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288 Cards in this Set

  • Front
  • Back
blood type:
universal recipients
AB+
blood type:
universal donor
O-
MCC of anemia
IDA
best initial test for anemia
CBC
symptoms when hematocrit is:
>30
none
symptoms when hematocrit is:
25-30
dyspnea
fatigue
symptoms when hematocrit is:
20-25
light headed
angina
symptoms when hematocrit is:
<20
syncope
what is the only anemia with high reticulocyte count
alpha thalassemia with 3 gene deletion
when should a px with anemia be transfused
symptomatic
very low hct in elderly
heart disease
what is meant by symptomatic in anemia
shortness of breath
light headed, confused, syncope
hypotension and tachycardia
chest pain
how much does each unit of packed RBC increase the Hct
3 points per unit
what should be suspected if a patients hematocrit does not rise after giving packed RBC
px is still bleeding
what is fresh frozen plasma used for
replace clotting factors
what is cryoprecipitate rich in
factor 7, 13, vW and fibrinogen
Dx:
anemia with RA
AOCD
Dx:
anemia with no symptoms
thalassemia
symptoms of lead poisoning
abdominal pain
arthralgia
peripheral neuropathy
gingival lead lines
what can cause lead poisoning in children
early or excess use of cows milk
serum iron, ferritin and TIBC in:
IDA
dec
dec
inc
serum iron, ferritin and TIBC in:
lead poisoning
N/inc
N
N
serum iron, ferritin and TIBC in:
AOCD
dec
N/inc
dec
serum iron, ferritin and TIBC in:
sideroblastic anemia
inc
inc
dec
serum iron, ferritin and TIBC in:
thalassemia
N/inc
N
N
MCC of siderblastic anemia
alcohol
what lab value can help suggest AOCD
inc ESR
how much iron is needed in:
normal person
1-2 mg
how much iron is needed in:
menstruating women
2-3
how much iron is needed in:
pregnant women
5-6
how much iron can the duodenum absorb in a day
4
what should always be done in px with IDA
check for occult blood in stool
anemia a/w:
low ferritin
IDA
anemia a/w:
high iron
sideroblastic anemia
anemia a/w:
normal iron studies
thalassemia
what is the next best test after microcytic anemia is determined by CBC
iron studies
what is the MCC of death by poison
iron
pathophys of iron toxicity
accumulates in mitochondria
causes lactic and citric acidosis
what does hepcidin do
inhibits absorption and mobilization of iron
symptoms of iron toxicity after:
3-6 hours
mucosal damage
symptoms of iron toxicity after:
6-72 hours
met acidosis
symptoms of iron toxicity after:
12-96 hours
hepatotoxicity
most accurate test for IDA
BM biopsy
what do reticulocytes stain with
methylene blue
most accurate test for sideroblastic anemia
BM biopsy
w/ prussian blue staining for ringed sideroblasts
most accurate test for thalassemia
hemoglobin electrophoresis

(alpha thalassemia is genetic studies)
findings in a thalassemia with 1 gene deletion
normal
findings in a thalassemia with 2 gene deletion
mild anemia
normal electrophoresis
findings in a thalassemia with 3 gene deletions
hemoglobin H (beta 4 tetrads)
findings in a thalassemia with 4 gene deletions
hemoglobin Bart (gamma 4 tetrad)
hydrops fetalis
findings in B thalassemia with 1 gene deletion
increased hemoglobin F and A2
Rx for IDA
oral ferrous sulfate or intramuscular iron
SE of giving iron
black stool
constipation
Rx for sideroblastic anemia
B6

adults:
-succimer
-EDTA

children
-dimercaprol
-succimer
-EDTA
Rx for B thalassemia major
lifelong transfusion
Rx for iron overload because of chronic transfusion
deferoxamine
deferasirox
what should be suspected if px has a microcytic anemia that doesnt respond to iron
thalassemia
once a macrocytic anemia has be determine what is the next best test
peripheral smear
what levels are unique to B12 and not folate def
increased methylmelonic acid
Rx for B12 def caused by D latum
praziquantel
MCC of megaloblastic anemia
alcoholics
what vitamin are vegetarians def in
B12
what vitamin are is goats milk def in
folate
MC vitamin def
folate
Rx for folate def caused by MTX
folinic acid (Leucovorin)
how long do B12 stores last
4-5 years
what are px with B12 def caused by gastritis at increased risk for
gastric cancer
how does B12 def cause neurological problems
causes defective myelin synthesis
what can cause subacute combined degeneration of the cord
B12 def
syphilis
what neurologic problems do px with B12 def have
posterior column damage
-position
-vibration
next best step in px with macrocytic anemia and equivical B12 and folate levels
methylmalonic acid level
potential complication when replacing B12 or folate
hypokalemia
why are pancreatic enzymes needed to absorb B12
removes R protein from B12
what lab changes are found in all forms of hemolysis
increased LDH, indirect bilirubin and reticulocytes
decreased haptoglobin
what does haptoglobin do
takes newly released hemoglobin and recycles it
MCC of autoimmune hemolysis
warm agglutinins
what hematological pathologies cause bilirubin gallstones
chronic hemolysis
sickle cell
spherocytosis
what is the mutation in SC
point mutation in position 6 of beta globin chain

