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98 Cards in this Set

  • Front
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Anemia -
What is it
Low Hct and Hb relative to
gender and age
Anemia -
History/PE
weakness
DOE
high-output CHF
severe - angina and syncope
iron-deficient -
pica (clay, ice chips)

PE-
pallor of skin & conjunctiva
tachy
tachypnea
inc. pulse pressure
systolic flow murmur
jaundice (hemolytic anemia)
pos. stool guaiac (GI bleed)
Anemia -
Evaluation
CBC - Hgb, Hct, MCV
folate
B12
retic count
haptoglobin
LDH
bilirubin
iron
ferritin
TIBC
Coombs'
DIC panel -
D-dimer
fibrinogen
fibrin split products
occult stool
Anemia -
Tx
Treat underlying cause

iron-def. -
oral iron supplements
iron-def in elderly male -
suspect colorectal cancer

B12 def - monthly B12 shots
chronic renal dis. - EPO

lwr threshold for
transfusions in CAD pts
(anemia can worsen
cardiac ischemia)
Anemia -
Types of
Microcytic Hypochromic
Iron deficiency
Anemia of Chronic Disease
Lead poisoning
Sideroblastic anemia
Thalassemias
Anemia -
Types of
Megaloblastic,
Macrocytic
Megaloblastic -
B12 deficiency
folate deficiency

Macrocytic -
alcohol
drugs -
anticonvulsants
sulfa drugs
AZT
chemo
radiation
myelodysplastic syndrome
G6PD Deficiency -
What is it
X-linked recessive
Most affected -
Black & Mediterranean men
G6PD in HMP shunt => NADPH
NADPH needed to keep
glutathione reduced to
detox free radicals
dec. NADPH = poor protection
against oxidative damage
G6PD Deficiency -
History/PE
Triggered by?
Acute, self-limited h. anemia
fatigue
jaundice
dark urine
hemoglobinuria

Triggered by oxidative
stress/agents -
viral or bact. infections
metabolic acidosis
fava beans
sulfonamides
primaquine
INH
dapsone
nitrofurantoin
aspirin

Most common type of
oxidative stress - infections
G6PD Deficiency -
Evaluation
CBC - Hct & retic count
bilirubin
haptoglobin
Heinz bodies
spherocytes

G6PD enzyme test -
can be false negative
during or right after episode
G6PD-def. RBC hemolyzed
young RBCs have normal enzyme
(enzyme activity deficient
in older cells)
G6PD Deficiency -
Tx
Sxs usually self-limited
avoid meds that cause
transfuse - if severe anemia
MAHA -
What is it
Narrowing of blood vessels
due to fibrin
(from inc. coag activity)
mechanical damage to RBC
and platelets
as they squeeze through
plat. also caught in fibrin
DIC
TTP
HUS
schistocytes
burr, helmet & triangle cells
HUS -
What is it
MCC of ARF in kids
gastroenteritis
Most cases from E Coli
O157:H7
HUS -
Manifestations
MAHA
thrombocytopenia
ARF
Renal failure dominates
HUS -
Lab Findings
Anemia
thrombocytopenia
HUS -
Tx
Dialysis for ARF
TTP -
Causes
HIV
pregnancy
OCP
individuals < 50 y/o
TTP -
Manifestations
Fever
thrombocytopenia
MAHA
transient neuro deficits
(thrombi in brain)
neuro deficits dominate
renal failure
(thrombi in kidney)
splenomegaly
TTP -
Lab Findings
CBC - thrombocytopenia & MAHA
schistocytes
LDH - inc.
indirect bilirubin - inc.
retic count - high
TTP -
Tx
Large-vol plasmapheresis
corticosteroids
ASA - (controversial use)
splenectomy
DIC -
Causes
obstetric complications -
(amniotic embolus
septic abortion
placenta abruptio)
sepsis
transfusion reaction
neoplasia
trauma
pancreatitis
rhabdomyolysis
burns
snakebites
DIC -
Manifestations
blding from venipuncture site
epistaxis
hematemesis
digital gangrene
hypotension
DIC -
Lab Findings
anemia
thrombocytopenia
inc. PT/PTT
inc. bleeding time
inc. D-dimer
inc. fibrin split products
dec. fibrinogen
DIC -
Tx
Tx underlying condition
transfuse with platelets
and cryoprecipitate
Sickle Cell Disease -
What is it (biochemically)
What triggers it
Autosomal recessive
valine for glutamic acid
in 6th position of B-globin

