Path Winter 5 Wbc

Front 1

whats the normal

WBC
PMN
EO
Baso
Lymph

Back 1

WBC- 5-10,000
PMN- 1500-6500
EO- 0-500
Baso 0-200
Mono 1-600
Lymph- 1200-3400

Front 2

what is granulocytopenia
what is the numebr

Back 2

granulocytes are PMN, EO, and baso.

often just refers to PMN bc the other are in such low numbers

<1500

aka:
neutropenia
agranulocytosis

Front 3

what can cause granulocytopenia

Back 3

1. Ineffective granulopoiesis: decreased myeloid stem cells, drugs, DNA defects

2. Decreased Production: myelopthisic anemia- Cancer, infection, Leukemia, lymphoma, Gauchers
, Aplastic anemia, large granular lymphocytic leukemia

3. Destruction/Removal: SLE, drug reaction, splenic sequestration, infections

Front 4

what is granulocytopenia, what else is it called

Back 4

low PMN (granulocytes)

Neutrophilia <1500
Agranulocytosis <500

<1500

Front 5

what is leukopenia, what 2 general things cause it

Back 5

decreased peripheral WBC

1. Granulocytopenia (low PMN)
2. Lymphopenia (bc of HIV or drugs)

Front 6

in general granulocytopenia is low PMN and can be caused by decreased production or increased destruciton. waht are some specific causes

Back 6

1. Myelopthisic Anemia: this gives a pancytopenia (Cancer, Infection in BM)

2. Aplastic Anemia: stem cell failure, also pancytopenia

3. Large Granular Lymphocytic Leukemia: pancytopenia, neoplastic cells suppresses BM

4. B12/Folate deficit: megaloblastic anemia

5. Myelodysplastic syndromes:

5. SLE

7. Splenic sequestrayion

8. Bacterial infection

9. Drugs/EtOH: most common!

10. Acute Leukemia. myelopthisic disease

Front 7

if you have a pneumonia wht can happen to PMN

Back 7

increased removal --> neutropenia!

<1500
left shift (more bands)

Front 8

what is the most common reason a person will have LOW PMN

Back 8

drugs

ex chemo, immunologic, idiosynchratic, EtOH

Front 9

what is the clinical course of someone with decreased PMN (neutorpenia, granulocytopenia)

Back 9

Agranulocytic Angina

**get ulcers in mucosa (mouth is common)

**risk of deep bacterial/fungal infection. aspergillious, candida

Front 10

if what numbers are low can you get oral (mucosal) ulcers and increased risk for deep bacterial and fungal infection

Back 10

neutropenia

Front 11

At age 6, Amber was treated for acute leukemia.
During course of therapy, she developed a
sinus/facial infection with fungus
(probable Aspergillus)
Extensive surgery required to excise necrotic
infected tissue including Amber’s eye
Aspergillus invades the walls of blood vessels in
severely neutropenic patients; causes tissue necrosis
Why does Amber have neutropenia?

Back 11

1. leukemia decreases PMN
2. chemo drugs kill PMN

**

Front 12

whats the deal

WBC 3000
Hb 7
Hct 22 (normal is 40-50)
MCV 122
Platelets 90,000

Back 12

WBC low
Anemic
Platelets low

**combination called pancytopenia

Front 13

whats pan cytopeni

Back 13

low plate, PMN, and anemia

thrombocytopenia, neutropenia, anemia

Front 14

if we are missing B12 and folate is the only thing we get megaloblastic anemia

Back 14

nope, ALL lines are decreased. will get a leukopenia

Front 15

if you see decreased WBC, Anemia and low platelets what do you do?

whats the ddx

Back 15

call hematologist, pancytopenia

1. Acute leukemia
2. myelophthisic anemia
3. Aplastic anemia
4. myelodysplastic syndrome

Front 16

what do lymphocytes do in pancytopenia

Back 16

NOTHING, lymph have nothing to do with pancyto (thrombopenia, anemia, neutropenia)

Front 17

whats hte ddx for pancytopenia

Back 17

1. acute leukemia
2. aplastic anemia
3. myelopthesic anemia
4. myelodysplastic syndrome

Front 18

Patient is a 33 y/o accompanied to the ER in an
ambulance by her 13 year old daughter. She
is unarousable and unable to give a history.
• Her daughter relates that she has been getting
progressively weaker for the past few days and
has not been out of bed for the past 24 hours.
She occasionally retches and has been vomiting
for 2 days
• Daughter relates a history of ETOH abuse and
smoking tobacco.

Hb 10
Hct 34 (low)
MCV high (103)
Plate 1800
Lymph 60
3nRBC/100WBC

what is a common cause of these findings?
what fo test should be done?

Back 18

macrocytic anemia and thrombocytopenic,

LYMPHOPENIC (recall this doesnt mean pancytopenia)- no lymphs means:
1. HIV
2. Drugs

Front 19

what numbers define lymphocytopenia

Back 19

when absolute is <1200 adults or <3000 in children

1. malnutrition
2. HIV
3. Corticosteroids
4. TB
5. Radiation, SLE

Front 20

what will malnutrition do to CBC, what else will cause the same thing

Back 20

NO lymphs! <1200 adult or <3000 kid

also caused by:
1. HIV, congenital immune deficit
2. corticosteroids
3. TB
4. Radiation, SLE

Front 21

what is reactive leukocytosis

Back 21

1. reactive means its inflammatory

>10,000 WBC in circulation

increased PMN common for adults
increased lymph common for kids

Front 22

what do corticosteroids do to:

1. Lymphs
2. PMN

What do glucocorticoids do to Eosinophiles

Back 22

1. decrease lpmyhs
2. increase PMN (prevent PMN from entering tissue so it stays in periphery)

Glucocorticoids will decrease Eisionophiles (given to treat Asthma which has high IgE/eosionophiles)

Front 23

why might one have neutrophilia

Back 23

1. infection (bacteria)
2. sterile inflammation/necrosis/burns
3. glucocorticoids- keeps PMN in circulation and prevent them from entering tissue
4. exercise
5. catecholamines

Front 24

ok so we know and MI will increase PMN, as will exercise and catecholamines. what cytokines cause more PMN to pour out of the BM?

what makes you INCREASE production (rather than just push them out)

Back 24

TNFa, IL1

**will see a fast reaponse, incerased bands. we pull from BM when there is infection, sterile inflammation (MI), or endotoxin

((MAKE more we need GF and its a slow response

Front 25

with severe inflammation we know we have increased PMN, do they look different

Back 25

Toxic Granulation: dark staining

Dohle Bodies: blue stained bits of ER

will also see cytoplasmic vacuoles

Front 26

when and where do we see Dohle bodies

Back 26

they are dilated ER that stain blue, seen when there in an infection that increases PMN counts

**will also have toxic granules (dark staining) and cytoplasmic vacuoles

Front 27

what PMN changes are seen with severe inflammation

Back 27

1. increased numbers
2. Dohle body- dilated ER, stains blue
3. Toxic granulation- dark staining
4. vacuolization

Front 28

what is leukemoid reaction
what is left shift

Back 28

1. Leukemoid reaction- granulocyte counts >50,000 (recall really low granulocyte counts was agranulocytosis)

2. left shift- too many bands

Front 29

if we see a peripheral smear and we see WAY too many PMN what might we call it

Back 29

neutrophilia but also leukemoid reaction

recall leukemoid reaction is >50,000! TONS

**typically with leukemoid we also see increased band cells

Front 30

A 61 y/o woman presents to the emergency
department with fever, rigors and tachycardia.
Blood cultures demonstrate gram negative bacilli
identified as Enterobacter cloacae.
Review pictured smear. What leukocytes
are present?
What is her total WBC count likely to be
what cytokines are active