valine replaces glutamic acid
what occurs in px with SC and are pregnant
frequent UTI
MCC of sepsis in SC
S pneumo
what should be given to children with SC
prophylactic penicillin until 5 yo
how do children with SC present
symmetric dactylitis
(inflammation of fingers)
what can cause acute painful vasoocclusive crisis
hypoxia
dehydration
infection
cold temperature
what is defective if spleens are missing in px with SC
Ab mediated opsonization (phagocytosis)
what should be done in SC px with low reticulocyte count
check parvo virus

check for folate def
what are some common complications of SC
retinopathy
stroke
enlarged heart with systolic murmur
lower extremity skin ulcer
avascular necrosis
MCC of stroke in children
SC
what are the common vessels to have avascular necrosis in SC and where are they found
ascending and foveal artery

lie in ligamentum teres
best initial test for SC
peripheral smear
most accurate test for SC
electrophoresis
what is seen on radiology in SC
fish mouth vertebrae

lung infiltrates
what causes strokes in SC px
RBC adherence to endothelium
activation of vWF
hyperviscosity
what are howell jolly bodies
precipitated remnants or nuclear material inside RBC
Dx
morulae seen inside neutrophils
ehrlichia
why do SC px need high levels of folate
to maintain reticulocytes
what should be done in SC px with fever
immediate antibiotics
how is aplastic crisis prevented in SC px
folate replacement
how is sickle cell crisis prevented
hydroxyurea
what vaccine should all SC px recieve
pneumococcal vaccine
best initial Rx for SC px
oxygen/hydration/analgesics
when is exchange transfusion needed in SC px
severe vasoocclusive crisis with:
-acute chest pain
-priapism
-stroke
-visual disturbance
what is the first clue to parvo infection in SC px
sudden drop in reticulocyte count
HbS concentration in SC trait px
35-40%
complications of SC trait
isosthenuria (clogged vasa rectae)

hematuria (papillary necrosis)
most accurate test for hereditary spherocytosis
osmotic fragility test
Rx for hereditary spherocytosis
folic acid replacement

splenectomy
why is it important to do a coombs test to px with hereditary spherocytosis
autoimmune hemolysis also has spherocytes
what lab levelsare found in hereditary spherocytosis
low MCV

high MCHC (>36%)
causes of warm autoimmune hemolysis
CLL
lymphoma
SLE
drugs
most accurate diagnostic test for autoimmune hemolytic anemia
coombs test
best initial therapy for warm hemolysis
steroids

if not, IVIG
Rx for recurrent warm hemolysis
splenectomy

if not, rituximab
what is a/w cold agglitinins
EBV
waldenstrom
mycoplasma
diagnostic test for cold agglutinins
direct coombs
Rx for cold agglutinins
stay warm
RBC transfusion
rituximab
immunosuppressive agents
what are some features of cryoglobulinemia
hep C
joint pain
glomerulonephritis
Rx for cryoglobulinemia
Rx the hep C that caused it
what is the pathophys of G6PD def
inability to generate glutathione reductase
what is found in the urine after a hemolytic episode in G6PD def
hemosiderin
what stains hemosiderin
prussian blue
what is used to stain heinz bodies
crystal violet
best initial test for G6PD def
blood smear looking for heinz bodies and bite cells
most accurate test for G6PD def
G6PD level after waiting 1-2 months after an acute episode of hemolysis
what are heinz bodies
aggregates of denatured hemoglobin
symptoms of HUS
renal failure
thrombocytopenia
hemolytic anemia
symptoms of TTP
renal failure
thrombocytopenia
hemolytic anemia
neurologic problems
fever
Rx for HUS/TTP
plasmapheresis or plasma exchange
how can recovery be measured in HUS/TTP
normalization of platelet count and LDH level
what can cause HUS
e coli
shigella
what can cause TTP
AIDS
drugs (ticlopidine, clopidogrel, cyclosporine)
what is the best test for HUS/TTP
blood smear
-looking for schistocytes
pathophys of paroxysmal nocturnal hemoglobinuria
deficiency in CD 55 and 59 (DAF)

