Hematology Flashcards
Protein most responsible for iron transport in plasma
Transferrin
Protein that represents an indirect measure of total body iron stores
Ferritin
Iron supplements should be taken in an empty or full stomach?
Empty since food inhibits iron absorption
After iron supplementation, reticulocyte begin to increase in how many days
4-7 days
Tx must be sustined for 6-12 months
When do you transfuse blood for IDA?
reserved for symptomatic anemia, cardiovascular instability and continued and excessive blood loss and who require immediate intervention
Formula for amount of iron needed by an individual patient
Body weight x 2.3 x (15-px hb in g/dL) + 500 or 1000 for iron stores
Pathogenic changes in the ____ protein can cause spherocytosis
Ankyrin
whereas changes in junctional complexes = elliptocytosis
Stomatocytosis= abnormalities of channel molecules
Other notes: anemia of hereditary spherocytosis is usually NORMOcytic
When do you advocate for splenectomy in patients with hereditary spherocytosis
Mild - avoided
Moderate - delayed until puberty
Severe - 4-6 yrs of age
must vaccinate against encapsulated organisms BEFORE splenectomy
Most typical feature of G6PD deficiency on blood film
Poikilocytes
Expected levels of the following in G6PD deficiency
Hemoglobin
LDH
Haptoglobin
Decreased hemoglobin due to hemoglobinuria
Hig LDH due to hemolysis
Low to absent haptoglobin because haptoglobin binds free hemoglobin hence low in intravascular hemolysis
Tx for acute hemolytic anemia in G6PD deficiency
No specific tx unless severe which may require transfusion
If there is acute renal failure, HD may be necessary
If with chronic non severe hemolytic anemia, regular folic acid supplements and surveillance will suffice
Next line of tx when steroids do not improve autoimmune hemolytic anemia
Rituximab
If very rare and severe refractory case, may need high dose cyclophosphamide OR anti CD52 Alemtuzumab
Predictors or probability of relapse of autoimmune hemolytic anemia (AIHA)
Severe anemia (Hb <6 g/dL)
Certain characteristics of Ab
Acute renal failure
infection
Which presents as chronic and indolent : cold agglutinin dse or warm AIHA ?
Cold
C= chronic
No predominance of spleen in Cold agglutinin dse
First line tx for cold agglutinin dse
Rituximab
Prednisone and splenectomy = NOT effective
Poisoning from which susbance is associated with Burton’s line?
Lead poisoning
Burton’s lie = bluish pigmentation of gum tooth line
Also assoc with basophilic stippling
so mga assoc with blue = lead
Paroxysmal nocturnal hemoglobinuria is due to deficiency of what surface proteins?
CD 59 and 55
Tx for PNH
Allogeneic transplant - defiinitive
Eculizumab for INTRA not extravascular hemolysis
do NOT give steroids- no effect on chronic hemolysis
Any px with venous thrombosis should be on anticoag prophy
How does pernicious anemia cause cobalamin deficiency?
anti IF antibodies decrease the availability of IF that binds to cobalamin
How does ZES cause cobalamin deficiency?
Inc acid causes inactivation of trypsin resulting in failure to release cobalamin from R binding protein
How does GVHD cause cobalamin deficiency?
Malabsorption of cobalamin due to abnormal gut flora and ilealmucosa damage
Characteristic peripheral blood smear pattern of thalassemia
Target cells
Another way of differentiating thalassemia from IDA is that IDA has low ferritin and obv low iron
Most common hypoproliferative anemia
IDA
next: anemia of inflammation
Mechanism most contributory to anemia in px with CKD
Dec EPO and reduction in red cell survival
First line for severe aplastic anemia
HSCT
Only tx that offers a chance of cure for MDS
HSCT
However due to significant tx related mortality and morbidity that increases with recipient age, only small proportion undergo this
The only symptoms that would differentiate polycthemia vera from other causes of erythrocytosis
Aquagenic pruritus
Erythromelalgia
1st step in the evaluation of elevated hemoglonin
Measure RBC mass
What would you consider when px has elevated rbc mass and low hb
Polycthemia vera
Confirm JAK2 mutation
Next step when px has elevated hb, rbc mass and elevated EPO
Measure arterial o2 sat
If low: diagnostic eval for heart or lung dse
if normal: ask if smoker or not.
