Hematology Flashcards

1
Q

Protein most responsible for iron transport in plasma

A

Transferrin

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2
Q

Protein that represents an indirect measure of total body iron stores

A

Ferritin

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3
Q

Iron supplements should be taken in an empty or full stomach?

A

Empty since food inhibits iron absorption

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4
Q

After iron supplementation, reticulocyte begin to increase in how many days

A

4-7 days , peak at 1-1/2 weeks

Tx must be sustined for 6-12 months

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5
Q

When do you transfuse blood for IDA?

A

reserved for symptomatic anemia, cardiovascular instability and continued and excessive blood loss and who require immediate intervention

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6
Q

Formula for amount of iron needed by an individual patient

A

Body weight x 2.3 x (15-px hb in g/dL) + 500 or 1000 for iron stores

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7
Q

Pathogenic changes in the ____ protein can cause spherocytosis

A

Ankyrin

whereas changes in junctional complexes = elliptocytosis

Stomatocytosis= abnormalities of channel molecules

Other notes: anemia of hereditary spherocytosis is usually NORMOcytic

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8
Q

When do you advocate for splenectomy in patients with hereditary spherocytosis

A

Mild - avoided
Moderate - delayed until puberty
Severe - 4-6 yrs of age

must vaccinate against encapsulated organisms BEFORE splenectomy

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9
Q

Most typical feature of G6PD deficiency on blood film

A

Poikilocytes

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10
Q

Expected levels of the following in G6PD deficiency

Hemoglobin
LDH
Haptoglobin

A

Decreased hemoglobin due to hemoglobinuria

Hig LDH due to hemolysis

Low to absent haptoglobin because haptoglobin binds free hemoglobin hence low in intravascular hemolysis

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11
Q

Tx for acute hemolytic anemia in G6PD deficiency

A

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

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12
Q

Next line of tx when steroids do not improve autoimmune hemolytic anemia

A

Rituximab

If very rare and severe refractory case, may need high dose cyclophosphamide OR anti CD52 Alemtuzumab

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13
Q

Predictors or probability of relapse of autoimmune hemolytic anemia (AIHA)

A

Severe anemia (Hb <6 g/dL)
Certain characteristics of Ab
Acute renal failure
infection

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14
Q

Which presents as chronic and indolent : cold agglutinin dse or warm AIHA ?

A

Cold

C= chronic

No predominance of spleen in Cold agglutinin dse

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15
Q

First line tx for cold agglutinin dse

A

Rituximab

Prednisone and splenectomy = NOT effective

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16
Q

Poisoning from which susbance is associated with Burton’s line?

A

Lead poisoning

Burton’s lie = bluish pigmentation of gum tooth line

Also assoc with basophilic stippling

so mga assoc with blue = lead

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17
Q

Paroxysmal nocturnal hemoglobinuria is due to deficiency of what surface proteins?

A

CD 59 and 55

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18
Q

Tx for PNH

A

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

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19
Q

How does pernicious anemia cause cobalamin deficiency?

A

anti IF antibodies decrease the availability of IF that binds to cobalamin

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20
Q

How does ZES cause cobalamin deficiency?

A

Inc acid causes inactivation of trypsin resulting in failure to release cobalamin from R binding protein

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21
Q

How does GVHD cause cobalamin deficiency?

A

Malabsorption of cobalamin due to abnormal gut flora and ilealmucosa damage

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22
Q

Characteristic peripheral blood smear pattern of thalassemia

A

Target cells

Another way of differentiating thalassemia from IDA is that IDA has low ferritin and obv low iron

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23
Q

Most common hypoproliferative anemia

A

IDA

next: anemia of inflammation

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24
Q

Mechanism most contributory to anemia in px with CKD

A

Dec EPO and reduction in red cell survival

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25
Q

First line for severe aplastic anemia

A

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

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26
Q

Only tx that offers a chance of cure for MDS

A

HSCT

However due to significant tx related mortality and morbidity that increases with recipient age, only small proportion undergo this

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27
Q

The only symptoms that would differentiate polycthemia vera from other causes of erythrocytosis

A

Aquagenic pruritus
Erythromelalgia

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28
Q

1st step in the evaluation of elevated hemoglonin

A

Measure RBC mass

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29
Q

What would you consider when px has elevated rbc mass and low hb

A

Polycthemia vera

Confirm JAK2 mutation

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30
Q

Next step when px has elevated hb, rbc mass and elevated EPO

A

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.

