hematology 2006

8/12/2019 Hematology 2006

1/29

Integrated Hematology Exam 11/21/06 Name:_______________________ID#:___________

Integrated Hematology:Pathophysiology/Pharmacology/Pathology (BI350/BI279)

CASE 1: (Questions 1-3)

A 65 year-old gentleman presents to your office it! a c!ief complaint of !eadac!e" #e states

t!at !e !as !ad intermittent !eadac!es for t!e last mont! or to" $!e !eadac!es are typically

relie%ed &y $ylenol" #e also notices t!at !e de%elops itc!ing' especially after !e taes a arms!oer" #e !as tried taing enadryl' &ut it! only partial impro%ement" #is ife notices

t!at !is comple*ion !as &ecome increasingly ruddy o%er t!e last se%eral mont!s" $!e patient

!as for t!e most part en+oyed good !ealt!" #e does not smoe" #e !as no non cardiac orrespiratory pro&lems" ,e%ie of systems is positi%e only for some soreness in !is left upper

uadrant"

.!ysical e*am: /ital signs are normal" #EE0$ re%eals eryt!ematous con+uncti%a" $!ere isno a&normal adenopat!y" ungs clear" #eart: S1' S2 normal no murmurs' ru&s or gallops"

#is a&domen is soft and non-tender" #e !as a 2-finger&readt! spleen" $!e li%er is not

palpa&le" E*amination of !is e*tremities is unremara&le"

#is !emoglo&in is 14 gmdl' !ematocrit 5' !ite count 12'777' platelet count 567'777" #is

perip!eral smear re%eals an increased num&er of platelets' &ut is ot!erise unremara&le"

1" $!is patient most liely !as a:

A" cra&l translocation"" #ig! eryt!ropoietin le%el"

C" 8A9-2 mutation"

" .roliferation of clonally-related fi&ro&lasts"E" #ypercellular marro due to an infectious process"

2" ;f t!is patient !ad a splenectomy for anot!er reason (suc! as trauma from a motor %e!icleaccident)' t!e !istology of t!e spleen ould most liely:

A" Appear to &e normal"" emonstrate an increased num&er of plasma cells"

C" e infiltrated &y s!eets of myelo&lasts"

" emonstrate myeloid metaplasia (e*tramedullary !ematopoiesis)"

E" 0one of t!e a&o%e"

Note that the correct answer is D. Myeloid metaplasia would be the expected finding

in the spleen of a patient with P.Vera. However, splenic congestion !. "None of the

#bove$% would be an appropriate answer as well for a patient in the early

!rythrocytotic Phase of P.Vera% & so we will accept answer !. as well.


2/29


$!is patient as treated appropriately and did ell for t!e ne*t years" #e no presents to !is

p!ysician it! symptoms of s!ortness of &reat! and dyspnea on e*ertion" #e is found to &e

anemic it! a #& of ' #ct 2s syndrome)"" isease progression to myelofi&rosis"

C" ?etastatic carcinoma unrelated to t!e initial diagnosis"

" $ransformation to Acute ymp!o&lastic euemia"

E" Adeuate cellularity' suggesting perip!eral red cell destruction as ane*planation for t!e anemia"

CASE 2" (Questions < @ 5)

A 55 year-old layer !o !as en+oyed e*cellent !ealt! presents to your office complaining of

easy &ruising" #e !as noted t!at o%er t!e last fe ees !e alays seems to !a%e an ecc!ymoticlesion some!ere on !is e*tremities or !is trun" $!ere as no non trauma" Additionally' !e

states t!at !e is fatigued more easily t!an is !is norm" An a%id golfer' !e finds it difficult to al

1 !oles" #e often notes s!ortness of &reat! it! e*ertion and occasional palpitations"

n e*amination' %ital signs are normal" #EE0$: con+uncti%al pallor' e*tensi%e selling of t!e

gums (see =igure 2A &elo) and palatal petec!iae" 0o a&normal adenopat!y" ungs are clear"

#eart: S1' S2 normal' ;/; systolic murmur" A&domen is soft' non-tender' no masses" #ise*tremities are negati%e e*cept for a diffuse petec!ial ras! on &ot! loer e*tremities &elo t!e nee"

"

2

=igure 1"(? &iopsy on left

reticulin stain on rig!t)"

=igure 2A"


3/29


a&oratory Studies:

