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OMS III > IM Hematology > Flashcards

IM Hematology Flashcards

1
Q

anemia

what is the mos tlikley dx

A

sx are generaaly bast not on the etiology but on the severity of dz so you cant answer this one

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2
Q

anemia

best initial test

A

cbc

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3
Q

Hct >30-35%

symptoms

A

none

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4
Q

Hct 25-30% sx

A

dypsnea (worse on exertion), fatigue

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5
Q

hct 20-25% sx

A

lightheadedness and angina

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6
Q

hct under 20-25% sx

A

syncope and chest pain

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7
Q

what kills you in anemia

A

cardiach ishcemia

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8
Q

anemia

mean corpuscular volume

A

although cbc establishes the presence of anemai mcv si the first clue to the etiology

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9
Q

anemia

low mcv causes

A

microcytosis:

iron deficinecy

thalassemia

sideroblastic anemia

anemia of chronic disease

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10
Q

low mcv overvies

A

microcytic anemias generally have a low reticulocye count. mos t causes of microcytosis are production problems. production problems are nearly synonymous with low retic counts

only alpha thalaseemia with 3 genes deleted has an elevated reic count

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11
Q

low mcv smear

A

doesnt tell you diference between the types of microcytosis bc they will all be hypchroic and can all have target cells

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12
Q

anemia

macrocytic anemia causes

A

low mcv from:

b12 and folate dieficiency

siderblastic anemia

alcoholism

antimetabolite meds such as asathioprine 6 mercaptopurin or hydroxyurea

liver dz or hypothyroidism

zidovudine or phenytoin

myelodysplastic syndrome

cold agglutinins can elevefate mcv falesly by clumping the cells

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13
Q

sideroblastic anemia can be

A

macro or microcytic

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14
Q

macrocytic anemias all give

A

a low reticulocyte count

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15
Q

blod loss and hemolysis will raise the

A

reticulocyte count

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16
Q

anemia

normocytic overview

A

acute blood loos or hemolysis can give a drop in hct so rapid that there is no time for mcv to change. blood loss ultimatley leads to iron deficiency and microcytosis. eventually hemolysis will increase reitc count and this will raise the mcv since reiculocytes are slitghtly larger than normal cells

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17
Q

methotrexate and ra

anemia

A

methotrexate causes macrocytic anemia

ra causes monocytic anemia

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18
Q

anemia

treatment

A

if severe treate with packed red blood cells

is the pt symptomatic then transufes

is the hct very low in an elderly pt or in a pt with heart disease, then transfuse

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19
Q

very low hct in the elderly or those with heart disease

A

25-30

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20
Q

sypmtomatic from aneami

A

sob

lighheaded confused and sometiems syncope

hypotension and tachycardia

chest pain

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21
Q

use ifa deificient donor ffp for

A

iga deficienty receipients

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22
Q

packed red blood cells

A

a concentrated form of glooc. ths blood product is a unit of whole blood with about 150ml of plasma removed. the hct of prbs is about 70-80% bc of the removeal plasma

each unit of prbcshould reaise the hct by 3 ponts per unit or 1g/dl of hg

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23
Q

Fresh frozen plasma

A

rplaces clotting factors in those with an elevated prothromibni time, actiaved parital thromboplastin time or inr and bleeding

ffp is sued as replacement with plasmapheresis

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24
Q

when is ffp not used

A

hemophili a or b

vonwillebrand disease

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25
Q

cryoprecipitate

A

used to replace fibrinogen and has some utility in disseminated intravascular coagulation. it provied high amounts of clotting factors in a smaller palsma volume. high levels of factor 8 and vwf are found in it

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26
Q

platelets

A

pooled from donations of mulitple donors. give to bleeding pt if platelet count is <50000. platelet infusion is ci in TTP

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27
Q

when is whole blood correct

A

never it is divided into prbc or ffp

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28
Q

Microcytic Anemia

definition/ etiology

A

mcv that is lower than 80 fl

most common causes are:
   iron deficiency
   chronic disease
   sideroblastic anemia
   thalassemia
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29
Q

Microcytic Anemia

iron deficiency

A

caused by blood loss

only need 1 to 2 mg per day

menstruating women need 2-3 mg per day

pregnant women need 5-6 per day

duodenum can absorb about 4 mg per day, which is why even a little bit of blood loss every day will lead to iron deficiency over time

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30
Q

Microcytic Anemia

chronic disease

A

includes any form of cancer or chronic infection

iron is locked in storage or trapped in macros or in ferriting

hv synthesis will not occur bc the rion just does not moved forward

precise mechanism is clear only in renal faulure in which there is a dericiency or erythropoietin

initially mcv is normal and then dexreases

hepcidin regulated iron absorption and its levels are low in anemai of chornic disease

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31
Q

Microcytic Anemia

sideroblastic anemia

A

can be macrocytic also when it is associaed with myelodysplasia, a preleukemic syndrome

most common cause is alcohols suppressive effect on the bone marrow

less common causes are lead poisoning, inh and b6 deificncy

reuslts fromt eh inability of iron to be incorporated with heme

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32
Q

Microcytic Anemia

thalassemia

A

extremely common cause of microcytosis. mos tpts with thalassemia trait alone are asymptomatic

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33
Q

Microcytic Anemia

presentation/what is the most likely dx

A

cant tell from sx but hx might give you a clue

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34
Q

Microcytic Anemia
what is the most likely dx

blood loss (gi bleeding)

