Hematology Exam 2

1. If you had a 2 year old and he had low H and H and sick prior to this... What does he have?

A. Transient erythroblastopenia. So rememebr that especially if viral problem.

2. What do you do for that?

A. Nothing

3. What is happening in this disorder?

A. Ab being used and its preventing EPO from doing what it should do

4. Does heparin have anything to do with vit K?

A. No

5. Starry sky?

A. Burkitts Lymphoma

6. Philadelphia chromosome

A. 22,9 translocation CML. Then CML goes to AML.

7. Should know and look over closely what cyclic neutropenia, something hagashi, and make sure you
understand them

8. Aids virus

A. RNA virus

9. What is cell that is hit by that?

A. CD4

10. Hemolytic uremia syndrome

A. E.coli. Hockey puck. Causes bleeding disorder.

11. Know cyclic neutropenia as said

A. Benign kid that presents with sores

12. What does VWF carry?

A. Factor 8

13. Do we have oncogene in us?

A. Yes and they are normal.

14. Benz jones protein is where?

A. Multiple Myeloma.

15. If PTT and bleeding time and PT are all prolonged, everything is prolonged, what is the probable dx?

A. DIC

16. If just PTT is prolonged, you have a deficiency of what possible factors?

A. 12,11,9,8. One of those 4 are missing

17. If just PT is prolonged?

A. Factor 7 or platelet factor 3

18. If both Pt and PTT

A. Factor 5,10,3,2,1 (down in the common pathway). Have to get this scheme down

19. Retinoblastoma

20. Philadelphia chromosome

21. Should know that factors 8 and 9 are inherited as

A. X-linked

22. V. Wildebrand is..

A. Autosomal dominant

23. 2 cancers that youa re repeatedly subjective to once you have CA and you get tx for that CA?

A. AML or NHL (acute myelogenous leukemia or non Hodgkins) are both associated with chemo

24. Know what vit k is used for

A. Hemolytic ds of the newborn

25. What facotrs are x linked or autosomal recessive

26. 2,7,9,10 are produced in the liver

27. Polycythemia vera

A. HA, blurred vision, BV broken in eyes. Hydroviscosity causes the HA. Volume is overloaded

28. Protamine sulfate counteracts

A. Heparin

29. Cyclic neutropenia

A. KOSTMANS. Inherited disorder and it gives you no nuetrophils. Incompatible with life and you last
 about 2 years from an infection. Very rare.

30. HSP should know that (purpura)

31. CML goes with philadelphia chromosme and it changes into AML

32. What cell matrues to plasma cells?

A. B-cells

33. ITP

A. Platelet problem. Takes place in spleen and wind up with petechia

34. DDAVP

35. What is cell in. Hodgkins disease?

A. Reed Sternberg cell

Heme Reviews

1. NOT phagocytes-- LYMPHOCTYES

2. Chediak-Higashi
- autosomal recessive

- giant granules
- neutro/thrombocytopenia

- partial albinism

3. Neutropenia ANC- AT LEAST 1500

4. Infectious Mononucleosis
- jaundice? YES

- test for syphilis positive? YES
- splenomegaly? YES

- ITP or low platelet count? YES can develop later on 2-3 months
-Mengitic picture? YES

5. what cell is involved in mono? MONOCYTE

6. Complement system-
-IgG and IgM DOES NOT activate it. they come out of it

7. AIDS virus -RNA

8. AIDS 2 proteins
- 1 reverse transcriptase
- 2 integrase

9. target cell that it attaches to in AIDS--- CD4

10. AIDS virus
- needles? YES
- semen? YES
- blood? YES
- shaking someones hand? NO

11. Complications of AIDS -- NOT RENAL CELL CARCINOMA
- shingles? YES
- kaposi sarcoma? YES
- TB? YES
- renal cell carcinoma? NO

12. How many drugs to make a cocktail? 3

13. Bence-Jones Protein in urine- MULTIPLE MYELOMA

14. Which leukemia do you offer a bone marrow transplant as soon as they go into remission? AML

15. What the lag phase in HIV? 3 MONTHS

16. Hemophilia A- whats prolonged? PTT IS PROLONGED

17. Von Willebrand disease--PTT abnormal, platelets can be affected, factor 8 is low (its being made it just doesn't have the protective
von willebrand factor to protect it.)

