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 , peak at 1-1/2 weeks
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
but most lack suitable donor hence immunosupression is the tx of choice (Antihymocyte globulin + cylclosporine)
Age and severity of neutropenia: impt factors when deciding between transplant and immunosupression
Older: ATG + cyclosporine
Profound neutropenia: transplant
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 hemoglobin affinity
Search for tumor as source of EPO
(cerebellar hemangioma, uterine leiomyoma, hepatoma)
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
Also addresses other constitutional ssx of PV
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
a-LIMA-2-zumab
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
nagccrystallize ang meth
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
inc also risk for ALL
Genetic abnormalities when present will classify a patient to have AML regardless of marrow blast count %
t (15;17)
t (8;21)
inv(16)
t (16;16)
otherwise, need blast count >=20%
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
Watchful waitng for most px except if with the ff
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
An MCHC > ___ on ordinary blood count should raise suspicion of hereditary spherocytosis
34
PNH is an acquired mutation of ___ gene
PIG-A gene
Most common early symptom of aplastic anemia
bleeding
Thalidomide derivative that is effective in reversing anemia of MDS with 5q- syndrome
Lenalidomide
LIMAlidomide
Nilotinib is not recommended in this subset of px
DM px on insulin
higher rates of hyperglycemia 10-20%
Other adverse effects: PAOD, Panc
Optimal TKI if with T315I mutation
Ponatinib
Typical immunophenotype of B cell lymphomas would test positive for these markers
CD 19, 20,23
CD 5 (although this is a T cell marker)
What is Richter’s transformation
Transformation of CLL to more aggressive lymphoma eg. DLBCl
Most sensitive test for DIC level
FDP
These are seen in what disease conditions?
Thalassemia and liver dse
A patient presents at the outpatient clinic with pallor. An initial blood count done in their barangay diagnostic center is as follows: Hgb 7.5g/dL, Hct 20%, reticulocyte count 15%. Which of the following is a likely possible cause of the anemia based on the given data?
A. Intravascular hemolysis
B. Myelodysplasia
C. Renal disease
D. Thalassemia
A. Intravascular hemolysis
since reticulocyte index is > 2.5 or high in px with hemorrhage/hemolysis
Which of the following is the most common mechanism of acquired neutropenias?
A. Bone marrow invasion by solid/ hematologic malignancies
B. Decreased production of progenitor cells due to cytotoxic/ immunosuppressive therapy
C. Immune-mediated peripheral destruction by drug haptens
D. Peripheral destruction due to circulating anti-neutrophil antibodies
C. Immune-mediated peripheral destruction by drug haptens
Most common cause of neutropenia: iatrogenic
Which stage in the evolution of IDA is characterized by decreasing ferritin, normal to high TIBC and normal serum iron?
A. Iron-deficient erythropoiesis
B. Iron-deficiency anemia
C. Iron-sufficient erythropoiesis
D. Negative iron balance
D. Negative iron balance
A 35-year old is seen for easy fatigability for many months. She is now 24 weeks pregnant with her 3rd child in 3 years. She does not see any obstetrician and does not take any vitamins. Lately, she has developed a taste for eating ice. She has no other complaints. Family and past history are negative. She does not smoke nor drink. Physical examination is pertinent for pale conjunctiva, mild spooning of nails, and a grade II/VI systolic murmur at the left lower sternal border. Stools are negative for occult blood. Labs are as follows: Hgb 7.1 gm/dL, Hct 23%, WBC 5,400/uL, Plt 450,000/uL; MCV 74 fL (85-95); RDW is 17.1% (13-15). What other laboratory finding will be consistent with your diagnosis?
A. Low ferritin
B. High RBC protopophyrin
C. High reticulocyte index
D. Normal total iron-binding capacity (TIBC)
A and B
Dx: IDA
What laboratory test will best differentiate anemia of chronic disease from true iron-deficiency anemia?
Serum ferritin
IDA- low <15
Anemia of chronic dse - normal to high 30-200
Unless with severe megaloblastic anemia due to folate deficiency, folate supplementation is generally avoided in which of the following conditions?
A. Chronic dialysis
B. Chronic hepatitis
C. Hemolytic anemias
D. Malignancy
D. Malignancy
b9 may feed tumors
A 45-year-old man presents to the outpatient clinic with pallor. A CBC done reveals the following: Hgb 6.3 g/dL, Hct 21%, WBC 4.5, Plt 167, MCV 106 fl, MCH 32 pg, MCHC 30 gm%. Medical history is pertinent for a partial gastrectomy done 6 months prior for recurrent bleeding gastric ulcers. What should be supplemented in this patient?
A. Cobalamin
B. Copper
C. Folic acid
D. Iron
A. Cobalamin
What medication may induce hemolysis in patients with G6PD deficiency undergoing treatment for tumor lysis syndrome?
A. Allopurinol
B. Febuxostat
C. Rasburicase
D. Sodium Bicarbonate
C. Rasburicase
Which treatment for ITP require monitoring for 8 hours post infusion due to the rare complication of severe
intravascular hemolysis?
a. Prednisone
b. Rho (D) immune globulin
c. IVIg
d. Rituximab
b. Rho (D) immune globulin
Gold std for PNH
Flow cytometry
What is the most common cause of death among patients with myelodysplasia syndrome (MDS)?
A. Complications of pancytopenia
B. Complications of treatment
C. Diseases unrelated to MDS
D. Leukemic transformation
A. Complications of pancytopenia
Only one cytogenetic abnormality in acute myelogenous leukemia has been invariably associated with the t(15;17)(q22;q12) cytogenetic rearrangement. What would you recommend for the treatment of this condition?
A. Cytarabine + daunorubicin
B. Gemtuzumab ozogamicin
C. Imatinib
D. Tretinoin
D. Tretinoin
Dx: Acute promyelocytic leukemia
Which of the following findings in chronic myelogenous leukemia (CML) would suggest a worse prognosis?
A. Eosinophilia
B. Increased reticulin fibrosis
C. Presence of bands
D. Thrombocytopenia
D. Thrombocytopenia
Thrombocytosis is common but thrombocytopenia is rare and suggests worse prognosis
At what percentage of peripheral or marrow blasts is blastic-phase CML defined?
A. 20%
B. 25%
C. 30%
D. 35%
C. 30%
A 64-year-old woman presents with a 6 month history of weight loss, recurrent low-grade fevers and night sweats. PE is pertinent for cervical and axillary lymphadenopathies. An excision biopsy of a cervical node is consistent with diffuse large B-cell lymphoma. A PET/CT scan reveals enlarged cervical and axillary nodes, as well as involvement of the thyroid and GI tract. What is the recommended mode of treatment in this patient’s case?