A

iron deficiency

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35
Q

Microcytic Anemia
what is the most likely dx

mensturation

A

iron deficiency

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36
Q

Microcytic Anemia
what is the most likely dx

cancer or chornic infection

A

chronic disease

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37
Q

Microcytic Anemia
what is the most likely dx

ra

A

chronic disease

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38
Q

Microcytic Anemia
what is the most likely dx

alcoholic

A

sideroblastic

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39
Q

Microcytic Anemia
what is the most likely dx

asymptomatic

A

thalassemia

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40
Q

Microcytic Anemia

smear

A

no useful bc all cuases are hypochromic and can have target cells

target cells are most common with thalassemia

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41
Q

Microcytic Anemia diagnostic tests

iron deficiency

A

a low ferritin is extremely specific

nearly 33% of all pts ahvce an increased ferritin bc it is an acute phase reactant

low serum iron level and an increase in total iron binding capacity (TIBC) (undbound sites on transferrin)

iron divided by tibc equals tranferring saturation

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42
Q

Microcytic Anemia diagnostic tests

chronic disease

A

the srum iron is low in circulation bc iron is trapped in storage

ferritin (stored iron) is elevated or normal

TIBC is LOW

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43
Q

Microcytic Anemia diagnostic tests

sideroblastic anemia

A

only form of microcytic aneami in which the circulating iron level is elevated

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44
Q

Microcytic Anemia diagnostic tests

thalassemia

A

genetic disease with normaml iron studies

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45
Q

chronic renal failure gives you

A

normocytic anemia

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46
Q

Microcytic Anemia unique lab features

Iron deficiency

A

the red cell distribution of width (RDW) is increased bc the newer cells are more iron deficienty and smaller. as the body runds out of iron, the newer cells have less hb and get progressively smaller

elevated platelet count

most accuarte test is a bone marrow biopsy for stainable iron which is decreased (rarely done but most accurate)

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47
Q

Microcytic Anemia unique lab features

sideroblastic anemia

A

prussion blue staining for ringed sideroblasts is the most accurate test

basophilic stipp;ing can occur in any cause of sideroblastic anemia

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48
Q

Microcytic Anemia unique lab features

thalassemia

A

hb electrophoresis is the most accurate test. for alpha thalassemia genetic studeis are the most accurate test

only 3 gene deletion alpha halassemia is assocaited wtih hb h and an increased retiulocyt count

all forms have a normal RDW

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49
Q

how is alpah thalassemia diagnosed

A

dna analysis

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50
Q

Alpha thalassemia

A

one gene dleleted: normal

two genes deleted: mild anemia, normal electrophoresis

3 genes deleted: moderate aneami with hb h, which are beta 4 tetrads, increased retics

4 genes deleted: gamma 4 tetrads or hb Bart. chf causes death in utero

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51
Q

Beta thalassemia

A

one gene deleted: incrased hb f and a2

2 genes delted: n/a

3 genes deleted: beta thalasseami intermedia
normal hb f
no transusion dependence

4 gene delted: n/a

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52
Q

only 3 gne deltion alpha talassemia has high

A

reticulocytes

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53
Q

Microcytic Anemia treatment

iron deficiency

A

replace iron with oral ferrous sulfate. if this is insufficient pts may be treated iwth im iron

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54
Q

Microcytic Anemia treatment

chronic disease

A

correct the underlying disease. only the anemia assocatied with end stage renal failure routinely responds to epo replacement

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55
Q

Microcytic Anemia treatment

siderblastic anemia

A

correct the cause. may give b6 or pyridoxine prelacement

inh can lead to this

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56
Q

Microcytic Anemia treatment

thalassemias

A

trait is not treated

beta major (cooley anemia) is managed with chronic transusion for life

iron overload is managed with deferasirox or deferiprone (oral iron chelators)

derexoamine is a parenteral version of an iron chelator

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57
Q

Macrocytic Anemia

initial test

A

get a smear first to detect hypersegmented neutros then get a b12 and folate levels

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58
Q

megaloblastic anemia is the presence of

A

hypersegmented neutrophils. many factors rease the mxv but only b12 and folate deficiency and antimetabolite meds cause hypersegmentation

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59
Q

Macrocytic Anemia

Vit b12 deficiency is cuased by:

A

pernicious anemia
pacreatic insufficieny
dietary deficiency (vegan/strict vegetarian)
chron dz, celiac, tropical spru, radiation, or any dz damaging the terminal ileum
blind loop syndrome (gastrectomy or gastric bypass for weigh tloss)
diphyllobothrium latum, hiv

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60
Q

Macrocytic Anemia

folate deficiency is caused by:

A

dietary deficicency (goats milk has not folate and provieds only limited iron and b12)

psoriasis and skin loss or turnover

phenytoin and sulfa

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61
Q

celiac dz causes

A

b12 folate and iron deficiency

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62
Q

what drug in ra cuases folate deficiency

A

methotrexate

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63
Q

Macrocytic Anemia

what is the most likely dx/presentation

A

alcohol can give macrocytic anemia and leuro problems it will not give hypersegmented neutros

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64
Q

b12 deficiency eruo abnormality

A

can give any neruo abnormality but peripheral neuropathy is the most common

dementia is the elast common

posterior column damage to position and vibratory sensation or subacute combined degeneraion of the cord is classic

look for ataxia

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65
Q

Macrocytic Anemia

diagnostic tests

A

megaloblastic anemia

increased ldh and incrased indiret bilirubin levels

decreased reticulocyte count

hypercellular bone marrow

macroovalocytes

increased homocysteine levels

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66
Q

b12 and folate deficiency on smear

A

are identical

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67
Q

only b12 deficiency is associated with

A

increased methylmalonic acid level

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68
Q

b12 deficiency mechanism

A

high ldh + high bilirubin + low retics = ineffective erythropoiesis

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69
Q

b12 and folate both increase

A

homocysteine levels

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70
Q

Tested facts about macrocytic anemia

A

schilling test is never the right answer

pernicious anemai is confirmed with antiintrinsic factor and antiparietal cell antibodies

red cells are destroyed as the leave the marrow so the reticulocyte count is low

b12 and folate deficiency can cause pancytopenia as well as macrocytic anemai

pancreatic enzymes are needed to absorbb12 they free it from carrier proteins

neurological abnormalities will improve as long as they are minor (peripheral) and of short duration

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71
Q

Macrocytic Anemia

treatment

A

replace what is deficient

folate replacement corrects the hematologic problems of b12 deficiency butno the neuro problems

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72
Q

b12 can give either

A

hematological or neurological abnormlaities alone it does not give both at the same time

