most accurate)
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32
Q

what is low in B12 deficiency?

A

reticulocytes

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33
Q

what to do if you suspect B12 deficiency, but B12 is normal?

A

order a methylmalonic acid level

homocysteine levels go up in both vitamin B12 deficiency and folate deficiency

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34
Q

next best test after finding a low B12 or elevated methylmalonic acid?

A

confirm etiology with antiparietal cell and anti-intrinsic factor antibodies
- confirm pernicious anemia as etiology (allergy to parietal cells)

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35
Q

Schilling test

A

older way to confirm etiology

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36
Q

why does B12 deficiency raise LDH and indirect bilirubin?

A

destroying red blood cells early as they come out of the bone marrow
“ineffective erythropoiesis” and is why retic count is low

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37
Q

what happens after B12 replacement therapy?

A

reticulocytes will improve first (in iron deficiency)

neurological abnormalities will improve last

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38
Q

presentations of hemolytic anemia

A

sudden onset of weakness and fatigue associated with anemia

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39
Q

hemolysis shows the following in labs:

A 
elevated indirect bilirubin level
elevated reticulocyte count
elevated LDH 
decreased haptoglobin level
spherocytes on smear
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40
Q

what else gives spherocytes?

A

autoimmune hemolysis

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41
Q

mechanism of lab abnormalities in hemolysis

A

when cells are destroyed, they release indirect bilirubin. the liver has limited capacity to glucuronidate it into direct (water-soluble) bilirubin. indirect bili never gets into the urine because it’s attached to albumin and cannot be filtered. haptoglobin is a transport protein for newly released indirect bili and is rapidly used up during hemolysis. LDH increases from any form of tissue breakdown and is extremely nonspecific

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42
Q

what to order in hemolysis

A

peripheral smear, LDH, bilirubin level, reticulocyte count, haptoglobin level

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43
Q

what to watch out for when treating B12 deficiency?

A

low potassium

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44
Q

what do you see in intravascular hemolysis?

A

abnormal peripheral smear (schistocytes, helmet cells, fragmented cells)
hemoglobinuria

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45
Q

sickle cell presentation

A

acute severe pain in the chest, back, and thighs

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46
Q

diagnostic tests for sickle cell anemia

A

peripheral smear
LDH, indirect bili, reticulocytes: elevated
hemoglobin electrophoresis

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47
Q

best initial test for sickle cell

A

peripheral smear - showing sickles

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48
Q

most accurate test for sickle cell

A

hemoglobin electrophoresis

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49
Q

initial management of sickle cell:

A

oxygen, fluids, analgesics, and antibiotics

complete physical exam

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50
Q

physical exam findings in sickle cell

A

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

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51
Q

best next step for sickle

A

oxygen, hydration with normal saline continuously and pain medication
if fever, give ceftriaxone, levofloxacin, or moxifloxacin with the first screen (more important than waiting for results of testing)

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52
Q

answer for a sickler with a fever

A

blood cultures, urinalysis, reticulocyte count, CBC, and chest x-ray along with oxygen, hydration, IVF, and antibiotics

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53
Q

when is the answer ‘exchange transfusion’ in sickle cell disease?

A

eye: visual disturbance from retinal infarction
lung: pulmonary infarction leading to pleuritic pain, pulmonary, hypertension, and abnormal x-ray
penis: priapism from infarction of prostatic plexus of veins if local drainage does not work
brain: stroke

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54
Q

most common cause of osteomyelitis in sickle cell disease

A

salmonella

* requires a biopsy

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55
Q

if a patient is on folate replacement therapy, what is the diagnosis?

A

parvovirus B19

- invades the marrow and stops production of cells at the level of the pronormoblast

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56
Q

most accurate diagnostic test for parvo

A

PCR for DnA

- more accurate than IgM or IgG

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57
Q

treatment of parvo

A

transfusions and IV immunoglobulins

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58
Q

further management of parvovirus

A

folate replacement
vaccinations: pneumococcal (13-PV and 23-PV), hemophilus influenzae, meningococcal
hydroxyurea to prevent further crisis (if >3 per year)
voxelotor or crizanlizumab to prevent pain crises when hydroxyurea is not working

