hematologic Flashcards
anemia
- is a sign, not a dz
- correct us of tests is paramount
- dx of iron deficiency anemia –> locate the etiology
morphologic approach to anemia
micro- MCV 100
folic acid, ETOHic, liver disease
normocytic- MCV 80-100
- anemia of chronic dz
- anemia of chronic renal failure
- multifactorial anemia
RBC destroy after 120 days- hemolysis in the spleen
retic count: baby RBC cells, increase to erythropoeitein
- help evaluate bone marrow function
kinetic approach:
low retic count- problem in marrow, lack of nutrients
bone marrow suppression
high retic count- blood loss, hemolytic anemias, sick cell, autoimmune, drug-induced , thallassemias
check WBC and plts
if low retic- acute blood loss
renal dz, drug effects
low plts and low WBC- sign bone marrow failing
leukemia, myelosuppresion, TB in marrow
iron metabolism
serum iron is free
TIBC- how hungry is our blood for iron.
ferritin- stores iron in liver and RES.
signs of iron deficiency-most common cause of anemia
- spoon nails
- glossitis
- esophageal web- Plummer-Vinson syndrome
- restless legs , check ferritin
- pica- chewing ice
- hair loss
etiology: bleeding, GI bleed, cancer, menses, blood donation, growth periods, pregnancy, lactation, gastric surgery, malabsorption, calcium inhibits GI
tx: oral iron : ferrous sulfate 325 mg bid -constipaion -6-8 weeks to recheck level -vitamin C helps -IV iron -check ferritin in 3-4 weeks
sickle cell
substitution of AA valine from glutamic to form beta s globin. Hbs
-8-10% AA carry gene
-use hemoglobin electrophoresis to dx
( presence of Hbs, higher levels of HbF)
- autosomal recessive
pathophys: deoxygenated hb s polymers causes sickling and damage to membrane
disease: functional splenic ( trivalent vaccine to prevent infections)
sickle cell trait
- no symptoms
- high O2 demand/ dehydration states can develop crisis
- over years can have microinfarts causing kidney damage/cardiac damage
sickle cell anemia
chronic hemolysis -moderate anemia -reticulocytosis - elevated t.billi -incr endothelial RF: if hgb > 8.5- more crisis pregnant pt cold weather high altitude
CHF/cardiac problems.
acute sickle cell crisis
-caused by stress syndrome
-last 1-2 weeks
fever, elevated WBC, acute chest syndrome ( infants, wheezing)
exchange transfusion
- splenic sequestration (LUQ–> hypovolemia, emergecny splenctomy)
-bone infarcts
-asvascular necrosis
-oxgyen, hydrate, and pain meds.
-get strep pneumo/ H.fu
-leg ulcers
-parvoviru 19-fatal
hand/ foot syndrome
maintenance: chronicle followed with CBC, given folic acid,
screening transcranial dopplers, retinal exams, multivalent vaccine
Beta-thalassemia trait
mild-mod anemia
- hgb: 9-11
- mishapen cells
- no sxs
- don’t give erythropotein
beta thalassemia major
- sever anemia
- infants: develop anemia in first weeks
- cardiac stress, hepatosplenomegaly, chipmunk facies ( bone deformity)
tx: allogenic bone marrow transplant
alpha thalassemia syndromes
alpha thalassemia 2 trait- loss of 1/4 alpha globin genes
- no abn MCV
alpha thalassemia-1 trait (minor)- loss of 2/4 alpha globin genes
- mild anemia, MCV is often
Anemia of chronic dz
- reduction is RBC production in bone marrow
- ferritin increased
- iron not available to make new hemoglobin
- low serum iron, TIBC is low, low iron saturation, normal or elevated ferritin
causes: chronic UTI, BC
met cancer, chronic leukemia, RA, UC, thyroid , DM
dx: hgb 8-10
retic count-low
reduced iron and and low TIBC, elevated ferritin
exclude renal failure
tx: manage underlying dz
- EPO
- only given iron if iron deficiency
- transfusion for acute issue
acute variant anemia
- infection, MI
after surgery, major trauma
low serum iron
-high ferritin
hemolytic anemia
caused by premature breakdown of RBC
-severity of anemia related to rate of RBC destruction and ability of bone marrow produce reticulocytes
s/s: acute pallor splenomegaly jaundice -incr LDH -low haptoglobin -incr. retic
hereditary spherocytosis
forms spheroctic cells are destroyed on spleen.