CC: BC 1'777 it! 15D polys' 17D lymp!s' 5D monos' 7D &lasts #& " gmdl

?C/ 5 platelet count 27'777".$ and .$$ are normal"

$!e perip!eral smear is s!on in =igure 2"


4/29


CASE 3" (Questions 6-4)

A patient it! asimilar presentationto t!e one descri&ed in Case 2 (a&o%e) as found to !a%e

t!e folloing cells on perip!eral smear (=igure 3)"

6" B!ic! of t!e folloing molecular defects ould &e most liely in t!is disorder

A" C!romosomal translocation in%ol%ing a C= (Core inding =actor)"" %ere*pression of cyclin 1"

C" t(1


5/29


CASE


6/29


13" B!ic! of t!e folloing accurately reflects t!e pat!op!ysiological &asis of t!is disease

A" ;t is associated it! t!e t(1s lymp!oma is treated it! a c!emot!erapy regimen"

Si* mont!s after completing !er treatment' s!e de%elops symptoms of s!ortness of &reat!

and is found to !a%e rales on lung e*am' and ele%ated 8/ (+ugular %enous distention)"

CJ, is consistent it! congesti%e !eart failure" $!is could represent a to*ic effect of:

A" o*oru&icin" /incristine

C" ,itu*ima&" =ludara&ine

E" Cyclop!osp!amide"

15" $!e t!ree most common types of 0on-#odgin>s lymp!oma in c!ildren (in t!e I"S") are:

A" ymp!o&lastic lymp!oma' =ollicular lymp!oma' C

" ymp!o&lastic lymp!oma' C' uritt>s lymp!oma

C" =ollicular lymp!oma' Small Cell ymp!ocytic ymp!oma' C" uritt>s lymp!oma' .erip!eral $ Cell lymp!omas' ?antle Cell lymp!oma

E" =ollicular lymp!oma' ?arginal Gone lymp!omas' uritt>s lymp!omas"

16" $!e to most common types of 0on-#odgin>s lymp!oma in adults in t!e I"S" are:

A" C and Small Cell ymp!ocytic ymp!oma

" =ollicular lymp!oma and CC" ?antle cell lymp!oma and uritt>s lymp!oma

" ?arginal Gone lymp!oma and C

E" #odgin>s lymp!oma and C"

1" A 3< year old oman it! Acute ?yelogenous euemia underent induction c!emot!erapy

and is no in remission" Cytogenetic studies re%ealed t!at s!e as at a !ig! ris of relapse" S!e

is no considering consolidation treatment it! #ig! ose c!emot!erapy @ stem cell trans-plantation" B!ic! of t!e folloing descri&es t!e properties of !ematopoietic stem cells $!ey:

A" Can repopulate t!e marro it! all cell lineages including !ematopoietic and

stromal supporting cells"" Are meta&olically indolent ie not !ig!ly proliferati%e cells"

C" E*press C33"

" E*press -cell and $-cell specific marers' since t!ey can differentiate intolymp!oid or myeloid cells"

E" Are easily identifia&le in &one marro aspirates"

6


7/29


1" B!ic! of t!e folloing is 0$ typically due to a disorder of pluripotential stem cells

or multipotential progenitorsA" Aplastic anemia

" ?yelodysplastic syndromes (?S)

C" C?" ."/era

E" ?ultiple ?yeloma

14" A 24 year old oman !o complains of fatigue is found to !a%e a !emoglo&in of 4"4 gdl

(normal 12-16)' a lo ?C/ and a lo ?C#C"

B!ic! of t!e folloing is t!e most appropriate drug t!erapy

A" Cyanoco&alamin" Epoetin

C" =errous gluconate

" =ilgrastin

E" =olic acid

0$E: QIES$;0S 27 @ 21 A,E ,EA$E"

27" A 6 year old man presents it! se%ere t!oracic &ac pain' fatigue and eig!t loss"

a&oratory studies: #& "7 gmdl' #ct 2


8/29


B!ic! of t!e folloing statements regarding t!ese cells is 0$ correct

A" $!ey are slig!tly larger cells (!ig!er ?C/) t!an normal r&cs"

" $!ey are more resistant to osmotic lysis t!an normal cells"C" $!eir ina&ility to c!ange s!ape leads to splenic trapping"

" $!is type of cell may &e seen in a %ariety of !emoglo&inopat!ies"

E" ;t is a manifestation of e*cess mem&rane"

'his smear shows target cells that are typically seen in liver disease and in

thalassemias and hemoglobinopathies. 'his rbc shape is due to excess membrane !

is a true statement%. !xcess membrane would result in slightly larger cells # is a

true statement% that are lessli(ely to undergo osmotic lysis ie there is more roominside the cell to ta(e in water before lysis & so they are more resistantto osmotic

lysis% ) is a correct statement%. *ncreased splenic trapping occurs in cells that are

less flexibleand unable to change shape to navigate the microcirculation. 'he classicexample we gave you of this was Hereditary +pherocytosis where there is a decrease

in the membranehemoglobin ratio, less rbc flexibility, and more splenic trapping

with rbc destruction.