A. Chemotherapy alone
B. Combination chemotherapy and radiation therapy
C. Field radiation therapy alone
D. Palliative treatment
A. Chemotherapy alone
Chemotx offers potentially curative x for DLBCL REGARDLESS of stage
A PET/CT scan reveals enlarged cervical and axillary nodes, as well as para-aortic and iliac nodes. Based on the Ann Arbor Staging System, what is the patient’s stage?
A. Stage II
B. Stage III
C. Stage IV-A
D. Stage IV-B
B. Stage III since both sides
most common cause of renal failure in patients with multiple myeloma?
hypercalcemia
A 65-year-old woman presents with a 2-month history of low back and extremity pain, associated with weight loss, easy fatigability and decreasing urine output. She has no co-mordbid illnesses. On PE, she appears pale, with normal chest and abdominal examination. There is grade 2 bipedal edema. Pertinent in her laboratories are the following: Hgb 8.2 g/dL, Hct 24.6%, WBC 4,700, Plt 104,000; BUN 36 mg/dL, creatinine 2.54 mg/dL, Na 124, K 5.2 Cl 102, total calcium 11.2 mg/dL, In addition to these clinical features, which of the following diagnostic tests will confirm your impression?
A. 24-h urine electrophoresis and immunofixation
B. Bone marrow aspirate and biopsy
C. Serum albumin and beta-2 macroglobulin
D. Skeletal radiography (x-rays/ CT scan)
B. Bone marrow aspirate and biopsy
Dx: multiple myeloma
What is the most common reaction associated with transfusion of blood components?
A. Acute hemolytic transfusion reaction
B. Allergic (urticarial) reaction
C. Febrile non-hemolytic transfusion reaction
D. Transfusion-related acute lung injury
C. Febrile non-hemolytic transfusion reaction
A 44-year-old presents at the ER for vaginal bleeding due to multiple myoma uteri. She has hypertension and is on twice-daily enalapril. Her hemoglobin on admission is 8.5 g/dL. A blood transfusion is ordered. Prior to the transfusion, her vital signs are as follows: BP 130/80 mmHg HR 85/min RR 16/min T 36.5C. She tolerates transfusion of 1 packed RBCs. 1 hour post- transfusion, vitals are: BP 90/60 mmHg HR 90/min RR 15/min T36.5C. She denies any symptoms, and her BP 2 hours post-transfusion is 120/80 mmHg without intervention. What is the most likely mechanism of her hypotension?
A. Anaphylactoid reaction
B. Anti-HLA class II antibodies
C. Immune mediated hemolysis
D. Increase in bradykinin levels
D. Increase in bradykinin levels
Blood products contain bradykinin normally degraded by ACE hence those on ACE-I –> inc bradykinin –> transient hypotension
Indications for irradiated blood components
What is the initial site of presentation of petechiae and purpura in patients with markedly decreased platelet counts?
A. Abdomen
B. Distal lower extremities
C. Mucous membranes
D. Upper chest/ trunk
B. Distal lower extremities
Petechiae first appear in areas of inc venous pressure eg ankles and feet
Which of the following concepts regarding heparin-induced thrombocytopenia (HIT) is TRUE?
A. HIT is not associated with bleeding but paradoxically markedly increases the risk of thrombosis.
B. Most patients develop HIT after two weeks of exposure to heparin.
C. The diagnosis of HIT requires IgG-specific ELISA or serotonin release assay.
D. When heparin is given in the last 100 days, HIT will occur within 5- 14 days after re-exposure.
A. HIT is not associated with bleeding but paradoxically markedly increases the risk of thrombosis.
B. Most patients develop HIT after two weeks of exposure to heparin. – occurs 5-14 days after exposure (days 5-10 in Ch114); HIT after 14 days is rare
C. The diagnosis of HIT requires IgG-specific ELISA or serotonin release assay. – HIT remains a clinical diagnosis.
D. When heparin is given in the last 100 days, HIT will occur within 5-14 days after re-exposure. – it will occur before 5 days
**additional info: thrombocytopenia not usually severe with nadir counts rarely <20k
A 24-year-old college student presents at the emergency room with weakness. 2 weeks prior, she recalls having loose bloody stools that resolved with a course of antibiotics. On PE, she is hemodynamically stable and afebrile. She is seen awake, oriented, with pale conjunctivae, clear breath sounds, soft abdomen, minimal bipedal edema, and petechiae on her lower extremities. Pertinent in her diagnostics is a hemoglobin of 9.5 mg/dL, platelet count of 88,000, and a creatinine of 2.2 mg/dL. Her peripheral blood smear reveals schistocytes. What is the mainstay of treatment in this clinical scenario?
A. Corticosteroids
B. Plasma exchange
C. Rituximab
D. Treatment is primarily supportive
D. Treatment is primarily supportive
Schistocytes is characteristic of MAHA
Dx: HUS
How much factor VIII does a bag of cryoprecipitate contain?
80 IU
Which of the following antiplatelet drugs is CORRECTLY matched with its mechanism of action?
A. Abciximab: inhibits final common pathway of platelet aggregation by blocking fibrinogen binding to Gp IIb/IIIa
B. Aspirin: inhibits thromboxane synthesis by targeting protease- activated receptor-1 (PAR-1) C. Ticagrelor: irreversibly blocks P2Y12 receptors on platelet surfaces
D. Tirofiban: decreases platelet recruitment by acetylating cyclooxygenase-1 (COX-1)
A.
How do you treat Heparin induced thrombocytopenia?
A 73-year-old woman presents at the ER following a fall at home. She has ischemic heart disease and atrial fibrillation and is compliant to warfarin. All trauma workup (cranial CT, chest, pelvic x-rays) are unremarkable. Aside from some minor right thigh pain, she is asymptomatic. A CBC is normal, but her INR is 8.2. There are no hematomas nor signs of bleeding. You discontinue the warfarin. How is the INR managed?
A. Administer IV vitamin K
B. Administer oral vitamin K
C. Transfuse 4-factor prothrombin complex concentrate
D. No additional therapy is required
D. No additional therapy is required
since INR < 10 and no bleeding
Examination of CSF is an essential routine diagnostic measure for acute lymphocytic leukemia (ALL), but should be performed only when the platelet count is >___ x 10^9/ L
20
What is the most common autoimmune complication in chronic lymphocytic leukemia (CLL)?