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31
Q

Next step when px has elevated hb, rbc mass and elevated EPO, NON smoker and has normal hemoglobin affinity

A

Search for tumor as source of EPO
(cerebellar hemangioma, uterine leiomyoma, hepatoma)

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32
Q

Maintaining hemoglobin and hematocrit in polycythemia vera

A

<=14 g/dL and <45% in men
<=12g/dL and <42% in women

1445
1242

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33
Q

Tx for symptomatic splenomegaly in polycythemia vera

A

Ruxolitinib - a JAK 2 inhibitor

PEG IFN a is an alternative

Also addresses other constitutional ssx of PV

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34
Q

How does survival of px with essential thrombocytosis compare with general population

A

Same

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35
Q

Major hormone regulator of plt production

A

Thrombopeitin

synthesized primarily in the liver parenchymal cells

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36
Q

Most common cause of NON iatrogenic thrombocytopenia

A

Infection

if iatrogenic –> medications

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37
Q

deficient protease in TTP

A

ADAMTS13

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38
Q

What constitutes TTP pentad

A

CNS symptoms, fever, renal failure, MAHA, thrombocytopenia

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39
Q

Mainstay of tx for TTP

A

TPE

therapeutic plasma exchange

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40
Q

Primary tx for HUS

A

Supportive

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41
Q

Target of antibodies that cause HIT

A

PF4 and heparin/ other GAG

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42
Q

Risk of developing HIT is highest after exposure to heparin for ______ days

A

5-14

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43
Q

Most common inherited bleeding disorder

A

von willebrand dse

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44
Q

what constitues ISTH scoring for DIC

A
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45
Q

central mechanism of DIC

A

uncontrolled generation of thrombin

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46
Q

Butt cells with auer rods in PBS is seen in what condition

A

Acute promyelocytic leukemia

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47
Q

Cases in which whole blood may be transfused

A

massive blood loss and coagulopathy
trauma casualties requiring massive transfusion
hemorrhagic shock
neonatal exchange transfusion

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48
Q

Hemoglobin transfusion thresholds for the ff
hemodynamically stable
preexisting cardiovascular disease
acute coronary artery dse

A

hemodynamically stable <7 (except for px undergoing orthopedic/cardiac surgery)

preexisting cardiovascular disease <8

acute coronary artery dse <9-10

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49
Q

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

A

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

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50
Q

Indications for FFP transfusion

A

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

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51
Q

Cause of febrile non hemolytic transfusion rxn

A

Anti HLA antibodies and cytokines released from leukocytes in non leukoreduced blood

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52
Q

Cause of acute hemolytic transfusion rxn

A

preformed antibodies that lyse donor erythrocytes

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53
Q

Cause of DELAYED hemolytic transfusion rxn

A

Sensitization to RBC alloantigens

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54
Q

Cause of allergic rxns during blood transfusion

A

plasma proteins

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55
Q

Cause of GVHD during blood transfusion

A

donor T lymphocytes that attack host HLA antigens

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56
Q

What should be given in pregnant px with Rh neg blood transfused with Rh positive blood to prevent allosensitization

A

Anti D Ig

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57
Q

Treatment for TRALI

A

supportive

may occur within 6 hrs although delayed cases may occur up to 72h later

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58
Q

Recipient risk factors assoc with inc risk of TRALI

A

Smoking, chronic alcohol use
Shock
Liver and cancer surgery
Mech vent
positive fluid balance

