Hematology/Oncology Flashcards
compensatory mechanisms in anemia
increased CO
increased extraction ratio
RIGHT shift on Oxy-Hb dissociation curve (increased 2,3 BPG)
expansion of plasma volume
general indications for blood transfusion
- Hb < 7 g/dL
2. pt requires increased O2 carrying capacity - i.e. pt with CAD or cardiopulm dz
what is pseudoanemia?
decrease in Hb and Hct secondary to dilution (acute volume infusion or overload)
MCC of death due to anemia
myocardial ischemia
formula for converting Hb to Hct
Hb x 3 = Hct
how does 1 Unit of packed RBCs affect Hb and Hct levels?
increases Hb level by 1 point and Hct by 3 points
if pt has low Hb and Hct, what are the next tests to determine cause of anemia?
reticulocyte count
mean corpuscular volume
reticulocyte index > 2%
excessive RBC destruction and blood loss - the BM is responding to increased RBC requirements
reticulocyte index < 2%
inadequate RBC production by BM
how do you administer Packed RBCs?
mixed with Normal Saline and infuse 1 unit over 90-120 minutes; check CBCs once complete
what does fresh frozen plasma contain?
all of the clotting factors - no WBCs, RBCs or platelets
what is FFP given for ? (3)
- high PT/PTT
- coagulopathy
- deficiency of clotting factors
how do you assess response of FFP?
look at PT and PTT
what is cryoprecipitate and what is it used for?
- contains factor VIII and fibrinogen
- used for: hemophilia A, DIC and vWD
1 unit of platelets raises platelet count by how much?
10 000
what is the ONLY time you use whole blood?
massive blood loss
- in a ratio of 1:1:1 of platelets:FFP:PRBCs and warmed
a pt who recently received a blood transfusion suddenly develops fever, chills, NV, flank pain, chest pain and dyspnea - what should you be worried about?
intravascular hemolysis due to ABO-mismatched blood
management of acute hemolytic reaction due to ABO-mismatched blood
- stop transfusion
- aggressive fluid replacement to avoid shock/renal failure
- Epi. for anaphylaxis
- dopamine/NE to maintain BP
a pt who received a blood transfusion 2 weeks ago develops mild fever, jaundice and anemia - what should you consider?
delayed hemolytic transfusion reaction (Extravascular hemolysis) due to mismatched minor RBC antigens
what is the next step in assessing anemia if the reticulocyte index is < 2?
exam smear and RBC indices- ie. MCV
differential diagnosis of microcytic anemia (MCV < 80)
iron deficiency
anemia of chronic disease
thalassemias
ring sideroblastic anemias
differential diagnosis of macrocytic anemia (MCV > 80)
vit B12 /folate deficiency
liver disease
stimulated erythopoiesis
differential diagnosis of normocytic anemia (MCV 80-100)
aplastic anemia BM fibrosis tumor anemia of chronic disease renal failure
what do you need to R/O in elderly pt with iron deficiency anemia?
colon cancer
major causes of iron deficiency anemia
- chronic blood loss - menstrual or GI loss
- dietary deficiency
- increased requirements - i.e. growth, pregnancy, lactation
what is the most reliable test for diagnosing iron deficiency anemia?
decreased serum ferritin (below 10 ng/ml)
what lab findings are characteristic of iron deficiency anemia?
low serum ferritin
low serum iron
high TIBC and low TIBC saturation
high RDW
what can cause a falsely elevated serum ferritin
malignancy and inflammatory states - ferritin is also an acute phase reactant
what is the gold standard for dx. iron deficiency anemia?
bone marrow biopsy
- rarely performed unless no
first step in tx. of iron deficiency anemia?
oral iron replacement w/ ferrous sulfate
- give trial to menstruating woman but in everyone else, attempt to determine point of chronic blood loss
young patient is brought in with massive hepatosplenomegaly, expansion of marrow space, growth retardation and failure to thrive; CBC reveals microcytic anemia - dx?
B-thalessemia major (aka. Cooley’s anemia)
how do you dx. B-thalassemia major?
hemoglobin electrophoresis –> HbF and HbA2 are elevated
Tx. of B-thalessemia major?
frequent PRBCs transfusios with desferrioxamine to prevent iron overload
if you suspect iron deficiency anemia, but it does not respond to iron - what should you do next?
Hb electrophoresis to R/O thalassemias
what happens if you have deletion of 2 alpha globin loci?
mild, microcytic hypochromic anemia
common in African-Americans
usually asx. w/ no tx. needed
pt comes in with hemolytic anemia, splenomegaly, and significant microcytic hypochromic anemia; Hb electrophoresis shows Hb H - what should you consider?
Hb H disease - mutation/deletion of 3 alpha globlin loci
what is Hb H
tetrads of B-globin chains
what is Hb-Barts?
tetrads of Y-globin chains form when there is deletion or mutationof all four alpha-globin loci resulting in fatal hydrops fetalis
which alpha-thalassemia requires tx and what is the tx?
