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125 Cards in this Set

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poikilocytosis
varying shapes
RBC membrane contains ________ antiport that allows RBC to transport CO2
chloride-bicarb
platelet contains
dense granules (ADP, Ca) and alpha granules (vWF, fibrinogen)
PMN
multilobed, azurophilic granules with hydrolytic enzymes, lysozyme, myeloperoxidase, lactoferrin (binds iron during ifxn)
monocyte
large kidney shaped nucleus, extensive frosted glass cytoplasm
marcophage activated by
gamma interferon
eosinophil
bilobate, major basic protein, phagocytic for Ag-Ab complexes, produces histaminase and arylsulfatase to help limit mast cell degran
neoplasms can elevate
basophil
allergy. bilobate. contain heparin (anticoag), histamine (vasodil), vasoactive amines, LTD4
mast cell
contains histamine, heparin, eosinophil chemotactic factors
in tissue
langerhans
DC
extrinsic coag factor
VII
antithrombin inhibis
VIIa, IXa, Va, XIa, XIIa
epoxide reductase
activates VitK, warfarin inhibits
tPA
activates plasmin
thrombomodulin
activates Protein C
protein S
req by Protein C to cleave Va and VIIIa
Factor V Leiden
factor V resistant to APC inhibition
after platelets bind vWF...
they release ADP and Ca. ADP helps platelets adhere to endothelium and ADP binding to receptor induces GpIIb/IIIa expression
fibrinogen binds GpIIb/IIIa and links platelets
pro-agg: TXA2 from platelets
anti-agg: PGI2 and NO from endothel
ticlopidine and clopidogrel MoA
inhibit ADP-induced expression of GpIIb/IIIa
abciximab MOA
inhibits GpIIb/IIIa directly
acanthocyte, spur cell
liver dz, abetalipoproteinemia
basophilic stippling
thalassemias, anemia of chronic dz, iron def, lead poison
BASte the ox TAIL
schistocyte
DIC, TTP, HUS, traumatic hemolysis
teardrop cell
myelofibrosis
heinz bodies
alpha thal, G6PD
alpha thal
Asian and African
1-2 gene deletion, no anemia
3 is HbH dz (beta4)
4 is hydrops fetalis, death
beta thal
minor is hetero, asym, incr HbA2 on electrophoresis
major is homo, req transfusion. no beta chain. marrow expansion shows crew cut on xray, chipmunk face
point mutations in splice sites and promoter seq. Mediterranean. aniso, poikilo, micro, hypo, targets, schistos. both major and minor incr HbF, HbS/beta thal hetero is milder than SS.
lead poison
inhibits ferrochelatase and ALA dehydratase. lead lines on gingivae (burtons) and epiphyses of long bones on xray. Encephalopathy and Erythrocyte basophilic stippling (aggreg of ribosomes) abdominal colic and sideroblastic anemia. wrist and foot drop
tx: dimercaprol and EDTA
succimer for kids
sideroblastic anemia
defect in heme sunth. XL defect in ALA synthase. reversible if from alcohol or lead
ringed sideroblasts with iron-laden mitochondria. incr iron, normal TIBC, incr ferritin.
tx: B6
cause of B12, neuro sx
fish tapeworm Diphyllobothrium latum
periph neurop with sensorimotor dysfxn
posterior columns (vibration and proprioception)
lateral corticospinal (spasticity)
dementia
nonblastic macrocytic anemia
liver dz, alcohol, reticulocytosis
blastic macrocytic anemia (not B12 or folate)
orotic aciduria
intravascular hemolysis findings
decr haptoglobin, incr LDH, Hb in urine
extravasculat hemolysis findings
incr LDH, incr UCB, jaundice
ACD
incr hepcidin
aplastic anemia
failure or destruction of myeloid stem cells
sx: fatigue, malaise, pallor, purpura, mucosal bleeding, petechiae, ifxn
tx: immunosupp, allogeneic bone marrow transplant, RBC and platelet transfusion, GCSF, GMCSF
hereditary spherocytosis
no central pallor
positive osmotic fragility test
pyruvate kinase def
AR. leads to decr ATP and rigid RBCs

hemolytic anemia in a newborn
HbC defect
glutamic acid to lysine mutation at position 6
paroxysmal nocturnal hemoglobinuria
impaired synth of GPI anchor of decay accelerating factor in RBC mem
labs: incr urine hemosiderin
complication: thrombosis
sickle cell
glutamic acid becomes valine at position 6
low O2 or dehydration ppts sickling
crew cut on xray
complic: aplastic crisis d/t parvoB19
renal papillary necrosis d/t low O2 in papilla and microhematuria d/t medullary infarcts
AI hemolytic anemia
warm agglutinin, IgG in SLE, CLL, alpha methyldopa

