final Flashcards
causes of prolonged PT and/or prolonged aPTT
-normal PT / abnormal aPTT- hemophilia A and B
-PTT- 8, 9, 11, 12 (intrinsic)
-PT- 7 (extrinsic)
-Vitamin K- 2, 7, 9, 10 - LIVER
heparin induced thrombocytopenia
-potentially devastating immune mediated ADR caused by emergence of antibodies that activate platelets in presence of heparin
-non immune type 1
-or type 2
hereditary hemochromatosis
-mutation in HFE gene- autosomal recessive
-increase iron absorption
-hereditary
-hepatomegaly
-cardiomyopathy
-hyperpigmentation
-arthropathy
-diabetes
-hypopituitarism
-tx:
-phlebotomy!!
-erythrocytapheresis
-iron chelation
-dietary iron intake
sickle cell ds
-when dehydrated -> cells sickle -> crisis
-carrier cant concentrate urine
-increase risk for - hematuria, thromboembolic ds, PE, splenic infarction
-Auto-splenectomy and splenectomy -> infections from encapsulated bacteria
-Strep pneumoniae, Haemophilus influenzae, and Neisseria meningitidis
-daily hydroxyurea tx -> immunosuppressive
-decrease WBC
-monitor RBC, WBC, reticulocytes, platelets
-increases HbF -> more F means less HbS
autoimmune hemolytic anemia
WARM ANTIBODY INDUCED
-IgG
-idiopathic, drugs, lymphoma, leukemia
-Coombs- +IgG or IgG and C3
-TX- Steroid, splenectomy!
-immunosuppressive: cyclophosphamide or cyclosporin
COLD ANTIBODY INDUCED
-IgM (mittens)
-idiopathic, mycoplasma, mono, waldenstroms macroglobulinemia!
-Coombs- ONLY C3 +
-TX- NO steroid/splenectomy -> immunosuppressive tx: cyclophosphamide or chlorambucil
paroxysmal nocturnal hemoglobinuria (PNH)
-rare, acquired, life threatening
-destruction of RBC, formation of clots, and impaired bone marrow
-PIGA gene mutation
-compliment regulatory proteins not linked membrane
-intravascular hemolysis
-hemoglobinuria
-dark/cola urina
myeloproliferative disorders
-uncontrolled clonal proliferation or 1 or more cell lines in bone marrow (erythroid, myeloid, megakaryocyte)
-JAK 2 kinase -> all can have it
-1. polycythemia vera
-high RBC, EPO
-JAK2 gene + or 2ndary (hypoxia)
-HTN, bruising, blurry vision, dizzy, tired, HA, tingling, itchy after shower
-tx- phlebotomy and hydrea!, ASA, myelosuppressive tx, fluids
-Hct < 45%
-platelets < 400,000
-2. essential thrombocythemia
-too many platelets
-MI, stroke
-JAK2 gene mutation and MPL mutation
-cellular phase- increase megakaryocytes with fibrosis of bone marrow
-fibrotic phase- collagenous fibrosis with lack of marrow elements
-3. Myelofibrosis
-chronic neoplasm
-increase fibrosis of bone marrow
-primary or in late essential thrombocythemia or polycythemia vera
-hyperplasia of megakaryocytes -> release fibroblast stimulating factors (platelet derived GF) -> increased fibrosis
-myeloid metaplasia in liver, spleen, etc.
-4. Chronic myeloid leukemia
-myeloproliferative neoplasm
-philadelphia chromosome (9,22 translocation) !!!!!!!
-middle aged
-tx- tyrosine kinase inhibitors!!! - imatinib, “nibs” -> monotherapy
-no chemo
waldenstrom macroglobulinemia and IgM multiple myeloma
-lymphoplasmacytic lymphoma (LPL)
-lymphoplasmacytic infiltration in bone marrow or lymphatic tissue and IgM monoclonal gammopathy in blood
-slow growing
-tumor infiltration- cytopenias, fever, night sweat, wt loss, LN, HSM
-M-protein- hyperviscosity!!! (ulcers, raynauds, stroke), cryoglobulinemia, cold agglutinin, neuropathy, amyloidosis
-macroglobulinemia- brain and eye hemorrhage, retinal ds, ulcer, raynauds, stroke
-CD56 -ve
-CD19, and CD20 + ve
hodgkin lymphoms
-1. classic HL:
-reed sternberg cells- binucleate or bilobed central nucleus - owl eyes
-supraclavicular nodes
-ITCH
-ETOH -> PAIN
-4 types:
-nodular sclerosis- MC
-mixed cellularity- MC in HIV
-lymphocyte depleted- least common
-lymphocyte rich
-2. nodular lymphocyte-predominant HL (NLPHL)
-no reed sternbergs
-cells express CD20(+)
-extranodal involvement
-noncontiguous spread
ann arbor system
-4 stages of lymphoma
a- w/o sx
-b- with sx
-stage 1- 1 spot
-stage 2- 2 spots on one side of diaphragm
-stage 3- both sides of diaphragm
-stage 4- bone, liver, lungs -> outside the lymph node
burkitt lymphoma
-extremely aggressive B cell lymphoma
-fastest growing human tumor
-pt die within weeks w/o tx
-dx in children and adults (uncommon in adults)
-EBV! assoc
-starry night appearance
-sub-african
aspirin
7-10 days of antiplatelets
-coated aspirin for GI
multiple myeloma
-skeleton survey- look for lesions
->60% plasma -> dx
-CRAB
-C >10
-R- Cr>2
-A- hmg <10
-bone ds- skeleton survey
-end organ damage
superior vena cava syndrome
-dyspnea
-distended veins
-edema !