Autoimmune hemolytic anemia
A 71-year-old man is referred to you for an incidental finding of an elevated WBC count (22,000/ uL) with lymphocytic predominance. He has no other co-morbidities and aside from urinary retention, denies any other symptoms. Aside from mild anemia (Hgb 11.0 g/dL), the rest of the laboratory investigation is normal. Extensive workup later reveals chronic lymphocytic leukemia. What is the most appropriate treatment at this time?
A. Allogenic stem cell transplant
B. B-cell receptor signaling inhibitors
C. Monoclonal antibodies +/- chemotherapy
D. No treatment indicated at present
D. No treatment indicated at present
“Dry taps,” or inability to aspirate bone marrow, occur in about 4% of attempts with the most common differential diagnosis being:
Metastatic carcinoma filtration
Target pre transfusion Hb in px with beta thalassemia intermedia/minor
9-10.5
Which of the following is true regarding anemia of inflammation or chronic disease?
A. Most commonly associated with nutritional deficiencies.
B. Corrected reticulocyte count is elevated.
C. High serum iron levels with low stores of iron.
D. Low serum iron while stores of iron are adequate.
D. Low serum iron while stores of iron are adequate.
Which infection of this virus is a risk factor for developing Hodgkin’s lymphoma?
A. COVID virus
B. Hepatitis B virus
C. Influenza virus
D. Human immunodeficient virus (HIV)
D. Human immunodeficient virus (HIV)
most common presentation of Hodgkin’s lymphoma
non tender lymphadenopathy
Needed iron per day to replace RBCs lost through senescence
20mg/day
Causes of inc protoporphyrin
IDA, lead poisoning
due to inadequate Fe supply to erythroid precursors to support Hb synthesis
Dose of folic acid for pregnant px
400 mcg if no hx of NTD
if with hx, 5mg/d
Gold std for proving dec RBC lifespan
RBC survival study
Diagnostic test for hereditary spherocytosis
osmotic fragility test
Most frequent infectious cause of hemolytic anemia
endemic areas- malaria
non-endemic Shiga toxin producing E.coli O157:H7
Triad of AIHA
Large drop in hb
jaundice
splenomegaly
Triad for PNH
intravascular hemolysis
pancytopenia
inc risk of venous thrombosis
Most common cause of death for PNH
venous thrombosis
Highly reliable test for PNH
Acidified serum (Ham test)
sucrose hemolysis test : UNRELIABLE
Gold std: flow cytometry
Mandatory supplement needed for PNH
Folic acid at least 3 mg/d
Cardinal feature of BM failure
Hypoproliferative anemia
Major prognostic determinant of aplastic anemia
CBC
Severe dse is defined by presence of any 2 of the ff
ANC < 500 u/L
Plt < 20K u/L
corrected retic count <1% or absolute retic count <60k
Predictor of response to tx and long term outcome
Absolute retic count >25K
Lymphocytes >1000/ul
Mutations in MDS associated with good prognosis
-Y
del 5q
del 20 q
mnemonic: GOOD date: maY 20 (5/20)
Causes of MICROcytic erythrocytosis
beta thalassemia trait
polycythemia vera
hypoxic erythrocytosis
Most impt risk factor for thrombosis in essential thrombocytosis
Smoking
Most common cause of incompatibility during pre transfusion
Lewis
Occasional px with mononucleosis or M.pneumoniae may develop agglutinins against this blood group system antigen
anti- I or anti- i
Sometimes px with syphilis/ viral infection that lead to paroxysmal cold hemoglobinuria has autoantibody to this blood group system antigen
P system
P antigen also expressed on urothelial cell and may be a receptor for E. coli binding
Blood group antigen that serves as receptor for P. vivax
Duffy
Whole blood transfusion is indicated if total blood volume loss is >= ___%
25
What can be done to prevent febrile non hemolytic transfusion rxn?
Leukoreduction
What can be done to prevent allergic rxn during transfusion?
premedicate with antihistamine if with known hx of allergy
Use washed RBC
Most common non iatrogenic cause of thrombocytopenia
viral and bacterial infection
To which anticoag should you shift px who developed HIT?
direct thrombin inhibitors like argatroban, lepirudin
Wafarin may bbe used but should be overlapped with direct thrombin inhibitors or fondaparinux, given for 3-6months due to high risk of thrombosis in HIT
AML commonly assoc with DIC
Acute promyelocytic leukemia
How much platelets must be transfused in px with DIC and severe thrombocytopenia
1-2u/10 kg
How do you define severity of hemophilia
Severe <1%
Moderate 1-5%
Mild 6-30%
most common mutation: inv of intron 22
severe and moderate forms may present with hemarthrosis
Leading cause of death in px with hemophilia with replacement tx
HIV, Hep C
Target factor activity for hemophilia for the ff:
Mild bleed
Severe hemarthroses
Large hematomas or bleeds into the deep muscles
Serious bleeds (oropharyngeal spaces, CNS, retroperitoneum)
Mild bleed= 30-50%
Severe hemarthroses =15-25% for 2 or 3 days
Large hematomas or bleeds into the deep muscles: 50% or even higher if clinical ssx do not improve and factor replacement for 1 week
Serious bleeds (oropharyngeal spaces, CNS, retroperitoneum)= 100% for 7 days
Target factor activity for hemophilia as prophylactic replacement for surger
100% for 7-10 days
** oral surger requires factor replacement for 1-3 days coupled with antifibrinolytics
Confirmatory test for presence of factor inhibitor
aPTT with mixing studies
if positive for inhibitor, aPTT on a 1:1 mix is abnormally prolonged
Tx: immune tolerance induction
if not responsive, may give rituximab + factor viii
Treatment for factor XI (eleven) inhibitor
do not give FFP and Factor XI
iv platelet concentrate or recombinant activated factor VII (seven)
mnemonic: 7/11
Iron deficiency anemia next to normal red blood cells. Microcytes (right panel) are smaller than normal red blood cells (cell diameter <7 μm) and may or may not be poorly hemoglobinized (hypochromic).
Polychromatophilia. Note large red cells with light purple coloring.
Hypochromic microcytic anemia of iron deficiency. Small lymphocyte in field helps assess the red blood cell size
Macrocytosis. These cells are both larger than normal (mean corpuscular volume >100) and somewhat oval in shape. Some morphologists call these cells macroovalocytes.
