pure erythroid leukemia= no significant myeloblastic component
What is AML M7?
Acute megakaryoblastic leukemia, dry tap due to fibrosis from increase in megakaryocytes, ID'd by immunophenotyping and cytochemistry
What is the cell specific to Hodgkin's lymphoma?
Reed Sternberg cells
What are non-hodgkin's lymphomas?
B-cell neoplasms
small cell lymphocytic lymphoma- nodes
chronic lymphocytic leukemia- PB and BM
What does CD tell you about the lymphocytes effected?
<10 (excluding 5)= T cell
>10 (plus 5)= B cell
If you see a smudge cell in a case study you think...
CLL
What cells predominate in Waldenstrom Macroglobulinemia?
Plasma cells (B cells)
What is Multiple Myeloma
malignant proliferation of plasma cells, lytic bone lesions, elevated serum calcium, bence jones proteins
What is MGUS?
Monoclonal ab present in the absence of plasma cell dyscrasia, can progress to multiple myeloma
What is sezary cell syndrome?
mycosis fungoides, T cell derived tumors of the skin, only bad prognosis if it progresses beyond the skin
What is ALL?
Most common in young children, typical findings are neutropenia, thrombocytopenia, anemia
What are the different types of ALL?
Immature B cell (most common)
Pre B cell
T cell
B cell
When is the TdT stain positive?
early B cell ALL
Hemostasis
balancing of clotting and bleeding
Cardiovascular system
deliver O2 and nutrients, take away waste
lymphatic system
transport lymph fluid, water and cells
Thromboresistant
inert to platelets and coag factors, (i.e. blood vessels)
Primary hemostasis
formation of the primary platelet plug
adhere via vWF
Secondary hemostasis
stabilize and strengthen via XIII
activate the coag cascade via VIIa after exposure to TF
Platelet production
in the BM by megakaryocytes
pseudothrombocytopenia
in-vitro sampling, anticoag clumps
remedy by using sodium citrate (blue top) tubes
Platelets shape change
disk-->spiky-->pancake
3 stages of clot formation
adhersion, aggregation, secretion
Adhesion
platelets roll and cling to non platelet surfaces to seal endothelial gaps
vWF required
Reversible
Aggregation
platelets adhere to each other
requires fibrinogen
irreversible
Secretion
Platelets secrete granular contents, essential for coagulation
\irreversible
Types of platelet granules
Alpha- larger, factor V, factor XI, fibrinogen, vWF, etc.
Delta- smaller, Ca, Mg, serotonin, etc.
Dense bodies
AKA delta granules
Gray platelet deficiency
alpha granule deficiency
GP Ib/IX/V
vWF receptor, facilitates adhesion to exposed collagen
GP IIb/IIIa
Fibrinogen receptor, allows platelets to bind to each other.
Where are most coag factors made
Liver
Von willebrand Disease
most common bleeding disorder in the US
quantitative or qualitative vWF defect
Types of vWFD
1: you just don't have as much (most common)
2A: chopped up faster
2B: cleared out quicker
2M: less GP Ib, no primary plug
2N: factor VIII deficiency
3: absence of all multimers
Pseudo vWFD
platelet type, mutation in GP Ib gene instead of vWF gene
Bernard Soulier Syndrome
deficiency/dysfunction of GP Ib (vWF receptor)
Qualitative PLT disorder--> giant platelets
Glanzmann's Thrombasthemia
deficiency/dysfunction of GP IIB/IIIA
aggregation cannot occur unless treated with ristocetin
PFA 100
assessment of PLTs for adequate function via exposure to collagen and ADP or epi
Thrombin
induces full aggregation and secretion of granular components
conversion of fibrinogen to fibrin
ADP
binds PLT membrane to stimulate dense granule release and shape change
ITP
Immune Thrombocytopenia Purpura
PLT destruction due to autoantibodies
labeled PLTs are removed quickly from the system leaving the patient with thrombocytopenia
TTP
Thrombic Thrombocytopenia Purpura
single acute episode in which clots form in the small vessels of the body
leaves body with fewer clotting resources, PLT and vWF thrombi w/o fibrin
clotting cannot occur without ristocetin
HUS
Hemolytic Uremic Syndrome
resembles TTP but in children
T/F Coag specimens should be kept refridgerated
False
coag factors precipitate in the cold
If you see a fibrinogen level of <25..
be suspicious of a clot!
Instrinci pathway
XII-->XI-->IX(+VIII)-->X(+V)-->II-->I
Extrinsic pathway
III(TF)-->VII-->X(+V)-->II-->I
Stago
type of coag analyzer, if iron ball stops moving signifies a clot
Sysmex
scattered light detection to look for clots
Lupus anticoagulant
prothrombotic agent
causes clotting in vivo, prolongs clotting in vitro
anti-phospholipid protein complexes
TT
Thrombin Time
Time to clot with thrombin added
qualitative measurement of fibrinogen
heparin interference
Reptilase time
Like thrombin time but with reptilase
PT
prothrombin time
evaluate extrinsic pathway using thromboplastin
can be used to measure warfarin
INR
used to normalize PT results regardless of strength of reagent
PTT
Partial thromboplastin time
measures the intrinsic pathway
Heparin
accelerates inactivation of thrombin, AKA antithrombin
Coumadin/warfarin
vitamin K antagonist
effects II, VII, IX, X, C+S
HIT
heparin induced thrombocytopenia
IgG antibody binds to heparin/platelet compound activating the platelet and starting the clotting cascade. Patient must be pulled off of heparin to remedy
DTI
Direct thrombin inhibitor
Can be used to avoid HIT
binds thrombin active site on platelets without activation
Hemophilia A
Factor XIII defiiency
x-linked recessive disorder, "classic" hemophilia
Hemophilia B
Factor IX disorder
x linked recessive
Christmas disease
Hemophilia C
Factor XI deficiency
autosomal dominant
Factor V Leiden
greater protein C resistance
Plasmin
cuts fibrin
forms split products like D dimers
D-Dimer
fibrin degradation product
can be used to disagnose thrombosis
DIC
disseminated intravascular coagulation
Over-active coag factors can cause random small clots throughout the body and uses up factors and platelets, leaving the body low on them when they may be needede to stop bleeding. Can cause diffuse bleeding throughout the body