Triggered by -
infection
dehydration
hypoxia
hypoxemia (pneumonia)
Sickle Cell Disease -
History/Sx
Vaso-occlusive crises
splenic crises -
splenomegaly =>
autosplenectomy ~ age 6
risk of encapsulated org.
(S. pneumo, H. influenzae,
N. meningitides)
sequestration crises
pain crises
aplastic crises - B19
crew haircut on XR
aseptic necrosis of fem. head
osteomyelitis - Salmonella
acute chest syndrome
vasa recta - vulnerable
extramedullary hematopoiesis
reticulocytosis
acute dactylitis
seizure
stroke
leg ulcers
painful priapism
Sickle Cell Disease -
Tx
Hydration and O2 -
dec. sickling
analgesia
transfusion -
(severe anemia
acute chest syndrome with
respiratory distress)
Abx in acute chest syndrome
(often have resp. infection)
hydroxyurea
23-valent pneumococc vaccine
prophylactic pcn < 5 y/o
Beta Thalassemia -
What is it
point mutation
Mediterranean, Asians, Blacks
major = Cooley's Anemia,
no beta globin chains
minor = dec. beta globin
Beta Thalassemia -
History/Features
ineffective erythropoiesis -
RBCs destroyed in bm
splenomegaly
hepatomegaly
crew haircut
bone deformities
growth retardation
jaundice
target cells
transfusion => secondary
hemochromatosis & CHF
Alpha Thalassemia -
What is it
gene deletions -
1 - silent carrier
2 - trait
3 - HbH disease
chronic hemolytic anemia
splenomegaly
4 - hydrops fetalis
Hb Bart = excess g chains
Alpha Thalassemia
Beta Thalassemia -
Tx
transfusions
splenectomy
folic acid
bm transplant
deferoxamine subq -
inc. urinary excretion of Fe
use to prevent iron overload
PCV -
What is it
Myeloproliferative disease
primary polycythemia
Inc. in RBC production
WBCs and platelets often inc.
> 60 y/o
inc. risk of conversion to -
AML, CML, myelofibrosis

hypercellular bm =>
bm burned out
replaced by fibrosis
extramed hematopoiesis
myeloid metaplasia with
myelofibrosis
can => Budd-Chiari

MCC of erythrocytosis -
secondary polycythemia
chronic hypoxia secondary
to lung dis.
PCV -
History/PE
Malaise
fever
pruritis (esp. after a
warm shower)
vascular sludging -
(headache
stroke
blurred vision
angina
MI
hepatic vein thrombosis
claudication)
plethora
large retinal veins
splenomegaly
PCV -
Dx
O2 sat - normal
Hct > 50%
RBC mass inc.
EPO undetectable
WBC & platelets - norm or inc
LAP high
ferritin not inc.
Secondary Polycythemia -
Features
Due to hypoxia
Inc. in RBCs
ferritin not inc.
EPO inc.
PCV -
Tx
. Serial phlebotomy -
dec. Hct and sludging
. daily ASA -
prevent thrombosis
. anagrelide - dec. platelets
(inhib maturation of megak)
. hydroxyurea - myelosuppress
Hyperacute Rejection -
What is it
How to prevent
Pt. has preformed antibodies
thrombi form immed.
organ can show signs of
ischemia before op finished
to prevent -
check ABO compatibility
give lymphocytotoxic agents
Hodgkin's Lymphoma -
What is it
Malignant
orderly spread from one
lymph node group to another
contiguous spread
extranodal rare
Reed-Sternberg cells
bi-modal incidence curve -
15-40 y/o and > 60 y/o
50% of cases associated
with EBV
more common in men except NS
good prognosis - more lymphoc
Types -
NS
mixed cellularity
lymphocyte-predominant
lymphocyte-depleted
Hodgkin's Lymphoma -
Hx/PE
Painless lymphadenopathy
or mediastinal adenopathy
B symptoms
pruritis
fatigue
hepatosplenomegaly
Hodgkin's Lymphoma -
What are B symptoms
What do they mean
Fever > 38.5 C,
night sweats,
10% weight loss over 6 months

systemic Sxs
marker for more advanced dis.
systemic instead of
local involvement
neg. prognostic for Hodgkin's
reflected in staging
A = no systemic Sxs
B = presence
Acute Rejection -
What is it
Tx
Occurs bet. 5 days & 3 months
liver rejection -
inc. GGT, alk phos, bilirub
kidney rejection -
inc. BUN/Cr
confirm with Bx
Tx -
steroids
OKT3 (antilymphocyte ab)
Hodgkin's Lymphoma -
Dx
Bx largest node
then CXR
consider bm Bx and LP
Hodgkin's Lymphoma -
Tx
Radiation for localized
chemo for advanced/widepread-
ABVD or MOPP