Hypersegmented neutrophils. Hypersegmented neutrophils (multilobed polymorphonuclear leukocytes) are larger than normal neutrophils with five or more segmented nuclear lobes. They are commonly seen with folic acid or vitamin B12 deficiency.
Spherocytosis. Note small hyperchromatic cells without the usual clear area in the center.
Sickle cells. Homozygous sickle cell disease. A nucleated red cell and neutrophil are also in the field.
Target cells. Target cells are recognized by the bull’s-eye appearance of the cell. Small numbers of target cells are seen with liver disease and thalassemia. Larger numbers are typical of hemoglobin C disease.
Acanthocytosis. Spiculated red cells are of two types: acanthocytes are contracted dense cells with irregular membrane projections that vary in length and width; echinocytes have small, uniform, and evenly spaced membrane projections. Acanthocytes are present in severe liver disease, in patients with abetalipoproteinemia, and in rare patients with McLeod blood group. Echinocytes are found in patients with severe uremia, in glycolytic red cell enzyme defects, and in microangiopathic hemolytic anemia
Elliptocytosis. Small lymphocyte in center of field. Elliptical shape of red cells related to weakened membrane structure, usually due to mutations in spectrin.
Howell-Jolly bodies are tiny nuclear remnants that normally are removed by the spleen. They appear in the blood after splenectomy (defect in removal) and with maturation/dysplastic disorders (excess production).
Teardrop cells and nucleated red blood cells characteristic of myelofibrosis. A teardrop-shaped red blood cell (left panel) and a nucleated red blood cell (r
Heinz bodies. Blood mixed with hypotonic solution of crystal violet. The stained material is precipitates of denatured hemoglobin within cells
Giant platelets. Giant platelets, together with a marked increase in the platelet count, are seen in myeloproliferative disorders, especially primary thrombocythemia.
Stippled red cell in lead poisoning. Mild hypochromia. Coarsely stippled red cell.
Pelger-Hüet anomaly. In this benign disorder, the majority of granulocytes are bilobed. The nucleus frequently has a spectacle-like, or “pincenez,” configuration.
Döhle body. Neutrophil band with Döhle body. The neutrophil with a sausage-shaped nucleus in the center of the field is a band form. Döhle bodies are discrete, blue-staining nongranular areas found in the periphery of the cytoplasm of the neutrophil in infections and other toxic states. They represent aggregates of rough endoplasmic reticulum
Chédiak-Higashi disease. Note giant granules in neutrophil.
Uremia. The red cells in uremia may acquire numerous regularly spaced, small, spiny projections. Such cells, called burr cells or echinocytes, are readily distinguishable from irregularly spiculated acanthocytes
Erythroid hyperplasia. This marrow shows an increase in the fraction of cells in the erythroid lineage as might be seen when a normal marrow compensates for acute blood loss or hemolysis. The myeloid/erythroid (M/E) ratio is about 1:1.
Myeloid hyperplasia. This marrow shows an increase in the fraction of cells in the myeloid or granulocytic lineage as might be seen in a normal marrow responding to infection. The myeloid/erythroid (M/E) ratio is >3:1
Causes of anemia with reticulocyte production index of > 2.5
Methods used for percussing for splenic dullness
- Nixon’s method: The patient is placed on the right side so that the spleen lies above the colon and stomach. Percussion begins at the lower level of pulmonary resonance in the posterior axillary line and proceeds diagonally along a perpendicular line toward the lower midanterior costal margin. The upper border of dullness is normally 6–8 cm above the costal margin. Dullness >8 cm in an adult is presumed to indicate splenic enlargement.
- Castell’s method: With the patient supine, percussion in the lowest intercostal space in the anterior axillary line (8th or 9th) produces a resonant note if the spleen is normal in size. This is true during expiration or full inspiration. A dull percussion note on full inspiration suggests splenomegaly.
- Percussion of Traube’s semilunar space: The borders of Traube’s space are the sixth rib superiorly, the left midaxillary line laterally, and the left costal margin inferiorly. The patient is supine with the left arm slightly abducted. During normal breathing, this space is percussed from medial to lateral margins, yielding a normal resonant sound. A dull percussion note suggests splenomegaly.
Clues
Posterior axillary line = Nixon
Mid axillary line = Traube
Anterior axillary line = Castell
Which of the following genetic changes characterizes Primary Myelofibrosis (PMF)?
a. t(15;19) translocation
b. JAK2 activating mutation
c. PDGFRa deletion or translocation
d. t(9;22)(q34:11) translocation
b. JAK2 activating mutation
A 50-year-old man, otherwise asymptomatic, shows you a CBC with the following findings: Hb 140mg/dL, Hct
0.38, WBC 4,500/uL, Platelet count 100,000/uL. What will you do next?
a. Assure patient that the values are normal
b. Repeat CBC in 24 hours
c. Request for peripheral blood smear
d. Refer for bone marrow biopsy
c. Request for peripheral blood smear
r/o pseudothrombocytopenia
What is the central mechanism of DIC?
Uncontrolled thrombin generation
A 48-year-old male diagnosed with ALL completed his induction therapy. He was advised to undergo
maintenance therapy which usually consists of 6-mercaptopurine and methotrexate. For this patient, how long would
you give the treatment?
a. 1 – 2 years
b. 1.5 – 2 years
c. 2 – 2.5 years
d. 2.5 – 3 years
c. 2 – 2.5 years
The standard duration of maintenance therapy for ALL
in adults is approximately 2–2.5 years.
A patient presented with the characteristic “chipmunk” facies, easy fatigability, dizziness, pale skin and requires
intensive blood transfusion support to survive. What is the possible diagnosis?
a. β Thalassemia Major
b. β Thalassemia Intermedia
c. β Thalassemia Trait
d. β Thalassemia Minor
a. β Thalassemia Major
Chipmunk” Facies:
This is caused by bone marrow expansion due to ineffective erythropoiesis. The expansion leads to characteristic craniofacial deformities, including:
Prominent frontal bones.
Maxillary overgrowth.
Flattened nasal bridge.
Patients with β Thalassemia Major require regular blood transfusions to maintain hemoglobin levels and prevent complications such as growth retardation and organ damage.
