WBC Disorders

Question 1

Mnemonic for multiple myeloma?

Answer 1

Calcium elevated
Renal failure
Anemia
Bone lytic lesions

Old age.

Question 2

Presentation of multiple myeloma?

Answer 2

Back pain, compression fractures, may have signs of hyper calcium, foamy urine

Look for punched out lesions in skull on X-ray.

Question 3

Why is MM so hard to treat?

Answer 3

It is highly differentiated - hard to kill without killing whole immune system. Less rapid division (which is usually a good thing but not in this case)

Question 4

ALL Pathophysiology?

Answer 4

Young patients, young cells (lymphoblast) - maturation arrest will see in peripheral smear.

Question 5

Presentation of AML / ALL

Answer 5

Anemia, tiredness, easy bleeding, frequent infections, bone pain, gum swelling (AML). Abrupt onset, very sick very fast

Question 6

Risk factors for AML

Answer 6

Down’s syndrome, previous cancer tx, adult ~65

Question 7

What is leukostasis?

Answer 7

AML - blasts clogging up vessels causing end organs damage - kidneys, liver etc. This is an emergency! Give them lots of fluids. Make sure they are hooked up with an oncologist.

Question 8

What is tumour lysis syndrome?

Answer 8

Large tumours, or very responsive to tx - cells burst - release K+ and Phosphate into the blood. Also all the cytokines - huge inflammatory. Consequences of hyperkalemia - cardiac

Question 9

Chromosome implicated in CML?

Answer 9

Philadelphia chromosome

Question 10

Epstein-Bar associated with?

Answer 10

Hodgkin’s lymphoma, Non-Hodgkins

Question 11

Reed-Sternberg cells?

Answer 11

Hodgkin’s lymphoma, look for super-clavicular LNs, no other symptoms

Question 12

Febrile Neutropenia work up?

Answer 12

CBC, Blood cultures X2, urinanalysis/cultures. Culture any sites of infection.

Tx with Abx RIGHT AWAY, non-responder - anti-fungal therapy and transfusion with neutrophils

Question 13

leukemia vs lymphoma

Answer 13

Leukemia - originating in blood and bone marrow

Lymphoma - solid tumor or mass of the lymph nodes, MALT, spleen, or bone marrow

Question 14

Leukocytes

Answer 14

Leukocytes: all white blood cells

Myeloid and lymphoid

Question 15

Neutrophils Function

Answer 15

Multilobed nucleus (2-5), granulated 
Function-  integral in innate immunity; main cell in acute inflammation (5-6 lifespan), phagocytosis

Question 16

Basophils Function

Answer 16

Granules contain mediators of acute inflammation (histamine)

Role in hypersensitivity rxn

Question 17

Eosinophil

Answer 17

Two-lobed, red-orange staining

Cytotoxic cells that play a role in immune, inflammatory and parasitic responses

Question 18

Monocytes

Answer 18

Innate immunity, become macrophages in tissue

Phagocytosis

Question 19

B Lymphocytes

Answer 19

Produce antibodies against a specific extracellular antigen (humoral immunity)
Stay in secondary lymphoid tissue

Question 20

T-lymphocytes function

Answer 20

Cell-mediated immune response, antigen specific

Question 21

Primary Lymphoid Organs

Answer 21

Bone Marrow and thymus

Question 22

Secondary Lymphoid Organs

Answer 22

Lymph node, spleen

Question 23

Multiple Myeloma Pathophysiology

Answer 23

Cancer of plasma cells that produce monoclonal immunoglobulins (antibody)

Question 24

Multiple Myeloma Immunoglobulin

Answer 24

M protein (messed up immunoglobulins )
Most common form of the M Protein:
IgG > IgA > IgD > IgM > Pure light chain myeloma

Question 25

MGUS

Answer 25

Premalignant plasma cell proliferative disorder | Risk factor for MM

Question 26

Leukemia (ALL) Pathogenesis

Answer 26

Early lymphoid precursors (lymphoblasts) grow and crowd bone marrow - are stuck in immature phase

Question 27

ALL - classic presentation?

Answer 27

Child, abrupt onset - bleeding, anemia, thrombocytopenia, pain, infections, rashes - vague sx. Small people, small blasts, small mortality

Question 28

AML Pathophysiology

Answer 28

Myeloblasts - myeloid progenitors, older adults, may see Auer rods on smear.

Question 29

AML classic presentation

Answer 29

Adults over 65, anemia, bleeding, bone pain, leukaemia cutis, gum swelling Large people, Large cells, Large mortality

Question 30

CML

Answer 30

Too many mature granulocytes - Philadelphia chromosome (BCR-ABL) not able to get rid of cells correctly

Question 31

CML - classic presentation

Answer 31

Any age, slow onset, spleenomegaly, BSx, bleeding and quadrant pain

Question 32

CLL - Pathophysiology

Answer 32

Too many mature B-cells, smudge cells,

Question 33

CLL - classic presentation

Answer 33

Asymptomatic, may have anemia and BSx, do not treat symptomatic, common in adults over 70

Question 34

Hodgkin’s Lymphoma - classic presentation

Answer 34

Asx, Reed-Strenberg cells, mainly B cell based, in lymph nodes and other lymph tissues

Question 35

Hodgkins prognosis?

Answer 35

Good prognosis, limited extra-nodal involvement

Question 36

Non-Hodgkin’s lymphoma - classic presentation

Answer 36

Mature B or T cells, from Epstein-Barr, H-pylori, HIV, environmental, autoimmune - goes to extra nodal sites - so may have associated sx that way (bowel obstruction, marrow failure, spinal cord)

Question 37

Febrile Neutropenia Tx

Answer 37

Must admit febrile neutropenia, get cultures and treat aggressively with abx. Consider neupogen

Question 38

Criteria for Febrile Neutropenia?

Answer 38

Fever >38 for an hour, or a fever >38.3 And one of them following: Absolute neutrophils <0.5 or less than 1 trending downwards

Question 39

Reasons for decreased neutrophils?

Answer 39

Decreased production - due to lack of B12/ folate, or malignancy or drugs (chemo) Or increased peripheral destruction due to autoimmune

Question 40

When is your white cell count lowest following chemo?

Answer 40

Usually 5-10 days

Question 41

What should you not do on exam in febrile Neutropenia?

Answer 41

DRE! Risk of introducing infection

Question 42

Myeloma kidney

Answer 42

Often lots of WBC/ Ig chains, but doesn’t usually cause albuminuria - this indicates some other cause of kidney disease. Hypercalcemia also leads to renal vasoconstriction and intratubular deposits

Question 43

Tx for myeloma kidney

Answer 43

Dc nephrotoxins, correct calcium, tx myeloma with chemo and Dexamethasone, extracorporeal methods, dialysis if needed

Question 44

Mastocytosis

Answer 44

Flushing, hypotension, syncope, mast cells in blood cells, skin lesions,