Back 30

1. gram (-) can be endotoxin, this causes INCERASE in PMN

will have left shift, lots of bands (also have dohle body, vacuouls and toxic granulation)

increased WBC- leukemoid reaction

IL1, IL6, TNF, CSF

Front 31

The patient is a 55‐year‐old white man who
presents to the emergency room complaining
of chest pain and labored breathing. His
temperature is 98.6°F. He is overweight and
smokes two packs of cigarettes a day. His
EKG demonstrates ST segment elevation. Labs
include increased CK‐MB and troponin
compatible with an acute myocardial infarction

His complete blood count (CBC) shows:
Hemoglobin (Hb) 13 gm/dL (nl 13.7‐16)
White blood cells (WBC) 15,000/mm3
(nl 5,000‐10,000)
Platelets (Plt) 350,000/mm3 (nl 150,000‐450,000)
Differential of white cell count
80% neutrophils (PMNs) 10% band neutrophils
9% lymphs; 1% monos
What does the neutrophil count indicate?
• At what stage of development are granulocytes
released into peripheral blood?
• What are the mechanisms underlying
neutrophilia?
• What is “left shift”
• What is the significance of blasts on CBC

Back 31

WBC are high normal is 5-10,000
this is a L shift neutropenia- this is bc of the necrosis associated with MI

released normally as a seg, released early as a band when the BM is stim to push them out fast

Front 32

what is the leukoerythroblastic reaction? what usually causes it

Back 32

nRBC in periphery. you should NEVER see this. also will see immature PMN with it

caused by these space occupying leisions:
malignancy
myeloproliforative disorder
granulomas
gaucher disease

Front 33

what space occupying leision in the BM will cause leukoerythroblastic reaction

Back 33

nRBC in periphery

1. primary/mets cancer
2. myeloproliferative disorder
3. granulomas

Front 34

A 71 y/o woman with history of chronic
respiratory disease with wheezing presented
with productive cough, fever and shortness of
breath that began 4 days earlier.
• The WBC was 18,400 (nl 5,000‐ 10,000)
with 38% eosinophils, 48% neutrophils,
4% monos, 10% lymphs
• What abnormality of the CBC is present?
• What is the likely cause?
• What is the mechanism of WBC change?

Back 34

WBC- leukocytosis
EO- HIGH

Caused by IgE- asthma, allergy, worm

IL5 drives eosinophiles, treat with glucocorticoids

Front 35

an asthmatic will have what increased? what IL stim it? how can we decrease it

Back 35

Eosinophiles, IgE
stim by IL5
Eosinophiles decrease with glucocotricoid
Lymphocytes decrease
PMN increase

Front 36

What are the causes of eosinophilia?

Recognize morphology and breakdown products. What is the mechanism of eosinophilia

Back 36

Type I Hs reactions
-asthma, allergies, atopic dermatitis, hay fever

EO increase is driven by IL5

charcot leyden crystal is the remnant

treat with cortocosteroid

Front 37

charcol leyden crystal

Back 37

Eosinophilia remnant

*eo increase bc of allergic, type I HS reaction,

Front 38

what are hte causes of monocytosis

Back 38

1. TB, cocci, maleria
2. Subacute bacterial endocarditis
3. SLE, RA
4. IBS

**any granulomatous disease causes monocytosis
**recall in inflammation the first responder is PMN but then we get monocytes

Front 39

58 yo female with...

WBC 14600
Hb 10
Hct 31
MCV 103
Plate 30,000

Patient seen for fatigue and concern regarding mild
scleral icterus and bruising.
Bilirubin 2 4 dL (normal < 1 2) with indirect
67
2.4 mg/1.2) bilirubin 2 mg/dL. DAT positive

PMN 2628
Lymph 10950
Mono 6%
Eos 1%
3+ macrocytes with polychromasia

Back 39

WBC high, increased biliruben bc of hemolysis

macro with polychromasia: RETICULOCYTES

Front 40

An 18 y/o male presented with 3 day history
of fever, sore throat and neck swelling.
• His temperature was 39.5oC, (103oF), throat
was erthythematous with enlarged tonsils
covered by white exudates. Cervical lymph
nodes were enlarged, shotty and tender with
a 3x4 cm right posterior cervical node.
• Throat swab was negative for group A
B‐ hemolytic streptococcus

24 hours later, he returned with a maculopapular
rash over the whole body.
• WBC 13,500 (normal 5,000‐10,000)
 78% lymphocytes, 15 neutrophils, 7 monos
 50% reactive (“atypical”) WHAT ARE THESE lymphocytes
• Platelets 150,000
• He was seen 3 days later. The rash had faded.
The spleen was palpable 2 cm below costal
margin. Diagnosis

Back 40

if its not strep its mono

Atypical lymphs are CTL T8 cells that go after viral infection. not unique to mono, any viral infection

Front 41

what are the causes of lymphocytosis
what is atypical lymphocytosis- what do they look like

Back 41

1. Lymphocytosis: viral infection, chronic lymphocytic leukemia

2. Atypical Lymphs: CTL T8 cells seen in any viral infection. old condensed chromatin, WAY too much cytoplasm. get a ballerina skirt

Front 42

what cell has a ballerina skirt

Back 42

atypical lymphs associated with mono (its the CTL T8 cells)

Front 43

what are the 2 things that cause lymphocytosis

Back 43

viral infections (reactive lymphs, indicate viral infection)

chronic lymphocytic leukemia

Front 44

atypical lymphs are what, what is another name for them

Back 44

the ones that have ballarina skirts and indicate viral infection

also called REACTIVE, activated

Front 45

Are reactive lymphocytes specific for infectious mononucleosis?

• What is the nature of reactive lymphocytes in infectious mononucleosis?

What are the diagnostic possibilities in a patient p with IM like disease who lacks heterophile antibodies

• What hematologic conditions other than lymphocytosis may be assoc with IM?

• What are the serologic findings allowing a diagnosis of infectious mononucleosis

Back 45

nope, any viral infection (also called reactive or active)

CD8 T cytotoxic

Mono- monospot, heterophile AB

if its not mono it can be cold hemolysins disease, DAT, IgM or autoimmune thrombocytopenia

Front 46

when do you see lymphocytosis with reactive or atypical lymphs? what if you find that its NOT due to one of these causes

Back 46

1. mono
2. CMV
3. toxoplasmosis
4. Viral hepatitis

Persistant lymphocytosis >4000 in adults: work
patient up for lymphoproliferative disorders
(malignancy of lymphocytes)
‐ Chronic lymphocytic leukemia– most common

Front 47

why might you have nucleated RBC in the peripheral smear (3)

Back 47

1. anemia- severe, <5 Hb
2. after splenectomy
3. BM replacement- (leukoerythroblastic)

Front 48

what are my lymphoid cells?
what are my myeloid cells?

Back 48

LYMPHOID: B, T, NK

MYELOID: all others, RBC, plate, eos, baso

Front 49

what are the lymphoid neoplasms

what are the myeloid

Back 49

Lymphoid- B, T, NK, lymphocytic leukemia/lymphoma

Myeloid- acute myeloid leukemia, myelodysplasia, chronic myeloproliforative disease

Front 50

what is often the underlying cause of a WBC neoplasm

Back 50

genetic issues

chromosomal tranlocation
AG R rearrangement errors

Front 51

in general what are hte clinical sx of Acute leukemia and whats the lab finding associated with it (ALL and AML)

Back 51

1. fatigue, anemia
2. never, PMN
3. petechiea, thrombocytopenia
4. painful bones, hypercellular BM
5. CNS sx, meningeal involvment, CNS bleeding

Front 52

A five y/o boy has been too tired to play with
his friends for 2 months. His mother is worried that, whenever he , falls or bumps into anything, a big bruise forms. For the past 2 days he has had a fever A CBC is ordered

WBC 2300 (low)
Hb 9 (low)
Hct 28 (low)
MCV 95
Platet 74,000 (low)

what does this mean, what test

A bone marrow was performed.
• There was marrow replacement by primitive
cells that have large nuclei with delicate
chromatin and indistinct nucleoli. There is scant
cytoplasm with no granules and no Auer rods.
These cells mark for CD10 (CALLA) and TdT.
• What is the most likely diagnosis?
• What is the most likely outcome of this child’s
disease with standard therapy?