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59
Q

what is voxelotor

A

HgS polymerization inhibitor

increases oxygen-carrying capacity

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60
Q

what is crizanlizumab

A

inhibits P-selectin inhibitor

controls platelet aggregation with RBCs

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61
Q

mechanism of hydroxyurea in sickle cell disease

A

increases the percentage of hemoglobin that is hemlogobin F, or fetal hemoglobin
increased fetal hemoglobin dilutes the sickle hemoglobin and decreases the frequency of painful crises

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62
Q

hemoglobin sickle cell disease

A

mild version of sickle cell disease with fewer crises
visual disturbances are frequent
no painful crises
renal problems are the only significant manifestation - hematuria, isosthenuria (inability to concentrate or dilute the urine), and UTIs
no specific tx of hemoglobin SC disease

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63
Q

autoimmune hemolysis

A

clues:
look for SLE or rheumatoid arthritis
CLL, lymphoma, or medications (PCNs, alpha-methyldopa, quinine, or sulfa drugs)

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64
Q

diagnostic testing of autoimmune hemolysis

A

LDH, indirect bilirubin level, and reticulocyte count (all elevated)
haptoglobin level can be decreased in both intravascular and extravascular
peripheral smear - spherocytes
Coombs test

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65
Q

most accurate test of autoimmune hemolysis

A

Coombs test

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66
Q

treatment of autoimmune hemolysis

A

steroids (prednisone)
if ineffective: splenectomy
rituximab works on IgG and IgM

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67
Q

warm antibodies

A

IgG

only IgG antibodies respond to steroids and splenectomy

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68
Q

cold-induced hemolysis (cold agglutinins)

A 
mycoplasma or EBV in hx
standard Coombs is negative
complement test is positive
treatment: rituximab
if fails, try bendamustine
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69
Q

mechanism of spherocytes in autoimmune hemolysis

A

normal RBC is biconcave. when antibodies attack the RBC membrane, they pull pieces out of it, which decreases the surface area and turns the RBC into a sphere
it takes more surface area to maintain biconcave disc than a sphere

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70
Q

rituximab

A

antibody against CD20 receptor on lymphocytes
CD20+ make antibodies
improves disease (and rheumatoid arthritis) by removing antibody-producing cells

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71
Q

G6PD deficiency

A

sudden onset of severe hemolysis

X-linked

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72
Q

most common form of oxidative stress in G6PD

A

infection

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73
Q

causes of oxidative stress in G6PD

A 
oxidizing drugs (sulfa, primaquine, dapsone)
fava bean ingestion
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74
Q

best initial diagnostic test for suspected G6PD

A

Heinz body test - bite cells

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75
Q

most accurate test for G6PD

A

G6PD level - but only after 2 months have passed

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76
Q

Heinz bodies and bite cells

A

collections of oxidized, precipitated hemoglobin embedded in the RBC membrane
bite cells appear when pieces of RBC membrane have been removed by spleen

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77
Q

tx of G6PD

A

no specific treatment

avoid triggers

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78
Q

pyruvate kinase deficiency

A

presents same as G6PD in terms of hemolysis
not provoked by meds or fava beans
unsure the provocation

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79
Q

hereditary spherocytosis presentation

A

recurrent episodes of hemolysis
splenomegaly
bilirubin gallstones
elevated MCHC

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80
Q

all with chronic hemolysis (sickle cell, spherocytosis) need:

A

lifelong folate replacement

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81
Q

most accurate diagnostic test for suspected hereditary spherocytosis

A

eosin-5-maleimide (EMA)

more accurate than osmotic fragility

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82
Q

treatment for hereditary spherocytosis

A

splenectomy
- prevents hemolysis since cells are destroed in spleen
give folate

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83
Q

hereditary spherocytosis genetics

A

loss of both ankyrin and spectrin in RBC membrane, which are the basis of the cytoskeleton that maintains the RBC membrane in its biconcave disc

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84
Q

HUS cause

A

E.coli 0157:H7 in history

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85
Q

TTP cause

A

medication use, such as ticlopidine

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86
Q

diagnosis of HUS based on

A

intravascular hemolysis with abnormal smear
elevated BUN and creatinine
thrombocytopenia

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87
Q

diagnosis of TTP based on

A 
intravascular hemolysis with abnormal smear
elevated BUN and creatinine
thrombocytopenia
fever
neurological abnormalities
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88
Q

what to never use in HUS/TTP

A

platelets

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89
Q

what test should be ordered in HUS/TTP?