Defective PIG-A (GP1 anchor protein)
where do thrombus usually form in paroxysmal nocturnal hemoglobinuria
hepatic vein
features of paroxysmal nocturnal hemoglobinuria
episodic dark urine on first urination

pancytopenia

iron deficiency
MCC of death in paroxysmal nocturnal hemoglobinuria
thrombosis
what is the most accurate test for paroxysmal nocturnal hemoglobinuria
flow cytometry (decreased CD 55 and 59 levels)
best initial therapy for paroxysmal nocturnal hemoglobinuria
steroids
only way to cure paroxysmal nocturnal hemoglobinuria
BM transplant
what can be used to inhibit compliment in paroxysmal nocturnal hemoglobinuria
eculizumab
-inactivates C5
how does aplastic anemia present
pancytopenia
-infection
-anemia
-bleeding
most accurate test for aplastic anemia
BM biopsy
what is seen on BM biopsy in aplastic anemia
hypocellularity with fatty infiltrates
MCC of congenital aplastic anemia
fanconis anemia
features of faconis anemia
kidney malformation (horseshoe)
hypo/hyper pigmentation
abnormal thumb
short stature
ear conduction defect
how is fanconis anemia diagnosed
genetic analysis shows chromosomal breaks
what causes the chromosomal breaks in fanconis anemia
diepoxybutane
what is the pathophys in aplastic anemia
T cells attack patients own marrow
Rx for aplastic anemai
supportive therapy
-transfusions
-platelets
-antibiotics

BM transplant
-if older than 50 or no match, Rx with antithymocyte globulin and cyclosporine (or tacrolimus)
MCC of increased RBC
chronic hypoxia
what must be excluded in polycythemia vera
chronic hypoxia
what is the pathophys of polycythemia vera
JAK2 protein mutation
why do px with polycythemia vera develop pruritus after hot showers
increased basophils release histamine
what are some lab abnormalities in polycythemia vera
increased Hct (above 60%)
MCV is low
Normal O2
decreased EPO
ESR close to 0
most accurate test for polycythemia vera
check for JAK2 mutation
MCC of polycythemia in newborn
delayed clamping of umbilicus
what nutrient abnormalities do px with polycythemia have
increased B12
decreased iron
Rx for polycythemia
phlebotomy
hydroxyurea
antihistamines (pruritus prevention)
ASA (thrombus prevention)
what is essential thrombocytosis
platelet count above 1 million
when essential thrombocytosis treated
only treat if
-symptomatic
-platelets are above 1.5 million
->60 yo and thrombosis
best initial Rx for essential thrombocytopenia
hydroxyurea
Rx for RBC suppression by hydroxyurea
anagrelide
Rx for erythromelalgia
aspirin
what is erythromelalgia
painful red hands
Rx for myelofibrosis
thalidomide and lenalidomide

if under 50-55 BM transplant
what is seen on BM biopsy in myelofibrosis
hypocellular fibrotic BM
a/w tear drop shaped cells
myelofibrosis
how does acute leakemia present
symptoms of pancytopenia
-fatigue
-infection
-bleeding

previous history of myelodysplastic syndrome
which leukemia is a/w DIC
M3 AML
best initial test for acute leukemia
blood smear showing blasts

(25% lymphoblasts is diagnostic)
most accurate test for acute leakemia
flow cytometry
translocation in M3 AML
15:17
what is defected in AML
retinoic acid receptor
associated with auer rods
AML
leukemia a/w bleeding gums
M5 AML
leukemia positive for a-naphthyl esterase
M5 AML
leukemia postive for myeloperoxidase
M3 AML
best initial Rx for acute leukemia
chemo

if poor prognosis give BM transplant
if good prognosis give more chemo
best indicator of prognosis in acute leukemia
cytogenetics
which leukemia should recieve an additional MTX
ALL
presentation of CML
high WBC count that is all neutrophils
pruritus
splenomegaly
how can CML and leukomoid reaction be differentiated
LAP is low in CML