Back 52

anemia, thrombocytopenic, leukopenic= pancytopenia

get a BM for pancytopenia

Dx: ALL (young, TdT, CALLA,

Outcome: 90% remission :) good, hyperploidy is good, B is good, young is good)

Front 53

what is ALL (Acute Lymphocytic Leukemia)

1. Age
2. Molecular
3. Prognostic markers/Prognosis

Back 53

1. Age: 5-15

2. Molecular: TdT, PAS+, CALLA, can have CD<10 if T- mediasteinal mass

3. Prognostic markers/Prognosis:
Good: hyperploidy, young, preB
BAD: t9:22, older, preT (mediasteinal compression)

90% remission

Front 54

what cancer affects young kids and it reliably cured 90%

Back 54

Acuke Lymphocytic Leukemia (can be B or T, B is better prognosis)

*look for CALLA

Front 55

what is seen in the CBC for a kid will Acute Leukemia

Back 55

Pancytopenic so CBC:
anemia
thrombocytopenic
leukopenic
BLASTS are seen

Front 56

whats the BM for Acute leukemia

Back 56

hypercellular, >20% blasts. primitive cells with large nuclei and nucleoli`

Front 57

what is leukemia
what is lymphoma

Back 57

Leukemia: lymphocyte cancer in the BM

Lymphoma: lymph nodes/extra nodal tissue outside BM

Front 58

A kid is anemic, thrombocytopenic and neutropenic. You do a BM and realize this 5 yo has ALL and can see nucleoli. what are the markers for B adn T involvements

Back 58

B- better prognosis, more common. CALLA (CD10), TdT, CD19

T- TdT, CD any less than 10. worse prognosis

Front 59

whats the most common cancer in kids

Back 59

ALL

5 yo, male >female
B is better prognosis, T is worse t9:22
cure is like 90%

Front 60

what are the clinical and lab features and prognosis of pre T lympboblastic neoplasia, is it leukemia or lymphoma

Back 60

**typically LYMPHOMA! mediastinal mass involving thymus- respiratory sx (can progress to leukemic)

**common in little white boys
**worse probnosis than b

(ALL)

Front 61

what leukemia has lots of blasts in BM and primitive cells with nucleili and delicate chromatin

Back 61

ALL

TdT + blasts on smear also

young kids get it, common, get better

will have CALLA- CD10 or CD19

Front 62

in what leukemia is t9:22 seen, is this good

Back 62

ALL, BAD marker
other bads: being older than 15, T cell lineage

Good: hyperploidy, young, B cells affected.

90% remission :)

Front 63

in ALL is the BM hypercellular or hypocellular

Back 63

HYPER! bone is tender. lots of blasts

**neoplastic blasts accumulate in BM and suppress normal hematopoiesis by crowding out the normal cells *myelopthisis*
this causes peripheral blood to be...1. anemic, 2. neutropenic, 3. thrombocytopenic

Front 64

what are hte sx of the Acute leukemias (ALL, AML)

Back 64

abrupt onset
Fatigue, anemia
fever/infection, neutropenia
bleeding, ecchymosese, thrombocytopenia

Bone pain there are tons of leukemic infiltrates packing into BM and kicking out the normal cells -- pancytopenia. the BM is hypercellular. Nucleoli, delicate chromatin

Front 65

in what acute leukemia do you see

1. hepatosplenomegaly
2. mediastinal mass
3. CNS/testicular infiltrates

Back 65

ALL for all.

mediasteinal if T cells are affected (TdT, CD <10_

Front 66

A 7 y/o boy presents to your office c/o easy
fatigability. g y His mother also tells you that she
has noticed that his gums bleed when he brushes
his teeth. There are no specific abnormalities on
physical examination
Hb 10.5 gm/dL 11.5‐14
WBC 75,000 μL 5,000‐10,000
• Chest x ray mediastinal mass You order
, /μ
Platelets 25,000 / μL 150,000‐450,000
x‐shows a mass. bone marrow biopsy and mediastinal biopsy

BM shows blasts

What stain/assay would be helpful in distinguishing
atypical lymphocytes from lymphoblasts?
• What is the diagnosis?
• What methods are available for distinguishing
between the types of acute leukemia?
• What lab tests aid in establishing the prognosis
• Does this patient have a leukemia or a lymphoma

Back 66

gums bleed- thrombocytopenia
anemic
leukophillic

1. stain- TdT on blasts only
2. Dx: ALL, t involvement
3. distinguish ALL- young. AML- older, auer rods
4. determine prognosis with translocation. t9:22 is bad
5. both leukemia nad lymphoma. the BM is blasts and the thymus is affected

Front 67

AML

1. Age
2. Molecular
3. Prognosis

Back 67

1. older 15-39 (ALL was <15)

2. Myeloperoxidase +, auer rods

3. Prognosis:
BAD- development of MDS, got it bc of therapy

tx with retinoic acid, BM is curative, 60% remission and 15-30% cure

Front 68

aeur rods and myeloperoxidase

TdT, CALLA,

Back 68

AML

ALL

Front 69

This 30 y/o male had noticed progressive
weakness for 1 month. On physical examination,
the tip of the spleen was palpable. There was
also sternal tenderness.
WBC 67,000 5,000‐10,000
Hb 10.2 gm 13.5‐16
Hct 30.6% 42‐50
• Blasts with cytoplasmic granules and Auer rods
Platelets 36,000 150,000‐450,000
were noted on the peripheral smear.

Back 69

Hb/Hct- anemic
Platelets- low

BLASTS- means leukemia til proven otherwise, auer rods are AML

Front 70

this is a cancer that ppl get when they are 30 (15-40). There are blasts that accumulate in BM and cause pancytopenia. Often this is due to a translocation. What do you think it is. how can we be sure

Back 70

acute MYELOGENOUS leukemia

**auer rod and myeloperoxidase +

**affects BM most

Front 71

what is the prognosis of the different classificaitons of AML

Back 71

1. AML with myelodysplasia like features is a poor prognosis. 5q 7q 20a aberrations

2. AML that is related to therapy is VERY poor prognosis

Front 72

AML classification
1. M0
2. M1
3. M2
4. M3

whats most aggressive, whats most common

Back 72

classificaiton depends on maturation of PMN

M0- CD34, minimal differntiated. MOST aggressove

M1- AML w/o maturation. myeloperoxidase +, few auer rods

M2- AML- blasts with auer rods, myeloperoxidase +. MOST COMMON

M3- DIC, t15:17, myeloperoxidase +, auer rods, treat with retinoic acid to prevent DIC

Front 73

what type of AML (classification) can cause DIC but is treated with retinoid acid to prevent DIC

Back 73

M3

this is called acute promyelocytic leukemia)

Front 74

Acute promyelocytic leukemia (m3, AML)

clinical
lab

Back 74

auer rods, can have DIC adn bleed.