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73
Q

what is a compication of b12 replacement

A

hypokalemia, bc of rapid cell production you use it up

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74
Q

what removes b12 from the r protein so it can bind with intrinic factor

A

pancreateic enzymes

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75
Q

Hemolytic anemia

can lead to

A

sudden diseacrea in hct

increaseed levels of LDH, indirect bilirubin, and reticulocytes

decreased haptoglobin level

slight increase in mcb bc reticulocytes are larger than normal cells

hyperkalemaifrom cell breakdown

folate deificiency from increased cell production using it up, folate stores are limited

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76
Q

chronic hemolysis is associated with

A

bilirubin gallstones

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77
Q

Sickle Cell Disease

definition

A

chronic, usually well-compensated hemolytic anemia with a reticulocyte coutn that is always high

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78
Q

Sickle Cell Disease

acute panful vasoconstrictor crisis is caused by

A

hypoxia

dehydration

infection/fever

cold temperatures

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79
Q

Sickle Cell Disease

what is the most likely diagnosis

A

look for an african american pt with sudden, severe pain in the chest backa nd thighs that may be acopmanied by fever

it is rare for an adult to present with an acute crisis without a clear hx of sickle cell dz

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80
Q

Sickle Cell Disease

common manifestations

A

bilirubin gallstones from chornically elevated bilirbuin levels

increased infection from autosplenectomy, particularly encapsulated organisms

osteomyelitis, most commonly from salmonella

retinopathy

stroke

enlarged heart with hyperdynamic features anda systolic murmur

lower extremity skin ulcers

avascular necrosis of the femoral head (xray is the first test, mri is most accurate)

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81
Q

Sickle Cell Disease

how do children present

A

with dactylitis (inflammation of fingers)

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82
Q

Sickle Cell Disease

kidney

A

papillary necrosis of the kidney happens from chronic kidney damage

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83
Q

Sickle Cell Disease

best initial test

A

peripheral smear

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84
Q

Sickle Cell Disease

most accurate test

A

hemoglobin electrophoresis

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85
Q

does sickle cell trait (AS) give sickled cells on smear

A

no

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86
Q

what lowers mortality in sickle cell disease

A

hydroxyurea in prevention

antibiotics with fever

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87
Q

why can you see howell-jolly bodies on peripheral smear in Sickle Cell Disease

A

bc they are seen in pts who dont have a spleen

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88
Q

morulae

A

seen inside neutrophils in ehrlichia infections

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89
Q

when do you see nucleated red blood cells

A

with premature release of precursor blood cells

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90
Q

Sickle Cell Disease

treatment (5)

A
  1. begin with oxygen/hydration/nalagesia
  2. if fever or a white cell count higher than usual is present, then abs are given. use ceftriaxone, levofloxacin, or moxifloxacin
  3. folic acid replacement is necessary on a chornic basis
  4. give pneumococcal vaccination bc of autosplenectomy
  5. hydroxyurea prevents recurrecnces of sickle cell crises by increasing hemoglovin F
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91
Q

dont waite for reults of testing to start abs if there is a fever with Sickle Cell Disease

A

the absence of a functional spleen leads to overwhelming infection

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92
Q

Sickle Cell Disease

exchange tranfusion is used if there is severe vasoocclusive crisis presenting with

A

acute chest syndrome

priapism

stroke

visual disturbance from retinal infarction

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93
Q

Aplastic crisis

A

pts with sickle cell dz usually have very high reticulocyte counts bc of the chronic copmensated hemolysis. parvovirus b19 causes an aplastic crisis which freezes the rought of the marrow. nothing will be visible on blood smear.

the bone marrow will show fiant pronormoblasts this would not be done routinely and is never the initial test

the first clue to parvovirus is a sudden drop in reticulocyte level

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94
Q

Hereditary Spherocytosis

etiology

A

this is a defect in the cytoskeleton of the red cell leading to an abnormal round shape and loss of the normal flexibility characteristic of the biconcave disk that allows red cells to bend in the spleen

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95
Q

Hereditary Spherocytosis

what is the most likey dx

A

recurrent episodes of hemolysis

intermittent jaundice

splenomegaly

family hx of anemia or hemolysis

bilirubin gallstones

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96
Q

Hereditary Spherocytosis

diagnotstic tests

A

low mcv

increased mean corpuscular hemoglobin concentration (MCHC)

negative coombs test

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97
Q

Hereditary Spherocytosis

most accurate test

A

osmotic fragility. when cells are placed in a slightly hypotonic solution, the increased swelling of hte cells leads to hemolysis

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98
Q

Hereditary Spherocytosis

treatment

A

chronic folic acid replacement supports red cell production

splenectomy stops the hemolysis but does not eliminate the spherocytes

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99
Q

Autoimmune (warm or igg) hemolysis

etiology

A

fifty percent of cases have no identified etiology clear cases are causes by:

chronic lymphocytic leukemia (CLL)

lymphoma

systemic lupus erythematous (SLE)

drugs: penicillin, alpha-methyldopa, rifampin, phenytoin

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100
Q

Autoimmune (warm or igg) hemolysis

diagnostic tests

A

most accurate test is the coombs testh, which detects igg antibody on the surgace of the red cells. the direct an dindirect coombs coombs tests tell basically the same thing, but the indirect test is associated with a greater amount of antibody

autoantibodies remove small amounts of red cell membrane and lead to a smaller membrane, forcing the cell to become round. biconcave disks need a greter surface area than a sphere. autoimmune hemolysis is assocaited with microspherocytes

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101
Q

autoimmune hemolysis is associated with

A

spherocytes

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102
Q

why does a smear not show fragmented cells in autoimmune hemolysis

A

bc the red cell destruction occursin side the psleen or liver not in the blood vessel

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103
Q

Autoimmune (warm or igg) hemolysis

treatment (4)

A
  1. glucocorticoids such as prednisone
  2. recurrent erpisodes respond to splenectomy
  3. severe, acute hemolysis not responding to prednisone is controlled with ivig
  4. rituximab, azathiprine, cyclophosphamide, cyclosporine is used when splenectomy does not control hemolysis
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104
Q

Autoimmune (warm or igg) hemolysis

alternate treatments to diminish the need for steroids in general are:

A

cyclophosphamide

cyclosporine

azathioprine

mycophenolate mofetil

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105
Q

Cold agglutinin disease

definition/etiology

A

coldagglutinins are igm antibodies against the red cell developing in association with epstein-barr virus, waldenstrom macroglobulinemia or mycplasma pneumonia