cold agglutinin, IgM, in CLL, mycoplasma, mono
direct coombs
pt RBC, anti-IgG added
indirect coombs
pt serum, RBC added
microangiopathic anemia
schistocytes. DIC, TTP HUS, SLE, malig HTN
macroangiopathic anemia
prosthetic heart valves and aortic stenosis
schistocytes
lead poisoning accumulates
protoporphyrin in blood
acute intermittent porphyria
porphobilinogen deaminase (aka uroporphyrinogen I synthase) defective
porphobilinogen, ALA, uroporphyrin acc in urine
sx: painful abdomen, red wine colored urine, polyneurop, psych disturb, can be from drugs
tx: glucose and heme, which inhibit ALA synthas
porphyria cutanea tarda
uroporphyrinogen decarboxylase defect

uroporphyrin acc in urine (tea color)
blistering cutaneous photosens. most common
substrates for ALA synthase, rate limiting and first step of heme synthesis
glucine and succinyl CoA and B6
PT tests
I, II, V, VII, X (extrinsic)
PTT tests
all except VII and XIII (intrinsic)
hemophilia A or B
intrinsic path defect, incr PTT only
platelet abnormalities
microhemorrhage: mucus mem bleeding, epistaxis, petechiae, purpura, incr bleed time, possible decr platelet count
bernard soulier
decr platelet count
glanzmann's thrombasthenia labs
blood smear shows not platelet clumping
ITP
decr PC, incr BT
defect: anti-GpIIb/IIIa Abs
lab: incr megakaryocytes
TTP
dec PC, incr BT
decr platelet survival
def of ADAMTS13 (vWF metalloprotease) leads to decr degradation of vWF multimers
incr large vWF multimers leads to incr platelet aggreg and thrombosis
labs: schistocytes, incr LDH
sx: neuro and renal sx, fever, thrombocytopenia, microangiopathic hemolytic anemia
von willebrands dz
PTT may be incr. intrinsic defect. vWF carry/protects VIII. AD.
tx: DDAVP (desmopressin), which releases vWF stored in endothelium
DIC
decr PC, incr BT/PT/PTT
schistos, incr D-dimers (fibrin split products), decr fibrinogen, decr V & VIII
widespread activation of clotting leads to def in clotting factors, which creates bleeding
Causes - STOP Making New Thrombi: Sepsis (GN), Trauma, OB probs, acute Pancreatitis, Malignancy, Nephrotic Syn, Transfusion
factor V leiden
most common cause of inherited hypercoag
prothrombin gene mutation
mutation in 3' UTR assoc with venous clots
antithrombin def
inherited, reduced incr in PTT after heparin
protein C/S def
decr inability to inactivate V and VIII. incr risk of thrombotic skin necrosis with hemorrhage after warfarin
FFP for
DIC, cirrhosis, warfarin OD
cryoprecipitate
contains fibrinogen, VIII, XIII
treats coag factor defs
leukemoid reaction
incr wbc count with left shift (80% bands) and incr leuk alk phos usu d/t ifxn
Hodgkins
50% EBV
bimodal young/old
good prog when incr Ls decr RS
Non Hodgkins
assoc with HIV and IS
extranodal, noncontig
fewer constitutional sx
peak incidence 20-40yo
Reed-Sternberg
CD30, CD15
variant include lacunar cells in nodular sclerosis variant
nodular sclerosing hodgkins
65-75%, lots of Ls. most common. collagen banding, lacunar cells. women, young adults
mixed cellularity hodgkins
25%, lots of RS and Ls, intermed prog
lymphocyte predominant hodgkins
6%. less then 35yo men
lymphocyte depleted
rare. older men with disseminated dz. bad prog
t(8;14)
burkitts. c-myc moves next to HC Ig (14)
diffuse large B cell lymphoma
usually older adults, but 20% kids
most common adult NHL
20% T cell
t(11;14)
mantle cell lymphoma. older men. transloc of cyclin D regulatory gene. CD5. poor prog.
t(14;18)
bcl-2. difficult to cure, indolent course. follicular lymphomas. adults.
adult T cell lymphoma
cutaneous lesions, japan, W Africa, Caribbean. aggressive
mycosis fungoides/sezary syndrome
cutaneous patches/nodules. indolent CD4+
multiple myeloma
CRAB: hyperCa, renal insuff, anemia, bone lytic lesions/pain
rouleaux
ALL
TdT+, CALLA+, can spread to CNS and testes. t(12;21) is a better prog
SLL/CLL
>60, asym, smudge cells. warm agglutinins.
CLL has incr periph blood lymphocytosis than SLL
hairy cell leuk
mature B cell tumor in elderly. TRAP
AML
60yo. Auer rods. myeloblasts circ. adults. t(15;17) is M3 AML which responde to all-trans retinoic acid (vitA), inducing diff of blasts. sx: DIC
CML
30-60yo. Philly chromo (t(9;22), bcr-abl. myeloid stem cell prolif. incr PMNs, metamyelocytes, basophils, Smeg, may --> blast criss
very low leuk alk phos d/t immature granulocytes