Front 75

A 40‐y/o male presented to the emergency
department with severe hematochezia. On
questioning, he reported the gradual onset of
fatigue over the last several weeks prior to the
bleeding, as well as the appearance of a “rash”
the day before.
On PE, there were numerous petechiae; pallor;
tachycardia tachypnea and orthrostatic
hypotension. CBC and other labs were ordered

Hemoglobin (Hb) 6.5 g/dL (13.5‐ 16)
• Hematocrit (Hct)17.9% (42‐50)
• White Blood Cells (WBC) 2,200/μL (>5000)
• Platelets (Plt) 4,000 μL/ (150,000‐450,000)
• Prothrombin time (PT)14.6 sec (11 – 12)
• Partial thromboplastin time (PTT) 42.4 sec (<32)
• Fibrinogen 118 mg/dL (ref. range 190 ‐ 400)
• D‐dimer 7.8 μg/mL (< 0.5)

Peripheral blood smear revealed 11% abnormal
immature cells with prominent cytoplasmic
granules and Auer rods.
A bone marrow aspirate and biopsy were
subsequently performed.
• What is the diagnosis in this case?
• What associated syndrome is indicated by the clinical presentation and laboratory data, and how does it relate to the patient's primary diagnosis?

Back 75

auer rods- AML

AML- M3 classification, acute promyelotic leukemia

DIC- treat with retinoic acid

Front 76

DIC can be associated with what leukemia

Back 76

AML- M3

treat with retinoic acid

Front 77

What is the molecular abnormality associated with the t(15;17), and how does it relate to disease pathogenesis and treatment?

• What is the primary criterion for a diagnosis of acute leukemia?

• What are Auer rods, and what is their
significance?

• Why is it necessary to distinguish between ALL and AML?

Back 77

t15:17- translocation seen in AML- the promyelocytic type, M3

dx acute leukemia with >20% blasts in BM

auer rods- fused granules, ID AML

AML and ALL have dif therapy

Front 78

26 y/o male with M1 (AML) diagnosed in January. Chromosomes
unfavorable; no remission; palliation only. Headache; cranial
palsies in Dec.; blasts in CSF. Visual loss in April- retinal hemorrhages
WBC 195,000, 99% blasts, platelets 20,000. Died in Aug.

Back 78

AML- age, super high blasts

Front 79

what are the common labs with AML

Back 79

anemia, Hb, Hct decreased
thrombocytopenia, platelts low
WBC- varies
Blasts in peripheral smear

myeloperoxidase +, Cd34, CD33 (TdT and CALLI are on ALL)

Front 80

whats teh prognosis of AML?

tx

Back 80

60% remission, 15-30% cure

poor prognosis when you are older than 50

Tx with retinoic acid, BM transplant, combo chemo

Front 81

what are the common labs with AML

Back 81

anemia, Hb, Hct decreased
thrombocytopenia, platelts low
WBC- varies
Blasts in peripheral smear

myeloperoxidase +, Cd34, CD33 (TdT and CALLI are on ALL)

Front 82

whats teh prognosis of AML?

tx

Back 82

60% remission, 15-30% cure

poor prognosis when you are older than 50

Tx with retinoic acid, BM transplant, combo chemo

Front 83

what is CLL

clinical
labs
treatment
prognosis

Back 83

1. clinical, super common, older folks, M more than women. enlarged LN

2. lymphocytosis, small lymphs, anemia/thrombocytopenia due to autoimmunity in late disease. smudge cells

3. treatment:

Front 84

what is the morph of CLL

Back 84

smudge cells

Front 85

can you do elective surgery with acute leukemia, wht about chronic

Back 85

acute- no
chronic- ya

Front 86

This 65 y/o male was in good health except for
mild hypertension. At check‐up, a CBC showed
a markedly elevated WBC. Physical exam
revealed slightly enlarged lymph nodes in
the neck and the axillae. Spleen was palpable.
Labs
WBC 130,000 /μL 5,000‐10,000
Hb 12.8 gm 13.7‐16 gm
Platelets 330,000 150,000‐450,000
90% small uniform lymphs and smudge cells

whats the results of CBC, what are some key features in the vingnettet. wahts the prognosis

Back 86

older male, enlarged nodes, large spleen. and smudge cells

CBC: leukocytosis, anemic, thrombocytopenic +/-

CLL- chronic lymphocytic leukemia

**mild treatment. ZAP - is better prognosis (hypermutated receptor)

Front 87

WHAT IS THE MOST common adult leukemia in the US

Back 87

CLL

>50
M>F
enlarged lymph nodes
lymphocytosis (small cells)

SMUDGE cells
zap - is better prognosis

Front 88

whats the relationship btwn CLL nad SLL

Back 88

PERIPHERAL B CELL CANCER

CLL- chronic lymphocytic leukemia: peripheral blood lymphocytosis is predominate

SLL- small lymphocytic lymphoma: when large LN are the predominate.

**super common. older ppl, M more than women

**good prognosis

Front 89

the B cell markers on ALL were CD19 CD20 and calla, what are they for CLL

Back 89

CD19 Cd20 CD5

Front 90

zap 70

Back 90

ZAP +: poor prognosis in CLL

ZAP -: better prognosis in CLL, hypermutated heavy chain

Front 91

does the spleen and liver enlarge in CLL

Back 91

yes

**this is the common one in old males in US. it makes LN large but there are lots of small b cells (CD19, CD20, CD5) if you have ZAP its a worse prognosis than ZAP negative. tx is gentle

Front 92

ok so there are LOTS of lymphs and they may be a little small but the giveaway is the smudge cell. wahts the...

1. DDX
2. CBC
3. prognosis
4. affected?
5

Back 92

1. CLL- SMUDGE
2. CBC- lymphocytosis, can get some autoimmune anemia/thrombocytopenia
3. ZAP+ bad prognosis. ZAP - is good prognosis (hypermutated receptor)
4. older men

Front 93

58 y/o female seen in ER with petechiae, ecchymoses

WBC 14,600 /μL 5000‐10,000
Hb 10 g /dL 13.5‐16
Hct 31.3%
MCV 103 80‐100
Platelets 13,000 /μL 150 ,000‐450,000
Diff: 20% neutrophils ; 75% lymphs, 5% monos
• 3+macrocytosis with polychromasia
• What additional lab tests should be ordered?

Chemistry profile: ALT and alkaline
phosphatase p p normal; LDH and bilirubin elevated
• Haptoglobin decreased
• Direct antihuman globulin (coombs test)
positive
• What pathologic process is present?
• What is the underlying cause?

Back 93

petecheia, ecchymosis- bleeder. PTT, PT,

**biliruben increased, haptoglobin decreased

Pathology- autoimmune hemolysis
Cause: CLL

**pt appears well but the CLL shows on CBC as leukocytosis and +/- thrmbocytopenia/anemia

Front 94

CML

clinical
Lab/CBC
smear
lab test

treatment
prognosis

Back 94

1. any age, common myeloproliforative disease, splenomegaly

2. granulocytosis with left shift (even baso and eos are increased) anemia, +/- platelets

3. t 9:22, BCR:ABL

4.

5. LAP is low

6. treat: long remissions with RTK inhibitor, BMT is cure

7. survive 3 years w/o therapy

Front 95

This 52 y/o male had gradually increasing fatigue g together with discomfort in the left upper quadrant. Physical exam revealed an easily palpable spleen and liver edge.