2


9/29


ability to concentrate urine the acidic and hypertonic environment in the renal

medulla results in sic(ling with microinfarcts%. +plenomegaly is N' a common

finding in adults with ++ disease because of splenic infarction which reduces the si/e

of the spleen by the time of adolescence "autoinfarction$%. 0emember that sic(le

patients are at increased ris( of infections with encapsulated organisms because of

this complication. 1inally, there is an increased thrombotic ris( but not because ofnormal platelets2

25" E*amination of perip!eral &lood smears may identify r&c a&normalities t!at may trigger ap!ysician to consider a particular diagnosis" B!ic! association is ;0C,,EC$

A" Sp!erocytesarm autoanti&odies

" ,ouleau*cold agglutinin disease

C" Sc!istocytes;C

" #oell-8olly &odiessplenectomy

E" asop!ilic stipplinglead poisoning

26" A patient presents it! a #& of "5' #ct of 26' and a normal &c and platelet count" Koususpect iron deficiency anemia &ecause s!e reports !ea%y menses" B!ic! of t!efolloing ould &e consistent it! t!is diagnosis

A" ecreased =e' decreased $;C' decreased ferritin"

" ecreased =e' increased $;C' increased ferritin"

C" ecreased =e' increased $;C' decreased ferritin"" ecreased =e' ?C/ of 45' and decreased ferritin"

E" ecreased e*pression of ?$-1"

2" A


10/29


24" A 54 year old oman !ad an isc!emic stroe 5 years ago' and is on clopidogrel (.la%i*)

to decrease !er ris of su&seuent isc!emic e%ents" $!is drug functions as an anti-platelet drug %ia !ic! mec!anism ;t:

A" ;n!i&its cycloo*ygenase"

" ;n!i&its t!e Hp;;&;;;a reaction"C" ;n!i&its t!e A. reaction"

" ;n!i&its Hp;&"

E" All of t!e a&o%e"

37" A 67 year old ;talian man presents to t!e E, it! profound fatigue and !emoglo&inuria after

en+oying Easter dinner (!ic! included fa%a &eans)" #& 6"7' #ct 1 &c and platelet counts

ere normal # 1277' ili


11/29


S!e most liely !as:

A" .ositi%e urinary !emosiderin"

" Acrocyanosis"C" .ositi%e #einF &ody prep it! supra%ital stain"

" .ositi%e irect Coom&s test utiliFing Anti ;gH"

E" ;ncreased life span of circulating red &lood cells"

33" $!e initial treatment of c!oice for t!is patient is:

A" Steroids"

" .lasmap!eresis"C" A%oidance of cold"

" An immunosuppressi%e drug suc! as aFat!ioprine (;muran)

E" ,itu*ima&"

3


12/29


'his is not a ma7or hemolytic reaction, which would present with symptoms occurring after

only a small amount of blood has been transfused and almost always before the unit is

transfused completely. 'hough the symptoms may include fever and chills and thus may

initially be difficult to distinguish from a non5hemolytic transfusion reaction%, there may

also be symptoms of nausea, chest tightness, restlessness, apprehension, 7oint pain, bac(

pain. 'he patient may develop tachycardia, tachypnea, hypotension, and oliguria. Ma7ortransfusion reactions are typically due to #) incompatibility reactions. #gain, these

occur more 8uic(ly than do the non5hemolytic febrile reactions.

#naphylactic reactions can occur in patients who are *g# deficient and have developed *g#

antibodies. 3hen they are exposed to donor *g# in the blood product, they can have an

anaphylactic reaction.

Proteins in donor plasma can cause minor allergic reactions these would be "anaphylactoid$

and might present with urticaria or s(in manifestations in addition to fever and chills%.