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59
Q

Definition of massive transfusion

A

Transfusion of 50% of the patient’s total blood volume over 3 hrs OR

> 5-10 units of RBC

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60
Q

What diagnostic tests can be requested to assess for iron overload

A

Serum ferritin
MRI
Liver biopsy

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61
Q

Alemtuzumab is a an anti CD ___

A

52

marami akong alam. 52 ang alem ko
a-LIMA-2-zumab

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62
Q

Blinanutumab is a an anti CD ___

A

19

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63
Q

Rituximab is a an anti CD ___

A

20

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64
Q

Moxetumumab is a an anti CD ___

A

22

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65
Q

Brentuximab is a an anti CD ___

A

30

bRENTuximab - tRENTa

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66
Q

Mechanism of action of Bevacizumab

A

inhibits VEGF to block angiogenesis

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67
Q

Mechanism of action of Denosumab

A

RANK ligand inhibitor

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68
Q

Mechanism of action of Everolimus

A

mTOR inhibitor

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69
Q

Mechanism of action of Venetoclax

A

inhibtis Bcl-2

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70
Q

Cardioprotectant that may be given with doxorubicin

A

Dexrazoxane

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71
Q

Most feared pulmonary complication of bleomycin

A

Pulmonary fibrosis

Earliest indicator of adverse effect is a dec in DLCO or coughing

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72
Q

Dose of doxorubicin assoc with cardiomyopatht

A

> 550 mg/m2

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73
Q

Dose of bleomycin that inc incidence of pulmo fibrosis

A

> 300 cumulative units

minimally responsive to glucocorticoids

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74
Q

MOA of renal toxicity of methotrexate

A

from crystallization in renal tubules

nagccrystallize ang meth

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75
Q

Mesna lessens toxicity of which chemo agent

A

Cyclophosphamide

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76
Q

Treatment for acute promyelocytic leukemia

A

ATRA

assoc with t(15;17) chromosomal tanslocation

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77
Q

Supportive tx of choice for nausea and vomiting for high riskagents

A

Serotonin antagonisis (eg. odansetron) and neurokinin 1 antagonists (aprepitant)

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78
Q

Plerixafor blocks what receptor

A

CXCR4

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79
Q

most commonly mutated oncogene in cancers

A

RAS

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80
Q

Trisomy 21 inc or dec risk of AML?

A

inc

inc also risk for ALL

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81
Q

Genetic abnormalities when present will classify a patient to have AML regardless of marrow blast count %

A

t (15;17)
t (8;21)
inv(16)
t (16;16)

otherwise, need blast count >=20%

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82
Q

Most impt prognostic factor that predict outcome of AML

A

Chromosome and molecular investigations

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83
Q

Most common PE finding in CML

A

Splenomegaly

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84
Q

Most common symptom of myeloma

A

Bone pain

affecting nearly 70% of px

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85
Q

Criteria for initiation tx for CLL

A

Watchful waitng for most px except if with the ff

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86
Q

Single most powerful predictor of survival in myeloma

A

Serum beta 2 microglobulin

can also substitute for staging

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87
Q

Criteria for diagnosis solitary plasmacytoma

A

> 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

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88
Q

Criteria for dx multiple myeloma

A

> 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

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89
Q

What class of drugs do bortezomib, carfilzomib and ixazomin belong to

A

Proteoseoe inhibitors

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90
Q

Class of drug: Panobinostat

A

Histone deacetylase inhibitor

tx for myeloma

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91
Q

Class of drug: Selinexor

A

Selective inhibitor of nuclear export (SINE)

tx for myeloma

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92
Q

An MCHC > ___ on ordinary blood count should raise suspicion of hereditary spherocytosis

A

34

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93
Q

PNH is an acquired mutation of ___ gene

A

PIG-A gene

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94
Q

Most common early symptom of aplastic anemia

A

bleeding

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95
Q

Thalidomide derivative that is effective in reversing anemia of MDS with 5q- syndrome

A

Lenalidomide

LIMAlidomide

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96
Q

Nilotinib is not recommended in this subset of px

A

DM px on insulin

higher rates of hyperglycemia 10-20%

Other adverse effects: PAOD, Panc

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97
Q

Optimal TKI if with T315I mutation

A

Ponatinib

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98
Q

Typical immunophenotype of B cell lymphomas would test positive for these markers

A

CD 19, 20,23
CD 5 (although this is a T cell marker)

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99
Q

What is Richter’s transformation

A

Transformation of CLL to more aggressive lymphoma eg. DLBCl

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100
Q

Most sensitive test for DIC level

A

FDP

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101
Q

These are seen in what disease conditions?

A

Thalassemia and liver dse

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102
Q

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

A. Intravascular hemolysis

since reticulocyte index is > 2.5 or high in px with hemorrhage/hemolysis

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103
Q

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

A

C. Immune-mediated peripheral destruction by drug haptens

Most common cause of neutropenia: iatrogenic

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104
Q

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

A

D. Negative iron balance

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105
Q

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

A and B

Dx: IDA

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106
Q

What laboratory test will best differentiate anemia of chronic disease from true iron-deficiency anemia?