• WBC 46,000 Platelets 754,000
• Hb 13.2gm Hct 39.6%
• Diff: segs 46%, bands 11, metas 10, myelos 3,
pros 2, eos 9, monos 2 , lymphs 17


1. dx
2. CBC
3. key things from case
4. what will the genes/ID agenst be

Back 95

1. CML- chronic myelogenous leukemia

2. Leukocytosis- left shift, thrombocytosis, anemia

3. any age, splenomegaly, leukocytosis with left shift, anemic. plates will be up or down

**LAP low, t 9:22, BCR ABL

Front 96

what is the msot common chronic myeloproliforative disease

Back 96

CML

**any age, splenomegaly, granulocytosis with L shift (even eos and baso increased) anemia, t9:22, BCR ABL. Long remission when treated with RTK inhibitor

Front 97

BCR able
t 9:22

Back 97

CML- LAP also low

**9:22 translocation also seen in ALL and is a BAD prognostic marker

Front 98

what do we see on CBC for CML

Back 98

anemia
platelets do thier one thing
GRANULOCYTOSIS WITH LEFT SHIFT, PMN are always increased
LAP- leukocyte alkaline phosphatase is decreased
HYpercellular BM with <20 blasts

Front 99

if your BM is hypercellular with MORE than 20% blasts is it chronic or acute leukemia

Back 99

acute

chronic- less than 20% but still hypercellular

Front 100

are leukocytoes ALWAYS increased in chronic leukemia

Back 100

yes! and hte BM is hypercellular with less than 20% blasts

contract to acute leukemia: WBC can be up down or normal and BM is hypercellular with more than 20% blasts

Front 101

in what leukemia will imatinib be useful

Back 101

CML- the RTK inhibitor turns off the signaling

Front 102

This 44 y/o woman c/o 2 month history of
fatigue, weight g , g loss and a 2 wk. history of low
grade fever, early satiety, and LUQ pain. She
had no palpable lymphadenopathy but
splenomegaly is noted.
• WBC count is 225,000/mm3 (5,000‐10,000)
and consists mostly of granulocytes in various
stages of maturation and myelocytes,
myeloblasts, basophils and eosinophils

What is the reason for this patient's early satiety
and for her night sweats, fever, and weight loss?

what will cytogenics show

Back 102

splenomegaly, leukocytosis, granulocytosis,

splenomegaly- causes fever, night sweats, early satiety

CML!!
BCR:ABL, t9:22

**tx with RTK inhibitor like imatinib

LAP is low

Front 103

This 44 y/o woman c/o 2 month history of
fatigue, weight g , g loss and a 2 wk. history of low
grade fever, early satiety, and LUQ pain. She
had no palpable lymphadenopathy but
splenomegaly is noted.
• WBC count is 225,000/mm3 (5,000‐10,000)
and consists mostly of granulocytes in various
stages of maturation and myelocytes,
myeloblasts, basophils and eosinophils.

CML

4 years later
• Patient c/o bone pain, weight loss, night sweats
and fever and early satiety. On physical exam,
massive splenomegaly and lymphadenopathy.
• Immature cells in the peripheral smear are
predominantly lymphoblasts of B lineage by
flow cytometry: CD10 (CALLA positive); TdT
positive; CD19 positive; surface immunoglobulin
negative; and negative CD2, CD7. Immunoglobulin gene rearrangement by
Southern blot demonstrates clonality in this
specimen but not the specimen from 4 yrs. ago.
• Southern blot analysis both samples contain the
same BCR/ABL chromosome abnormalities.
Additional chromosomal abnormalities are seen
in the second sample

Is this second presentation a new leukemia or
does this leukemia arise from a pre‐existing
malignancy?
• Diagnosis?

Back 103

CALLA
TdT

Looks like ALL

**CML can progress into ALL or AML

Front 104

whats hairy cell leukemia

clinical
lab
morph
immunophenotypic

Back 104

1. splenomagale, LN normal.
2. Pancytopenia
3. TRAP stain to see the cells

**common to get an atypical mycobacterial infection

Front 105

A 41 y/o man has a 5 month history of
fatigue and persistent fever. On physical
examination, he has marked splenomegaly
but no lymphadenopathy. CBC reveals that
he is pancytopenic.
Peripheral smear is pictured. Right side is stained by tartrate‐resistant acid
phosphatase.

What is your diagnosis?

What is the immunophenotype of these
cells?
• What could his fever represent?
• What is his prognosis

Back 105

hairy cell leukemia

B cell luekemia so: Cd19, CD20 always. CD11 CD22


Prognosis: long term survival is GREAT
fever- common to have atypical mycobacterial infections

TRAP stain

Front 106

A 49 y/o male c/o dyspnea on exertion for 1
mon. It has worsened over the past 2 days.
He has had 2 episodes of epistaxis and
increasing abdominal distension w/o pain.
No history of infection or weight loss.
• Skin and conjunctiva pale; abdomen distended;
tachycardia of 120/min.
• CT of abdomen reveals massive splenomegaly
of 26 x 15 x 35 cm.

• Hb 6 g/dl (nl 13.5‐ 16)
• Hct 21%
• Reticulocytes 2.7%
• WBC 2400 (5,000‐10,000); Differential:
11% segs; 61% lymphs and 6% mononuclear
cells uncertain etiology, 22% monos.
• Platelets are 21,000/uL (150,000‐450,000)
• Chemistry profile and coagulation tests are
normal.

Flow cytometry: CD19 CD
20 CD11 CD22

whats the dx?
how might you stain the cells

Back 106

pancytopenia: anemia, neutropenia, thrombocytopenia

Hairy cell leukemia
TRAP stain

Patient treated with cladribine (2‐CdA),
epoietin and filgrastrim.
• The spleen shrunk to 10 x 12 x 24 cm and Hb
and platelet count returned to normal.

Front 107

whats the leukopeia

pancytopenia, splenomegaly, indolent, good treatment, CD 11 CD22, atypical mycobacteria infection

Back 107

Hairy cell

Front 108

what does myelodysplastic syndrome mean

Back 108

pre leukemia of hte myeloid line

Front 109

what is myelodysplastic syndrome?
How is it different from Myelodproliforative?

Back 109

Myelodysplastic: pre cancer, stem cell problems mean there is ineffective erythropoiesis, not enough cells in periphery, cytopenia. progress to AML

Myeloproliforative: too many cells in the blood

Front 110

what is the natrual history of MDS (myelodysplastic syndrome)

Back 110

can become acute leukemia

**recall myelodysplastic is where there is a stem cell disorder that leads to ineffective hematopoiesis which leads to cytopenia and increased risk of AML

Front 111

who might have an increased risk of AML, and some cytopenias bc they have a stem cell problem that gives ineffective erythropoiesis (ie MDS)

what is the cayse

Back 111

more likely in older ppl, more common than Acute leukemia in this patient age group

M>F, insidious

Cause: insidious common, therapy related, genetic stem cell damage

Front 112

what are some morphologies of MDS (myelodysplastic syndrome)

BM
Peripheral

Back 112

MDS- inefective hematopoiesis, increased risk of AML

BM: Hypercellular, megaloblastoid maturation, ringed sideroblasts


Peripheral Blood: pancytopenia, MCV increased, pseudo-Pelger-Huet cells (binucleate granulocytes)

Front 113

if you see pleger huet cells in the peripheral smear what might you find inthe BM

Back 113

Pleger Huet- common in myelodysplasia, its a binucleate granuloctye

BM: hypocellular, ringed siderophages, megaloblastoid maturation (dysplastic magakaryocyte)

**recall MDS is bad stem cells that make hematopoiesis ineffective. we get pancytopenia and increased risk for AML

Front 114

when is this seen: Bone marrow aspiration:
Megaloblastoid dyserythropoiesis

what else is seen in BM

what is seen on peripheral

Back 114

myelodysplasia

also see:
dysplastic megakaryocyte
ringes siderophages- Fe, prussian blue

Peripheral blood: binucleate granulocytes called Pelger Huet

Front 115

what is 5 q syndrome

Back 115

its seen in older women with MDS (remeber there were stem cell defects so no hematopoiesis, and there were lots of karyotypes)

there is megaloblastic anemia with ringed sideroblasts, platelets are normal and have a GOOD PROGNOSIS

