heme Flashcards
mechanism of polycythemia vera
EPO: high/low
jak2 mutation causing proliferation of bone marrow stem cells
EPO low( jak2 mutation don’t care about EPO
high Hb, pain in fingertips, pruritus with exposure to hot water: Dx & treatment
Phlebotomy
Polycythemia vera
old, fatigue, Hb low: cause
GI bleed
diverticular bleed
colorectal cancer
angiodysplasia
smoker, COPD+high Hb mechanism?
EPO level?
2nd polycythemia
high EPO d/t low O2
smoker, Polycythemia , hypercalcemia, high creatinine
Renal cell carcinoma
d/t paraneoplastic PTHrP secretion
Bleed Time increase (2-7min)
Bleeding on surfaces
vWF
platelet adhesion
school age kids with viral infection
+epistaxis, bruising/petechiae, low platelet.
ITP
Low platelet, high bleeding Time, random bruising 30-40F
ITP
30F random bruise+BT 6,
domestic abuse
40F bruise +BT 9
ITP(idiopathic/immune thrombocytopenia purpura
35F +viral infection+epistaxis ITP DX confirm with:
low platelet
13M + epistaxis +BT 9
ITP
Teen cough, coryza, epistaxis
antibodies against
glycoproteins IIB/IIIA
HS type II
Teen nose bleed, very low platelet manage with
Initial treatment?
No response?
steroid
Non responsive: IVIG
Teen nose bleed, very low platelet , effective way to decrease recurrence
splenectomy
NOT steroid (only initial treatment)
Teen +viral infection+ low neutrophil
next best step
viral induced neutropenia
immediate IV broad-spectrum antibiotic
Teen +viral infect. Neutropenia, fever +antibiotics. Restore Neutrophill with?
GM-CSF (IL3)
child +pancytopenia+low platelet+viral DX
B19
aplastic anemia
32F+ arthritis+ Hb 9+WBC 3k+ Platelet 90k mechanism
note no viral infection
increased peripheral destruction
autoantibodies
antiphospholid
overactive thyroid treatment low WBC(neutrophil 5%
Drug-induced neutropenia
methimazole & propylthiouracil
drug causing agranulocytosis
ganciclovire (CMV)
Clozapine (anti-psychotic0)
methortrexat(DMARD)
ticlopidine(anti-platelet )
child with absent or hypo plastic thumb low Hb, WBC, platelet
falconi anemia
AR aplastic anemia
pure RBC aplasia
triphalangeal thumb (Diamond-blackfan anemia
Thymoma associated with anemia
pure RBC aplasia
(myasthenia gravis)
daycare worker with lacy rash, no aplastic anemia, check
Parvo IgM titers
44F chemotherapy low RBC, WBC, platelets next step
bone marrow aspiration
chemo with all cell line low (neutropenia)
IG broad spectrum antibiotic
PT high
PTT high
BOTH high
extrinsic (III, 7,5,10
intrinsic( 12,9,8, 10
common (10,5, prothrombin(II), thrombin(IIa)–>fibrinogen(1)–>fibrin(1a)
Bleeding time high with aspirin why?
lower platelet function
inhibit CoX1/2
decrease thromboxane A2 production
Increase fibrin degradation product seen with
DIC and PE
treatment for BT9, petechiae, PTT 42 , Normal platelet
DDAVP (desmopressin) increases vWF
Hemarthrosis in school age boy
Hemophilia A/B
the child with hemarthrosis, PTT 90
Hemophilia A/B
inheritance pattern for vWF
AD
hemophilia inheritance
X-LR
cause of Hemophilia A/B
deficient production of factor 8/9
Hemophilia A mc
Hemophilia A treatment
IV desmopressin/factor 8 replacement
Hemophilia B
factor 9 replacement
repeated factor replacement(like repeated antibody venom treatment)
PTT very high
factor 8/9 less effective
Low HbA, high HBA2 and HbF in 8 month old boy
Insufficient production of Beta globin
symptomatic after 6 month
How is Nephrotic syndrome associated with acquired clotting state
loss of natural anticoagulants
antithrombin III
antithrombin III function
natural anticoagulant
inhibit factors IIa (thrombin) and 10a
Restocitin neg dz , normal platelet
vWF and Bernard-Soulier ds
BT high with pos Restocitin, Normal platelet
Glanzmann thrombasthenia
AoCD mechanism
inflammatory state –>high IL6 liver–>___hepcidin–>dec ferroportin activity–>___iron released by gut enterocytes and macrophages—> iron transport___—->ferritin level____—–>Transferrin and TIBC____
elevated hepcidin
decrease all others
12F+chronic renal failure +epistaxis, Hb low? explain epistaxis
renal failure cause uremic platelet dysfunction
high BUN
Note: epistaxis is seen with platelet dysfunction
82M+ M spike IgG+epistaxis. explain epistaxis?