A

Serum ferritin

IDA- low <15
Anemia of chronic dse - normal to high 30-200

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107
Q

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

A

D. Malignancy

b9 may feed tumors

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108
Q

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

A. Cobalamin

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109
Q

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

A

C. Rasburicase

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109
Q

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

A

b. Rho (D) immune globulin

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110
Q

Gold std for PNH

A

Flow cytometry

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111
Q

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

A. Complications of pancytopenia

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112
Q

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

A

D. Tretinoin

Dx: Acute promyelocytic leukemia

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113
Q

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

A

D. Thrombocytopenia

Thrombocytosis is common but thrombocytopenia is rare and suggests worse prognosis

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114
Q

At what percentage of peripheral or marrow blasts is blastic-phase CML defined?
A. 20%
B. 25%
C. 30%
D. 35%

A

C. 30%

115
Q

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

A. Chemotherapy alone

Chemotx offers potentially curative x for DLBCL REGARDLESS of stage

116
Q

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

A

B. Stage III since both sides

117
Q

most common cause of renal failure in patients with multiple myeloma?

A

hypercalcemia

118
Q

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)

A

B. Bone marrow aspirate and biopsy

Dx: multiple myeloma

119
Q

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

A

C. Febrile non-hemolytic transfusion reaction

120
Q

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

A

D. Increase in bradykinin levels

Blood products contain bradykinin normally degraded by ACE hence those on ACE-I –> inc bradykinin –> transient hypotension

121
Q

Indications for irradiated blood components

A
121
Q

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

A

B. Distal lower extremities

Petechiae first appear in areas of inc venous pressure eg ankles and feet

122
Q

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

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

123
Q

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

A

D. Treatment is primarily supportive

Schistocytes is characteristic of MAHA
Dx: HUS

124
Q

How much factor VIII does a bag of cryoprecipitate contain?

A

80 IU

125
Q

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

A.

126
Q

How do you treat Heparin induced thrombocytopenia?

A
127
Q

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

A

D. No additional therapy is required

since INR < 10 and no bleeding

128
Q

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

A

20

129
Q

What is the most common autoimmune complication in chronic lymphocytic leukemia (CLL)?

A

Autoimmune hemolytic anemia

130
Q

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

A

D. No treatment indicated at present

131
Q

“Dry taps,” or inability to aspirate bone marrow, occur in about 4% of attempts with the most common differential diagnosis being:

A

Metastatic carcinoma filtration

132
Q

Target pre transfusion Hb in px with beta thalassemia intermedia/minor

A

9-10.5

133
Q

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.

A

D. Low serum iron while stores of iron are adequate.

134
Q

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)

A

D. Human immunodeficient virus (HIV)

135
Q

most common presentation of Hodgkin’s lymphoma

A

non tender lymphadenopathy

136
Q

Needed iron per day to replace RBCs lost through senescence

A

20mg/day

137
Q

Causes of inc protoporphyrin

A

IDA, lead poisoning

due to inadequate Fe supply to erythroid precursors to support Hb synthesis

138
Q

Dose of folic acid for pregnant px

A

400 mcg if no hx of NTD
if with hx, 5mg/d

139
Q

Gold std for proving dec RBC lifespan

A

RBC survival study

140
Q

Diagnostic test for hereditary spherocytosis

A

osmotic fragility test

141
Q

Most frequent infectious cause of hemolytic anemia

A

endemic areas- malaria
non-endemic Shiga toxin producing E.coli O157:H7

142
Q

Triad of AIHA

A

Large drop in hb
jaundice
splenomegaly

143
Q

Triad for PNH

A

intravascular hemolysis
pancytopenia
inc risk of venous thrombosis

144
Q

Most common cause of death for PNH

A

venous thrombosis

145
Q

Highly reliable test for PNH

A

Acidified serum (Ham test)

sucrose hemolysis test : UNRELIABLE
Gold std: flow cytometry

146
Q

Mandatory supplement needed for PNH

A

Folic acid at least 3 mg/d

147
Q

Cardinal feature of BM failure

A

Hypoproliferative anemia

148
Q

Major prognostic determinant of aplastic anemia

A

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

149
Q

Mutations in MDS associated with good prognosis

A

-Y
del 5q
del 20 q

mnemonic: GOOD date: maY 20 (5/20)

150
Q

Causes of MICROcytic erythrocytosis

A

beta thalassemia trait
polycythemia vera
hypoxic erythrocytosis

151
Q

Most impt risk factor for thrombosis in essential thrombocytosis

A

Smoking

152
Q

Most common cause of incompatibility during pre transfusion

A

Lewis

153
Q

Occasional px with mononucleosis or M.pneumoniae may develop agglutinins against this blood group system antigen