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106
Q

Cold agglutinin disease

presentation

A

sx occcur in colder parts of the body such as nubmness or mottling of ht enose, ears, fingers, and toes. sx resolve on warming up the body part

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107
Q

Cold agglutinin disease

diagnostic tests

A

the direct coombs test is positive only for compleemnt. the smear is normal or may show only spherocytes. cold agglutinin titer is themost accurate test

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108
Q

Cold agglutinin disease

treatment

A
  1. stay warm
  2. administer rituximab and sometimes plasmapheresis
  3. cyclophosphamide, cyclosporine, or other immunosuppressive agents stop the production of the antibody
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109
Q

most common wrong answer in Cold agglutinin disease

A

prednisone is the most common wrong answer, dont do steroids or spleenctomy

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110
Q

cryoglobulines are often mixed up with cold agglutinins. although both are igm and do not respond to steroids, cryoglobulins are associated with

A

hepatitis c

joint pain

glomerulonephritis

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111
Q

Glucose 6 phosphate dehydrogenase deficiency

etiology

A

x linked recessive disorder leading to an inability to generate gluathione reductase and protect the red cells from oxidatn stress. the most common oxidant stress is infection. other causes are dapsone, quinidine, sulfa drugs, primaquine, nitrofurantoin, and fava beans

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112
Q

Glucose 6 phosphate dehydrogenase deficiency

what is the most likely dx

A

look african american or mediterranean men with sudden anemia and jaundice who have a nomral sized spleen with an infection or are using one of the drugs previously listed

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113
Q

Glucose 6 phosphate dehydrogenase deficiency is almost exclusively in

A

men

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114
Q

Glucose 6 phosphate dehydrogenase deficiency

diagnostic tests

A

best initial test is form heinz boeids and bite cells, the g6pd level will be normal after a hemoytic event

the most accurate test is the g6pd level after waiting 1 to 2 months after an acute episode of hemolysis

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115
Q

what stain shows heinz bodies

A

methylene blue

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116
Q

Glucose 6 phosphate dehydrogenase deficiency

treatment

A

nothing reverses the hemolysis, aboid oxidant stress

117
Q

Hemolytic Uremic Syndrome and Thrombotic Thrombocytopenic Purpura

A

they are different versions of hte same basic disease cuased by deficiency of metalloproteinase ADAMTS 13.

118
Q

HUS is assocaited with

A

E Coli 0157h7 and more frequent in children

119
Q

TTP is associated with

A

ticlopidine clopidogrel cyclosporine AIDS and SLE

neurological disorders and fever and is more common in adults

neurological sx include confusion and seizures

120
Q

Hemolytic Uremic Syndrome and Thrombotic Thrombocytopenic Purpura

are characterized by

A

intravasuclar hemolysis with fragmented red cells (schistocytes)

thrombocytopenia

renal insufficinecy

121
Q

Hemolytic Uremic Syndrome and Thrombotic Thrombocytopenic Purpura

diagnostic test

A

no specific test

normal pt/aptt and negative coombs tests

122
Q

Hemolytic Uremic Syndrome and Thrombotic Thrombocytopenic Purpura

treatment

A

plamapheresis or plasma exhange

steroids if not related to drugs or diarrhea

123
Q

Hemolytic Uremic Syndrome and Thrombotic Thrombocytopenic Purpura

if there is a delay to plasmapheres

A

infuse ffp

124
Q

why dont you transfuse platelets into pts with hus or ttp

A

bc they make the disease worse

125
Q

Paroxysmal Nocturnal Hemoglobinuria

etiology

A

a clonal stem cell defect with increased sensitivity of red cells to complement in acidosis. this is from deficiency of the complement regulatory proteins CD55 and 59 also known as decay accelerating factor. the gene for phosphatidylinositol class a (PIGA) is defective. this leads to overactibation of the complement system . this does nothing to an unaffected person but in pnh it leads tohemolysis and thrombosis

126
Q

Paroxysmal Nocturnal Hemoglobinuria

presenation/ what is the most likely dx

A

episodic dark urine

pancytopenia and iron deficiency anemia

clots in unusual places (not just DVT or pulmonary embolism)

127
Q

most common cause of death in Paroxysmal Nocturnal Hemoglobinuria

A

thrombosis

128
Q

Paroxysmal Nocturnal Hemoglobinuria

diagnostic tests

A

cbc often shows pancytopenia in addtion to anemia. the momst accurate test is a decreased level of CD55 and CD59. the ham test and the sucrose hemolysis test are obolete. flow cytometry is another way of saying cd55/cd59 testing

129
Q

Paroxysmal Nocturnal Hemoglobinuria

treatment (4)

A
  1. prednisoneis hte best initial therapy for hemolysis. the mechanism is not clear
  2. allogeneic bone marrow trasplant is the only method of cure
  3. eculizumab inactivates c5 in the com;lement pathway and decreases red cell destruction. complement overactivation is the mechanism of pnh. eculizumab is for hemolysis and thrombosis
  4. give folic acid and replacement with transfusions as needed
130
Q

pnh is a stem cell defect that

A

may cause aplastic anemia, myelodysplasi or acute leukemia

131
Q

most common site of thrombosis in Paroxysmal Nocturnal Hemoglobinuria

A

large vessel thrombosis of the mesentreic and hepatic veins

132
Q

Aplastic Anemia

Definition

A

aplastic anemia is pancytopenia of unclear etiology

any infection or cancer can invade the bone marrow causing decreased production or hypoplasia

133
Q

Aplastic Anemia

etiology

A

radiation and toxins such s toluene, insecticides (DDT), and benzen

drug effect: sulfa, phenytoin, carbamazepine, chloramphenicol, alcohol chemo

sle

pnh

infection: hiv, hepatitis, cmv, ebv

b12 nd folate deficiency

thyroid inhibiting medications such as ptu and methimazole

134
Q

Aplastic Anemia

presentation

A

pts present with th fatigue of anemia, infections from low htie cell counts, and bleeding from thrombocytopenia