s/s: splenomegaly and jaundice
dx: on blood smear
tx: splenectomy but vaccinate
G-6-PD deficiency
RBC depend on anaerobic metabolism
10% black have this
bite cells and Heinz bodies
fauvism: -ingestion of Fava beans cause problems in patient
aplastic anemia
- pancytopenia
- rx: hematopoietic cell transplant of HLA compatible
-cyclosporine
-supportive
-IVIG- help immune sysmtpe
ATG-immunosuppresan
causes: chemo tx and rad tx
megaloblastic anemia
ETOHism elevated MCV
- vitamin b12 low
- folic acid deficiency
- chemo tx
b 12 defieicny
- s/s: neuro sxs
- tx with folic acid will improve anemia
elevated MMA
homocysteine elevated in both b12 and folate deficiency
s/s: dementia, depression,
tx; SQ B12 7 days , 7 weeks, and then mostly
orally: 1-2 mg daily
pernicious anemia
- autoimmune gastritis
- attack on gastric intrinsic factor
- incr riskigastric cancer
- 25% autoimmune thyroid disorders
Labs: RBC show macrocytosis ( MCV> 100)
folic acid deficient
caused by nutritional deficiency, ETOH abuse, malnutrition
s/s: like pernicious anemia
dx: serum folic acid love
tx: 1 gm daily supplement
all child-bearing ladies should take .4 mg
ITP
-idiopathic, autoimmune
s/s: petechial hemorrhage, mucosal bleeding and thrombocy
Plt count
TTP
-rare, severe infection, HIV, plalvix
FAT RN F-evere A- anemia T-thrombocytopenia R-renal impairment N-neurologica
tx: plasmapheresis
Von Willebrand
4 types
- most common bleeding disorder
labs : PTT and BP slightly elevated
VWF levels are low
s/s: mucosal bleeding
tx; DDAVP, plt transfusion, factor IX
hemophilia
hemophilia A- affect mailes - defective clotting factor -PTT prolonged dx: quant/qual fictive VII test tx; mild DDVAP
hemophilia B: Christmas tree
- factor IX
dx: quant IX tes
tx: replace IX concentrate
factor xi deficiency
ashkenazi jewish
-bleeidng after trauma
-very rare
tx with Factor XI
DIC
systemic- thrombosis and hemorrhage
- d/s results from;
tissue damage, burns,
sepsis, severe allergic reaction - can be seen by pancreatic cancer
tx: blood thinners or tx bleeding ( FFP, plt transfusion)
check fibrinogen level daily
Acute leukemia
RF: rad tx, chemo, down syndrome, organic solvents
ALL-
kids 3-5
-20% leukemia, most are kids
- philadelphia chromosome ( incr risk for patients)
TX: induction chemotherapy X 2 years and then trasplan
cure rate high
AML- age 60
-auer rods from myeloid granules
labs: higher WBC ( 100, 000, leukophoresed)
TX: induction chemo, stem cell transplant if failed relapse
sx: bone marrow failure
( infection, bleeding, sepsis)
CLL
- most common leukemia
- age 60
- therapy only if recurrent infection, double of WBC,
tx: chemo and rad tx
CML
- chromosone 22
- can remain in chronic phase ( wBC 20-100, 00)
- plastic crisis> 20% blasts
tx: Gleevec is new tx
-80-85% go to remission
life long
labs: WBC
elevate basophils and e
multiple myeloma
- accumulation of plasma cells in bone marrow or visceral soft tissue
- sxs: CRAB
C-caclium elevation
R-renal insufficiency
A- anemia BM suppression
B-bone lytic lesions
dx: 1 major and 1 minor major- tx: incurable (2-7 years) stem cell transplant decahedron chemo tx monitor closely for infections
NHL
- incr age and males
- chronic immunosuppression
- chemo /rt
- chronic infections ( EBV, mono, HIV, Hpylor, HHV)
- SLE, RA
s/s: weight loss, painless lymph nodes, night sweats, fever, pruritus, chest/ pain
dx: CT, PET
elevated WBC, LDH
bx nodes
tx: chemo/ rad tx
Rituxan
stem cell transplant
hodgkins lymphoma
younger men and older men
- HIV, fix
- head and neck nodes
- weight loss
- prutis
dx: LN bx with Reed Sternberg cells, high LDh
- imaging -bulky LAD, orderly spread
B-sx -night sweats
course: indolent or aggressive
tx: chemo, rt, stem cell for refractory dz
px: good , 75% curable