A

ADAMTS-13 level - decreased in TTP

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90
Q

PT/aPTT in HUS and TTP

A

normal

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91
Q

treatment for HUS/TTP

A

plasmapheresis for severe cases
HUS w/o infection = eculizumab (antibody against complement C5)
caplicizumab (ab against VWF acts like ADAMTS-13 and removes VWF and stops platelet aggregation)

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92
Q

what not to use in tx of HUS

A

antibiotics

platelet transfusion

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93
Q

what should you do before starting eculizumab?

A

vaccinate for meningococcus

- complement deficiency predisposes to meningococcal infection

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94
Q

mechanism of HUS/TTP

A

ADAMTS-13 is a metalloproteinase that breaks down von Willebrand factor (VWF) to release platelets from one another. When VWF is not dissolved, the platelets form abnormaly prolonged strands that serve as a barrier to RBCs, which run into the strands and break down/are destroyed. plasmapheresis in tx of severe TTP is to replace ADAMTS-13
giving platelets increases the size of the abnormal platelet strands

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95
Q

paroxysmal nocturnal hemoglobinuria

A

presents with pancytopenia and recurrent episodes of dark urine, particularly in the morning

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96
Q

most common cause of death in paroxysmal nocturnal hemoglobinuria

A

large vessel venous thrombosis, such as portal vein thrombosis

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97
Q

most accurate diagnostic test of paroxysmal nocturnal hemoglobinuria

A

CD 55 and CD 59 antibody (decay accelerating factor)

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98
Q

treatment of paroxysmal nocturnal hemoglobinuria

A

glucocorticoids

eculizumab for transfusion-dependent patients with severe illness

99
Q

unprovoked portal or hepatic vein (budd-chiari) thrombosis?

A

look for JAK2 mutation and CD55/59

100
Q

long term consequence of paroxysmal nocturnal hemoglobinuria

A

aplastic anemia or acute myelogenous leukemia (AML)

101
Q

methemoglobinemia

A

occurs when the blood is locked in an oxidized state and cannot pick up oxygen

102
Q

methemoglobinemia symptoms

A

shortness of breath for no clear reason, with clear lungs on exam and a normal chest xray
look for brown blood

103
Q

what to look for when suspecting methemoglobinemia

A

drugs: nitroglycerin, amyl nitrate, nitroprusside, dapsone, or any anesthetic drugs that end in -caine

104
Q

treatment of methemoglobinemia

A

methylene blue

105
Q

twenty minutes after a patient receives a blood transfusion, the patient becomes short of breath. there are transient infiltrates on the chest x-ray. all symptoms resolve spontaneously

A

TRALI - presents with acute SOB from antibodies in the donor blood against recipient white cells
no treatment

106
Q

as soon as a patient receives a transfusion, he becomes hypotensive, short of breath, and tachycardic. LDH and bilirubin levels are normal

A

IgA deficiency - presents with anaphylaxis

in the future, use blood donations from an IgA-deficient donor or washed red cells

107
Q

during a transfusion, a patient becomes hypotensive and tachycardic. she has back and chest pain and there is dark urine. LDH and bilirubin are elevated and the haptoglobin level is low

A

ABO incompatibility

108
Q

a few days after a transfusion, a patient becomes jaundiced. the hematocrit does not rise with transfusion, and he is generally without symptoms

A

minor blood group incompatibility to Kell, Duffy, Lews, or Kidd antigens or Rh incompatibility presents with delayed jaundice

109
Q

a few hours after a transfusion, a patient becomes febrile with a rise in temperature of about 1 degree. there is no evidence of hemolysis

A

febrile nonhemolytic reaction

reactions against donor white cell antigens

110
Q

acute leukemia symptoms

A

signs of pancytopenia: fatigue, bleeding and infections from white cells that don’t work
have functional immunodeficiency

111
Q

what would you see on bone marrow biopsy for AML

A

auer rods

112
Q

best initial test if suspecting leukemia

A

peripheral smear showing blasts

113
Q

treatment for acute myelogenous leukemia

A

chemotherapy with idarubicin and cytosine arabinoside (best initial therapy)