LAP is high in leukomoid reaction
what is the mutation in CML
BCR-ABL 9:22
tyrosine kinase
best initial Rx for CML
tyrosine kinase inhibitors (bind to ATP binding site)
(imatinib, dasatinib, nilotinib)
which myeloproliferative disorder is most like to transform to acute leukemia
CML
how can CML transformation to acute leukemia be prevented
tyrosine kinase inhibitors (bind to ATP binding site)
(imatinib, dasatinib, nilotinib)
what is most effective Rx to cure CML
BMT
what is usually abnormal in myelodysplastic syndrome and how does this effect the prognosis
5q deletion

(better prognosis when present)
MCC of death in myelodysplastic syndrome
infections and bleeding
how does myelodysplastic syndrome present
px over 60 with asymptomatic pancytopenia despite and hypercellular BM
a/w pelger huet cells
myelodysplastic syndrome
what determines the severity of myelodysplastic syndorme
percentage of blasts
what are pelger huet cells
bilobed nucleus

neutrophils with nuclei with 2 lobes connected by a thin strand
Rx for myelodysplastic syndorme
transfusion
EPO
lenalidomide
what can decrease transfusion dependence in those with myelodysplastic syndorme
lenalidomide
presentation of CLL
proliferation of B lymphocytes
lymphadenopathy
hepatosplenomegaly
infection
a/w smudge cells
CLL
a/w WBC count over 20,000 (sometimes >100,000)
CLL
how can CLL be confirmed
flow cytometry
what is richter phenomenon
conversion of CLL into high grade lymphoma
Stages of CLL
0=increased WBC
1=lymphadenopathy
2=hepatosplenomegaly
3=anemia
4=thrombocytopenia
Rx for CLL
stage 2-4 fludarabine or rituximab
Rx for refractory cases of CLL
cyclophosphamide
Rx for severe infection in CLL
IVIG
Rx for autoimmune thrombocytopenia or hemolysis in CLL
prednisone
Rx for CLL with just high cell count
chlorambucil
hairy cell leukemia is also known as
leukemia reticuloendotheliosis
most accurate test for hairy cell leukemia
TRAP or CD11c
Rx for hairy cell leukemia
cladribine
presentation of hairy cell leukemia
pancytopenia
splenomegaly
dry, fibrotic, hypercellular BM
what should CLL px be prophylaxed for
PCP
what is non hodgkins lymphoma
proliferation of lymphocytes in the lymph nodes and spleen
how does non hodgkiins present
painless lymphadenopathy (not warm/red/tender)
fever
night sweats
weight loss
best initial test for non hodgkins
excisional biopsy
what correlates with a worse prognosis in nonhogkins
high LDH
mutation in follicular small cell lymphoma
14:18
mutation in burkitts
8:14
how is nonhodgkins staged
BM biopsy

CT scan of chest, abdomen and pelvis
staging of non hodgkins lymphoma
1=lymph
2=2 or more lymphs
3=both sides of diaphragm
4=widespread
Rx for nonhodgkins
1-2=local radiation

3-4=chemo + rituximab + CHOP
cyclophosphamide, adriamycin, vincristine, prednisone
Rx for MALT
eradicate H pylori

if not, Bleomycin + CHOP
cyclophosphamide, adriamycin, vincristine, prednisone
MCC of hodgkins lymphoma
nodular sclerosing
presentation of hodgkins lymphoma
painless cervical or supraclavicular lymphadenopathy

reed sternberg cells
Rx for hodgkins lymphoma
1 and 2 = local radiation or chemo

3 and 4 or anyone with B symptoms = ABVD
-adriamycin (doxorubicin)
-bleomycin
-vinblastine
-dacarbazine
Rx for hodgkins if relapse after radiation
chemo
Rx for hodgkins if relapse after radiation and chemo
extra high chemo with BMT
complications of radiation therapy
increased risk for solid cancer and premature CAD
what test should be done to determine dosing of chemotherapy in hodgkins
MUGA or nuclear ventriculogram
SE of vincristine
neuropathy
SE of cisplatin
renal and ototoxicity
Presentation of mononucleosis
posterior cervical lymphadenopathy
palatal patechiae
splenomegaly
why cant px with mononucleosis play sports and when can they start
splenomegaly

they can start when PE is normal (usually 1-3 months)
MC presentation of MM
bone pain
what causes the hypercalcemia in MM
osteoclast activating factor
what causes the bone lysis in MM
plasmocyte released humoral factors and expanding plasma cell mass
MCC of death in MM px
renal failure and infection
best initial test for MM
x ray
what is seen on x ray of MM
lytic lesions
why does MM have a decreased anion gap
MM has increased amounts of IgG which is cationic