18. Reed-Sternberg cell (RS) --HODGKINS DISEASE

19. disorder where you get ulcerations of the mouth, anus in a 6-week period..every 5-6 weeks you get fevers--CYCLIC NEUTROPENIA

20. What cells produce immunoglobulins? B CELLS and PLASMA CELLS

21. what do B cells become? PLASMA CELLS

22. Philadelphia chromosomes? 9 and 22 CML (chronic myelogenous leukemia) A RECIPROCAL TRANSLOCATION BETWEEN
CHROMOSOME 9 AND 22.

23. Patient presents with rash on lower extremities (swelling of the ankle or knee) look like bruises, what would you want to know or
worry about? URINARY PROBLEMS AND/OR GI PROBLEMS (blood in urine or stool)

24. Pseudotumors with hemophilia? HEMOPHILIA A & B (not von willebrand)

25. What age does the ALL take place? children ( 4-6, 4-7, 3-5) NOT 14-15 YEAR OLDS

26. what is more common ALL or AML? ALL (L comes before M in the alphabet-L most common)

27. What are sanctuary in leukemia? where do they hide? TESTICLES AND CNS (ALWAYS LOOK AT CSF TO SEE IF THEY HAVE A
SANCUTARY)

28. Which leukemia undergoes the transformation of metamorphosis? CML (CML GOES TO AML)

29. starry - sky? whats the diagnosis? BURKITT'S LYMPHOMA

30. Osteolytic skeletal area or lytic bones lesions? whats the diagnosis? MULTIPLE MYELOMA

31. Any kind of leukemia- can you relapse? YES
could there be testicular relapse? YES
relapse good or poor prognosis? POOR
certain definitive time you treat? right away but go by Protocol. usually 2 - 2 1/2 YEARS could last longer so no really definitive time

32. Hemarthroses are in what disorder? HEMOPHILIA A & B THATS IT

33. can you get splenic rupture in infectious mono? YES
can it reoccur? YES 6 % RELAPSE
you can get hepatitis? YES
can you get encephalopathy? YES

34. KNOW PT/PTT SCHEMATIC

35. how can you treat ITP? immunoglobulins, RHOGAM "john answers then "BOOYA" ancona response "you talk about that as if you
were a defective product" OWNED

35. The complement system when it get done, what does it give you? IgG and IgM (ITS OPSOMIZING AND PUTTING IN BACTERIA
AND LYZING THE BAD CELLS- GETTING RID OF THEM)

36. What are the two most common types of cancers that you can develop after having cancer? at risk for NON-HODKINS LYMPHOMA
& AML

37. what the agent in HUS? E. COLI (CONTAIN A VEROTOXIN)

38. Von Willebrand factor- what does it attach to? FACTOR 8 (VIII)

39. Which Immunoglobulin comes from the gut? IgA

40. CAT-SCRATCH DISEASE??

41. If you have nodes in your neck and nodes axilla area..what is the stage of hodkins? STAGE 2 TWO SEPARATE AREAS ABOVE THE
DIAPHRAGM
what about if axillary and groin? STAGE 3 ONE ABOVE ONE BELOW DIAPHRAGM

42. KNOW PT/PTTs….HEMOPHILIA A &B, VON WILLEBRANDS, VIT K AND DIC!









what cell is not a phagocyte

LYMPHOCYTES

growth factors

what are the growth factors that the cells respond to?

what do we use them for?

something syndrome?

how is it inherited?

autosomal recessive - FATAL

neutropenia

what is the number the ANC has to be below?

1500

what is not true of mono?

B cells attacking T cells

aids

what is true?

ALL OF THE ABOVE

aids is transmitted every way except

hand shaking

secondary infection complication of aids except

renal cell carcinoma

bens-jones protein and other proteins associated with which disease?

multiple myeloma

??? after first remission

AML

lag phase in HIV?

2-3 mos

hemophilia a

factor 8 deficiency

PTT being infected

ONLY PTT IS PROLONGED

von wilibr???

attaches to factor 8

what is not true

PT IS PROLONGED

14 yo female - ulcerations of mouth and anal area

fever 102, occur frequently (about every 8 weeks)

what is likely diagnosis

cyclic neutropenia

immunoglobulins produced by what cell?

B cells

pseudotumors of long bones

what disease

hemophilia a and b

what is false about ALL?

incidence is 9-12 yo

philadelphia chromosome

chromosomes 9 and 22

everything about leukemia is true except

sanctuaries (they are in AML, not ALL)??

lytic bone lesions....and monoclonal proteins

seen in what disease?

multiple myeloma

23 male with history of 4 days fever, lethargy, stiff neck, etc.