114
Q

treatment for acute promyelocytic leukemia (M3 leukemia)

A

add all trans retinoic acid to idarubicin and cytosine arabinoside
arsenic trioxide is extremely effective when combined with ATRA

115
Q

treatment for acute lymphocytic leukemia

A

add intrathecal methotrexate

116
Q

most important prognostic finding in acute leukemia

A

cytogenetic abnormalities, such as specific karyotype

117
Q

what is associated with acute promyelocytic leukemia

A

DIC

118
Q

treatment of leukostasis

A

leukapheresis and hydroxyurea

119
Q

elderly patient with pancytopenia, elevated MCV, low reticulocyte count, and macroovalocytes and normal B12, <20% blasts

A

myelodysplasia

120
Q

pelger-huet cell

A

neutrophil with two lobes

121
Q

myelodysplasia

A

mild, slowly progressive preleukemia syndrome
may progress to acute leukemia
most common cause of death is infection or bleeding

122
Q

treatment of myelodysplasia

A

largely supportive
transfusions as needed
specific therapies: azacitidine (increases survival), decitabine, lenalidomide (for those with 5q minus syndrome)
luspatercept (erythroid maturation stimulant): helps RBCs grow, reducing the frequency of transfusions

123
Q

chronic myelogenous leukemia (CML)

A

elevated white count that is predominantly neutrophils
splenomegaly
early satiety

124
Q

mechanism of early satiety in CML and CLL

A

spleen anatomically right on top of the stomach and compresses it, which makes a person feel full right after eating

125
Q

diagnostic testing for CML

A

elevated neutrophil count with low leukocyte alkaline phosphatase (LAP) score
- rx high wbc from infection give elevated LAP score
- LAP is up in normal cells, not CML
philadelphia chromosome by PCR of blood or BCR/ABL by FISH (most accurate test)

126
Q

most accurate test for CML

A

philadelphia chromosome by PCR of blood or BCR/ABL by FISH

127
Q

treatment for CML

A

imatinib
which leads to 90% hematologic remissionwith no major adverse effects
if not effective, try dasatinib and nilotinib

128
Q

wrong answers for CML

A

interferon -> much less effective, causes uncomfortable, flu-like symptoms
hydroxyurea -> never makes the philadelphia chromosome negative
busulfan -> never right for anything, unless the exam asks what causes pulmonary fibrosis

129
Q

when would you use interferon in CML?

A

in pregnant patients

130
Q

what myeloproliferative disorder has the highest risk of transformation into acute leukemia?

A

CML

131
Q

chronic leukocytic leukemia (CLL)

A

exclusively age >50 with elevated white count that is described as “normal appearing lymphocytes”
- often asymptomatic

132
Q

diagnostic testing for CLL

A

peripheral blood smear: smudge cells (ruptured nuclei of lymphocytes) - best initial diagnostic test

133
Q

best initial diagnostic test for suspected CLL

A

peripheral blood smear

134
Q

stages of CLL

A 
stage 0: elevated white cell count alone
stage 1: enlarged lymph nodes
stage 2: enlarged spleen
stage 3: anemia
stage 4: low platelets
135
Q

mechanism of infection and hemolysis in CLL

A

the lymphocytes in CLL produce abnormal or insufficient immunoglobulins

  • when IgG produced is abnormal, it is inappropriately directed against RBCs or platelets, causing immune thrombocytopenia or hemolysis
  • when IgG supply is insufficient, it leads to infection
136
Q

treatment for CLL

A

based on stage of the disease

137
Q

treatment for early stages CLL

A

stage 0 and stage 1

no therapy required

138
Q

treatment for more advanced stages of CLL

A

fludarabine + rituximab (an antibody against CD20) to extend survival
chlorambucil is less effective

139
Q

first line therpay for CLL

A

there is no clear first line therapy

140
Q

when is venetoclax the answer for treatment of CLL?

