final Flashcards
(32 cards)
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