'0#9* refers to "transfusion5associated lung in7ury$ and symptoms begin suddenly whilethe blood product is being transfused or up to 6 hours after transfusion. 'he presenting

symptom is D:+PN!#.

3" .atients it! -t!alassemia !o !a%e a one gene deletion ill !a%e:

A" A mild microcytosis it!out anemia"" Ele%ated le%els of # t!roug!out adult life"

C" Ele%ated le%els of #& arts t!roug!out adult life"

" Splenomegaly &ecause of c!ronic lo le%els of !emolysis"

E" 0one of t!e a&o%e"

!ither # or ) is an acceptable answer.

3" A p!ysician on t!e ards is trying to decide if it is appropriate to gi%e cryoprecipitate to apatient !o is &leeding" ;n maing t!is decision' it ill &e !elpful to remem&er t!at

cryoprecipitate does 0$ contain !ic! of t!e folloing factors:

A" =i&rinogen" =actor J;;;

C" =actor /;;;

" =actor /

E" %onBille&rand factor

'he correct answer is D. -ryoprecipitate contains the high molecular weight factors

fibrinogen, factor ;***, factor V***, and von3illebrand factor, but does not contain

factor V.

34" Bit! fi&rinolytic t!erapy' t!e goal is to directly or indirectly en!ance t!e generation of !ic! enFym

A" =actor /;;a " $!rom&in

C" .lasmin

" =actor Ja

E" =i&rinogen

12

Dropped


13/29



14/29


count' normal .=A and a normal .$' &ut !is .$$ is 67 seconds (normal 37 sec)" #e most liely

!as:

A" =actor J;; deficiency" =actor /;;; deficiency

C" =actor ;J deficiency

" %onBille&rand>s diseaseE" =actor /;; deficiency


15/29


'he vast ma7ority of the class got this answer right based on the intent of the 8uestion

#nswer !%. However, it is true that there is an error in ). >antiplasmin binds to

P9#+M*N when it is no longer bound to fibrin. +o in reality, it may have been difficult to

answer !. +ince #, -, and D were all correct, it is difficult to say which is more correct. #llanswers will be accepted to this 8uestion because of this error.

s disease" B!ic! of t!e folloing is most lielyA" #e ould &e e*pected to !a%e normal factor /;;; le%els' &ut decreased

%onBille&rand factor and decreased %onBille&rand antigen"

" #e s!ould !a%e a proportionate decrease in factor /;;;' %onBille&rand factor and%onBille&rand antigen"

C" #e ould !a%e impaired platelet Hp;;&;;;A function"

" #e ould &e e*pected to !a%e little or no response to A/."

E" #e ould !a%e an ele%ated prot!rom&in time (.$)"5


16/29


" =actor / deficiency

C" o le%el of .rotein C

" =actor J in!i&itorE" 0one of t!e a&o%e"

'he (ey here was recogni/ing that a ma7or source of Vitamin ? is endogenous production by

bacteria in the 4.*. tract. !radication of normal gut flora because of prolonged treatment withantibiotics can lead to Vitamin ? deficiency. #s you (now, Vitamin ? is needed for the

synthesis of factors **, V**, *;, and ; as well as Protein - and Protein +. 'hus the correct

answer is -. Note that the patient cannot have D*- & she has a normal platelet count.

56" A surgeon plans to prop!ylactically treat a patient !o !as undergone nee replacement

surgery it! !eparin to decrease t!e ris of deep %enous t!rom&osis" B!ic! of t!e

folloing statements concerning !eparin and lo molecular eig!t !eparin is correctA" ot! need freuent monitoring it! a.$$ to ensure proper t!erapeutic le%el"

" ;n correct t!erapeutic dose' &ot! !a%e eual antiJa acti%ity"

C" ot! !a%e comparati%e ris of patient de%eloping #;$"

" ?B !eparin !as a longer !alf-life t!an unfractionated !eparin"E" Eno*aparin is t!e trade name for unfractionated !eparin"

5" B!ic! of t!e folloing statements regarding arfarin is correct:

A" locs gamma-car&o*ylation of factors ;;' /;;' /' and J"" ;n initial


17/29


54" A


18/29


62" B!ic! of t!e folloing is t!e most liely diagnosis in t!is patientA" Acute ymp!o&lastic euemia

" #odgin>s disease it! marro in%ol%ement

C" Acute ?yelogenous euemia' ?3" ;$.