A

when there is CLL that fails initial therapy and there is a 17p deletion

141
Q

venetoclax

A

increases apoptosis in CLL when there is a 17p deletion

142
Q

hairy cell leukemia

A

pancytopenia
massive splenomegaly
middle-aged patient (50s)

143
Q

hairy cell leukemia peripheral smear

A

hairy cells and immunophenotyping (or flow cytometry)

most accurate test

144
Q

treatment of hairy cell leukemia

A

cladiribine (2-CDA)

145
Q

myelofibrosis

A

presents with pancytopenia, massive splenomegaly in a middle-aged patient but a normal TRAP level

146
Q

myelofibrosis peripheral smear

A

teardrop shaped cells

147
Q

myelofibrosis bone marrow biopsy

A

fibrosis

148
Q

myelofibrosis genetics

A

JAK2 mutation

149
Q

myelofibrosis treatment

A

bone marrow transplantation can be curative

if not possible, ruxolitinib or fedratinib (inhibits JAK2)

150
Q

polycythemia vera (Pvera)

A

elevated RBCs
headache, blurred vision, dizziness, fatigue
pruritus, often after hot bath or shower due to release of histamine from basophils
splenomegaly

151
Q

what to order in Pvera

A

B12, LAP and a CBC

152
Q

most accurate diagnostic test in pvera

A

CBC

- markedly high hct in the absence of hypoxia with a low MCV

153
Q

lab findings in Pvera

A 
low erythropoietin
poss elevated white cell and plt
elev B12 and LAP
high hct -> thrombosis (order ABG to exclude hypoxia as cause)
JAK2 mutation
154
Q

treatment for Pvera

A

phlebotomy
hydroxyuria - lower cell count
daily aspirin
anagrelide or ruxolitinib if hydroxyurea fails

155
Q

essential thrombocytopenia

A 
found in asymp pt on CBC
markedly elevated plt
headache, visual disturbace, pain in the hands (erythromyalgia)
thrombosis and bleeding
CALR mutation
156
Q

treatment of essential thrombocytopenia

A

hydroxyurea to lower plt count

daily aspirin

157
Q

multiple myeloma presentation

A

bone pain caused by a fracture occurring under normal use

158
Q

causes of death in MM

A

most common are infection
- immunodeficient
and renal failure

159
Q

initial diagnostic testing for suspected MM

A

skeletal survey - punched out or osteolytic lesions are suggestive of metastatic prostate
serum protein electrophoresis (SPEP) - elevated monoclona antibodies (usually IgG)
urine protein electrophoresis (UPEP) - Bence-Jones protein
peripheral smear - rouleaux formation of blood cells, mpv elevated
calcium - elevated (osteolytic lesions)
beta 2 microglobulin level - prognostic indicator
BUN and Cr - detect the frequent occurrence of renal insufficiency; bortezomib reverses renal dysfunction

160
Q

other testing for MM

A

serum free light chain (FLC) ratio 100:1 highly consistent

low AG - IgG is cation and raises the chloride level, narrowing the gap

161
Q

mechanism of renal failure in myeloma

A

hypercalcemia -> nephrocalcinosis
hyperuricemia -> directly toxic to kidney tubules
Bence-Jones protein clogs up glomeruli and is toxic to kidney tubules
amyloid occurs in myeloma

162
Q

most specific test in MM

A

bone marrow biopsy

high number of plasma cells

163
Q

treatment for MM

A

no single clear treatment
melphalan and steroids
consider adding thalidomide, lenalidomide, bortezomib, or daratumumab
- give ppx against clotting when using these agents
- bort high risk neuro complications
- darat can be used in adults unable to receive other therapies

164
Q

most effective therapy in MM

A

autologous stem cell bone marrow trnasplantation

- reserved for young patients (<70) with advanced disease

165
Q

things to remember to treat with MM

A 
hypercalcemia (hydration)
bone fractures (bisphosphonates)
renal failure (hydration)
anemia (erythropoietin)
ppx against infections (pneumovax, flu)
166
Q

smolding myeloma

A

10-60% of bone marrow is plasma cells
high M-spike on serum protein electrophoresis (SPEP)
urine monoclonal protein level is elevated
FLC ratio is increased
no CRAB organ damage (no hypercalcemia, renal failure, anemia, or bone lesions)

167
Q

tx smolding myeloma

A

no specific therapy, close follow up

168
Q

monoclonal gammopathy of unknown significants (MGUS)