Front 116

whats the prognosis and tx of myelodysplastic syndrome (MDS)

Back 116

refractory anemia, up to 5 year surviva, but usually a 1-2 year survival

**die due to:
Acute leukemia
PMN low- infection
Bleeding- thrombocytopenia

Front 117

what is myeloproliforative disorder (MPD)

Back 117

Multipotent myeloid clonal transformation (CML is pluirpotent)

*hypercellular BM bc of increased hematopoiesis
**splenomegaly
**splenomegaly- extramudullary hematopoiesis

Front 118

what are some common type of MPD

Back 118

CML
Polycythemia vera
thrombocytothemia
primary myelofibrosis

Front 119

A 50 y/o woman has purpura, headaches, and abdominal fullness. Her BP is 160/85 and is slightly blue. Overall she looks bloated (plethoric)

Her hemoglobin is
20g/dL (nl 12‐15.6),
platelet count is 550,000/μL
(150,000‐450,000) and WBC 31,000/μL (5,000‐10,000).
Abdominal CT shows massive splenomegaly

What is the Dx?
Explain the CT finding.
What complications may occur?

Back 119

Hgb is HIGH- polycythemia
Platel high
WBC high

MPD- polycythemia vera (EPO normal, get proliforation bc of JAK mutation)

**worry that with the granulocytosis you have increased basos- their histamine can cause itching, and peptic ulceration

**risk for both bleeding and thromboses

Front 120

polycythemia

1. pathogenesis
2. labs
3. clinical

Back 120

1. pathogenesis: JAK mutation leads to constant erythroid production, EPO is NOT high

2. labs- Hgb/WBC/Plate all are high (polycythemia, granulocytosis, thrombocytosis) rich thick blood

3. clinical: splenomegaly, hypercellular BM. older ppl. excess body fluid (plethoric) cyanotic, HTN, HA, Histmince from basophils makes you itch and peptic ulcers and skin ulcers

Front 121

which MPD comes about bc of JAK mutation?

who might we see this in, what is their clinical presentaiton

Back 121

polycythemia vera- EPO independent increase in RBC production, also get granulocytosis and thrombocytosis. its a really rich thick blood

seen in older ppl, they have excess body fluid (plethoric) and are cyanotic. splenomegaly, HTN, HA, Histamine from excess basophils leads to peptic ulcers and puritis. skin ulcers

Front 122

ok so polyvythemia vera is an increase in RBC and you get thick rich blood. what is the risk of bleeding

Back 122

increased bc of stagnant flow- and you have lots of platelets so can also thombose

Front 123

how is polycythemia treated

Back 123

you bleed them!

**the problem is lots of RBC production bc of a JAK mutation (NOT EPO) so get rid of some of the blood

**keep in mind with PVC you have increased risk of BOTH bleeding and thrombosing

Front 124

whats the lab in polycythemia vera

Hgb
Hct
Plasma volume
WBC
Platelets
LAP
EPO
other

Back 124

Hgb high
Hct high
Plasma volume high
WBC high
Platelets high
LAP normal (low in CML, another MPD)
EPO LOW
other: JAK mutation

Front 125

what is essential thrombocytosis

Back 125

its a myeloproliforative disorder

its high platlets with no cause (not a JAK mutation as in PCV)

*dx of exclusion, reactive thrombocytosis is in the ddx

**can live a long time with this

Front 126

what are some common causes of reactive thrombocytosis

Back 126

common!

**its anything that will increase acute phase protein: fever, bleed, surgery, infection/inflammation, Fe deficit anemia, solid tumors

Front 127

describe primary myelofibrosis (its a myeloproliforative disease)

clinical
lab
morphology

Back 127

fibrosis of BM bc there are cancer megakaryocytes that release PDGF and TGFb which are profibrosis

1. Clinical: extramedularry hematopoeisis, splenomegaly, fatigue, weight loss, night sweats, lots of infections, bleeding, can turn to AML

2. Lab

3. Morphology: nRBC, tear drop, Howell Jolly body

Front 128

EW is an 88 y/o female admitted for SOB and
dependent edema of 2‐3 days duration.
• She has a history of CHF and was hospitalized
for that diagnosis 2 years earlier. She has been
transfused every 2‐3 months for the past 2 years.
Blood in stool led to endoscopy
during which angiodysplasia (fragile blood vessels)
of the stomach were found.
• She has a decreased appetite due to early satiety
• She reports a 35# wt. loss in 1 year to 105#.
One month earlier, she was transfused with 4
units of blood. BP 132/57; heart rate 102
• Hypoxia with pO2 of 62; 90% sat.
WBC 5680 /μL 5,000‐10,000
Hb 8.6 gm / dL 13‐16
Hct 28.3 %
MCV 83.3 80‐100
Platelets 528,000 150,000‐450,000
Smear morphology: 1 NRBC/100 WBC; many teardrop RBCs; occasional Howell-Jolly bodies
giant platelets

She was seen by a hematologist who found that
the spleen was massively enlarged 14cm below
the costal margin and extending to the midline;
the liver was enlarged 8cm below the costal
margin.
• LDH 475 U/L (100‐195) Uric acid 6.7 (1.5‐6.5)
• What is the significance of clinical and lab
findings?
• What test is indicated and what is the likely
morphologic finding?

DX

Back 128

early satiety, teardrop RBC- spleen
anemic
thrombocytitic!

LDH- necrosis
Uric Acid- hematologic turnover

Lab- spleen, thrombocytosis
Do a BM: nRBC, howell jolly, tear drop, anemia, Leukoerythroblastosis

PRIMARY MYELOFIBROSIS

Front 129

A 38 y/o painter has a 2 mon. history of
worsening headaches, fatigue and abdominal pain. On exam, he has gray discoloration at the gingival margin of his teeth. Hemoglobin is 9 gm/dL (nl 13.7‐17); MCV 67 (80‐100); serum
creatinine is 3 mg/dL (nl <1.2). Occasional normoblasts with basophilic stippling (pictured) Are seen in the peripheral smear.

To what environmental toxin has he been exposed?
What accounts for the increased creatinine?
What findings might appear in a similarly exposed child?

Back 129

smear- nRBC with stippiling
**inhibit Fe from getting into hemme. inhibits ferrochelatase and ALAdehydrotase

1. Pb- lead based paint!! Pb- PlumBer

2. Renal injury

3. Kids- brain affected, bones affected more

Front 130

who is at risk for Pb exposure

Back 130

1. painter
2. plumber
3. battery makers
4. radiator repair
5. Air
6. water- plumbing
7. lead glazed ceramics
8. food, toys, candy wrapper
9. gun ppl

**the "natropaths" use this as tx
**store it in bone/teeth, 30 year half life

Front 131

what is the pathogenesis of Pb (lead) toxicity

Back 131

inhibits ferrochelatase: and gamma ALA dehydrogenase: now fe and be added to heme

interferes with epiphyseal remodling in kids, wont let fractures heal

Front 132

what inhibits gALA dehydrase adn ferrochelatase

Back 132

Lead- messes with Ca so kids growth plates affected and inhibits healing of fractures

**we get RBC hemolysis, renal damage and HTN

Front 133

what enzymes in heme synthesis are blocked by Pb

Back 133

1. gamma ALA dehydrase
2. Ferrochelatase

Front 134

what are the consequences of Pb exposure

Back 134

Brain, Blood, Bone all bc of Ca

Anemia- hypochromic, microcytic (Fe deficit bc the enzymes are blocked)
Basophillic stippling

*basophilic stippling (hypochromic microcytic anemia, looks like Fe dificit anemia.. BC IT IS! recall Fe cant be inforporated into heme)

Front 135

13 month old Bianca Mesa was found tobe to have a microcytic anemic during a regular checkup.