135
Q

Aplastic Anemia

diagnostic tests

A

confimred by excluding all cuases of pancytopenia

most accurate test is a bone marrow biopsy

136
Q

Aplastic Anemia

Treatment

A

transfusion for anemia, abs for infection, and platelets for bleeding

allogeneic bone marrow tranplantation

when the bt is older (above 50), or there is no donoruse anithymocyte globulin and cyclosporine

tacrolimus is alternative to cyclosporine

alemtuzumab is an anticd52 that suppresses t cells

137
Q

aplastic anemia acts as an autoimmune disorder in which the T cells

A

attack the pts own marrow. treatment is based on meds like cyclosporine that inhibit t cells. this brinkg sthe marrow back to life

138
Q

Polycythemia Vera

Definition

A

the unregulated overproduction of all 3 cell lines, but red cells overproduction is the mos tprominent. there is a mutation in the JAK2 protein which regulates marrow production. the red cells grow wildly despite a low erythropoeitin level

139
Q

Polycythemia Vera

wha tis the most likely dx

A

headache, blurred vision, and itnnitus

htn

fatigue

splenomegaly

bleeding from enjorged blood vessels

thrombosis from hyperviscosity

140
Q

Polycythemia Vera

pruritus

A

often follows warm showers bc of histamine release from increased numbers of basophils

141
Q

Polycythemia Vera

diagnostic tests

A

hct is elevated above 60%, platelets and wbc are elevated also

must exclude hypoxia as a cause of erythrocytosis

total rec cell mass is elevated

oxygen levels are normal

epo levels are low

B12 is elevated

iron levels are low bc you have so many red cells

142
Q

Polycythemia Vera

most accurate test

A

jak2 mutation

found in 95% of pts

143
Q

Polycythemia Vera

convert to

A

AML

a small number of them, basophils can be up

144
Q

Polycythemia Vera

treatment

A
  1. phlebotomy and aspirin prevent thrombosis
  2. hydroxyurea helps lower the cell count
  3. allopurinol or rasburicase protects against uric cid rise
  4. antihistamines
145
Q

Polycythemia Vera

target hct

A

45%

146
Q

Polycythemia Vera

vs

Renal cell cancer

A

both have elveated hct but the epo level is elevated with kidney cancer

147
Q

Polycythemia Vera

mcv

A

low

148
Q

platelet counts eleveate temporalily after

A

splenectomy

149
Q

Essential Thrombocytosis

A

markedly elevated platelet count above one million leading to both thormbosis and bleding. essential thrombocytosis can be very difficult to distingush from an elevated paltelet count as a reaction to another stress such as infection, cancer, or iron deficiency

150
Q

ruxolitinib

A

inhibits jak2

also suppresses myelofibrosis

151
Q

jak2 is found in

A

50% of ET cases

152
Q

Essential thrombocytosis

treatement

A

if pt isunder age 60 and asymptomatic with a platelet count under 1.5 million no treatment is necessary

if pt is above 60 and there are thromboses or platelet count is 1.5 million begin treatment

153
Q

Essential thrombocytosis

best initial therapy

A

hydroxyurea

anagrelide is used whe there is red cell suppression from hydroxyurea

aspirin is used for erythromelalgia

154
Q

Myelofibrosis

definition

A

disease of older persons with a pancytopenia associated with a bone marrow showing marked fibrosis

blood production shifts tot eh spleen and live rwhich become amrkedly enlarged

155
Q

Myelofibrosis

dx

A

look for teardrop shaped cells and nucleated red blood cells on blood smear

156
Q

Myelofibrosis

treatment

A

thaldiomide and lenalidomide are tumor necrosis factor inhibitore sthat inrease bone marrow production

if under 5 do allogneneic bone marrow transplant

157
Q

Acute Leukemia

presentation

A

signs of pancytopenia:
fatigue
infection
bleeding

wbc count is normal or increases

infection is common bc leukemic blasts do not function normally

158
Q

Acute Leukemia

most frequently tested

A

M3 or acute promyelocytic leukemia

associated with dic

no clincial difference in all of the all leukemias (3 types)

159
Q

Acute Leukemia

best initial test

A

blood smear showign blasts

160
Q

Acute Leukemia

most accurate test

A

flow cytometry which will distingush the different ssubtypes of acute leukemia

so you can find the specific cd sybtypes

161
Q

Acute Leukemia

myeloperoxidase

A

characteristic of acyte myelocytic leukemia (AML)

162
Q

Acute Leukemia

look for a hx of

A

myelodsyplastic syndrome

163
Q

m3 is associated with translocation between

A

15 and 17

164
Q

Auer rods

A

esosinophilic inclusions associated with aml. m3 or acute promyelocytic leukemia os mos tcommonly associated with auer rods

165
Q

Acute Leukemia

treatment

A

both aml and all are treated with chomto remove blasts form the peripheral blood smear, this is known as remission

166
Q

Acute Leukemia

if prognosis is poor

A

the proceed directly to bmt

167
Q

Acute Leukemia

if prognosis is good

A

give more chemo

168
Q

Best indicater of prognosis in acute leukemia is cytogenetics

A

specific chromosomal characteristicds found in each pt

169
Q

Acute Leukemia

treatement of m3

A

add all trans retinoic acid

170
Q

wha tdo you add to all treatment

A

intrathecal chom like methrotrexate to prevent relapseof all in the cns

171
Q

m3 gives

A

dic

172
Q

add atra to

A

m3

173
Q

auer rods=

A

aml

174
Q

add intrathecal methotrexate to

A

all

175
Q

rasburicase prevents

A

tymor lysis related rise in uric acid

176
Q

Chronic Myelogenous Leukemia

what is the most likely dx

A

persistnely high wbc that is all neutros

pruritus is common after showrs from histamine release from basophils

splenomegaly presents with early saitety abdominal fullness and luq pain

can present with vague sx of fatigue night seats and fever from hpyermetabolic syndrome

high wbc on routine exam

177
Q

Chronic Myelogenous Leukemia

diagnostic tests

A

after hgih neutrophil count is found, you must determine if it is a reaction to another infection or stress leukemoid rxn, or genuinely represents a leukemia

may find blasts but should be under 5% in CML

178
Q

Chronic Myelogenous Leukemia

what is the most accurate test

A

BCR-ABL, done by pcr or fish

179
Q

BCR-ABL

A

9:22 translocation=philly chormosome in 95% of cases

180
Q

Chronic Myelogenous Leukemia

treatment

A

tyrosine kinase inhibitors such as imatinib dastinip or nilotinib are the best initial therapy

only a bmt can cure cml but this should never the first therapy (it is the most effective cure