This is the major cause of morbidity and the second leading cause of death in hemophilia patients receiving clotting factor concentrates
HCV
Features responsible for high progression from smoldering multiple myeloma to multiple myeloma include bone
marrow plasmacytosis >10%, abnormal kappa/lamda free light chain ratio, and ___:
a. < 20 g/L hemoglobin, below the lower limit of normal
b. > 177umol/L serum creatinine
c. >30g/L (3g/dL) serum M protein
d. ≥ 200mg per 24h urinary monoclonal protein
c. >30g/L (3g/dL) serum M protein
A 22/F nursing student from Palawan consults you for body malaise and generalized weakness with easy fatigability for the past year. She has no fever, weight loss or cough. She has no vices and no comorbidities. Family history reveals frequent blood transfusion of her father. Her last trip back home to Palawan was last year. Vital signs are stable. Systemic findings are unremarkable except for slightly pale conjunctivae. Initial CBC results:
Hb 83 g/dl
Hct 25
RBC 1.9
WBC 7.8
PC 322
Hemoglobin electrophoresis showed
HbA: 93%
HbF: 3%
HbA2: 4%
You contemplate blood transfusion. What is the pretransfusion Hb concentration goal for the patient?
A. 8.0-9.5 g/dl
B. 9.0-10.5 g/dl
C. 10.0-11.5 g/dl
D. 12- 16 g/dl
B. 9.0-10.5 g/dl
For non-transfusion-dependent thalassemia like beta-thalassemia intermedia, the pretransfusion hemoglobin goal is typically 9.0–10.5 g/dL
Transfusion-transmitted bacterial infection remains a significant concern notably with what blood
product?
A. Packed RBC at room temperature
B. Platelet concentrate at room temperature
C. Washed RBC at room temperature
D. Fresh whole blood at room temperature
B. Platelet concentrate at room temperature
others are not really stored at room temp
Autoimmune hemolytic anemia is best characterized by which of the following?
A. Increased levels of plasma C3
B. Spherocytic red cells
C. Decreased osmotic fragility
D. Decreased unconjugated bilirubin
B. Spherocytic red cells
Warm AIHA –> Spherocytes
Which of the following is compatible with coagulopathy due to liver disease?
A. Elevated platelet count
B. Elevated levels of factor VIII
C. Low levels of von Willebrand factor
D. Normal levels of PT and aPTT
B. Elevated levels of factor VIII
A 50-year-old male consulted because of incidental finding of platelet count of 780,000/mm3 . He has a history of gouty arthritis and on intermittent pain medications. Physical examination showed no splenomegaly, no lymphadenopathy. What is the best step in the diagnostic approach to this patient?
A. Request for JAK-2 mutation assay.
B. Offer BMA biopsy to document a primary bone marrow disease.
C. Request for CRP, ESR, Ferritin.
D. Reassure patient and repeat CBC platelet count once inflammation subsided
D. Reassure patient and repeat CBC platelet count once inflammation subsided
Which of the following is true of the management of hemorrhagic manifestation in diffuse intravascular coagulation (DIC)?
A. Clotting factor concentrates are recommended for control of bleeding
B. Transfusion must be adjusted according to the patients clinical and laboratory evolution
C. Use of antifibrinolytic drugs is the cornerstone of treatment in DIC
D. Platelet concentrate at a dose of 10 U/ 10 kg is sufficient for DIC
B. Transfusion must be adjusted according to the patients clinical and laboratory evolution
A. Clotting factor concentrates are recommended for control of bleeding - NOT recommended
C. Use of antifibrinolytic drugs is the cornerstone of treatment in DIC - may precipitate thrombosis
D. Platelet concentrate at a dose of 10 U/ 10 kg is sufficient for DIC - 1-2 u/10kg
Which of the following is associated with Diffuse Intravascular Coagulation (DIC)?
A. Thrombocytosis
B. Normal prothrombin time
C. Elevated fibrin degradation products
D. Elevated fibrinogen
C. Elevated fibrin degradation products
it is not DIC if FDP not elevated
Drug-induced antibody- mediated thrombocytopenia is more commonly seen with which drugs?
A. Ibuprofen and naproxen
B. Sulfonamides and quinine
C. Carbamazepine and phenytoin
D. Amiodarone and quinidine
B. Sulfonamides and quinine
A 35-year-old female was diagnosed 1 year prior to consult with Immune Thrombocytopenic Purpura (ITP). Since then her monthly platelet counts ranged between 50,000 to 80,000/uL. She is now a primigravida, 15 weeks AOG. CBC showed Hgb 11 g/dL, WBC 7,000/uL, platelet count 55,000/uL. When during pregnancy would medical treatment of her ITP be needed?
A. Promptly, as her platelet count is subnormal
B. At a platelet count of less than 5,000/uL
C. At a platelet count of less than 50,000/uL
D. Near term, regardless of platelet count
B. At a platelet count of less than 5,000/uL
A 34-year-old male is recently diagnosed with Acute
Lymphoblastic Leukemia. He harbors the BCR-ABL
mutation. Aside from chemotherapy, what other
medicine should he be taking?
A. Ibrutinib
B. Lapatinib
C. Ponatinib
D. Ruxolitinib
C. Ponatinib
A 75-year-old male was recently diagnosed with Acute
Myelogenous Leukemia. He has hypertension and heartfailure with preserved ejection fraction. What is the most appropriate treatment for him?
A. High dose cytarabine
B. Idarubicin + Cytarabine
C. Decitabine + Venetoclax
D. Intermediate dose cytarabine
C. Decitabine + Venetoclax
Older patients and those unable to receive intensive therapy due to medical comorbidity may receive repetitive cycles of lower intensity therapy with a hypomethylating agent (HMA; decitabine or azacitidine) or low-dose cytarabine, in combination with daily venetoclax.
A 65-year-old male is recently diagnosed with chronic lymphocytic leukemia. CBC shows: hemoglobin 10.5 g/dL, WBC 110,000/mm3, platelet count 125,000/mm3. Spleen is palpable 2 cm below the left subcostal margin. There were no palpable lymph nodes and he has no symptoms. What is the most appropriate treatment for him?
A. Ibrutinib alone
B. Ibrutinib + Rituximab
C. Rituximab + Bendamustine
D. Active surveillance
D. Active surveillance
Currently, a watchful waiting strategy is used for most patients with CLL, with therapy reserved for patients with symptomatic disease.
.A 45-year-old male was diagnosed with acute myelogenous leukemia in 2015. He underwent induction chemotherapy using 7+3 regimen for which he achieved a complete response. He was given consolidation chemotherapy using high dose cytarabine after that. He did not undergo a bone marrow transplant. At present time, he is still in remission. Which of the following molecular abnormalities was most likely present?
A. IDH2
B. NPM1
C. DNMT3
D. FLT3-ITD
B. NPM1
AML with mutated NPM1 is associated with a more favorable clinical outcome.