A 
asymptomatic elevation of IgG on SPEP
elevated total protein, >70
peripheral neuropathy
10% plasma cells
no treatment
169
Q

waldenstrom macroglobulinemia

A

hyperviscosity from IgM overproduction
blurry vision, confusion, and headache
enlarged nodes and spleen

170
Q

best initial test if suspecting waldenstrom macrogloblinemia

A

serum viscosity level
- markedly increased
SPEP
- elevated IgM

171
Q

treatment for waldenstrom macroglobulinemia

A

plasmapheresis if symptomatic

for further treatment, same as CLL: fludarabine, chlorambucil, or rituximab

172
Q

aplastic anemia

A

pancytopenia with no identified etiology

173
Q

best treatment for aplastic anemia

A 
if young (<50) with a match: BMT
if >50 and no match: use antithymocyte globulin and cyclosporine
174
Q

causes of aplastic anemia

A

most are idiopathic

chronic Hep B and C can cause it

175
Q

lymphoma presentation

A

enlarged lymph nodes, most commonly cervical

176
Q

hodgkin disease

A

spreads centrifugally away from the center, starting at the neck

177
Q

non-hodgkin lymphoma

A

presents as widespread disease

178
Q

B symptoms in lymphoma

A

fever
weight loss
night sweats

179
Q

major difference between hodgkin’s and non-hodgkin’s

A

HD has reed-sternberg cells

180
Q

best initial test for HD and NHL

A

excisional lymph node biopsy

181
Q

after biopsy of lymph nodes, what’s next?

A

staging
stage 1: single lymph node group
stage 2: two lymph node groups on one side of the diaphragm
stage 3: lymph node involvement on both sides of the diaphragm
stage 4: widespread disease

182
Q

when does HD usually present?

A

80-90% present with stages I and II

183
Q

when does NHL usually present?

A

80-90% present with stages III and IV

184
Q

how to stage lymphoma?

A

chest x-ray, CT scan with contrast (chest, abdomen, pelvis, head), and bone marrow biopsy

185
Q

wrong answers for diagnostic tests in lymphoma:

A 
needle biopsy (useful for infections, but not sufficient for lymphoma)
lymphangiogram or ex lap of abdomen
186
Q

treatment of lymphoma

A 
localized disease (stage I and II) without B symptoms: radiation and low-dose chemotherapy
more advanced stages (stage III and IV): chemotherapy exclusively
187
Q

chemo for HD

A

adriamycin (doxorubicin), bleomycin, vinblastine, davarbacine (ABVD)

188
Q

treatment of chemo-induced nausea

A 
5HT inhibitors (ondansetron, granisetron, palonosetron, dolasetron) = best initial
glucocorticoids: dexamethasone
neurokinin-1R antagonists: aprepitant, rolapitant, netupitant) - use if QT prolongation
189
Q

VWD presentatin

A

epistaxis and/or petechiae

superficial bleeding from the skin and mucosal surfaces

190
Q

VWD findings

A

normal plt count

elevated aPTT because VWF destabilizes factor VIII

191
Q

most accurate test in suspected VWD

A

ristocetin cofactor assay and VWF level

192
Q

treatment of VWD

A

desmopressin or DDAVP is first line - releases subendeothelial stores of VWF and factor VIII, which will stop the bleeding
if not effective, use factor VIII replacement
if both ineffective, use recombinant VWF

193
Q

mechanism of ristocetin testing

A

acts as an artificial endothelial lining
if VWF present, platelets will stick to it
ristocetin is a functional test of VWF activity

194
Q

ITP

A

platelet bleeding with plt <10k-30k

195
Q

platelet-type bleeding

A 
petechiae
epistaxis
purpura
gingiva
vaginal
196
Q

factor-type bleeding

A

hemarthrosis

hematoma

197
Q

diagnostic testing of ITP

A

peripheral smear - large platelets
sonogram - normal spleen size
bone marrow - increased megakaryocytes

198
Q

treatment for ITP if plt >50k

A

no treatment

199
Q

treatment for ITP if plt <50k with minor bleeding

A

prednisone

200
Q

treatment for ITP if plt <10-20k with serious bleeding

A

IVIG or RhoGAM

201
Q

treatment for ITP if recurrent episodes

A

splenectomy

rituximab

202
Q

treatment for ITP if no response to splenectomy

A

avatrombopag, romiplostim, eltrombopag

203
Q

what doesn’t help ITP?