A

anti- I or anti- i

154
Q

Sometimes px with syphilis/ viral infection that lead to paroxysmal cold hemoglobinuria has autoantibody to this blood group system antigen

A

P system

P antigen also expressed on urothelial cell and may be a receptor for E. coli binding

155
Q

Blood group antigen that serves as receptor for P. vivax

A

Duffy

156
Q

Whole blood transfusion is indicated if total blood volume loss is >= ___%

A

25

157
Q

What can be done to prevent febrile non hemolytic transfusion rxn?

A

Leukoreduction

158
Q

What can be done to prevent allergic rxn during transfusion?

A

premedicate with antihistamine if with known hx of allergy
Use washed RBC

159
Q

Most common non iatrogenic cause of thrombocytopenia

A

viral and bacterial infection

160
Q

To which anticoag should you shift px who developed HIT?

A

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

161
Q

AML commonly assoc with DIC

A

Acute promyelocytic leukemia

162
Q

How much platelets must be transfused in px with DIC and severe thrombocytopenia

A

1-2u/10 kg

163
Q

How do you define severity of hemophilia

A

Severe <1%
Moderate 1-5%
Mild 6-30%

most common mutation: inv of intron 22
severe and moderate forms may present with hemarthrosis

164
Q

Leading cause of death in px with hemophilia with replacement tx

A

HIV, Hep C

165
Q

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)

A

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

166
Q

Target factor activity for hemophilia as prophylactic replacement for surger

A

100% for 7-10 days

** oral surger requires factor replacement for 1-3 days coupled with antifibrinolytics

167
Q

Confirmatory test for presence of factor inhibitor

A

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

168
Q

Treatment for factor XI (eleven) inhibitor

A

do not give FFP and Factor XI
iv platelet concentrate or recombinant activated factor VII (seven)

mnemonic: 7/11

169
Q
A

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).

170
Q
A

Polychromatophilia. Note large red cells with light purple coloring.

171
Q
A

Hypochromic microcytic anemia of iron deficiency. Small lymphocyte in field helps assess the red blood cell size

172
Q
A

Macrocytosis. These cells are both larger than normal (mean corpuscular volume >100) and somewhat oval in shape. Some morphologists call these cells macroovalocytes.

173
Q
A

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.

174
Q
A

Spherocytosis. Note small hyperchromatic cells without the usual clear area in the center.

175
Q
A

Sickle cells. Homozygous sickle cell disease. A nucleated red cell and neutrophil are also in the field.

176
Q
A

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.

177
Q
A

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

178
Q
A

Elliptocytosis. Small lymphocyte in center of field. Elliptical shape of red cells related to weakened membrane structure, usually due to mutations in spectrin.

179
Q
A

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).

180
Q
A

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

181
Q
A

Heinz bodies. Blood mixed with hypotonic solution of crystal violet. The stained material is precipitates of denatured hemoglobin within cells

182
Q
A

Giant platelets. Giant platelets, together with a marked increase in the platelet count, are seen in myeloproliferative disorders, especially primary thrombocythemia.

183
Q
A

Stippled red cell in lead poisoning. Mild hypochromia. Coarsely stippled red cell.

184
Q
A

Pelger-Hüet anomaly. In this benign disorder, the majority of granulocytes are bilobed. The nucleus frequently has a spectacle-like, or “pincenez,” configuration.

185
Q
A

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

186
Q
A

Chédiak-Higashi disease. Note giant granules in neutrophil.

187
Q
A

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

188
Q
A

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.

189
Q
A

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

190
Q

Causes of anemia with reticulocyte production index of > 2.5

A
191
Q

Methods used for percussing for splenic dullness

A
  1. 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.
  2. 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.
  3. 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

192
Q

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

A

b. JAK2 activating mutation

193
Q

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

A

c. Request for peripheral blood smear

r/o pseudothrombocytopenia

194
Q

What is the central mechanism of DIC?

A

Uncontrolled thrombin generation

195
Q

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

A

c. 2 – 2.5 years

The standard duration of maintenance therapy for ALL
in adults is approximately 2–2.5 years.

196
Q

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

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.