If smoker: measure carboxyhemoglobin levels.
Next step when px has elevated hb, rbc mass and elevated EPO, NON smoker and has normal hemoglobinaffinity
Search for tumor as source of EPO
Maintaining hemoglobin and hematocrit in polycythemia vera
<=14 g/dL and <45% in men
<=12g/dL and <42% in women
1445
1242
Tx for symptomatic splenomegaly in polycythemia vera
Ruxolitinib - a JAK 2 inhibitor
PEG IFN a is an alternative
How does survival of px with essential thrombocytosis compare with general population
Same
Major hormone regulator of plt production
Thrombopeitin
synthesized primarily in the liver parenchymal cells
Most common cause of NON iatrogenic thrombocytopenia
Infection
if iatrogenic –> medications
deficient protease in TTP
ADAMTS13
What constitutes TTP pentad
CNS symptoms, fever, renal failure, MAHA, thrombocytopenia
Mainstay of tx for TTP
TPE
therapeutic plasma exchange
Primary tx for HUS
Supportive
Target of antibodies that cause HIT
PF4 and heparin/ other GAG
Risk of developing HIT is highest after exposure to heparin for ______ days
5-14
Most common inherited bleeding disorder
von willebrand dse
what constitues ISTH scoring for DIC
central mechanism of DIC
uncontrolled generation of thrombin
Butt cells with auer rods in PBS is seen in what condition
Acute promyelocytic leukemia
Cases in which whole blood may be transfused
massive blood loss and coagulopathy
trauma casualties requiring massive transfusion
hemorrhagic shock
neonatal exchange transfusion
Hemoglobin transfusion thresholds for the ff
hemodynamically stable
preexisting cardiovascular disease
acute coronary artery dse
hemodynamically stable <7 (except for px undergoing orthopedic/cardiac surgery)
preexisting cardiovascular disease <8
acute coronary artery dse <9-10
Platelet transfusion thresholds for the ff:
Absence of fever or infection
With fever or infection
For surgery, with DIC or other invasive procedure
Neurosurg/ eye surgery
Absence of fever or infection <=5
With fever or infection <= 10-20
For surgery, with DIC or other invasive procedure <= 50
Neurosurg/ eye surgery <=80
Indications for FFP transfusion
Multiple coag factor deficiencies or DIC
Single coag factor def when factor replacement is NOT available
Trauma casualties with >30% blood loss
SEVERE bleeding due to warfarin OR taking warfarin for emergent surgery
Cause of febrile non hemolytic transfusion rxn
Anti HLA antibodies and cytokines released from leukocytes in non leukoreduced blood
Cause of acute hemolytic transfusion rxn
preformed antibodies that lyse donor erythrocytes
Cause of DELAYED hemolytic transfusion rxn
Sensitization to RBC alloantigens
Cause of allergic rxns during blood transfusion
plasma proteins
Cause of GVHD during blood transfusion
donor T lymphocytes that attack host HLA antigens
What should be given in pregnant px with Rh neg blood transfused with Rh positive blood to prevent allosensitization
Anti D Ig
Treatment for TRALI
supportive
may occur within 6 hrs although delayed cases may occur up to 72h later
Recipient risk factors assoc with inc risk of TRALI
Smoking, chronic alcohol use
Shock
Liver and cancer surgery
Mech vent
positive fluid balance
Definition of massive transfusion
Transfusion of 50% of the patient’s total blood volume over 3 hrs OR
> 5-10 units of RBC
What diagnostic tests can be requested to assess for iron overload
Serum ferritin
MRI
Liver biopsy
Alemtuzumab is a an anti CD ___
52