A

plasmapheresis because antibodies are already stuck to the platelets

204
Q

uremia-induced platelet dysfunction

A

uremia by itself prevents platelets from working properly, they do not granulate

205
Q

findings in uremia-induced platelet dysfunction

A

normal platelet count with platelet-type bleeding in a patient with renal failure
ristocetin test and VWF level will be normal

206
Q

treatment for uremia-induced platelet dysfunction

A

ddavp, dialysis, estrogen

207
Q

glanzmann thrombasthenia and bernard soulier

A

platelet-bleeding despite normal plt count and normal VWF

diagnosed by platelet studies

208
Q

platelet study in glanzmann thrombasthenia

A

like being on abciximab personality

209
Q

platelet study in bernard-soulier

A

giant platelets

210
Q

treatment of glanzmann thrombasthenia and bernard soulier

A

ddavp - releases subendothelial VWF and factor VIIIa
tranexamic and epsilon amino caproic acid inhibit fibrinolysis and plasminogen - acute bleeding
recombinant factor VIIIa
estrogen upregulates VWF

211
Q

clotting factor deficiencies

A

factor VIII
factor IX
factor XI
factor XII

212
Q

joint bleeding or hematoma in a male child

A

factor VIII

213
Q

diagnostic test in factor VIII

A

mixing study firsts, then specific factor level

214
Q

treatment of factor VIII

A

severe deficiency: factor VIII replacement

minor replacement: DDAVP

215
Q

joint bleeding or hematoma

less common

A

factor IX

216
Q

diagnostic test for Factor IX

A

mixing study first, then specific factor level

217
Q

treatment of factor IX

A

factor ix replacement

218
Q

rare bleeding with trauma or surgery

A

factor XI

219
Q

diagnostic test of factor XI

A

mixing study first, then specific factor level

220
Q

treatment of factor XI

A

FFP with bleeding episodes

221
Q

no bleeding

A

factor XII

222
Q

diagnostic test for factor XII

A

mixing study first, then specific factor level

223
Q

treatment for factor XII

A

no treatment

224
Q

factor VIII lab findings

A

prolonged aPTT and bleeding

225
Q

treatment of factor VIII

A

recombinant factor VIII

ddavp

226
Q

what is in prothrombin complex concentrate

A

reverses warfarin
contains vitamin-k dependent factors: factors II, VII, XI, and X and protein C and S
works faster than FFP and vitK

227
Q

heparin-induced thrombocytopenia

A

reduced platelets manifesting a few days after the start of heparin

228
Q

most common presentation of HIT

A

thrombosis - venous thromboses 3x more common than arterial thromboses

229
Q

best initial diagnostic test in HIT

A

platelet factor 4 antibodies or heparin-induced, antiplatelet antibodies

230
Q

most accurate test in HIT

A

serotonin release assay

231
Q

treatment of HIT

A

stop heparin
use fondaparinux or a DOAC
if fonda not avail, choose bivalirudin or argatroban
if both, choose fonda

232
Q

venous or arterial thrombosis
elevated aPTT and normal PT
spontaneous abortion
false positive VDRL

A

antiphospholipid syndromes

233
Q

diagnostic test for antiphospholipid syndromes

A

mixing study first

russel viper venom test is most accurate for lupus anticoagulant

234
Q

treatment of antiphospholipid syndromes

A

heparin followed by warfarin

INR target 2-3, lifelong

235
Q

skin necrosis with the use of warfarin

venous thrombosis

A

protein C deficiency

236
Q

diagnostic test in protein c deficiency

A

protein c level

237
Q

treatment for protein c deficiency

A

DOAC

238
Q

most common cause of thrombosis

venous thrombosis

A

factor V leiden mutation

239
Q

diagnostic test for factor V leiden

A

factor V mutation test

240
Q

treatemnt for factor V leiden

A

DOAC

241
Q

no change in aPTT with a bolus of IV heparin

venous thrombosis

A

antithrombin deficiency

242
Q

diagnostic test for antithrombin deficiency

A

level of antithrombin III

243
Q

treatment of antithrombin deficiency

A

DOAC or warfarin, lifelong therapy may be required

COMLEX Level 3 (5 decks)

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