197
Q

This is the major cause of morbidity and the second leading cause of death in hemophilia patients receiving
clotting factor concentrates

A

HCV

198
Q

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

A

c. >30g/L (3g/dL) serum M protein

199
Q

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

A

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

200
Q

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

A

B. Platelet concentrate at room temperature

others are not really stored at room temp

201
Q

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

A

B. Spherocytic red cells

Warm AIHA –> Spherocytes

202
Q

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

A

B. Elevated levels of factor VIII

203
Q

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

A

D. Reassure patient and repeat CBC platelet count once inflammation subsided

204
Q

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

A

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

205
Q

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

A

C. Elevated fibrin degradation products

it is not DIC if FDP not elevated

206
Q

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

A

B. Sulfonamides and quinine

207
Q

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

A

B. At a platelet count of less than 5,000/uL

208
Q

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

A

C. Ponatinib

209
Q

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

A

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.

210
Q

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

A

D. Active surveillance

Currently, a watchful waiting strategy is used for most patients with CLL, with therapy reserved for patients with symptomatic disease.

211
Q

.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

A

B. NPM1

AML with mutated NPM1 is associated with a more favorable clinical outcome.

212
Q

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

A

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.

213
Q

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

A

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)

214
Q

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

A

B. 3,500 IU

215
Q

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

A. Repeat CBC after 1 week since >30k and no bleeding

216
Q

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

A

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.

217
Q

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

A

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).

218
Q

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

A

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.

219
Q

Blast cut off for dx of AML

A

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

220
Q

Definition of complete response of AML to tx

A

ANC >1000/uL and plt >100K
Absent circulating blasts
Bone marrow blasts <5%
No Auer rods, no extramedullary leukemia

221
Q

Back pain and lower extremity weakness is commonly seen in AML with what mutation

A

8;21

222
Q

What should be tested prior to giving high dose cytarabine in AML px

A

Cerebellar testing due to irreversible toxicity

223
Q

most common induction for AML

A

Cytarabine + Anthracycline 7+3

224
Q

Target plt and hb during chemotx of px with AML

A

plt >10k
hb >8g/dL

transfuse to maintain these values

225
Q

Chromosomal abnormalities associated with poor prognosis in CML

A

trisomy 8
double pH
isochrome 17
17 p deletion
20q- (good prog in MDS)

226
Q

Major tx endpoint of CML

A

Achievement of COMPLETE cytogenetic response
the only endpoint associated with improvement in survival = approximates normal life expectancy

227
Q

Definition of erythrocytosis

A

Men
Hb >17 (Hct>50)

Women
Hb >15 (Hct >45)

228
Q

How do you diagnose CNS leukemia

A

Lumbar tap: >=5 leukocytes/uL or leukemic blast
must have plt at least 20k prior to procedure

LP: impt diagnostic in ALL

229
Q

Most common immunologic subtype of B- ALL

A

Common ALL

230
Q

Most impt prognostic factor during tx of ALL

A

Minimal residual dse

231
Q

The only ALL specific drug

A

L asparaginase
now more intensively used in adults

232
Q

Examples of TKI that can cross BBB

A

Dasatinib, probably ponatinib

233
Q

Intrathecal chemotherapy in ALL

A

methotrexate +/- cytosine arabinoside (AC) +/- glucorticoids

234
Q

MOA of Blinatumomab

A

CD19 inhibitor

235
Q

MOA of inotuzumab

A

CD 22 inhibitor

ino2zumab

236
Q

Mutation with good prognosis in CLL

A

del 13 (q14.3)

poorest prog: del 17 (p13.1)

237
Q

If clonal B cell count on flow cytometry >= ____ diagnosis is CLL, no further work up needed

A

5x10^9/L

238
Q

Leading cause of morbidity and mortality in CLL

A

infection

PCP pneumonia prophy for at least 6mos needed following nucleoside analog based tx

Varicella prophy if with prior hx of varicella

239
Q

In Richters transformation CLL commonly transforms into an aggressive lymphoma most commonly _____

A

DLBCL

240
Q

First step in dx Richter transformation

A

PET CT to localize an area for biopsy

SUV > 5

241
Q

Staging for CLL

A

No reqt for imaging or BM biopsy

242
Q

Primary tx for CLL

A

BTK or BCL2 targeted oral tx +/- CD20 monoclonal Ab

BTK inh - ibrutinib, acalabrutinib
BCL2 inh - venetoclax

243
Q

Most common subtype of Hodgkins in younger age groups

A

Nodular sclerosis

244
Q

Used for staging Hodgkins

A

PET scan

245
Q

Most common chemotx for Hodgkins

A

ABVD

Adriamycin, Bleomycin, Vinblastine, Dacarbazine

No benefit of adding RT in advanced stages

246
Q

Nilotinib can be given in all phases of CML except

A

Blastic phase

247
Q

laboratory test reflects the total erythroid mass and is expected to be elevated in true iron deficiency anemia?