-coughing
-hoarse
-chest pain
-difficulty swallowing
-cyanosis
-horners syndrome- small pupil, droopng eyelid, no sweating on one side
-paralyzed vocal cord
-headache
-bilateral upper extremity venography -> gold standard
cardiac tamponade
-dyspnea, orthopnea, raised JVP
-pericardial rub
-chest discomfort/pain
-cough
-becks- muffle heart sound, low BP, JVP
-electrical alternans
-POCUS- pericardial fluid, RV diastolic collapse, dilated IVC
hyperviscosity syndrome
-cause- wadenstrom’s macroglobulinemia (50%) and multiple myeloma (5%)
-neurological sx
-bleeding diathesis
-retinal hemorrhage and papilledema
-hypervolemia
-CHF
-tx-
-plasmapheresis (ASFA cat 2)
-chemo
-neutropenic fever- neutrophil are low
-TUMOR LYSIS SYNDROME!- what to order and look for if pt just had chemo
-hyperkalemia
-high uric acid
-ARF
-low Ca
-hyperphosphatemia
-AML- auer rod in blast- abnormally fused primary (azurophilic) granules forming needle shaped -> Strongly + for myeloperoxidase
-ALL- immature b cells
-CLL- indolin 1, older, not treated, smudge cells
-CML- TKI tx
RAIs clinical staging system
-CLL
-tx around stage 3
-starts with 0! - normal
-1- enlarged LN
-2- enlarged spleen and liver
-3- anemia
-4- anemia + thrombocytopenia
PT vs APTT
-mixing study differentiates btwn deficiency and inhibitor
-clot test normalizes -> factor deficiency
-clot test doesnt normalize -> factor inhibitor
diff dx hemophilia A and von willebrand ds
-VWD-
-MC hereditary bleeding ds
-bruising, nose bleed, menorrhagia, mucosal bleeding
-tx- DDAVP
-hemophilia A
-deficiency of factor 8
-males only- X-linked inherited
-joint/muscle bleeding!
-compartment syndrome
-PTT prolonged
-tx- replace factor, cryo, DDAVP (for mild)
-hemophilia B
-deficiency of factor 9
primary events in DIC
-thrombin (procoagulation) + plasmin (fibrinolysis)
-bleeding and clotting disorder
-NEED TO KNOW HOW TO DX
-SCREENING:
-aPTT- prolonged
-PT- prolonged
-fibrinogen- low
-platelet- low
CONFIRMATORY:
-d-dimer- high
-fibrin degradation products- high
-tx:
-underlying condition- antibx, surgery, chemo, embolization
-replace- platelets, FFP, cryo
-heparin- in certain situations
D-dimer
-contrast venography GOLD STANDARD for DVT
-CT spiral for PE
-measure of d-dimer liberated from fibrin by action of plasmin
-evidence of prior thrombin activity follow by fibrinolysis
-should be part of eval of DIC
-important screening and prognostic in venous thromboembolic disease -> good + predictive value for DVT and PE
thrombotic thrombocytopenic purpura
-deficiency or antibodies to ADAMTS13 which cleaves VWF
-platelet adhesion and aggregation
-thrombocytopenia
-microangiopathic hemolytic anemia
-fragmented RBCs and lab evidence of hemolysis and microvascular thrombosis
-includes microangiopathic hemolytic anemia, thrombocytopenia, renal failure, neurologic findings, and fever
-HAMS13 test
ITT vs HUS vs TTP
tx of warfarin/phenindione overdose/poisoning
-if INR too high
-vitamin K1 phytonadione
-FFP or factor 9 can be infused
-if mild- only stopping warfarin is okay
therapeutic INR for coumadin
-INR of 2.5
-between 2-3!!!
-increase bleed risk is exponential
-INR >5 is BAD
heparin induced thrombocytopenia: type 1 vs type 2
blood product tx
-factor 8
FFP and Cryo
FFP
-Contains all factors of the soluble coagulation system, including fibrinogen and labile factors V and VIII. FFP is used to treat patients with multiple factor deficiencies and bleeding.
Cryo
-A concentrated form of FFP that contains a subset of clotting factors, including fibrinogen, factor VIII, von Willebrand factor, and factor XIII. Cryo is used to treat bleeding disorders, hypofibrinogenemia, von Willebrand disease, and other conditions
DDAVP (desmopressin acetate)
-synthetic vasopressin
-MOA- release of stores from endothelial cells raising factor 8 and vWD serum levels
-IV, SQ, nasal
-for mild cases of hemophilia, VWB
coagulation deficiency summary