PE - cervical lymphadenopathy

what test to diagnose?

 look for heterophile antibody

all are complications that may occur

acute transformation

if PTT and PT are both prolonged, which factor is most likely to be affected?

factor II

ITP can be treated in which way

all of the statements are correct

HSP has all following except

E.coli verotoxin

which statement is true of non-hodgkins

predominately a disease of B cells

von w attaches to factor XIII

which immunoglobulin is produced by the gut?

IgA

mass on left side of neck - inc. gradually in size

nodes and herpes zoster

Reed-stern cell

hodgkins

what stage is pt?

stage 1a

mix and match

PTT and bleeding time are all prolonged - common pathway

which is in common pathway? vit k deficiency

PT is prolonged, PTT and bleeding are normal - 8, 9, 11, 12 - 8 is answer

23 yo sore throat - abx - tired

next step?

EBV titer

most pts with this require

nothing

cause of death can occur with

splenic rupture and ICH can occur

MC type of childhood leukemia

ALL

MC preventitive measure for something with uric acid

adf

mode of inheritance for factor XIII

sex linked

what factors are produced in liver

2, 7, 9, 10

also need vit k

most sensitive test for DIC

split products

vit k

used in everything except

neutralizing heparin

most likely diagnosis in septic pt that is bleeding

DIC

factors that affect prognosis in hodgkins

ALL OF THE ABOVE

chemotherapy used in hodgkins in all stages except

stage 1a

polycytenia

everything seen except

hypotension

protamine sulfate

inactivates heparin

HIV infects which cells

 CD4 - t helper cells

what is true about multiple myeloma

radiographs show bone lesions

45 yo female

LUQ pain, fatigue, weight loss

philadelphia chromosome

CML

78 yo male - progressive headaches, weakness, dizziness

polycythemia vera

what can we use to treat von w disease type 1

PDADP

14 yo female - adbdominal pain, diarrhea

HUS

4 yo male - intermittent fevers, pallor, enlarged cervical lymph nodes, splenomegaly

initial lab tests?

CBC with differential

painless swelling of cervical and inguinal nodes for 4months

hodgkins

test to prove diagnosis?

lymph node biopsy

65 yo male, bacterial pneumonia, fatigue, weight loss, nodes, etc.

CLL

true or false

false

HIV is RNA? true

more survivors for AML than ALL? false

ENV, GAG and ??? in AIDS virus?? true

protamine sulfate used for vit k? false

29 yo female -

HSP

what quesiton do you want to know?

abdominal pain and blood in urine

von w is carrier for what factor?

factor XIII

oncogenes

normal genes present in everyone

pt has leukemia in TOTAL REMISSION

what do you think leukemia cell population will be?

10^5

most sensitive test to detect DIC

fibrin split products

hemorrhagic disease of newborn is not secondary to what factor?

12

complications of BMT include all except

inc risk of splenic rupture

Burkitt

B cell

non hodgkin

same staging but not same histologically as hodgkins

CMV is a complication of infections commonly seen in transplants? true

christmas disease - factor 9

50 questions (end of review); White blood cells to the end

Clotting cascade slide

- Intrinsic pathway - PTT

- Extrinsic pathway - PT

Fibrinolysis - degradation products - D-Dimer

- Hemophilia

- vWF - platelet problems;

- Hematologic malig's - Diploid, Hap, Aneu, Hyperdiploid, Hyplo, etc

- Oncogenes; Normal? Yes

- Retinoblastoma; Suppressor genes (look thru pupil and its white; will
lose eyeball) - kids are missing BOTH tumor suppressor genes

- Philadelphia chromosome (swap - 9 & 22)!!!!!

- Leukemia; relapse does not mean new disease = same disease, need
new treatment....as the previous doesn't work!

- 3 drug cancer treatments; wanting to hit the cancer cells at all phases

- sex linked; autosomal dominant; autosomal recessive is NOT sex linked

- chemo leaves patients receptive to AML or Non-Hodgkin's lymphoma

(no matter what the previous cancer was)

- HIV is an RNA Virus; CD4 cells effected by HIV; Karposi's sarcoma

- treating of leukemia creates uric acid....want to use allopurinol to treat

the kidney's and not allow the ua to accumulate

- CASE; Reed-Sternberg cells = Hodgkins; Starry night slide = Burkett's

- Know the hodgkin's stages - ie: stage 4 because of jaundice, enlarged

organs, etc; Positive Heterophile = Mononucleosis

- 2 year old case; nothing wrong, just red cells are low; nothing abnormal
= red cell aplasia; transient - leave it alone!!