A 43-year-old male complains of frequent headaches. There were no focal neurologic deficits. He has no comorbid illness. On examination, he had hyperemic and vascular conjunctiva with the spleen palpable below the subcostal area. CBC showed hemoglobin 20 g/dL, hematocrit 60%, WBC 13,000/mm3, platelet count 480,000/mm3. JAK2 mutation was positive. What is the most appropriate treatment for him?
A. Aspirin
B. Ruxolitinib
C. Phlebotomy
D. Hydroxyurea
C. Phlebotomy
Phlebotomy serves initially to reduce hyperviscosity by reducing the red cell mass to normal while further expanding the plasma volume. Periodic phlebotomies thereafter serve to maintain the red cell mass within the normal range and induce a state of iron deficiency that prevents accelerated reexpansion of the red cell mass.
A 45-year-old female was recently diagnosed with chronic myelogenous leukemia. CBC showed hemoglobin 12.5 g/dL, WBC 130,000/mm3, neutrophils 30% lymphocytes 10% eosinophils 5% basophils 30% blast 15%, platelet count 98,000/mm3. FISH for BCR-ABL was 80% with no additional cytogenetic abnormalities on routine karyotyping. What is the phase of her disease?
A. Chronic phase
B. Accelerated phase
C. Blast crisis
D. CML in transformation
B. Accelerated phase
Criteria of accelerated-phase CML, historically associated with median survival of <1.5 years, include the presence of 15% or more peripheral blasts, 30% or more peripheral blasts plus promyelocytes, 20% or more peripheral basophils, cytogenetic clonal evolution (presence of chromosomal abnormalities in addition to Ph), and thrombocytopenia <100 × 109/L (unrelated to therapy).
Blast crisis >30% blasts peripheral or BM blasts or sheets of blasts in extramedullary dse (usually skin, soft tissue, lytic bone lesions)
A 24-year-old male weighing 70 kg diagnosed with severe congenital Hemophilia A. He went to the ER for severe abdominal pain and is found to have acute appendicitis. How much Factor 8 concentrate does he need?
A. 2,000 IU
B. 3,500 IU
C. 5,000 IU
D. 6,500 IU
B. 3,500 IU
A 50-year-old male presents with multiple ecchymoses.
CBC showed hemoglobin 13.5 g/dL, WBC 9,500/mm3,
neutrophils 70% lymphocytes 30%, platelet count
40,000/mm3. He has no bleeding episodes. Hepatitis
screen and HIV were negative. Ultrasound of the liver
and spleen were normal. What is the most appropriate
treatment?
A. Repeat CBC after 1 week
B. Start prednisone at 1mg/kg/day
C. Give dexamethasone 40 mg/day for 4 days
D. Initiate platelet transfusion
A. Repeat CBC after 1 week since >30k and no bleeding
A 58-year-old female diagnosed with Invasive Ductal
Carcinoma Stage IV was admitted at the ICU because of
febrile neutropenia and septic shock. CBC showed
hemoglobin 8.5 g/dL, WBC 870 with 0 neutrophils,
platelet count 38,000/mm3. PTT was 68 seconds, PT-INRwas 2.5. Fibrinogen was 0.3 g/L and D-dimer was 500. What blood component is most appropriate for her?
A. Packed RBC
B. Cryoprecipitate
C. Platelet concentrate
D. Fresh Frozen Plasma
B. Cryoprecipitate
Patient has DIC. A fibrinogen level <1.0 g/L (or <100 mg/dL) is a key indication for cryoprecipitate transfusion, especially in bleeding or at risk of bleeding.
A 43/F consulted at the clinic for 6 weeks history of low-grade fever, fatigue, and weight loss. She also noted pain, stiffness and swelling in her fingers and elbows, bilaterally. Further work-up revealed elevated ESR, CRP and positive RF. She was started on Methotrexate. She returned 2 weeks later because she complains of further fatigue, dizziness and muscle weakness. CBC showed macrocytic anemia. What is the BEST course of action for this patient?
A. Give cyanocobalamin 1000 ug IM every month.
B. Start folinic acid.
C. Add corticosteroids to patient’s regimen.
D. Increase the dose of methotrexatea
B. Start folinic acid.
Given the clinical vignette, the patient seems to have developed megaloblastic anemia secondary to folate deficiency from methotrexate use. The drugs that inhibit DHF reductase include methotrexate, pyrimethamine, and trimethoprim. Methotrexate has the most powerful action against the human enzyme, whereas trimethoprim is most active against the bacterial enzyme and is likely to cause megaloblastic anemia only when used in conjunction with sulfamethoxazole in patients with preexisting folate or cobalamin deficiency. The activity of pyrimethamine is intermediate. The antidote to these drugs is folinic acid (5-formyl-THF).
Patients with G6PD deficiency are at an increased risk
of hemolytic anemia when exposed to certain
medications. What is the pathophysiology behind this
condition?
A. Altered membrane-cytoskeleton complex
B. Abnormal red cell ion content
C. Deficient enzymes of the glycolytic pathway
D. Problems in redox metabolism
D. Problems in redox metabolism
G6PD is a housekeeping enzyme critical in the redox metabolism of all aerobic cells. In red cells, its role is even more critical because it is the only source of NADPH, which directly and via glutathione (GSH) defends these cells against oxidative stress. G6PD deficiency-related HA is a prime example of an HA due to interaction between an intracorpuscular cause and an extracorpuscular cause: indeed, in the vast majority of cases hemolysis is triggered by an exogenous agent. Although the G6PD activity is decreased in most tissues of G6PD-deficient subjects, in other cells the decrease is much less pronounced than in red cells, and it does not seem to impact on clinical expression.