** marami akong alam. 52 ang alem ko
Blinanutumab is a an anti CD ___
19
Rituximab is a an anti CD ___
20
Moxetumumab is a an anti CD ___
22
Brentuximab is a an anti CD ___
30
bRENTuximab - tRENTa
Mechanism of action of Bevacizumab
inhibits VEGF to block angiogenesis
Mechanism of action of Denosumab
RANK ligand inhibitor
Mechanism of action of Everolimus
mTOR inhibitor
Mechanism of action of Venetoclax
inhibtis Bcl-2
Cardioprotectant that may be given with doxorubicin
Dexrazoxane
Most feared pulmonary complication of bleomycin
Pulmonary fibrosis
Earliest indicator of adverse effect is a dec in DLCO or coughing
Dose of doxorubicin assoc with cardiomyopatht
> 550 mg/m2
Dose of bleomycin that inc incidence of pulmo fibrosis
> 300 cumulative units
minimally responsive to glucocorticoids
MOA of renal toxicity of methotrexate
from crystallization in renal tubules
Mesna lessens toxicity of which chemo agent
Cyclophosphamide
Treatment for acute promyelocytic leukemia
ATRA
assoc with t(15;17) chromosomal tanslocation
Supportive tx of choice for nausea and vomiting for high riskagents
Serotonin antagonisis (eg. odansetron) and neurokinin 1 antagonists (aprepitant)
Plerixafor blocks what receptor
CXCR4
most commonly mutated oncogene in cancers
RAS
Trisomy 21 inc or dec risk of AML?
inc
Genetic abnormalities when present will classify a patient to have AML regardless of marrow blast count %
t (15;17)
t (8;21)
in(16)
t (16;16)
otherwise, need blast count >=205
Most impt prognostic factor that predict outcome of AML
Chromosome and molecular investigations
Most common PE finding in CML
Splenomegaly
Most common symptom of myeloma
Bone pain
affecting nearly 70% of px
Criteria for initiation tx for CLL
Single most powerful predictor of survival in myeloma
Serum beta 2 microglobulin
can also substitute for staging
Criteria for diagnosis solitary plasmacytoma
> Biopsy-proven solitary lesion of bone or soft tissue with evidence of clonal plasma cells
Normal bone marrow with no evidence of clonal plasma cells
Normal skeletal survey and MRI (or CT) of spine and pelvis (except for the primary solitary lesion)
Absence of end-organ damage such as hypercalcemia, renal insufficiency, anemia, or bone lesions (CRAB) that can be attributed to a lymphoplasma cell proliferative
disorde
Criteria for dx multiple myeloma
> Clonal bone marrow plasma cells or biopsy-proven bony or extramedullary plasmacytoma
and any one or more of the following myeloma-defining events:
1.) Evidence of one or more indicators of end-organ damage that can be attributed to the underlying plasma cell proliferative disorder, specifically:
a. Hypercalcemia: serum calcium >0.25 mmol/L (>1 mg/dL) higher than the upper limit of normal or >2.75 mmol/L (>11 mg/dL)
b. Renal insufficiency: creatinine clearance <40 mL/minb or serum creatinine >177 μmol/L (>2 mg/dL)
c. Anemia: hemoglobin value of >20 g/L below the lower limit of normal, or a hemoglobin value <100 g/L
d. Bone lesions: one or more osteolytic lesions on skeletal radiography, CT, or PET-CTc
e. Any one or more of the following biomarkers of malignancy:
* Clonal bone marrow plasma cell percentage
≥60%
* Involved: uninvolved serum free light chain ratio ≥100
* >1 focal lesion on MRI studies
What class of drugs do bortezomib, carfilzomib and ixazomin belong to
Proteoseoe inhibitors
Class of drug: Panobinostat
Histone deacetylase inhibitor
tx for myeloma
Class of drug: Selinexor
Selective inhibitor of nuclear export (SINE)
tx for myeloma