HbH disease - tx. w/ frequent PRBC transfusions w/ desferrioxamine
what are the iron studies findings in thalassemia pts?
normal/high serum ferritin
normal/high serum iron
normal TIBC
normal/high RDW
what are the iron studies findings in sideroblastic anemia pts?
increased serum iron and ferritin
normal/low TIBC
TIBC saturation is normal/high
what are some acquired causes of sideroblastic anemia?
drugs - chloramphenicol, alcohol, INH
lead exposure
collagen vascular disease
myelodysplastic syndromes
what are the hereditary causes of sideroblastic anemia?
defect in ALA synthase
abnormal vit B6 metabolism
what are the iron studies findings in anemia of chronic disease?
low serum Fe
low TIBC
low serum transferrin
high serum ferritin
characteristic finding in aplastic anemia
BM is empty with no precursor cells leading to pancytopenia (anemia, neutropenia and thrombocytopenia)
causes of aplastic anemia
idiopathic radiation exposure drugs - chloramphenicol, NSAIDs, alcohol, sulfonamides, gold, carbamazepine viral infection chemicals - benzene, insecticides
how do you definitively dx. aplastic anemia?
bone marrow biopsy
what is the definitive tx. of aplastic anemia?
bone marrow transplantation
what two reactions is vit B12 necessary for?
conversion of homocysteine to methionine
conversion of methylmalonyl CoA to succinyl CoA
what is the characteristic neuropathy in vit B12 deficiency?
demyelination of posterior columns, lateral corticospinal tracts and spinocerebellar tracts
what kind of cancer are pts with pernicious anemia at higher risk for?
intestinal type - gastric ca.
gastric carcinoid
- perform periodic stool testing for FOB
diagnostic findings in vit. B12 deficiency
- hypersegmented neutrophils
- serum vit B12 level < 100
- serum methylmalonic acid and homocysteine levels elevated
- ab’s against IF
what Schilling test result implies dietary deficiency of vit B12
after giving radioactive Vit B12, if urine levels are > 5%
what do you do if in the Schilling test, urine levels are below normal (<5%)?
give radioactive vit B12 w/ IF –> if they go up after, then you know you have IF deficiency; if they do not go up, it is due to malabsorption
tx. of vit B12 deficiency
cyanocobalamin IM once per month
serum methylmalonic acid levels are only increased in what deficiency?
vit B12
- this test allows you to tell it apart from folate deficiency
what general lab findings do you see in hemolytic anemia?
elevated reticulocyte count
elevated LDH and bilirubin
decreased haptoglobin
decreased Hb/Hct
what kind of cells can be seen on PB smear in thalasemia?
target cells
what kind of cells on smear are characteristic of TTP, DIC, prosthetic heart valves and hemolytic anemias?
schistocytes
helmet cells
what characteristic clinical features (in addition to those of anemia) can be seen in hemolytic anemias?
jaundice
dark urine - esp. IV processes
what kind of cells are seen on smear in intravascular hemolysis)
schistocytes
which cells suggest extravascular hemolysis?
spherocytes
helmet cells
what types of cells characterize G6PD deficiency?
heinz bodies
what vitamin should be supplemented in hemolytic anemia?
folate
sudden drops in Hct are usually due to..
parvovirus B19 infection
folate deficiency - acute aplasia
what should you suspect in a young blank male who presents to you with painless hematuria?
sickle cell trait
what is really the only clinical feature seen in sickle cell trait?
isosthenuria and increased rate of UTIs
what does the prognosis of sickle cell disease depend on?
the frequency of vaso-occlusive crises
MC clinical manifestation of sickle cell disease vasoocclusive crises
painful crises involving bone - multiple sites
what is usually the first manifestation of sickle cell disease?
hand-foot syndrome (dactylitis) - painful swelling of dorsa of hands and feet seen in infancy
splenic sequestration crises
in sickle cell dz, sudden pooling of blood into spleen results in rapid development of splenomegaly and hypovolemic shock; potentially fatal
what is priapism due to in sickle cell crises?
infarction of prostatic plexus of veins
tx. that can potentially prevent recurrence of priapism in sickle cell dz
hydralazine
nifedipine OR
antiandrogen (stilbestrol)
what types of infections are sickle cell pts susceptible to?
encapsulated bacterias - SHiNS
salmonella osteomyelitis
screening test for sickle cell trait/disease
sickle cell prep - blood smear with chemical added to remove O2 from hemoglobin - looks for sickled cells
diagnostic test for sickle cell dz
hemoglobin electrophoresis
what should sickle cell pts receive prophylactically until age 6?
penicillin
folic acid supplments in definitely
which drug is used to enhance HbF levels and reduce incidence of painful crises in sickle cell dz?
hydroxyurea
which conditions in sickle cell dz warrant consideration for blood transfusion?
acute chest syndrome
priapism unresponsive to other measures
cardiac decompensation
stroke
causes of spherocytosis
hereditary G6PD deficiency ABO incompatability hyperthermia autoimmune hemolytic anemia
diagnostic test for hereditary spherocytosis
osmotic fragility test - RBC burst in hypotonic saline
lab findings in hereditary spherocytosis
elevated reticulocyte count
elevated MCHC
direct Coombs test result is negative
what is the Tx. for hereditary spherocytosis?
splenectomy
- vaccinate against pneumo, H.flu and meningococcus several weeks before