1. What are the causes of microcytic
anemia?

Serum iron was increased. Blood lead level was elevated.


• State health department worker helped parents identify the 15‐piece set of clay‐based traditional pottery from which the child had been fed.

Back 135

1. causes of microcytic anemia: Fe deficit, chronic disease, a/b thalassemia, sideroblastic anemia

in this case it was Pb exposure

Front 136

consequence of Pb exposure

1. Blood
2. Brain (adult and kid)
3. Peripheral Nerves
4. GI
5. ABD
6. Reproductive
7. bones

Back 136

1. Blood: microcytic anemia, basophillic anemia
2. Brain (adult and kid) : adult, HA, memnor loss. Kid- retard, encephalopathy
3. Peripheral Nerves- demyelin
4. GI- renal, HTN
5. ABD- pain, anorexia
6. Reproductive- delayed puberty, infertility
7. bones- epuphyseal plates bad, delayed healing of fracutres

Front 137

in what mcrocytic anemia is the TIBC (transferrin)increased

Back 137

total iron binding capcaity is INCREASED in Fe defict anemia

**Fe is deficit so we have a higher capacity to bind in all other microcytic anemias (Chronic disease, thalassemai, sideroblast) its decreased or normal

Front 138

for Fe deficit anemia what is

1. Serum Fe
2. TIBC
3. % saturation
4. Ferririn
5. "other"

Back 138

1. Serum Fe: down
2. TIBC: up (the only one)
3. % saturation: down
4. Ferririn: down
5. "other": down marrow Fe

**Chronic disease is the same but a DECREAESD TIBC, and INCREAED FERRITIN. Chronic disease increases Fe

Front 139

for a and b thal what is

1. Serum Fe
2. TIBC
3. % saturation
4. Ferririn
5. "other"

Back 139

1. Serum Fe N
2. TIBC N
3. % saturation N to high
4. Ferririn: N to high
5. "other"

Front 140

what is Fe like for sideroblastic anemia or Pb

1. Serum Fe
2. TIBC
3. % saturation
4. Ferririn
5. "other"

Back 140

1. Serum Fe up
2. TIBC down
3. % saturation up
4. Ferririn up
5. "other"ringed sideroblasts, basophilic stippling

Front 141

Langerhans Cell Histocytoses

1. Morphology
EM
Molecular

Back 141

immature dendritic cells that proliforate

BIRNECK granules @ EM
CD1a

*can have acute disseminated LCH called Letteerer swie disease where lots of organs are infiltrated with langerhans
OR
*can have eosionophilic granuloma with only a few organ systems affected

Front 142

what features are assoicated with acute disseminated LCH

Back 142

this is a category of langerhans histocytosis

**multisystem langerhan cell infiltrates
**eosionophilic granuloma

Front 143

when do you see Birbeck granules, what marker will it have

Back 143

langerhan cell histiocytosis

CD1a

Front 144

A 6 mon.‐old boy presented with an nonhealing
diaper rash of 2 mon. duration. Rash persisted
and
spread despite aggressive topical therapy. Fever
And increased fussiness developed.
PE: red, seborrhea‐like eruptions of diaper area,
Lower abdomen (pictured) and scaling dermatitis Of scalp, axilla and postauricular area. Abdominal Distension with marked heptosplenomegaly and Bilateral inguinal lymphadenopathy noted.

Back 144

liver and spleen are HUGE

Langerhans cell histiocytosis

Lots of Eos and birbeck granules

Front 145

what is eos granuloma
where are they found
what do they do

Back 145

langerhans with eos, lymphs, plasma, and PMN

found in bone, medullary cavity, ribs, femur
also have leision in skin, sotmach, lungs of smokers

**common in kids, regress on their own or need to be cut out. good prognosis

Front 146

whats dyscrasia

Back 146

abnormal material in blood

plasma cell neoplasm

Front 147

what is monoclonal gammapathy

Back 147

its M protein, NOT the same as IgM

seen in plasma cell neoplasm

Front 148

what is the defining feature of plasma cell neoplasm

what are the common B cell ones:

Back 148

**terminally differentiated B cells secrete monoclongal Ig, excess heavy or light chains can also be produced


MM, lymphoplasmacytic lymphoma, heavy chain disease, primary immunocyte associated amyloidosis, Monoclonal Gammopathy of Undetermied Significance (MGUS)
Common: follicular, large B, MGUS

Front 149

Case History # 24 A 69 year old female was admitted with
intractable low back pain, lethargy and confusion
Albumin 2.7 (3.5‐6)
Uric Ac 8.5 (2.2‐6.7)
T.Prot 10 (6‐8.5)
LDH 238 (<190)
Ca++ 13 (8.5‐10‐5)
Phos 4.1 (2.5‐4.5)
BUN 42 (7‐25) T.Bili 0.8 (<1.2)
Creat 2.3 (<1.2) Chol 145
WBC 4000 (5000‐10,000)
Hb 10.2 (13.5‐ 16gm)
Hct 30.6%
MCV 106 (80 100)
230
80‐Platelets 97,000 (150,000‐450,000)

Peripheral smear shows Rouleaux formation

Diff: 54% segs, 2 bands, 1 meta, 33 lymphs
 6 monos, 3 eos, 1 baso
• 1+ aniso, 1+ micro, rouleaux formation
• What is rouleaux? Is it the same as agglutination?
• What is the diagnosis?
• How is the diagnosis made?

Back 149

Ca is high
kidney is affected
macrocytic anemia

Rouleaux- RBC stick together bc they are not being repelled by albumin or there is TONS of Ig from plasma cells

Dx: multiple myeloma, confirm with BM

Front 150

in what disease do you see rouleaux?

Back 150

RBC stacked like pancakes bc of TONS of Ig, in MM

**Not the same as agglutinin- this required AB

Front 151

what is the pathogenesis of MM

Back 151

common, plasma cells in BM makes nodules

1. normal
2. infection or inflammation (HIV, osteomylitis, RA) paired with some genetic instability --->
3. BAD NEWS! there is monoclonal gammapathy of Uncertain significance
4. PLasma cells secrete IL6 and others to activate osteoclasts
5 leads to bone destriction and WAY to many Ig- thich serum, proteinuria-renal damage, suppression of normal humoral immunity- risk of inection

Front 152

in MM there is a single cell in a predisposed pt (HIV, RA, osteomylitis, genetic, radiation etc) who gets a genetic instability, what does this lead to

Back 152

MGUS- monoclonal gammaopathy of uncertain significance

**this is a PREcancer can turn into MM (or not) and secrete IL6 and others. leads to bone resorption and TONS og Ig (lots of Ig means- thick serum, renal dalage- too much protein, suppression of normal humeral immuniety)

Front 153

what does MM look like

bone
kidney
BM
systemic
late disease

Back 153

bone punch-outs

**IL6- osteolytic activity: vertebrae, ribs, SKULL. Bone pain HYPERCALCEMIA

KIDNEY: bence Jones light chain, clogs the pee so you get casts in teh pee

BM: Russel bodies, inclusions of Ig in plasma cells. Plasmacytosis

AMYLOID: congo red

Plasma cell Leukemia: plasma cell in the periphery, shoudl NEVER see this. ?