A

Transferrin receptor protein

248
Q

In acute infection or inflammation, anemia results from

A

Faster destruction of early senscent RBCs

249
Q

The functional murmur of anemia is systolic/diastolic?

A

mid systolic ejection type of murmur

250
Q

mechanism of anemia in liver dse

A

shortened RBC survival +inadequate compensatory EPO production

251
Q

T/F paresthesias and muscle weakness are manifestations of both Vit B 9 and Vit B12 deficiencies

A

False. Vit B12 only

252
Q

Pernicious anemia is associated with blood group ___

A

A

F>M, more common among >60yo
Normal expectancy in women with tx; shorter in men due to inc risk of gastric CA

253
Q

Abnormal hemoglobin form seen in excess in Beta thalassemia

A

HbA2

dec HbA and HbF

254
Q

Amount of elemental iron in 1 uprbc

A

250-300 mg

50-65 mg per tab of ferrous sulfate

255
Q

most common preceding infection in aplastic anemia

A

seronegative hepatitis

256
Q

Criteria for complete remission of AML

A

all must be satisfied

257
Q

Bortezomib and Lenalidomide require this/these prophy

A

Bortezomib = VZV prophy
Lenalidomide = DVT prophy

258
Q

Treatment for hyperviscosity syndrome in MM and Waldenstroms Macroglobulinemia

A

plasmapheresis

259
Q

Most common Non Hodgkins lymphoma

A

DLBCL (aggressive type)

260
Q

Mutations associated with the ff lymphomas:
Burkitt
Follicular
Mantle cell
MALT

A

Burkitt = t(8;14) Burk8t –> starry sky
Follicular = t (14;18) Fourteen, eighteen
Mantle cell= t(11;14) mantlELEVEN
MALT = t(11;18)

261
Q

Most common INDOLENT NHL

A

Follicular (2nd most common NHL)

262
Q

NHL that needs prophylactic CNS tx

A

Burkitt

Combination tx for Burkitt all contain cyclophosphamide

263
Q

Associated organisms with MALToma

A

H. pylori (gastric)
Borrelia burgdorferi (skin)
C. psitacci (eye)
C. jejuni (Small intestine)

264
Q

Lymphoma associated with Sezary syndrome

A

Mycosis fungoides/ Cutaneous T cell lymphoma

Consists of generalized erythroderma and circulating tumor cells

265
Q

Most common T cell lymphoma

A

Angioimmunoblastic T cell lymphoma

> 80% with advanced dse
+ polyclonal hypergammaglobulinemia , eosinophils, inc LDH, + Coombs, opportunistic infections

266
Q

Lymphoma with flower cells positive for CD 4 and 25

A

Adult T cell lymphomas

267
Q

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

A

D. 40-year-old with painless supraclavicular LAD, measuring 2.0 cm

268
Q

What is the mainstay of treatment for sickle cell patients with severe symptoms?

A

Hydroxyurea

It increases HbF

269
Q

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

A

C. Tropical sprue

270
Q

In px with megaloblastic anemia due to cobalamin deficiency, what is the usual level of cobalamin?

A

<100 ng/mL or <74 pmol/L

270
Q

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

A. Cobalamin

271
Q

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

A

D. Transfuse incompatible blood

272
Q

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

A

C. Request for bone marrow aspirate and biopsy

273
Q

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

A. Anti-thymocyte globulin + cyclosporine

Since elderly –> A is the better answer

274
Q

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

A. ε-aminocaproic acid can be given prophylactically before and after elective surgery to prevent bleeding.

275
Q

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

A

B. Acute myeloid leukemia

276
Q

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

A. HLA-matched platelets

277
Q

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

A

D. Thrombocytopenia

278
Q

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

A. Chemotherapy alone

279
Q

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

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

280
Q

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

A

D. Observe and monitor CBCs since >30k

281
Q

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

A

C. Every month

282
Q

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

A

D. No additional therapy is required

283
Q

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

A

D. Warfarin