- one stage in hodgkin's where no chemo is used: stage 1 = radiation

only!!!!!

- Heparin: IV only; halflife = 1 hour; Inactivated in the Liver; Nothing to do

with Vit. K

- CML: acute transformation into AML (very bad); CML has a great
response to tx (phila chromo - 9 to 22); Priaprism in it can present with
just that.....

- staging of lymph nodes: one region = stage 1; two regions = stage 2

(NO crossing of the diaphragm); two regions with crossing the diaphragm
= stage 3, etc..........hodgkins and non-hodgkins

- no bone marrow on ITB

- BM on ITP? No; They do it, but shouldnt

- Hemolytic uremic syndrome; low platelet count with bleeding through

the gut (HUS); ICU patients, etc....is NOT confined to just one spot;
Global; virotoxin that can go anywhere

- IV Ig at these patients

- cyclic neutropenia - up and down cycles of white count and infections

occur; limited thing - hormone offered to these patients to keep their
WBC up; Poetin; self injectable

- mult myeloma = Bence jones protein

- Vonwillebrand factor is attached to factor 8

- Pseudotumors are associated with: 8 or 9

- henloch scherline purpura - rash from buttocks down; chest down; after
dx, you want to hear = want to make sure not bleeding into the gut and
kidneys; check stool

- Plasma cells are NOT seen on BM, or in Peripheral; They are only found
in tissue

