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30 Cards in this Set

  • Front
  • Back
4 types of Primary Myeloproliferative Disorders
Polycythemia vera
Essential thrombocytosis
Chronic Myleogenous Leukemia
Myelofibrosis with myeloid metaplasia
Sign of fibrosis of the bone marrow seen in the peripheral smear
Tear drop cells
Leukoerythroblastic picture: many types of abnormal cells, L shift
Major differences between polycythemia vera and secondary polycythemia
(5)
As opposed to a secondary polycythemia (in response to tissue hypoxia or increased Epo), polycythemia vera shows:
marked splenomegaly
increased histamine level
panhyperplasia of the BM
decreased Epo
Normal oxygen saturation
Course and Tx of polycythemia vera
Benign if controlled - phlebotomy, chemo

Can progress to:
"spent phase": fibrosis & leukoerythroblastic picture, splenomegaly
acute leukemia: risk increases w/chlorambucil tx
Definition of an acute leukemia
Greater than 20% blasts in the bone marrow
Consequences of tumor lysis when treating AML or ALL w/ very high WBC counts
release of intracellular components may cause:
acidosis
renal failure due to uric acid deposition
hyperphosphatemia
hypocalcemia
AML subtype M3
Acute Promyelocytic Leukemia
t(15;17)
translocated retinoic acid receptor
treatment with retinoic acid unblocks maturation arrest
Bence-Jones protein
Ig light chain component found in the urine
Associated with multiple myeloma
What neoplasm is associated with lytic bone lesion?
Multiple Myeloma
due to high level of osteoclast activity
Multiple Myeloma Epidemiology
More common in males, AA, median age is 60 yo
Clinical Manifestations of Multiple Myeloma
Non-specific: fatigue, fractures, infections
Paraprotein: expands plasma volume, decreased anion gap
Hypercalcemia: due to IL-6, OAF, pt. is immobilized by pain of lytic lesions
Hematologic: rouleaux formation on blood smear, anemia, thrombocytopenia, platelet dysfunction
Renal Insufficiency
Immunodeficiency
Cord compression & neuropathy
Multiple Myeloma Treatment
Incurable - overall survival is poor
Melphalan & Prednisone
Maintain hydration
Transfusions, Vaccinations
Epo to treat anemia
Bisphosphonates
Thalidomide, Revlimid, Dexamethasone,
Bortezomib
Monoclonal Gammopathy of Unknown Significance (MGUS)
Asymptomatic.
Diagnosis: M protein less than or equal to 3g/dL in serum, fewer than 10% plasma cells in BM, no lytic bone lesion, anemia, hyperCa, renal insufficiency

Can develop into plasma cell proliferative disorder, e.g. multiple myeloma, AL amyloidosis
2 organs most commonly affected by AL amyloidosis
Kidneys - nephrotic syndrome
Heart - restrictive cardiomyopathy
Hallmark feature of AL amyloidosis
Macroglossia
Waldenstrom's Macroglobulinemia
Cause
Clinical effects
Treatment
Proliferation of B cells that mature into plasma cells

Lymphoplasmacytic infiltration of BM
Anemia
Neuropathy
Organomegaly
IgM gammopathy
Hyperviscosity Syndrome

Tx: Plasma exchange, alkylating agents, Rituximab (Ab versus CD 20), nucleoside analogs
Vitamin K-dependent clotting factors
2, 7, 9, 10
3 changes which occur in an activated platelet
1. phosphatidyl serine is exposed on surface - 'platform' where clotting factors bind
2. granules release ADP & thromboxane which activates more platelets
3. IIbIIIa receptor is expressed - binds fibrinogen, crosslinks platelets and PLATELET PLUG is formed
Most common hereditary bleeding disorder
von Willebrand disease

vWF is a carrier protein for factor VIII
and mediates adhesion of platelets to subendothelial tissue

*factor VIII is low & aPTT can be prolonged
Common infectious cause of DIC?
Meningococcal meningitis
5 signs of Thrombotic Thrombocytopenic Purpura
Fever
Mental Status Changes
Thrombocytopenia
Microangiopathic Hemolytic Anemia
Renal failure
General cause of TTP
Deficiency of von Willebrand factor metalloproteinase which causes the vWF to accumulate, promoting platelet microaggregation formation
Mechanism of aspirin
Irreversibly blocks COX enzyme
which converts arachidonic acid into prostaglandins
PGs are converted into thromboxane
therefore aspirin inhibits platelet activation
2 common causes of acquired platelet defects
Aspirin/clopidogrel
Uremia - corrected by drug DDAVP (desmopressin), dialysis
Immune Thrombocytopenia Purpura:
kids versus adults
Tx
In kids is self-limited, occurs post viral infection
In adults is a chronic condition
since are removed by macrophages in spleen, can Tx w/splenectomy
causes of DIC
(7)
Obstetric complications (most common)
Cancer
Sepsis (gram negative)
Trauma
Acute Pancreatitis
Nephrotic Syndrome
Transfusion
Test to distinguish leukemoid reaction from CML
LAP (leukocyte alkaline phosphatase) score
Alkaline phosphatase is an enzyme found in mature neutrophils.

With a left shift, such as in CML, there should less LAP and a lower LAP score
Significance of D-dimer in blood?
Is a fibrin degradation product
Present in blood after a blood clot is degraded by fibrinolysis.

Sign of DIC, DVT, PE
Direct Coomb's test versus Indirect Coomb's test
Direct Coomb’s test: Testing for antibody (IgG or complement) coating patient’s red cells

Indirect Coomb’s test: Testing for antibody in patient’s plasma/serum
Lab Findings in DIC
⇧ PT, PTT, D-dimer
⇩ Platelet count
Helmet Cells
Schistocytes