E" C!ronic ?yelogenous euemia

63" B!ic! of t!e folloing c!romosomal translocations ould you e*pect to find in t!ispatient

A" t(422)

" t(1


19/29


C" ;;A

" ;;

E" ;;;

6" $!is patient completed treatment for #odgin lymp!oma 1 mont! ago" S!e no presents

it! increasing dyspnea and a non-producti%e coug!" .!ysical e*am is remara&le for fine rales att!e lung &ases" Arterial &lood gases s!o !ypo*ia" CJ, s!os pulmonary fi&rosis" B!ic! of t!e

folloing c!emot!erapeutic agents most liely produced t!ese effects

A" leomycin" Cyclop!osp!amide

C" o*oru&icin

" Etoposide

E" /incristine

6" B!ic! type of #odgin>s lymp!oma does 0$ !a%e MClassicalN C15O and C37O

,eed-Stern&erg cells

A" ymp!ocyte-epleted" ymp!ocyte .redominance

C" ?i*ed Cellularity" 0odular Sclerosis

E" ymp!ocyte-,ic!

64" A 63 year old patient is diagnosed it! a myelodysplastic syndrome" An appro%ed drug

for treatment of t!is disease is:

A" Cyclop!osp!amide

" ?et!otre*ateC" o*oru&icin

" 5-aFacytidine

E" leomycin

D. is the correct answer. 'his was covered by Dr. Mega in the lecture on MD+.

However, @5a/acytidine was not listed on the pharmacology drug list.

7" A 5 year old man presents it! painless selling in !is nec" .!ysical e*amination isremara&le for a 2 cm cer%ical lymp! node @ splenomegaly" iopsy of t!e cer%ical node re%eals a

neoplasm containing small' clea%ed cells t!at recapitulate t!e normal follicular arc!itecture of

lymp! nodes" B!ic! of t!e folloing mec!anisms is most liely in%ol%ed in t!e de%elopment oft!is patient>s neoplasm

A" Amplification of -myc

" #omoFygous loss of p53C" %ere*pression of &cl-2

" .oint mutation in ras decreasing its H$.ase acti%ity

E" ,eciprocal translocation &eteen c!romosome 4 and 22"

14

Dropped


20/29


1" A 6 year old man presents it! progressi%e pruritic eryt!roderma' e*foliation' and

lymp!adenopat!y" E*amination of t!e perip!eral &lood smear re%eals lymp!ocyte-lie

cells it! cere&riform nuclei" $!ese cells are Cs lymp!oma" Se%eraldays after a treatment' s!e notices &lood in !er urine" B!ic! of t!e folloing

c!emot!erapeutic drugs is most liely responsi&le for t!is side effect

A" leomycin" Cisplatin

C" Cyclop!osp!amide

" o*oru&icin

E" /incristine

'he correct answer to this was -. -yclophosphamide, which causes hemorrhagic cystitis.

However this point was not emphasi/ed by Dr. 9a/o in his antineoplastics pharmacology

lecture, and it is clear that many of you answered ). -isplatinum because of its associated

renal failure. 'hough hematuria is not a typical presentation of cisplatinum renal toxicity, this

is not something you might (now. 'herefore we did accept answers ) and - for this 8uestion.

0$E: QIES$;0S 5- are related"

27


21/29


A 62 year old man presents to !is p!ysician for !is annual p!ysical e*am" #e is found to

!a%e an ele%ated !ite &lood cell count' it! a normal #& and platelet count" #is perip!eral

&lood smear is s!on in =igure 4"

5" B!at is t!e most liely diagnosis &ased on t!e presentation and perip!eral &lood smearA" C!ronic ymp!ocytic euemia

" #airy cell leuemiaC" 0on-#odgin>s lymp!oma

" ,ic!ter>s syndrome

E" SeFary syndrome

6" B!ic! of t!e folloing pattern of marers ould &e most consistent it! t!is diagnosis

A" C< and C3 positi%ity

" C5' C23' and C27 positi%ityC" C33 positi%ity

" $dt positi%ity

E" C14 positi%e' C27 positi%e' C5 negati%e

" B!ic! of t!e folloing statements a&out t!is disease is true

A" ;t is staged using t!e ,ai or inet Staging classifications"" $!is disorder arises solely from cells t!at !a%e unmutated ;g !ea%y c!ain %aria&le

region genes"

C" ;t is treated aggressi%ely using com&ination c!emot!erapy and ,itu*ima& it! an

aim for cure"" C!romosomal translocations are not found in t!is disorder"

E" .atients it! t!is disease in%aria&ly die from progression to acute leuemia"

" rug MsanctuariesN are potential pro&lems for curati%e treatment it! anticancer drugs"ne common p!ysiological cause for t!e de%elopment of drug sanctuaries is:

A" .rotein &inding"

" #ypot!ermiaC" Alopecia

" #ypo*ia

E" .ancytopenia

21

=igure

4"


22/29


4" An 7 year old male !as &een folloed for to years it! a !emoglo&in of 17' ?C/ 172'

&c 3777 it!