Front 154

who gets bence Jones? Is it the same person that gets russel

Back 154

YES! MM for both- the B cell neoplasms that punches your bones out

**the kidney gets clogged bc you make TONS of Ig- this excess protein is damaging

**the BM is making TONS of plasma cells and they get RUSSEL BODIES- inclusions of Ig in the malignant plasma cell (Mott cell)

Front 155

whats a Russel Body

Back 155

in MM its a plasma cell in the BM with Ig inclusions bc its trying to make SO MANY Ig

In MM we will have bone punch outs. Bence Jones in kidney, Russel in BM Mott cell (malignant plasma) and amyloidosis

Front 156

whats a Mott cell

Back 156

malignant plasma cell in the BM (seen in MM) it has the Russel body Ig inclusion

Front 157

Be able to recognoze a Mott Cell with Russel body

Back 157

malignant plasma cell with Ig inclusions seen in MM

**really blueish/purpleish and can he binucleate

Front 158

clinical features of MM

Back 158

*really common BM tumor

1. oler ppl
2. black ppl
3. can be from MGUS
4. Bone pain- low back, fracture
5. Hypercalcemia- confusion weakness
6. infections- too many Ig of one type, the body thinks you have lots so other B cells dont make AB
7. renal- bence jones
8. amyloid
9. hyperviscocity
10. Rouleaux RBC

Front 159

how to make Dx of MM

Back 159

1 M protein on electrophoresis- lots og IgG, IgD, light chain (NOT IgM)

2. Bence Jones in pee

3. Rouleaux RBC in peripheral smear

Officially:
plasmacytosis in B< >10%
monoclonal AB in serum or pee
CRAB- hypercalcemia, renal insuffiency, anemai, bone leision

Front 160

whats a CRAB?

Back 160

MM- your bones HURT

C- hyperCALCEMIA
R- Renal Insifficiency- Bence Jones
A- Anemia (reauleaux)
B- Bone leision

**for MM dx you need:
BM plasmacytosis >10%
Monoclonal AB
CRAB

Front 161

• A 69 y/o female was admitted with intractable low
back pain, lethargy and confusion.
• Chemistries T. Prot 10 H (6‐8.5) Ca++13 H (8.5‐10.5)
• Creat 2.3 H (<1.2)
• Hb 10.2 (12‐14gm); rouleaux formation

Dx

How is Dx made

Back 161

Dx: MM- bone pain, confision from hypercalacemia, anemic, renal problems. ROULEAUX (other buzzwords- bence jones, russel body)

Dx is made based on:
1. Protein electrophoresis M proteion. Lots of monoclonal AB (IgG often)
2. BM biopsy- russel body in Mott cell
3. at least one CRAB- hypercalcemia, renal insuffiency (bence jones), anemia, Bone leision

Front 162

A 76 y/o female presents with fracture of
her right arm without trauma.
• Two years earlier, she suffered from
compression fractures of the vertebra with
lytic lesions on x‐ray.
• Bone marrow demonstrated 36% atypical
plasma cells. There was Bence Jones
proteinuria and IgG paraproteinemia of
plasma.
Diagnosis? What supports this diagnosis?

Back 162

MM

Old,

compression fracture- lytic bone problems (IL6 osteoclast activity)

Atypical Plasma cells- Mott cell with russel body

Bence Jones- renal insuffiency

IgG

Front 163

when does solitary plasmacytoma predispose for MM, what are the locations

Back 163

Solitary Plasmacytoma- localaized plasma tumor

**can happen in BM and get MM in like 10 years

Front 164

whats MGUS (monoclonal gammapathy uncertain significance)

Back 164

its when you have M proteion but NO sx of MM (No CRAB)

**its a precancer that can turn into MM

Front 165

72 year old woman hospitalized because of
easy fatigability and blue‐red fingertips. No
history of malar rash, solar‐induced rash.
• On physical examination, she exhibits mild
palor and questionable scleral icterus.
• Lab:
• Hemoglobin 8.2 gm
• Reticulocyte count 5.2% ( normal ~ 1%).
• Positive direct antihuman globulin (Coombs) test.

Back 165

Raynauds phenomena- cold agglutinin disease

Front 166

what is the neoplasm with monoclonal IgM

Back 166

Lymphoplastic Lymphoma- indolent

when you secrete lots of IgM its thick adn you get hyperviscossity called Waldenstroms macroglobulinemia

**IgM is super thick! its NOT MM, recall that was IgG. also no renal failure or amyloid like was seen in MM

LN are involved
Autoimmune hemolyitc anemia due to cold agglutinin *IgM

Front 167

whats waldenstroms macroglobuinemia

Back 167

its the super thick serum you get bc of IgM proliforation. Lymphoplasmacytic lymphoma

LN are involved

Autoimmune hemolytic anemia due to cold agglutinin disease (IgM)

bleeding
cryoglobulinemia: ranauds

Front 168

72 year old woman hospitalized because of easy
fatigability and blue‐red fingertips. Hb 8.2 gm
• Positive direct antihuman globulin (Coombs) test.


• What is the finding of blue‐red fingers called?
What are the causes of this condition?

Which diagnosis is favored by the laboratory findings in
this patient?

Back 168

1. ranauds
2. vascular spasm, AI disease- scleroderma, crest, cold agglutinin
3. Cold agglutinins, cryoglobulins, positive DAT, anemia

Front 169

A 35 y/o man comes to the ED with meningeal symptoms. He says he was taken to a trauma center in rural Ecuador 2 yrs. ago after experiencing blunt abdominal trauma. He does
not remember exactly what was done, but he believes they took drastic measures to control intraabdominal bleeding. Before leaving the
trauma center, he was counseled about a new susceptibility to encapsulated organisms.
Which of the following findings would be seen in
a peripheral blood smear in this patient?

A. Heinz bodies, target cells, perhaps more
platelets than usual
B. Megaloblastic anemia
C. Schistocytes
D. Sickle cells
E. Spherocytes

Back 169

A- Heinz body, target cells, lots of platelets: THE SPLEEN IS GONE!!!

Front 170

what in teh Spleen WHite nad REd

Back 170

White- T cells
Red- sinusoids, macro

Front 171

what does the spleen do

Back 171

1. filter blood: clears old RBC, RBC+AB (extravascular hemolysis), Howell Jolly, Heinz body removal, clears bacteria

2. humoral immunity

3. Lymphorecticular cells
4. hematopoeiss with BM cant
5. store RBC and plate for reservs

Front 172

what causes splenomegaly, what are the sx

Back 172

1. Infection
2. Congestion- cardiac or liver problems
3. lymphohematogenous disorders
4. SLE, RA
5. Glycogen storage diseases

Sx:
early satiety, discomfort when eating, dragging in LUQ

Front 173

what is perisplitis

Back 173

spleen coated with fibrin

**can happen after a spleen infection, mono or CMV

Front 174

if a spleen weighs...

1. 150
2. 200-400
3. 500
4. 10000
5. 3000

Back 174

1. normal
2. splen infection
3. cardiac cauused congestion
4. hepatic congesion
5. Myeloproliforative disorders

**remember in SS it autoinfarcts nad gets really small

Front 175

spleen cancer

Back 175

mets in uncommon but is seen with:
1. Malignant Melanoma
2. Lung cancer

PRIMARY:
Lymphohematopoietic malignancy- systemic involvement, splenomegaly

1. Follicular Lymphome
2. Large B cell lymphome

Front 176

causes of ruptured spleen/splenectomy

Back 176

trauma- will have intraabd hemorrhage and hypovolumic shock
spontaneous in mono, maleria, leukemia

**when spleen is gone you get nRBC, howell jolly body, target cells, susceptible to encapsulated (strep pneumonia)

Front 177

if you have had MVA nad have intraabdominal hemorrhage, hypovolumic shock what can have happened

Back 177

ruptured spleen

**after splenectomy you have nRBC, Howell Jolly, target cells, heinz