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