imatinib
Auer bodies
peroxidase positive inclusions in granulocytes and myeloblasts. tx can release and lead to DIC
t(9;22)
Phily. CML
t(15;17)
M3 AML
t(11;22)
ewings
t(11;14)
mantle cell lymphoma
langerhans cell histiocytosis
prolif of immature DCs. S-100 and CD1a. Birbeck granules (tennis rackets)
polycythemia vera
incr RBC, WBC, platelets. JAK2 mutation
RCC, Wilms, cyst, HCC, hydronephrosis can cause ectopic erythropoietin
JAK2 mutations (GF signaling)
chronic myeloproliferative disorders
essential thrombocytosis and myelofibrosis (positive 30-50%)
LMWH
act on Xa, better bioavail, longer half life
HIT
heparin binds platelet factor IV, causing ab production that binds to and activates platelets leading to their clearance and resulting in thrombocytopenic hypercoagulable state
Lepirudin, bivalirudin
directly inhibit thrombin. for HIT
warfarin tox
skin/tissue necrosis
thrombolytics labs, tox tx
incr PT and PTT, no chage in PC
tx tox with aminocaproic acid, an inhibitor of fibrinolysis
clopodogrel, ticlopidine moa, use, tox
irreversibly block ADP receptors
use: acute coronary syn, coronary stent, decr stroke risk
tox: neutropenia (ticlopidine)
cilostazol, dipyridamole
phosphodiesterase III inhib, incr cAMP in platelets inhibits platelet aggreg, vasodilation
use: intermittent claudication, coronary vasodilation, prevent stroke and angina
tox: N, HA, flush, hypoten, abd pain
abciximab use
acute coronary syn, percutaneous transluminal coronary angioplasty
Mtx
leuk, lymph, choriocarcinoma, sarcoma, abortion, ectopic preg

inhibits DHF reductase
tox: macrovesic fatty change in liver, teratogen, mucositis
5-FU
colon cancer and other solid tumors, BCC

inhibits thymidylate synthase
tox: photosens, leucovorin doesn't work, need thymidine to rescue myelosup
6-MP
activated by HGPRTase
leuk, lymph but not CLL or hodg
tox: BM, GI, liver, incr with allopurinol
6-TG
acute lymphoid leukemia
tox: BM depression, liver, allopurinol ok
Ara-C
pyrimidine analog
AML, ALL, high grade NHL
tox: leukopenia, thrombocytopenia, megaloblastic anemia
dactinomycin
intercalates in DNA
Wilms, Ewings, rhabdo, kids' tumors (kids ACT out)
tox: myelosupp
doxorubicin
free radicals, noncov intercalate
hodg, myeloma, sarcoma, breast, ovary, lung
myelosupp, alopecia
dexrazoxane chelates iron to prevent cardiotox
bleomycin
free radicals
testicular CA, hodg
skin changes
etoposide
SCC, prostate, testic
myelosupp, GI, alopecia
cyclophosphamide, ifosfamide
alkylating. covalently crosslink DNA at guanine. req liver activ

NHL, breast, ovary. IS
myelosupp, hemorrhagic cystitis (mesna helps)
nitrosurease (carmustine, lomustine, semustine, streptozocin)
req bioactiv. CNS. alkylates DNA

brain tumors
dizzy, ataxia
busulfan
Alkylates DNA

CML, ablate BM before transplant
hyperpigment, pulm fibrosis
vincristine, vinblastine
hodg, wilms, chorio, ALL
tox: vincristine: neurotox and paralytic ileus
vinblastine blasts bone (BM sup)
paclitaxel
ovarian, breast
tox: myelosupp, hypersens
cisplatin, carboplatin
crosslink DNA
testic, bladder, ovary, lung
tox: nephro, acoustic nerve damage, prevent nephro with amifostine (free radical scavenger) and chloride diuresis
hydroxyurea
inhibits ribonucleotide reductase, decr DNA synth

melanoma, CML, sickle cell
BM supp, GI
prednisone
CLL, Hodg (part of MOPP)
tox: cataracts, PUD, psychosis
tamoxifen
may incr risk of endometrial CA bc partial agonist. hot flashes. not raloxifene.
trastuzumab
herceptin. tyrosine kinase
cardiotox
imatinib
also GI stromal tumors
edema
nephrotoxic, acoustic nerve damage
cisplatin, carboplatin
periph neuropathy
vincristine
cyclophosphamide
hemorrhagic cystitis