- IgA is in the mucosa of the intestine and gut; Payer's patches

- Polycythemia; primary vs secondary

HEME TEST2 REVIEW 
‐ Lytic bone lesions on 60 yr old man ‐= multiple myeloma  
‐ If you have 4 yr old that’s tired, fatigued, with bruising, bone pain, neutropenia = leukemia  
○ Think ALL because its more common, always consider it before AML  
‐ Adult female 45yo with LUQ pain (location of spleen), weight loss, philadelphia chromosome = leukemia  
○ This one is CML  
○ AML is the worst in kids, CML is worst in adults  
‐ Cervical adenopathy on R side, negative monospot (for infxious mono), node biopsy shows sternberg reed 
cells)  
○ Hodgkins 
○ Stage 1 because only one lyph node involved  
○ Treatment = radiation  
‐ 70‐80 yo with headaches, dizziness, weakness, pruritis splenomegaly, HTN, red facies, no lymphadenopathy, 
Hb 19, Hct 65, WBC elevated at 12, platelets elevated at 600  
○ Polycythemia vera  
‐ Factor 7,5,2,10,9 ‐‐> which is not vitK dependent  
○ Factor 5  
‐ What is not decreased in DIC ‐ factor 5, platelets, fibrinogen, split products  
○ Split products (this is what happens as a result of fibrin degradation)  
‐ Pt with thrombocytopenia ‐‐> what is impt in hisotyr ‐ HIV, chemo/radiation, liver disease, bleeding 
○ All of the above  
‐ Type 1 von willibrands disease ‐ you might be able to use what instead of factors?  
○ DDAVP?  
‐ Hemophilia B ‐ what factor is involved  
○ Factor 9  
‐ Teenager complaining of abd cramps, diarrhea 4 days, ate a lot of hamburgers, developed ecchymosis and 
petechiae ‐ what is it   
○ HUS  
‐ 28 yo woman with URI a month ago with bloody nose, petechiae. No fever. Bleeds when brushes teeth. No 
enlarged spleen. Platelets low, high megakaryocytes. What is it  
○ ITP  
‐ 65 yo male with pneumonia in past yr, has fatigue, weight loss, fevers over last months. PE shows 
hepatosplenomegaly, lymphadenopathy. Few platelets, high WBC. What is it  
○ CLL  
‐ Pt has painless lymphadenopathy of neck going on for months. Fever, chills, weight loss, fatigue. Whats most 
definitive test ot make diagnosis ‐ CXR, CBC, CT, biopsy  
○ Take biopsy  
‐ Reed sternberg cell ‐ know it  
‐ Pt with ITP ‐ what treatments can you use ‐ nothing if it’s a kid, can give IVIBG???, prednisone, or ??. Wont do it 
for the adult because its chronic. You'll probably take out the spleen more quickly, and you'll put them on suppressants  
‐ What can cause death in ITP  
○ Intracranial bleeds ‐‐> But they're rare  
‐ What is another cause of death in ITP  
○ Spleen rupture (bleeding from ruptured enlarged spleen)  
‐ MC type of childhood leukemia = ALL  
‐ Whats most effective way to treat uric acid formation  
○ Allopurinol  
‐ How do you inherit hemophilia 
 ○ Sex linked  
‐ Hypoprothrombinemia (AKA factor 2), factor 9 deficiency, factor 10 deficiency, factor 5 deficiency ‐ which one 
of these conditions will PTT be prolonged  
○ All of the above  
‐ vitK is effective in all of THESE conditions except  
○ Hypoprothrombinemia ‐ YES 
○ Neutralizing heparin ‐ NO 
○ Counteract warfarin ‐ YES  
○ Hemolytic disease of newborn ‐ YES  
‐ What factors produced in liver out of these ‐ 7,5,2,9  
○ Factor 5 is NOT produced in the liver, all the others are  
‐ Most sensitive test for DIC is what  
○ PT, PTT, and thrombin time can all be used for many things  
○ Split products is the most sensitive test for DIC  
‐ Most likely diagnosis for bleeding septic pt  
○ DIC  
‐ Philadelphia Chromosome is what disease  
○ CML  
‐ Stage 3s ‐ what disease is this?  
○ AKA stage 3 with spleen involvement (that’s the s)  
○ Hodgkins  
‐ M1 classification is what disease  
○ AML  
‐ Ben jones protein is what disease  
○ Multiple myeloma  
‐ Plastin transformation is what? Metamorphosis is what?  
○ CML that goes into AML?  
‐ Von willibrands disease is what sort of inheritance  
○ Autosomal dominant  
‐ Burkitts lymphoma  
○ Is a B cell lymphoma  
‐ Factor 8 is what type of inheritence  
‐ Hodgkins is what?  
○ Reed sternberg  
‐ CMV complications in what type of ppl  
○ Immunocomp 
‐ AML ‐ what percentage of leukemia in kids  
○ 15%  
‐ Cancer pts have chromosomal breaks, translocations, rearrangements. Why do we look for them?  
○ Because tells us severity of disorder and gives us clues of how we should treat them  
‐ Whats another name for factor 9  
○ Christmas disease  
‐ 14 yo with oral ulcerations, blisters, pus on tonsils, 102 fever, has it every 8‐10 weeks  
○ Shedeyak nagashi syndorme, pelvic hewitt, cyclic neutropenia?? 
○ Its cyclic neutropenia  
‐ Pt with 4 day history of ankle swelling, purpuric rash on lower extremities and buttocks ‐ what do we ask ‐ how 
long her fever was, chest pain, seizures, chest pain, palpitations, how long she's been sombulent, presence of abd pain 
and dark urine  
 ○ Presence of abd pain and dark urine ‐‐> we're worried about henloch schonlein purpura (we're worried 
about her kidney and gut bleeding)  
‐ If you have pseudotumors on toes, fingers, what disease is this 
○ Hemophilia A and B, NOT in von willibrands 
‐ Von willibrands factor is a carrier of what  
○ Factor 8  
‐ Oncogenes ‐ whats true about protooncogenes ‐ are they mutated genes, are they only present in cancer, do 
they only occur because of chemo, are they target genes we use for radiation, or normal genes in all of us  
○ Normal in all of us  
‐ Spleen in ITP ‐ is it always palpable?  
○ No. its always enlarged, but not always enough to be felt  
‐ AIDs is what sort of virus  
○ RNA   
‐ More survivors of AML or ALL  
○ ALL  
‐ If there is an env, gag, and pol gene? Is what virus  
○ Aids virus  
‐ Protamine sulfate is used to counteract  
○ Heparin  
‐ You can see all in polycytemia except ‐ polyviscosity, hypervolemia, headaches, blurry vision, injected 
conjunctiva, hypotension, gout  
○ hypotension  
‐ HIV infects  
○ CD4 cells  
‐ Which neutrophilic disorder is inherited ‐ Kossmans, cyclic neutropenia, drug induced neutropathic 
○ Kossmans ‐ inherited recessive trait, fatal   
‐