cationic substances increase chloride and bicarbonate levels, which decreased anion gap
why does MM cause rouleaux formation
IgG paraprotein sticks to RBC
what are bence jones proteins
K/y light chains
most specific test for MM
BM biopsy that shows more than 10% plasma cells
what is seen on bone scan in MM
nothing, lesions are only seen on x ray
what is needed to see bence jones proteins
immunoelectrophoresis (UPEP/SPEP)

urine dipstick will not show anything
best initial Rx for MM
dexamethasone with lenalidomide or bortezomib
what is used to Rx MM in older px who cannot tolerate SE
melphalan
most effective Rx for MM
BM and Stem cell Transplant if under 70
what is the M spike in MM
IgG or IgA
Dx
px with IgG or IgA spike that BM biopsy does not show MM
MGUS
what is the immunoglobulin spike in waldenstrom macroglobulinemia
IgM
presentation of waldenstrom
hyperviscosity
blurry vision (engorged vessels in eye)
vertigo
mucosal bleeding
reynauds phenomenon
best initial therapy for waldestrom
plasmaphoresis
what levels are altered when there is a platelet problem
BT
what levels are altered when there is a clotting problem
clotting factors
features of platelet bleeding
patechiae/purpura
mucosal bleeding
epistaxis
features of factor bleeding
joints and muscles
features of idiopathic thrombocytopenia purpura
platelets <50,000
isolated thrombocytopenia
normal sized spleen
when is a bleeding in idiopathic thrombocytopenia purpura considered severe
intracranial bleeding or GI bleeding
Rx for idiopathic thrombocytopenia purpura when no bleeding or platelets are >30,000
none
Rx for idiopathic thrombocytopenia purpura when mild bleeding or platelets <30,000
steroids
Rx for idiopathic thrombocytopenia purpura when severe bleeding or platelets <10,000
IVIG
anti Riho (anti D) (RhoGAM)
Rx for idiopathic thrombocytopenia purpura when recurrent episodes or steroid dependent
splenectomy
Rx for idiopathic thrombocytopenia purpura when splenectomy or steroids are not effective
rituximab
azathioprine
cyclosporine
mycophenolate
romiplostim
eltrombopag
what vaccines need to be given before splenectomy is performed
neisseria
h influ
pneumococcus
what can worsen vW disease
aspirin use
how does vW disease present
platelet def. type of bleeding with normal platelet count
how can vW disease be diagnosed
increased BT
vWF is decreased
ristocetin cofactor asay
best initial therapy for vW disease
desmopressin
Rx for vW if no response to desmopressin
replace factor 8 or vWF
what happens if recurrent bouts of hemarthrosis
hemophilic arthropathy

iron deposition with synovial thickening and fibrosis
most accurate test for hemophilia
specific assay for factor 8 and 9
Rx for hemophilia
desmopressing

severe cases need to replace specific factors
best initial test for hemophilia
mixing studies with normal plasma will correct the PTT
Rx for factor 11 def
FFP
lab findings in DIC
elevated PT, PTT, d-dimers, fibrin split products

low platelets and fibrinogen
what is seen on blood smear in DIC
helmet cells
Rx for DIC
replaced platelets if under 50,000

replace clotting factors with FFP
what is used to Rx DIC if FFP doesnt work
cryoprecipitate
Dx
px starts warfarin and develops skin necrosis
congenital protein C def
MCC of hypercoagulable state
factor 5 leiden mutation
how can HIT be confirmed
ELISA for PF4 antibodies
or
serotonin release assay
Rx for HIT
stop all heparin

switch to argatroban, lepirudin, danaparoid and bivalirudin

finally get px on warfarin after switching
Rx for severe cases of HIT
plasmaphoresis
best initial test to Dx antiphospholipid syndrome
mixing study (will not correct PTT)
most accurate test for antiphospholipid syndrome
russel viper venom test
Rx for antiphospholipid syndrome
herparin and warfarin
next step in a px who is pregnant, has already had a miscarriage and is currently pregnant
begin heparin to protect current pregnancy
Rx if px develops hypocalcemia after a transfusion
calcium gluconate
Dx
elderly px develops brownish discoloration after minor trauma
senile purpura
pathophys behind seniles purpura
perivascular connective tissue atrophy with hemosiderin deposits