181
Q

CML ahs the greatest likelihood of all myeloproliferative disorders to transform into

A

acute leukemia (blast crisis) if cml is untreated 20% turn into this within 1 year

182
Q

in an acute leukostasis rxn

A

do leukapharesis first

183
Q

Myelodysplastic Syndrome

Definition

A

mds is a preleukemic disorder presenting in older pts with a pancytpenia despite a hypercellular bone marrow

most pts never develop aml bc complications of infection and lbeeding lead to death before leukemia occurs

184
Q

Myelodysplastic Syndrome

presentation

A

asymptomatic pancytopenia on routine cbc

fatigue and weight loss

infection

bleeding

splenomegaly

no single pathognomonic finding in the hx or physcial examination

185
Q

Myelodysplastic Syndrome

diagnostic tests

A

CBC: aneiam with ain increased mcv, nucleated red ells, and a small number of blasts

hypercellular marrow

prussion blue stain shows ringed sideroblasts

severity is based on the percentage of blasts

5q deletion has an excellent response to lenalidomide

186
Q

Myelodysplastic Syndrome

treatment

A
  1. transfusion: support with blood products as needed
  2. epo gives about 20% response
  3. azaxitidine or decitabine
  4. lenalidomide for those with the 5q election can decrease transfusion dependence
  5. bone marrow transplant under age 50
187
Q

azacitidine decreases

A

transfusion dependence and increases survival in MDS

188
Q

pelger -huet cells are the

A

most distinct lab abnormality in mds (bilobed nucleus)

189
Q

5q deletion

A

the characteristic abnormality in mds, pts with 5q have a better prognosis than thosewithout it

190
Q

Chronic Lymphocytic Leukemia

presentation

A

can be asynptomatic at presentation with only an elevated wbc count

most common sympto is fatigue

lymphadneopathy (80%)

spleen or liver enlargement (50%)

infection from poor lymphocyte function

hemolysis sometimes

191
Q

Chronic Lymphocytic Leukemia

definition

A

a clonal proliferation of normal, mature-appearing B lymphocytes that function abnormally, occurs ove the age of 50 in 90% of those affected

192
Q

physical findings in cll

A

nothing unique

193
Q

richter phenomenon

A

conversion of cll into high-grade lymphoma, happens in 5% of pts

194
Q

smudge cell

A

lab artifact in which the fragile nucleus is crushed by the cover slip

195
Q

CLL

treatment

A

for stage 0 (elevated WBC), stage 1 (lymphadenopahty, and stage II (hepatosplenomegaly), there is no treatment

Stage III (anemia) and stage IV (thrombocytopenia) are treated with gludarabine, cyclophsophamide, and rituximab

if there is a choice that lists fludarabine, cyclophosphamide and rituximabe then this is the best initial therapy for advanced stage 3 and 4 or any pt who is symptomatic (severe fatigue, painful nodes)

alemtuzumab (anticd52) is used when fludarabine fails.

196
Q

for refractory cases of cll

A

cyclophosphamide (more efiicacy but more toxic)

197
Q

for mild cases of cll

A

chlorambucil (elderly)

198
Q

for severe infection

A

iv immunoglobulin

199
Q

autoimmune thrombocytopenia or hemolysis

A

prednisone

200
Q

Hairy Cell Leukemia

presents with

A

middle-aged men with:

pancytopenia
massibe splenomegaly
monocytopneia
inspirable “dry” tap desptie hypercellularity of the marrow

201
Q

Hairy Cell Leukemia

best initial test

A

smear showing hairy cells

202
Q

Hairy Cell Leukemia

most accurate test

A

immunoptyping by flow cytometry (CD11)

203
Q

Hairy Cell Leukemia

treat with

A

cladribine or pentostatin

204
Q

in ahir clel leukemia b cells are seen on smear

A

with filamentous projections

205
Q

Non-Hodgkin Lymphoma

Definition

A

is a proliferation of lymphocytes in the lymph nodes and spleen. nhl is most often widespread at presentation and can affect any lymph node or organ that has ;ymphoid tissue. nhl and cll are extrmely similar, but nhl is a solid mass and cll is liquied or circulating

206
Q

Non-Hodgkin Lymphoma

presentation

A

painlessl ymphadenopathy

may involve pelvic, retroperitonieal or mesenteric structures
nodes not warm red or tender
b symptoms: fever, weight loss, drenching night sweats

207
Q

Non-Hodgkin Lymphoma

best initial test

A

excisional biopsy

208
Q

Non-Hodgkin Lymphoma

diagnostic tests

A

cbc is normal in most cases

high ldh levels correlatee with worse severity

staging determiens the intnesity of therapy

statign ngl does not often change treatment bc in most causes disease is weidpsread

209
Q

Non-Hodgkin Lymphoma

staging steps

A

ct scan of the chest, abdomen, and pelvis

210
Q

infection not Non-Hodgkin Lymphoma gives nodes that are

A

warm red and tender

211
Q

Non-Hodgkin Lymphoma

stages

A

stage 1: 1 lymph node group
stage 2: 2 or more lymph node groups on the same side of the diaphragm
stage 3: both sides of the diaphragm
stage 4: widesperad disease

212
Q

Non-Hodgkin Lymphoma

treatment of local disease

A

stage 1a and 2a

local radiation and small dose/course of chemo

213
Q

Non-Hodgkin Lymphoma

treatement of advanced disease

A

compination chemo with chop and riguximab (antibody against CD20)

c=cyclophosphamide
h=adriamycin (doxorubicin)
o=vincristine (oncovin)
p=prednisone

