WBC Disorders Flashcards
Mnemonic for multiple myeloma?
Calcium elevated
Renal failure
Anemia
Bone lytic lesions
Old age.
Presentation of multiple myeloma?
Back pain, compression fractures, may have signs of hyper calcium, foamy urine
Look for punched out lesions in skull on X-ray.
Why is MM so hard to treat?
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)
ALL Pathophysiology?
Young patients, young cells (lymphoblast) - maturation arrest will see in peripheral smear.
Presentation of AML / ALL
Anemia, tiredness, easy bleeding, frequent infections, bone pain, gum swelling (AML). Abrupt onset, very sick very fast
Risk factors for AML
Down’s syndrome, previous cancer tx, adult ~65
What is leukostasis?
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.
What is tumour lysis syndrome?
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
Chromosome implicated in CML?
Philadelphia chromosome
Epstein-Bar associated with?
Hodgkin’s lymphoma, Non-Hodgkins
Reed-Sternberg cells?
Hodgkin’s lymphoma, look for super-clavicular LNs, no other symptoms
Febrile Neutropenia work up?
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
leukemia vs lymphoma
Leukemia - originating in blood and bone marrow
Lymphoma - solid tumor or mass of the lymph nodes, MALT, spleen, or bone marrow
Leukocytes
Leukocytes: all white blood cells
Myeloid and lymphoid
Neutrophils Function
Multilobed nucleus (2-5), granulated Function- integral in innate immunity; main cell in acute inflammation (5-6 lifespan), phagocytosis
Basophils Function
Granules contain mediators of acute inflammation (histamine)
Role in hypersensitivity rxn
Eosinophil
Two-lobed, red-orange staining
Cytotoxic cells that play a role in immune, inflammatory and parasitic responses
Monocytes
Innate immunity, become macrophages in tissue
Phagocytosis
B Lymphocytes
Produce antibodies against a specific extracellular antigen (humoral immunity)
Stay in secondary lymphoid tissue
T-lymphocytes function
Cell-mediated immune response, antigen specific
Primary Lymphoid Organs
Bone Marrow and thymus
Secondary Lymphoid Organs
Lymph node, spleen
Multiple Myeloma Pathophysiology
Cancer of plasma cells that produce monoclonal immunoglobulins (antibody)
Multiple Myeloma Immunoglobulin
M protein (messed up immunoglobulins )
Most common form of the M Protein:
IgG > IgA > IgD > IgM > Pure light chain myeloma
MGUS
Premalignant plasma cell proliferative disorder
Risk factor for MM
Leukemia (ALL) Pathogenesis
Early lymphoid precursors (lymphoblasts) grow and crowd bone marrow - are stuck in immature phase
ALL - classic presentation?
Child, abrupt onset - bleeding, anemia, thrombocytopenia, pain, infections, rashes - vague sx.
Small people, small blasts, small mortality
AML Pathophysiology
Myeloblasts - myeloid progenitors, older adults, may see Auer rods on smear.
AML classic presentation
Adults over 65, anemia, bleeding, bone pain, leukaemia cutis, gum swelling
Large people, Large cells, Large mortality
CML
Too many mature granulocytes - Philadelphia chromosome (BCR-ABL) not able to get rid of cells correctly
CML - classic presentation
Any age, slow onset, spleenomegaly, BSx, bleeding and quadrant pain
CLL - Pathophysiology
Too many mature B-cells, smudge cells,
CLL - classic presentation
Asymptomatic, may have anemia and BSx, do not treat symptomatic, common in adults over 70
Hodgkin’s Lymphoma - classic presentation
Asx, Reed-Strenberg cells, mainly B cell based, in lymph nodes and other lymph tissues
Hodgkins prognosis?
Good prognosis, limited extra-nodal involvement
Non-Hodgkin’s lymphoma - classic presentation
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)
Febrile Neutropenia Tx
Must admit febrile neutropenia, get cultures and treat aggressively with abx. Consider neupogen
Criteria for Febrile Neutropenia?
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
Reasons for decreased neutrophils?
Decreased production - due to lack of B12/ folate, or malignancy or drugs (chemo)
Or increased peripheral destruction due to autoimmune
When is your white cell count lowest following chemo?
Usually 5-10 days
What should you not do on exam in febrile Neutropenia?
DRE! Risk of introducing infection
Myeloma kidney
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
Tx for myeloma kidney
Dc nephrotoxins, correct calcium, tx myeloma with chemo and Dexamethasone, extracorporeal methods, dialysis if needed
Mastocytosis
Flushing, hypotension, syncope, mast cells in blood cells, skin lesions,