uremic platelet dysfunction 2nd to MM(renal amyloidosis)
effect of imatinib and dihydropyridine Calcium channel blocker(nifedipine, amlodipine)
fluid retention
Imatinib treats CML(9:22 bcr/abl)
10M + lytic bone lesion with pos CD1a,S100
Langerhan Cell histiocytosis
Birbeck granules
MOA of rituximab
Indication
CD20 inhibitor on B cells
RA, ITP, MS, AIHA, B cell Non-hodgkin(CLL)
Rituximab reactivate
JC virus
Toxicity of ___________ mitigated with saline (NaCl-) infusion and amifostine
cisplatin
important side effect with cisplatin
Oto and Neurotoxicity
decrease IL-2 responsiveness+ no effect on calcineurin
Sirolimus
decrease calcineurin
cyclosporine +tacrolimus
MOA of cyclosporine and tacrolimus
dec calcineurin+dec IL-2 transcription +nephrotosic
Sirolimus(mTOR) is save in
kidney failure, not nephrotoxic
gingival hyperplasia +nephrotoxicity +BP
cyclosporine
antagonist at cyclophilin receptor
cyclosporine
antagonist at FK506 receptor to decrease calcineurin
important side effect
Tacrolimus
Type II diabetes
Mesna is use to
reduce cyclophosphamide toxicity using thiol-SH group
hemorrhagic cystic due to acrolein
cyclophosphamide
guanine N7 alkylating agent
MOA of cyclophosphamide
inhibitor
topo I
Topo II
irinotecan/topotecan
etoposide/teniposide
HER-2/New(ERBB2) treatment
trastuzumab
trasTUzumab toxicity
Cardio
partial agonist in endometrium increasing endometrial cancer
tamoxifen
SERMs
Tamoxifene and raloxifene
Both antagonists at ER receptors in the breast
agonist on bone
fluid retention in CML treatment
Imatinib
bar/abl tyrosine kinase inhibitor for CML
inmatinib
Ab against EGFR
cetuximab
MOA of erlotinib
EGFR tyrosine kinase inhibitor
Notable indicator of erlotinib
non-small cell lung cancer
dexrazoxane chelation of free radicals
toxicity of doxorubicin/daunorubicin
dilated cardiomyopathy with a DNA intercalator
doxorubicin/daunorubicin
DNA intercalates + cause free radical
doxorubicin(Adriamycin)/daunorubicin
pulmonary fibrosis Drug?
Free radical formation
Bleomycine
vincristine toxicity
neuro
hyper stabilize microtubules
paclitaxel/docetaxel
inhibit microtubules formation
cochicine, -bendazoles (mebendazole, albendazole)
vincristine
vinblastine,
griseofulvin
Inhibits IMP dehydrogenase (purine synthesis)
mycophenolate mofetil
pyrimidine synthesis inhibitor (ribonucleotide reductase inhibitor
hydroxyurea
produces more fetal Hb
sickle cell pt
converted to 6-MP
inhibits purine synthesis
azothioprine
MOA of 6-mercaptopurine
PRPP amidotransferase inhibitor(purine synthesis inhibitor)
MOA of 5- fluorouracil
thymidylate synthase inhibitor
leucovorin rescue use in
methotrexate toxicity
reversible, competitive inhibitor of dihydrofolate reductase; 1st line DMARD for RA
Methotrexate
Methotrexate side effect
pulmonary fibrosis, hepatotoxicity, neutropenia
antifibrinolytic drugs (tPA: stroke)
tranexamic acid, aminocaproic acid
tPA fibrinolytic should be given
in ischemic stroke within 3-4.5 hr
MOA of apixaban
factor Xa inhibitor(band)
MoA of fondaparinux
inhibits factor Xa (activate antithrombin)
treatment for heparin induce thrombocytopenia
Bivalirudin,
lepirudin
dabigatran
argatroban
Both anti-platelet agents and vasodilators
dipyridamole +cilostazol, both cAMP and cGMP phosphodiesterase inhibitors
Gp2b/3a inhibitor
abciximab
eptifibatide
tirofiban
MOA of
clopidogrel
prasugrel
ticagrelor
ticlopidine
ADP2Y 12 receptor blockers
inhibit aggregation by dec expression of Gp2b/3a