ABVD -
adriamycin
bleomycin
vincristine
dacarbazine

MOPP -
mechlorethamine
oncovin (vincristine)
prednisone
procarbazine
Chronic Rejection -
What is it
Tx
Irreversible,
gradual loss of function
mos. to yrs. after transplant
no Tx
Bx to r/o late, treatable,
acute reaction
Non-Hodgkin's Lymphoma -
What is it
Malignant
involves lymphoreticular & GI
extranodal common
multiple, peripheral nodes
noncontiguous spread
incidence inc. with age
median age 50 y/o
risk factor - immunodeficient
Types -
lymphoblastic - T
lymphocytic - B
large - B
follicular - B
Burkitt's - B
Transfusion Reactions -
What are they
Hemolysis
allergic reactions
transfusion-related infection
Non-Hodgkin's Lymphoma -
Hx/PE
Painless adenopathy
B Sxs
systemic adenopathy
hepatosplenomegaly
Non-Hodgkin's Lymphoma -
Dx
Bx for Dx
CXR
whole body CT
consider bm Bx and LP
then staging
Non-Hodgkin's Lymphoma -
Staging
Based on number of nodes
does disease cross diaphragm
any B Sxs
Transfusion Reactions -
What is hemolysis from
Preformed Ab lyse donor RBC
MCC -
ABO incompatibility due to
clerical error
also, mislabeled specimens
reactions to Ag not tested
Non-Hodgkin's Lymphoma -
Tx
Radiation and chemo (CHOP)
CHOP -
Cytoxan
adriamycin
Oncovin (vincristine)
prednisone

Tx may be complicated by
tumor lysis syndrome
Transfusion Reactions -
Allergic reactions
IgA deficiency -
anaphylactic reaction
during transfusion
Non-Hodgkin's Lymphoma -
What is Tumor Lysis Syndrome
From tumor cell death
releasing intracellular
contents after chemo
mainly in acute leukemias
and non-Hodgkin's

hyperK+
hyperuricemia
hyperphosphatemia
hypoCa2+
ARF
Multiple Myeloma -
What is it
Risk factors
Complications
Malignancy of plasma cells
arises within bone marrow
they invade and destroy
adjacent bone tissue
> 50 y/o
Blacks more than whites
Risk factors -
radiation
chemical exposure
MGUS
most significant factor - age

Dis. usu recurs - poor prog
complications -
anemia
infection
neurological diseases
renal failure
Multiple Myeloma -
Hx/PE
Back pain
hypercalcemic Sxs -
"stones, bones, abdom moans
and psych overtones"
pathological fractures
fatigue
frequent infections -
esp. H. influ & pneumococcus

PE -
pallor
fever
bone tenderness
bone deformities
lethargy
Transfusion Reactions -
Infections
Rare
viruses -
HBV HCV, HIV, HTLV-1, -2, CMV

bacteria -
risk highest with
platelet transfusion
Multiple Myeloma -
Dx
Serum & urine protein
electrophoresis -
(monoclonal IgG gammopathy
M(onoclonal) spike, M prot.)
Bence-Jones proteinuria -
IgG light chains
CBC - anemia, thrombocytopenia
periph smear - rouleaux RBC
electrolytes - low-anion gap
full-body skeletal survey -
punched-out lytic lesions
bone scan - neg.
bone marrow Bx -
> 10% plasma cells confirms

UA -
detects albumin
need specific test for Bence-
Jones protein that involves
acidification of urine

staging based on -
degree of anemia
elevated calcium
XR findings
renal dysfunction
Multiple Myeloma -
Tx
Younger pts. - BMT
older - melphalan & prednisone

combination chemo -
vincristine
adriamycin (doxorubicin)
dexamethasone
use:
in prep for BMT
or if dis. progresses after
melphalan & prednisone

hypercalcemia -
hydration & loop diuretics
then bisphosphonate
Transfusion Reactions -
Hx/PE
Shortly after administered -
hypotension
tachypnea
tachy
fever/chills
hemoglobinuria
chest pain
discomfort
Transfusion Reactions -
Tx
Stop transfusion
retype pts. blood
BC
DIC labs
assess for free Hb in
blood and urine
IV fluids
mannitol
Hemophilias -
What are they
X-linked recessive
coagulopathy
Hemophilia A - dec. factor 8
Hemophilia B - dec. factor 9
Hemophilias -
Hx/PE
Within hrs. of trauma,
bleed into -
joints, muscles, GI, brain