214
Q

Non-Hodgkin Lymphoma

presents in advanced stages in

A

80-90% of cases

215
Q

Mucosal associated lymphoid tissue

A

lymphoma of the stomach assocaited with h pyloi

treat with clarithromycin and amoxicillin

216
Q

Hodgkin Disease

A

same as nhl but has reed-sternberg cells on pathology

217
Q

difference between hodgins and nhl

stage

A

hodgkins - stage 1 and 2 in 80-90% of disease

nhl - stage 3 and 4 in 80-90%

218
Q

difference between hodgins and nhl

locations

A

hodgkin - centers around cervical area

nhl - disseminated

219
Q

difference between hodgins and nhl

pathology

A

hodgkin - reed sternberg cells

nhl - no reed sternberg cells

220
Q

difference between hodgins and nhl

pathological classification

A

hodgkin - lymphocyte predominate has the best prognosis

lymphocyte depleted has the worst prognosis

nhl - burkitt and immunoblastic have the worst prognosis

221
Q

hodgkin disease

treatment stage 1a and 2a

A

local radiation and a small course of chemo

222
Q

hodgkin disease

treatment of stage 3 and 4

A

abvd

a=adriamycin (doxorubicin)
b=bleomycin
v=vinblastine
d=dacarbazine

223
Q

hodgkin disease

relapses ater radiation therapy

A

are treated with choemo. relapses after chom are treated with extra high dose chemo and bone marrow transplantation

224
Q

hodgkin disease

complications of radiation and chemo

A

radiation increases the risk of solid utmors such as breast, thyroid, or lung cancer. screening for breast cancer is recommended 8 years or more after treatmeent. radiation increases the chance of premature coronyar artery disease, the risk of acute leukemia, mds, and nhl as a compication of chemo is about 1% per year

225
Q

hodgkin disease

how to determine the dosing of chemo

A

uga or nuclear ventriculogram bc adriamycin or doxorubicin is cardiotoxic

226
Q

hodgkin disease

just radiation

A

never

227
Q

doxorubicin ae

A

cardiomyopathy

228
Q

vincristine ae

A

neuropathy

229
Q

bleamycin ae

A

lung fibrosis

230
Q

cyclophosphamide ae

A

hemorrhagic cystitis

231
Q

cisplatin ae

A

renal and ototoxicity

232
Q

Multiple Myeloma

definition

A

abrnomal proliferation of plasma cells. these plasma cells are unregulated in their production of useless immunoglobulin tht is usually igg or iga. igm is aseparate disease called waldenstrom macroglobulinemia. these immunoglobulins do not fight infection but clof up the kidney

233
Q

Multiple Myeloma

what is the most likely dx

A

most common presnetation is bone pain from pathologic fractures. a pathologic fracture means that the bone breaks under what would be considered normal use. this is from osteoclast activating factor (OAF), which attacks the bone, causing lytic lesions. oaf is also the reason for hypercalcemia. infection is common bc the abnormal plasma cells do not make immunoglobulins that are effective against infections

234
Q

Multiple Myeloma

presentation

A

hyperuricemia: from increased turnover of the nuclear material of palsma cells
anemia: from infiltration of the marrow with massive numbers of plasma cells

renal failure: from accumulation of immunoglobulins and bene-joens protein in the kidney; hypercalcemia and hyperuricemia also damage the kidney

235
Q

most common cuases of death in Multiple Myeloma

A

renal failure and infection

236
Q

Multiple Myeloma

first test

A

xray of the affected bone that will show lytic punched out lesions

237
Q

Multiple Myeloma

diagnostic tests

A

serum protein electrophoresis (SPEP) shows and IGG of 60% or an iga of 25% spike of a single type or clone (called an m or monoclonal spike) fifteen percent have light chains or bence jones protein only

also have:
hypercalcemia
bnec-jones protein on urin immunoelectrophoresis
beta2 microglobulin levels correspond to severity of disease
smear with rouleaux
elevated bun and creatinine
bone marrow biopsy: greater than 10% plasma cells
elevated total protein with normal albumin

238
Q

myeloma has a decreased

A

anion gap, igg is cationic and increased ationic substances will increase chlride and bicarb levels, this decreases the anion gap

239
Q

rouleaux

A

form when the igg paraprotein sticks to the red cells, causing them to adhere to each other in a stack or roll

240
Q

is bence-jones protein detected on dipstick?

A

no

241
Q

Multiple Myeloma

most accurate test

A

bone marrow biopsy with grater tha n10% plasma cells

242
Q

Multiple Myeloma

treatment

A

best initial therapy is a combo of dexamethasone and lenalidomide bortezomib or both

melphalan is useful in older fragile prs who cannot toelrate adverse effects.

most effective terhapy in those under age 70 is an autologus bone marrow traonplasnt with stem cell support, this is used fater induction chemo therapy with enalidomide and steroids

243
Q

Monoclonal gammopathy of unknown significancy

A

igg or gia spikes on an spep are common in older pts

main issue is to evaluate with bone marrow biopsy to exclude myeloma, this iwll have a smaller number of plasma cells

no therapy

1% per year transorm into myeloma

amount of ig in the spike is the main correlate of risk for myeloma, more mgus more myeloma

244
Q

Waldenstrom Macroglobulinemia

definition

A

overproduction of igm from malignant b cells leading to hyperviscosity

245
Q

Waldenstrom Macroglobulinemia

presents with

A

lethargy

blurry vision and vertigo

engorged blood vessels in the eye

mucosal bleeding

raynaud phenomenon

246
Q

Waldenstrom Macroglobulinemia

tests

A

anemia is common

igm spike on spep results in hyperviscosit

no bone lesions

247
Q

Waldenstrom Macroglobulinemia

treatment

A

plasmapharesis is the best initial therapy to remove igm and decrease viscosity

long temr treatment is with rituximab or prednison cyclophosphamide

control teh cell cells that make the abnormal ig, decrease the means of production

use bortezomib or lenalidoide like in myeloma

248
Q

bleeding disorders

platelet bleeding overvies

A

superficial

epistaxis, gingival, petechiae, purpura, mucosal surgaces like gums and vaginal bleeding

