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30 Cards in this Set
- Front
- Back
4 types of Primary Myeloproliferative Disorders
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Polycythemia vera
Essential thrombocytosis Chronic Myleogenous Leukemia Myelofibrosis with myeloid metaplasia |
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Sign of fibrosis of the bone marrow seen in the peripheral smear
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Tear drop cells
Leukoerythroblastic picture: many types of abnormal cells, L shift |
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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 |
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Course and Tx of polycythemia vera
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Benign if controlled - phlebotomy, chemo
Can progress to: "spent phase": fibrosis & leukoerythroblastic picture, splenomegaly acute leukemia: risk increases w/chlorambucil tx |
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Definition of an acute leukemia
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Greater than 20% blasts in the bone marrow
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Consequences of tumor lysis when treating AML or ALL w/ very high WBC counts
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release of intracellular components may cause:
acidosis renal failure due to uric acid deposition hyperphosphatemia hypocalcemia |
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AML subtype M3
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Acute Promyelocytic Leukemia
t(15;17) translocated retinoic acid receptor treatment with retinoic acid unblocks maturation arrest |
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Bence-Jones protein
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Ig light chain component found in the urine
Associated with multiple myeloma |
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What neoplasm is associated with lytic bone lesion?
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Multiple Myeloma
due to high level of osteoclast activity |
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Multiple Myeloma Epidemiology
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More common in males, AA, median age is 60 yo
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Clinical Manifestations of Multiple Myeloma
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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 |
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Multiple Myeloma Treatment
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Incurable - overall survival is poor
Melphalan & Prednisone Maintain hydration Transfusions, Vaccinations Epo to treat anemia Bisphosphonates Thalidomide, Revlimid, Dexamethasone, Bortezomib |
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Monoclonal Gammopathy of Unknown Significance (MGUS)
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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 |
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2 organs most commonly affected by AL amyloidosis
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Kidneys - nephrotic syndrome
Heart - restrictive cardiomyopathy |
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Hallmark feature of AL amyloidosis
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Macroglossia
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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 |
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Vitamin K-dependent clotting factors
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2, 7, 9, 10
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3 changes which occur in an activated platelet
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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 |
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Most common hereditary bleeding disorder
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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 |
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Common infectious cause of DIC?
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Meningococcal meningitis
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5 signs of Thrombotic Thrombocytopenic Purpura
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Fever
Mental Status Changes Thrombocytopenia Microangiopathic Hemolytic Anemia Renal failure |
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General cause of TTP
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Deficiency of von Willebrand factor metalloproteinase which causes the vWF to accumulate, promoting platelet microaggregation formation
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Mechanism of aspirin
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Irreversibly blocks COX enzyme
which converts arachidonic acid into prostaglandins PGs are converted into thromboxane therefore aspirin inhibits platelet activation |
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2 common causes of acquired platelet defects
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Aspirin/clopidogrel
Uremia - corrected by drug DDAVP (desmopressin), dialysis |
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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 |
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causes of DIC
(7) |
Obstetric complications (most common)
Cancer Sepsis (gram negative) Trauma Acute Pancreatitis Nephrotic Syndrome Transfusion |
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Test to distinguish leukemoid reaction from CML
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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 |
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Significance of D-dimer in blood?
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Is a fibrin degradation product
Present in blood after a blood clot is degraded by fibrinolysis. Sign of DIC, DVT, PE |
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Direct Coomb's test versus Indirect Coomb's test
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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 |
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Lab Findings in DIC
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⇧ PT, PTT, D-dimer
⇩ Platelet count Helmet Cells Schistocytes |