soft-tissue hemorrhage
hemarthroses
intramuscular bleeding
GI bleeding
excessive bleeding from
mild trauma, surgical/dental
procedures

mild deficiency -
(25%+ activity)
no Sxs or
Sxs during surgery or trauma

severe deficiency -
(< 5-10% activity)
spontaneous bleeding
Hemophilias -
Dx
Inc. PTT
Normal -
PT, bleeding time, platelets,
Ristocetin cofactor assay

mixing study -
50/50 mix
pt.'s blood with normal blood
if PTT corrected -
factor deficiency
if PTT not corrected -
Ab to factor
Hemophilias -
Tx
Give during episodes or
as prophylaxis -
factor 8 concentrate (Hemo A)
factor 9 concentrate (Hemo B)

desmopressin -
mild Hemo A
before minor surg procedure
inc. endog Factor 8 activity
von Willebrand's Disease -
What is it
AD
MC inherited bleeding d/o
qual. or quan. defect of vWF
aggregation normal
adhesion abnormal

vWF -
prot needed for plat adhesion
dec. platelet adhesion
dec. factor 8
von Willebrand's Disease -
Hx/PE
. Easy bruising
. mucosal bleeding -
epistaxis
oral bleeding
GI bleeding
menorrhagia
. postincisional bleeding
von Willebrand's Disease -
Dx
Normal -
PT, platelet no.

PTT - normal or inc.
BT - inc., especially
after aspirin

Ristocetin Cofactor Assay -
normal or dec. response
von Willebrand's Disease -
Tx
Desmopressin - if mild
OCP - menorrhagia
von Willebrand's factor -
now available

transfusion -
minimized to dec. risk of
transmitting viral infections
Hypercoagulable States -
What are the causes
Inherited -
factor V leiden - MC inherited
prot C deficiency
prot S deficiency
antithrombin 3 deficiency
homocystinemia
fibrinolysis defects

Acquired -
prolonged bed rest
immobilization
MI
tissue damage
DIC
hyperlipidemia
vasculitis
multiple myeloma
lupus anticoag
nephrotic syndrome
smoking
cancer
warfarin (on initiation)
OCPs
pregnancy
Idiopathic Thrombocytopenic
Purpura (ITP) -
What is it
Autoimmune platelet d/o
kids - postviral, selflimited
Ab against viral Ag
on platelets

adult - chronic
IgG against glycoproteins
in platelet membrane
females affected 2X as males
< 50 y/o

Evans' syndrome -
ITP with autoimmune h. anemia

Can be associated with -
Hodgkin's
NHL
CLL
HIV
SLE
RA
Idiopathic Thrombocytopenic
Purpura (ITP) -
Hx/PE
afebrile
no splenomegaly
petechiae
purpura
ecchymoses
mucosal bleeding -
(epistaxis
oral bleeding
menorrhagia)
oral hemorrhagic bullae
Idiopathic Thrombocytopenic
Purpura (ITP) -
Dx
CBC
normal - PT, PTT, DIC
BT inc.
pos.- platelet-assoc. IgG test
periph smear - megathrombocyte
bm aspiration -
normal or inc. megakaryocytes
Idiopathic Thrombocytopenic
Purpura (ITP) -
Tx
kids - self-limited

adults -
. corticosteroids -
may mask leukemia (CLL)
bm aspirate 1st
. platelets -
if severe, uncontrolled bldg
. IVIG & anti-RhoD (if Rh+) -
life-threatening hemorrh or
severe TCP (< 10,000)
. splenectomy -
if refractory to meds
Leukemias -
What are they
MC cancer in kids
categorized based on
cell origin & differentiation
major complications end-stage
bleeding
infection
anemia

acute -
blast
min. differentiation

chronic -
mature cells
more differentiated
Acute Lymphocytic Leukemia -
What is it
Lymphoblasts
pre-B or pre-T
kids
Acute Lymphocytic Leukemia -
Hx/PE
Hx-
limp
refusal to walk
bone pain
easy bruising
fever

PE -
pallor
widespread petechiae/purpura
ecchymoses
bleeding
adenopathy
hepatosplenomegaly
Acute Lymphocytic Leukemia -
Dx
Dec. bm elements
inc. LDH
inc. uric acid
TdT
PAS
CD10 (CALLA)
CXR
LP
CT
Dx based on bm aspirate
Acute Lymphocytic Leukemia -
Tx
Good prognosis with chemo
daunorubicin
vincristine
prednisone