Blast cut off for dx of AML
20%
Chromosomal arrangement that define AML regardless of number of blasts: t (15;17), t(8;21), inv (16), t(16;16)
Most common acute leukemia in older px
Definition of complete response of AML to tx
ANC >1000/uL and plt >100K
Absent circulating blasts
Bone marrow blasts <5%
No Auer rods, no extramedullary leukemia
Back pain and lower extremity weakness is commonly seen in AML with what mutation
8;21
What should be tested prior to giving high dose cytarabine in AML px
Cerebellar testing due to irreversible toxicity
most common induction for AML
Cytarabine + Anthracycline 7+3
Target plt and hb during chemotx of px with AML
plt >10k
hb >8g/dL
transfuse to maintain these values
Chromosomal abnormalities associated with poor prognosis in CML
trisomy 8
double pH
isochrome 17
17 p deletion
20q- (good prog in MDS)
Major tx endpoint of CML
Achievement of COMPLETE cytogenetic response
the only endpoint associated with improvement in survival = approximates normal life expectancy
Definition of erythrocytosis
Men
Hb >17 (Hct>50)
Women
Hb >15 (Hct >45)
How do you diagnose CNS leukemia
Lumbar tap: >=5 leukocytes/uL or leukemic blast
must have plt at least 20k prior to procedure
LP: impt diagnostic in ALL
Most common immunologic subtype of B- ALL
Common ALL
Most impt prognostic factor during tx of ALL
Minimal residual dse
The only ALL specific drug
L asparaginase
now more intensively used in adults
Examples of TKI that can cross BBB
Dasatinib, probably ponatinib
Intrathecal chemotherapy in ALL
methotrexate +/- cytosine arabinoside (AC) +/- glucorticoids
MOA of Blinatumomab
CD19 inhibitor
MOA of inotuzumab
CD 22 inhibitor
ino2zumab
Mutation with good prognosis in CLL
del 13 (q14.3)
poorest prog: del 17 (p13.1)
If clonal B cell count on flow cytometry >= ____ diagnosis is CLL, no further work up needed
5x10^9/L
Leading cause of morbidity and mortality in CLL
infection
PCP pneumonia prophy for at least 6mos needed following nucleoside analog based tx
Varicella prophy if with prior hx of varicella
In Richters transformation CLL commonly transforms into an aggressive lymphoma most commonly _____
DLBCL
First step in dx Richter transformation
PET CT to localize an area for biopsy
SUV > 5
Staging for CLL
No reqt for imaging or BM biopsy
Primary tx for CLL
BTK or BCL2 targeted oral tx +/- CD20 monoclonal Ab
BTK inh - ibrutinib, acalabrutinib
BCL2 inh - venetoclax
Most common subtype of Hodgkins in younger age groups
Nodular sclerosis
Used for staging Hodgkins
PET scan
Most common chemotx for Hodgkins
ABVD
Adriamycin, Bleomycin, Vinblastine, Dacarbazine
No benefit of adding RT in advanced stages
Nilotinib can be given in all phases of CML except
Blastic phase
laboratory test reflects the total erythroid mass and is expected to be elevated in true iron deficiency anemia?
Transferrin receptor protein
In acute infection or inflammation, anemia results from
Faster destruction of early senscent RBCs
The functional murmur of anemia is systolic/diastolic?
mid systolic ejection type of murmur
mechanism of anemia in liver dse
shortened RBC survival +inadequate compensatory EPO production
T/F paresthesias and muscle weakness are manifestations of both Vit B 9 and Vit B12 deficiencies
False. Vit B12 only
Pernicious anemia is associated with blood group ___
A
F>M, more common among >60yo
Normal expectancy in women with tx; shorter in men due to inc risk of gastric CA
Abnormal hemoglobin form seen in excess in Beta thalassemia
HbA2
dec HbA and HbF
Amount of elemental iron in 1 uprbc
250-300 mg
50-65 mg per tab of ferrous sulfate
most common preceding infection in aplastic anemia
seronegative hepatitis
Criteria for complete remission of AML
all must be satisfied
Bortezomib and Lenalidomide require this/these prophy
Bortezomib = VZV prophy
Lenalidomide = DVT prophy
Treatment for hyperviscosity syndrome in MM and Waldenstroms Macroglobulinemia
plasmapheresis
Most common Non Hodgkins lymphoma
DLBCL (aggressive type)
Mutations associated with the ff lymphomas:
Burkitt
Follicular
Mantle cell
MALT
Burkitt = t(8;14) Burk8t –> starry sky
Follicular = t (14;18) Fourteen, eighteen
Mantle cell= t(11;14) mantlELEVEN
MALT = t(11;18)
Most common INDOLENT NHL
Follicular (2nd most common NHL)
NHL that needs prophylactic CNS tx
Burkitt
Combination tx for Burkitt all contain cyclophosphamide
Associated organisms with MALToma
H. pylori (gastric)
Borrelia burgdorferi (skin)
C. psitacci (eye)
C. jejuni (Small intestine)
Lymphoma associated with Sezary syndrome
Mycosis fungoides/ Cutaneous T cell lymphoma
Consists of generalized erythroderma and circulating tumor cells
Most common T cell lymphoma
Angioimmunoblastic T cell lymphoma
> 80% with advanced dse
+ polyclonal hypergammaglobulinemia , eosinophils, inc LDH, + Coombs, opportunistic infections
Lymphoma with flower cells positive for CD 4 and 25
Adult T cell lymphomas
Based on retrospective analyses on patients with lymphadenopathy (LAD), which of the following clinical scenarios warrants lymph node biopsy the most? (C62 413)
A. 25-year-old with painless pre-auricular LAD, measuring 1.0 cm
B. 30-year-old with bilateral cervical LADs, largest at 2.0 cm who recently had a sore throat
C. 35-year-old with tender unilateral cervical LAD, measuring 1.5 cm
D. 40-year-old with painless supraclavicular LAD, measuring 2.0 cm
D. 40-year-old with painless supraclavicular LAD, measuring 2.0 cm
What is the mainstay of treatment for sickle cell patients with severe symptoms?
Hydroxyurea
It increases HbF
Which of the following causes of cobalamin deficiency can be severe enough to cause megaloblastic anemia?
A. Gastric bypass surgery
B. Use of proton pump inhibitors
C. Tropical sprue
D. Zollinger Ellison syndrome
C. Tropical sprue
In px with megaloblastic anemia due to cobalamin deficiency, what is the usual level of cobalamin?
<100 ng/mL or <74 pmol/L
A 45-year-old man presents to the outpatient clinic with pallor. A CBC done reveals the following: Hgb 6.3 g/dL, Hct 21%, WBC 4.5, Plt 167, MCV 106 fl, MCH 32 pg, MCHC 30 gm%. Medical history is pertinent for a partial gastrectomy done 6 months prior for recurrent bleeding gastric ulcers. What should be supplemented in this patient
A. Cobalamin
B. Copper
C. Folic acid
D. Iron
A. Cobalamin
A patient with HIV admitted to the wards for pneumonia develops dyspnea, jaundice, pallor and left upper quadrant pain. The hemoglobin dropped to 6.0 g/dL from a baseline of 10 g/dL in the span of one day. Coombs test is positive. All of the available blood units cross-matched are incompatible. What is the next best step in the management of this case?