249
Q

bleeding disorders

factor bleeding overvies

A

deep

joints and muscles

250
Q

Immune thrombocytopenic purpura

what is the most likely diagnosis

A

isolated thrombocytopenia (normal hct, normal wbc)

normal sized spleen

251
Q

Immune thrombocytopenic purpura

mild bleeding

A

so no intracranial or major gi bleeding

use prednisone

252
Q

Immune thrombocytopenic purpura

diagnostic tests

A

disgnosis of exlusion usually, can show:

antiplatelet antibodies lack specificity

us or ct to exclude hypersplenism

megakaryocytes are elevated in number

bone marrow not routine, indicated only before pslenectomy

increase in mean platelet volume

253
Q

Platelets are large in

A

ITP

254
Q

ITP Treatment

no bleeding count >30000

A

no treatment

255
Q

ITP Treatment

mild bleeding count <30000

A

glucocorticoids

256
Q

ITP Treatment

severe bleeding coutn <10000

A

ivig, antirho (anti-d)

257
Q

ITP Treatment

recurrent episodes and steroid dependant

A

splenectomy

258
Q

ITP Treatment

splenectomy or steroids not effectiv

A

romiplostin or elzombopag, rituximab azathioprine cyclosporine or mycophenolate

259
Q

romiplsotim and etlrombopag are synthetic

A

thrombopoietin for ITP

260
Q

before splenectom y vaccinate for

A

neisseria meningitidis
haemophilus influenzae
pneumococcus

261
Q

Von Willebrand Disease

definition

A

is the most common inherited bleeding disorder with a decrease in the level or functioning of Von Willebrand Disease. autosomal dominant

262
Q

Von Willebrand Disease

what is the most likely dx

A

look for bleeding related to paltelets (epistaxis, gingiva, gums) with a normal platelet count. Von Willebrand Disease is markedly worsened after the use of apsirin. the aptt may be elevated in half of the pts

263
Q

Von Willebrand Disease

diagnostic tests

A

vwf level may be decreased

ristocetin cofactor assay: detects vwf dysfunction also called vwf activity

factor 8 activity

bleeding time: increased duration (rarely done)

264
Q

Von Willebrand Disease

treatment

A

bets initial therapy is ddavp (demsopressin) which releases subendothelial stores of vwf. if there is no response use factor 8 replacement or vwf concentrated

265
Q

Hemophilia

presentation

A

look for delayed joint or muscle bleeding in a male child, since the condition is xlinked recessive. bleeding is delayed bc the primary hesotatic plug is with platelets

266
Q

hemophilia

tests

A

pt is normal and the aptt is prolonged

mixing sutdies with normal palsma will correct the aptt to rnomal

most accurate is a specific assay for factors 8 and 9

267
Q

hemophilia

treatment

A

treat mild cases with ddavp

severe blededing with replacement of specific factor

268
Q

Factor XI deficiency

A

most of the time thiere is no increase in bleeding with trauma or surery ther eis increased bleeding

look for a normal pt with a prolonged aptt, mixing study will correct the aptt to normal as occurs whenever there is a deficiency of clotting factors

uise ffp to stop bleeding

269
Q

Factor XII deficiency

A

these pts ahve an elevted aptt but there is no bleeding, there is no therapy needed

270
Q

Disseminated Intravascular Coagulation

bleeding is related to

A

clotting factor deficiency and throbocytopenia

271
Q

Disseminated Intravascular Coagulation

risk factors

A

sepsis

burns

placenta abruption

amniotic fluid embolus

snake bites

trauma resulting in tissue factor release

cancer

272
Q

Disseminated Intravascular Coagulation

diagnostic tests

A

elevation in both the pt and aptt

low platelet count

elevated ddimer and fibrin split products

decreased fibrinogen level (it has been consumed)

273
Q

Disseminated Intravascular Coagulation

treatment

A

if platelets are under 50000 and the pt has serious bleeding, replace platelets as well as clotting factors by using ffp

heparin has no definit benefit

cyroprecipitate may be effective to replace finbrinogen levels if ffp does not control bleeding

274
Q

Hypercoagulable States/Thrombophilia

overview

A

most common cause is factor V leiden mutation. there is no difference in the intensity of anticoaculation. use warfarin to an INR of 2 to 3 for 6 months

275
Q

the only thrombophilis improtant to test for with first clost is

A

antiphospholipid antibody syndrome

276
Q

HIT

A

more common wiht the use of unfractionated heparin, but can still occur with low moelcular weight heparin.

277
Q

HIT

presnetation

A

5 to 10 days after the start of heparin with a marked drop in platelet coutn (more than 30%). both venous and arterial thromboses can occur, benous clots are more common. rarely leads to bleeding. the platelet just precipitate out

278
Q

HIT

diagnostic tests

A

confirmed with elisa for platelet factor 4 antibodies or the serotonin release assay

279
Q

HIT treatment

A
  1. immediately stop all heparin containing products. you cnnot just witch unfractinated heparin to low molcular wieght heparin
  2. administer direct thrombin inhibitors: argatroban, lepirudin, bivalirudin, and fondaparinux
  3. warfarin should not be used first, but after a direct throbin inhibitor is started, use warfain, fondaparinux is safe
280
Q

platelet therapy in hit

A

dont do it it will only get worse

281
Q

apl i sthe one most likely to need lifelong

A

warfarin wiht only only clot

282
Q

Anti phospholipids syndromes

definition

A

2 main syndromes are lupus anticoagulatn and the anticardiolipin antibodies are associated with multiple spontaneous abortions.

283
Q

anticardiolipin antibodies are associated with

A

abortions

284
Q

Anti phospholipids syndromes

are the only cause of thrombophilis with

A

an abnormality in aptt

285
Q

Anti phospholipids syndromes

best initial test

A

mixing study bc it is a circulating inhibitor, ath aptt will remain elveated even after the ix

286
Q

Anti phospholipids syndromes

most accurate test for lupus anticoagulant

A

russell viper venom test

287
Q

Anti phospholipids syndromes

treatment

A

heprain and warfarin as you would for any cuase of dvt or pulmonary embolus. aply syndrome may require lifelong anticoag

288
Q

fondaparinux is safe in

A

hit

OMS III (21 decks)

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