Heme Flashcards
Diseases with macrocytosis
B12 def
folate def
liver dz
aplastic anemia
diseases with microcytosis
fe def
thalassemia
lead poisoning
“howell jolly bodies”
splenectomy or asplenism
“Heinz bodies” and “bite cells” (2)
thalassemia
G6PD def
“target cells” (2)
thalassemia
sickle cell
“basophilic stippling”
lead poisoning
“burr cells” (echinocytes)
uremia
“bands” seen
bacterial infx
Fe def anemia: high or low ferritin?
ACD: high or low ferritin?
Fe def: low ferritin
ACD: high ferritin
RBC life span
120 days
list (non-obvious) lab values for hemolytic anemia (5)
inc reticulocytes inc LDH inc indirect bili dec haptoglobin \+ schistocytes
Hgb S on electophoresis
sickle cell
microcytic anemia
normal Fe
electrophoresis normal
alpha thalassemia
microcytic anemia
normal Fe
electrophoresis with low HgbA, high HgbF
beta thalassemia
MC presentation of alpha thalassemia major?
stillbirth
When do sx appear for beta thalassemia major
6 months
treatment for beta thalassemia major?
deferoxamine (chelating agent)
bone marrow transplant
weekly transfusions
what will iron labs show in ACD (3)
low iron
low TIBC
high ferritin
treatment of ACD in addition to treating underlying disease?
epo
management for someone with G6PD symptoms
self-limiting, remove offending agent if possible
supplement or transfuse if severe
if someone is a carrier of the sickle cell trait, what is the only symptom they might have
episodic hematuria
when do sx start for sickle cell anemia and what are the sx
6 months, dactylitis MC 1st presentation
other than dactylitis, what are 3 other presentation of sickle cell anemia
- dactylitis
- infections (salmonella osteo, asplenia)
- hemolytic anemia
- thrombotic sx (chest pain syndrome, splenic crisis, AVN)
what pain med can you NOT use in sickle cell dz
meperidol (demerol)
management of sickle cell crisis
1st step IV hydration and O2*
Hydroxyurea
Folic acid
Vaccinate for spneumo, hinf, nmeningitis
diagnostic tests for hereditary spherocytosis
microspherocytes that are hyperchromic
+ osmotic fragility test
- coombs test
diagnostic tests for autoimmune hemolytic anemia
microspherocytes
+ coombs test
smear w/ agglutination of rbcs
tx for hereditary spherocytosis
folic acid
splenectomy TOC
warm v cold autoimmune hemolytic anemia
warm - caused by SLE, malignant
IgG Ab
cold- caused by the cold, infx
IgM Ab
tx for autoimmune hemolytic anemia
warm- steroids
cold - warm up
“cola cola urine in early am”
paroxysmal nocturnal hemoglobinuria
best test for paroxysmal nocturnal hemoglobinuria
flow cytometry
best tx for paroxysmal nocturnal hemoglobinuria
ecluzimab (anti complement ab)
list the extrinsic factors (PT)
1, 2, 5, 10
7
list the intrinsic factors (PTT)
1, 2, 5, 10
8, 9, 11, 12
which med prolongs PT
warfarin
which med prolongs PTT
heparin
happens to kids, following a viral illness, with mucocutaneous bleeds and petechiae.
labs show thrombocytopenia, PT/PTT normal
dx and treatment
ITP
tx kids by observing x 6 mos +/- ivig
adults needs ivig/steroids
a patient with s/s of mucocutaneous bleeds, hemolytic anemia, kidney failure.
labs show thrombocytopenia, normal PT/PTT, increased ind bili, decreased haptoglobin
what 2 disease should you suspect
TTP and HUS
what is the major symptom difference between TTP and HUS
TTP will have near symptoms and fever
HUS more likely to have kidney sx
what is the major patient population difference between TTP and HUS
TTP: females 20-50yo
HUS: kids after a diarrheal prodrome
what is the tx for TTP versus HUS
TTP: plasmapheresis and steroids
HUS: observe kids, +/- plasmapheresis
hemophilia a
what factor
intrinsic or extrinsic
factor 8
intrinsic
hemophilia b
what factor
intrinsic or extrinsic
factor 9
intrinsic
what is the treatment of hemophilias
A: Factor 8 replacement and DDAVP
B: Factor 9 replacement only
does VWD increase PT or PTT
increased PTT
due to decreased factor 8 associated with it
gold standard test for VWD
risotecin activity test
no platelet aggregation with risocetin
tx for mod to severe VWD
DDAVP
Cryoprecipitate (with factor 8, fibrinogen, vwf)
which lymphoma
- peaks in 20s and again in 50s
- peaks 50+ with hx immunosuppression
- hodgkins
2. non hodg
which lymphoma
- is peripheral with noncontiguous LAD
- is upper body with localized LAD
1 non hodg
2 hodgkins
which lymphoma has reed sternberg cells with owl eye appearance
hodgkins
which lymphoma shows diffusely large b cells or follicular
non hodgkins
which lymphoma commonly has b sx
hodgkins
which lymphoma is assoc with the “pel ebstein fever” 1-2 weeks of cyclical fevers
hodgkins
which lymphoma is more assoc with EBV
hodgkins
also Burkitt’s which is a NHL
leukemia assoc with kids with >30% blasts
ALL
auer rods
AML
+ philadelphia chromosome
CML
indolent chemo that doesn’t need treatment until there is a crisis
CLL
“smudge cells”
CLL
how do you treat a blastic crisis
like AML - combo chemo and BMT
“rouleaux formation” on CBC
multiple myeloma
tx for multiple myeloma
stem cell transplant
chemo, bisphosphonates
other than phlebotomy, what is med tx for polycythemia vera?
hydroxyurea
extra-liver sx of hemochromatosis (4)
heart failure
hypogonadism
pancreatic insufficiency
bronze skin