A. Give prednisone 1 mg/kg/day
B. Give rituximab
C. Refer to surgery for emergency splenectomy
D. Transfuse incompatible blood
D. Transfuse incompatible blood
A 34-year-old man with newly discovered pancytopenia presents for evaluation. He has been healthy until the last 3 to 6 months, when he noted a decline in his energy and easy bruising. He has no fever or weight loss. He has no other medical problems. He is a non-smoker and rarely drinks alcohol. He currently works in an accounting firm. Family history is unremarkable. Labs are as follows: Hgb 9.3 g/dL, WBC 3,600/uL (N32% L64%), Plt 24,000/uL, with normal liver and renal function tests. What is the next step in the management of this patient?
A. Admit for blood transfusion and further work-up
B. Initiate trial of glucocorticoids
C. Request for bone marrow aspirate and biopsy
D. Request for hemoglobin electrophoresis
C. Request for bone marrow aspirate and biopsy
A 60-year-old woman with unremarkable past medical history develops anemia (Hgb 7.8 g/dL), thrombocytopenia (Plt 20,000/uL), and neutropenia (ANC 500/uL). Reticulocyte production index is 1% and bone marrow cellularity is <25%. What is the most appropriate treatment option in this case?
A. Anti-thymocyte globulin + cyclosporine
B. Allogeneic bone marrow transplantation
C. Autologous bone marrow transplantation
D. Lenalidomide + decitabine
A. Anti-thymocyte globulin + cyclosporine
Since elderly –> A is the better answer
A 46-year-old woman admitted for elective surgery is referred to you for increased platelet count (750,000/uL) on routine CBC. On peripheral blood smear, large platelets are noted. She reports that she does not have any other symptoms. She does not smoke nor drink alcohol. What advice would you give the referring service regarding the surgery?
A. ε-aminocaproic acid can be given prophylactically before and after elective surgery to prevent bleeding.
B. Normalizing the platelet count with hydroxyurea is essential to prevent perioperative thrombotic events.
C. Plateletpheresis is the mainstay of therapy and is necessary in this case.
D. Surgery should be deferred, and she should be started on aspirin for at least 4 weeks.
A. ε-aminocaproic acid can be given prophylactically before and after elective surgery to prevent bleeding.
A 62-year-old man presents with a 6-month history of fatigue, weight loss, anorexia and increasing abdominal enlargement. Physical examination shows pallor, gingival hypertrophy, and splenomegaly. A peripheral blood smear shows granules in cytoplasm with fine, lacy chromatin in the nucleus. Abnormal rod-shaped granules are also seen. What is the most likely diagnosis?
A. Acute lymphoid leukemia
B. Acute myeloid leukemia
C. Chronic lymphocytic leukemia
D. Chronic myeloid leukemia
B. Acute myeloid leukemia
A 42-year-old woman is newly diagnosed with acute myeloid leukemia. She has been receiving platelet transfusions since her admission 2 weeks prior. Her platelet count two days ago was 8,000. 8 units of platelet concentrate is transfused, and her repeat platelet count was 11,000. Earlier this morning, the platelet count is 4,000, and the latest post transfusion platelet count is 8,000. No bleeding is noted. What will you transfuse?
A. HLA-matched platelets
B. Irradiated platelets
C. Washed platelets
D. No indication to transfuse for now; observe trends
A. HLA-matched platelets
Which of the following findings in chronic myelogenous leukemia (CML) would suggest a worse prognosis?
A. Eosinophilia
B. Increased reticulin fibrosis
C. Presence of bands
D. Thrombocytopenia
D. Thrombocytopenia
A 64-year-old woman presents with a 6 month history of weight loss, recurrent low-grade fevers and night sweats. PE is pertinent for cervical and axillary lymphadenopathies. An excision biopsy of a cervical node is consistent with diffuse large B-cell lymphoma. A PET/CT scan reveals enlarged cervical and axillary nodes, as well as involvement of the thyroid and GI tract. What is the recommended mode of treatment in this patient’s case?
A. Chemotherapy alone
B. Combination chemotherapy and radiation therapy
C. Field radiation therapy alone
D. Palliative treatment
A. Chemotherapy alone
In which of the following clinical scenarios will the transfusion of irradiated blood products be preferred? (CPG on Rational Use of Blood Products P35)
A. Aplastic anemia receiving immunosuppressive therapy
B. Confirmed deficiency of immunoglobulin A
C. Neonates and premature infants
D. Recurrent severe allergic-type events
A. Aplastic anemia receiving immunosuppressive therapy
A. Aplastic anemia receiving immunosuppressive therapy - IRRADIATED
B. Confirmed deficiency of immunoglobulin A - WASHED
C. Neonates and premature infants - LEUKOREDUCED D. Recurrent severe allergic-type events - WASHED
A 35-year-old woman presents with rash on the legs of recent onset. Platelet is 48,000 with no signs of overt bleeding. A repeat CBC 1 week later shows a platelet count of 46,000. There is no active bleeding. You suspect immune thrombocytopenic purpura (ITP). How should this patient be managed?
A. Eltrombopag
B. Prednisone
C. Prednisone + Rh0(D) immune globulin
D. Observe and monitor CBCs
D. Observe and monitor CBCs since >30k
Patients on warfarin with stable dose requirements should still have their INR determined at least:
A. Every week
B. Every 2 weeks
C. Every month
D. Every 2 months
C. Every month
A 73-year-old woman presents at the ER following a fall at home. She has ischemic heart disease and atrial fibrillation and is compliant to warfarin. All trauma workup (cranial CT, chest, pelvic x-rays) are unremarkable. Aside from some minor right thigh pain, she is asymptomatic. A CBC is normal, but her INR is 8.2. There are no hematomas nor signs of bleeding. You discontinue the warfarin. How is the INR managed?
A. Administer IV vitamin K
B. Administer oral vitamin K
C. Transfuse 4-factor prothrombin complex concentrate
D. No additional therapy is required
D. No additional therapy is required
A 32-year-old is referred for anticoagulation after spontaneous vaginal delivery. She was diagnosed with pulmonary embolism at 25 weeks AOG. She was managed with low-molecular weight heparin during the pregnancy that was discontinued at the onset of labor. She is now prepared for discharge. She wishes to breastfeed. Which of the following is the preferred agent?
A. Apixaban
B. Dabigatran
C. Rivaroxaban
D. Warfarin
D. Warfarin