CNS prophylaxis -
intrathecal methotrexate
Acute Myelogenous Leukemia -
What is it
Myeloblasts
neutrophils, eosinophils,
basophils (neb)
8 different types
adults
Acute Myelogenous Leukemia -
Hx/PE
Hx -
fatigue
dyspnea (from anemia)
fever
frequent infections
easy bruising
leukemia cutis
CNS Sxs
M3 - DIC
M4 - CNS
M5 - CNS and
gingival hyperplasia

PE -
lethargy
fever
bleeding
petechiae/purpura
hepatosplenomegaly
Acute Myelogenous Leukemia -
Dx
Dec. LAP
Auer rods
myeloperoxidase
CD33
Sudan stain
M3 - t(15;17)
Acute Myelogenous Leukemia -
Tx
Daunorubicin
ara-c

transfusions
ABx
allogeneic/autogeneic BMT
retinoic acid -
induce remission of
promyelocytic form, M3

neutropenic fever -
fever if neutrophil count
< 500 cells/microL
broad-spectrum ABx and
antifungals

complication -
tumor lysis syndrome
Chronic Lymphocytic Leukemia -
What is it
Lymphocytes
T & B
elderly (> 65 y/o)
slowly progressive
many pts. diagnosed by
incidental lymphocytosis
good short-term survival
poor long-term survival
Chronic Lymphocytic Leukemia -
Hx/PE
Lymphadenopathy
fatigue
hepatosplenomegaly
Chronic Lymphocytic Leukemia -
Dx
Absolute periph lymphocytosis
inc. lymphocytes in bm
malig transformed CD5+ B
smudge cells
associated with bcl-2 oncogene
CD19

stage 0 - lymphocytosis
stage 1 - lymphadenopathy
stage 2 - splenomegaly
stage 3 - anemia
stage 4 - TCP

stages 1 & 2 - if Sxs, treat
stages 3 & 4 - treat
Chronic Lymphocytic Leukemia -
Tx
Supportive

chemo -
chlorambucil & prednisone
fludarabine
once develop Sxs
fatigue, lymphadenopathy,
anemia or thrombocytopenia

splenectomy & steroids -
autoimmune HA and TCP
Chronic Myelogenous Leukemia -
What is it
Myeloid cells
often stable for several yrs.
then "blast crisis"
prior radiation exposure
40-60 y/o
Chronic Myelogenous Leukemia -
Hx/PE
Early stages -
asymp or mild
fatigue, fever, malaise

later stage -
early satiety
LUQ fullness - huge splenomeg
bleeding

blast crisis -
final phase
rapidly fatal for most
fever
bone pain
weight loss
inc. splenomegaly
Chronic Myelogenous Leukemia -
Dx
Myeloid with basophilia
LAP low
B12 - often high
definitive Dx -
Philly chromosome
bcr-abl
t(9;22)
Chronic Myelogenous Leukemia -
Tx
Poor prognosis
neg Philly chrom - worse prog

if have donor - BMT
otherwise, imatinib (Gleevac)

BMT - often => GVHD
MGUS -
What is it
Monoclonal Gammopathy
of Undetermined Significance

overproduction of particular
IG (by plasma cells)
no systemic manifestations
cause is unknown
MGUS -
Dx
Increased monoclonal IG spike
amounts lower than
Multiple Myeloma
BM < 5% plasma cells
(BM Bx > 10% plasma cells
confirms Multiple Myeloma)
MGUS -
Tx
Not necessary
Vitamin K Deficiency -
What does it lead to?
What is it from?
Leads to decreased production
of factors 2, 7, 9 & 10

from -
diet deficiency
malabsorption
ABx that kill bacteria in
colon that make it
Vitamin K Deficiency -
Sxs
Bleeding mimics hemophilia
can occur anywhere
check for oozing at
venipuncture sites
Vitamin K Deficiency -
Dx
Inc. PT & PTT

PT -
increases first
is more severe

confirm -
give vitamin K
PT & PTT then correct
Vitamin K Deficiency -
Tx
If severe - FFP and vitamin K
Liver Disease and Bleeding -
What is it
Factor 8 and VWF are not
made in liver

MC site of bleeding - GI
decreased platelets from
hypersplenism
Liver Disease and Bleeding -
Tx
Acute Tx - FFP