23/29


0$E: Questions 2 and 3 are related"

A 57 year old man presents it! fatigue" #e is found to !a%e a palpa&le spleen and a &c of 57'777'#& 4' and a platelet count of


24/29


5" ?et!otre*ate is one of t!e c!emot!erapeutic agents !ic! is acti%e against Acute ymp!ocytic

euemia" B!at is it>s mec!anism of action

A" $opoisomerase ;; in!i&itor"" Alylating agent"

C" locs tu&ulin polymeriFation"

" ;n!i&its ri&onuclotide reductase"E" ;n!i&its di!ydrofolate reductase"

6" A 65 year old man complains of eaness' eig!t loss' and &one pain" #e reports !a%ingprogressi%e difficulty seeing" .!ysical e*am is significant for !epatosplenomegaly and

lymp!adenopat!y" Serum protein electrop!oresis re%eals an ?-protein spie' it! an ele%ated

serum ;g? le%el" one marro aspiration s!os plasmacytoid lymp!ocytes" B!ic! of t!e

folloing is t!e most liely diagnosisA" #ea%y c!ain disease

" ?onoclonal gammopat!y of undetermined significance

C" ?ultiple ?yeloma

" .lasmacytomaE" Baldenstrom>s ?acroglo&ulinemia

0$E QIES$;0S and are related"

" A 7 year old man presents it! left sided a&dominal pain" #e is found to !a%e moderatesplenomegaly" CC: &c 7'777 it!


25/29


Melphalan and prednisone have been in use for ABC years. 'he other treatments are

all new therapies for MM.

47" A patient as recently diagnosed it! iffuse arge Cell ymp!oma" B!ic! of t!e

folloing is 0$ a significant prognostic factor in t!is disease

A" Age" Serum #

C" .erformance Status

" Hene e*pression profilingE" C!romosomal a&normalities

41" Anticancer drugs are freuently classified &y t!eir mec!anism of action"

Cyclop!osp!amide is an e*ample of aan:A" Alylating agents

" $opoisomerase ;; in!i&itor

C" Antimeta&olite

" Antisignaling agentE" ;ntercalating agent

42" B!ic! of t!e folloing statements is 0$ true regarding radiation &iology

A" $!e amount or uantity of radiation is e*pressed in terms of a&sor&ed dose"

" 1 Hray (Hy) is eui%alent to 177 rad"C" =ractionation gi%es normal cells a c!ance to repair during t!e time &eteen treatments"

" $!e most c!aracteristic let!al 0A lesion after a dose of ioniFing radiation is t!e single

&ase pair deletion"

E" Hreater doses of radiation are reuired under !ypo*ic conditions t!an in o*ic conditions tpro%ide eui%alent cell illing"

43" ;rradiation of ater can produce !armful products suc! as !ydro*ide radicals" f t!e totalcell eig!t' ater represents:

A" 5-D

" 22-2DC" 37-35D

" 57-55D

E" 7-5D

4


26/29


46" A 6 year old patient it! an < lytic lesion due to multiple myeloma is undergoing

radiation t!erapy" B!ic! of t!e folloing statements are true

A" =i&rosis may &e an acute to*icity t!at ill limit deli%ery of full doses of radiation"" Effects are most e%ident in rapidly di%iding tissues suc! as lung"

C" $o*icities do not resol%e' and are dose-limiting"

" A smaller dose per fraction causes more damage to normal tissue relati%e to tumor"E" ifferent dose fractionation sc!emes can result in a ider t!erapeutic indo"

4" A 55 year old man presents it! left upper uadrant a&dominal discomfort and fatigue" n e*am!e !as moderate splenomegaly and scattered petec!iae on t!e loer e*tremities" a&oratory

studies re%eal pancytopenia" An attempt at a &one marro aspirate as unsuccessful" ;f you are

a&le o&tain cells for analysis' t!e folloing ould confirm t!e diagnosis you suspect:

A" $!e presence of $-cell marers"" $!e presence of C33 and C13"

C" Cytoc!emical staining re%ealing resistance to $artrate Acid .!osp!atase"

" .resence of C37 and C15O"

E" .resence of Hp;;&;;;a"

4" B!ic! of t!e folloing lymp!omas is t!e only one to &e MgradedN !istopat!ologicallyA" iffuse arge Cell lymp!oma

" ?antle cell lymp!oma

C" ?arginal Fone lymp!oma" =ollicular lymp!oma

E" #odgin>s disease"

'his was covered by Dr. 4riffiths lecture. 1ollicular lymphoma is the only type

of lymphoma that is graded histologically. *m guessing that many of you

confused +taging and 4radingE 4rading is typically used to describe the degree

of differentiation of a tumor by histologic microscopic% examination. # 4rade *

tumor is typically well5differentiated. *n many tumor grading schemes, 4rades

*V5V are undifferentiated or anaplastic.

*n follicular lymphoma, grading is an indication of large cell component

remember that follicular lymphomas can transform into D9)-9 & a poor

prognosis event%. 1ollicular lymphomas are graded into three groups *, ** and

***. +tage *** has the highest large cell component and the highest li(elihood of

transformation.

F#s a reminder, +'#4*N4 refers to extent of spread of the primary tumorG.

Anser $rue or =alse to t!e folloing uestions (44-172):

44" ?antle cell lymp!oma is associated it! o%ere*pression of Cyclin 1"A" $rue

" =alse"

177" #;/-associated lymp!omas typically include follicular lymp!oma and S"A" $rue

" =alse

171" Adult $ Cell euemiaymp!oma is associated it! E/"

A" $rue" =alse

26

Dropped


27/29


172" ?arginal Gone lymp!omas !a%e an o%erall poor prognosis"

A" $rue

" =alse

173" B!ic! of t!e folloing is 0$ an accurate statement a&out treatment of =ollicular ymp!oma

A" ecause it is an indolent disease' Matc!ing and aitingN is an option"" ;t is typically cured it! standard c!emot!erapy agents"

C" =irst line treatment of pre%iously untreated patients may &e com&ination

c!emot!erapy (cyclop!osp!amide' %incristine' and prednisone) O ,itu*an"" ,adioimmunot!erapy it! tositumoma& (e**ar) and i&ritumoma& tiu*etan (Ge%alin)

are undergoing currently undergoing e%aluation for initial treatment of t!is disease"

E" .rogression to a more aggressi%e disease occurs at a rate of 5D per year

depending on degree of large cell component present"

17


28/29


A 6 year old c!ild presents it! a rapidly enlarging a&dominal mass and %isi&le ascites" ?om

says t!e c!ild only &ecame really sic in t!e last 3 ees" $!e &one marro &iopsy aspirate is

s!on in =igure 12"

17" $!is c!ild>s presentation and &one marro aspirate are most consistent it! a diagnosis of:

A" Acute ?yelogenous euemia (A?)

" uritt>s lymp!omaC" iffuse arge cell lymp!oma

" Bilm>s tumorE" 0euro&lastoma

174" B!ic! of t!e folloing is true of t!is diseaseA" $!is clinical picture of a rapidly groing a&dominal mass in a c!ild is t!e most

common presentation of t!is disease orld-ide"

" $!is is an indolent' or slo-groing disease"

C" $ingi&le-&ody macrop!ages are a prominent feature of t!e !istopat!ology"" $!is disease is not cura&le"

E" $!is disease is a mature' perip!eral $ cell malignancy"

117" $!e t!erapeutic action of imatini& (Hlee%ec) in%ol%es in!i&ition of:

A" ?icrotu&ule association

" $yrosine inase acti%ityC" $etra!ydrofolate uptae

" 0A polymerase acti%ity

E" ,0A polymerase acti%ity

111" A common side effect resulting from treatment it! eit!er cyclop!osp!amide or

do*oru&icin ould &e:

A" oss of taste sensation" toto*icity (ear to*icity)

C" ?yelosuppression

" ;ncreased appetiteE" 0ig!t &lindness

#DD!ND=M N0M#9 9#)0#'0: V#9=!+

B&c (cellsl)


29/29


11"5-15"5 (female)

#ematocrit (D)

hematology 2006

Documents