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68 Cards in this Set
- Front
- Back
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what is polycythemia
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erythrocytosis
inc RBC, Hb, Hct |
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what are the erythropoetin levels in a primary polycythemia
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Low
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what are the erythropoetin levels in a secondary polycythemia
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High
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what is relative polycythemia and what causes it
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caused by dehydration (loss of plasma)
Causes: vomitting/ diarrhea diuretics excess sweating Hanta virus (four corners flu) Gaisbocks synd (stress) |
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what is polycythemia vera
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Inc in RBCs, blood volume, neutro, and platelets
absolute, primary polycythemia caused by a JAK2 mutation in stem cells erythropoeiten levels are LOW |
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what are appropriate and inappropriate causes of absolute, secondary polycythemia
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HIGH erythropoietin for both
Approp: High altitude, cyanotic heart disease, pulmonary disease Inapprop: Renal cell carcinoma |
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what is bleeding diatheses and what causes it
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excessive bleeding
fragile vessels platelet deficiency derangements of coagulation |
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what is prothrombin time (PT) used to monitor and what is its typical time
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coumadin therapy
~12 sec |
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Reff time for aPTT
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~35 - 40 sec
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vasculitis
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Organism that infects the vessels
-meningiococcemia -ricketts infective endocarditis (subungual hemorrhage, petechiae) DIC |
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types of immune vasculitis
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collagen disease- RA, SLE
drug rxns- Hypersensitivity/leukocytoclastic vasculitis unk- antigen- Henoch-Schonlein purpura |
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sympt of Henoch-Schonlein purpura
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Symptoms have onset following URI:
‐ Palpable Purpuric rash ‐ Colicky abdominal pain (?focal bleed) ‐ Polyarthralgia ‐ Acute glomerulonephritis |
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pathogenisis of H-S purpura
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IgA immune complexes deposited in the vessels
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what are the diseases caused by impaired collagen support
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Scurvy (vit C)
Ehelers-Danlos (type I & III collagen) Cushings (corticosteroid excess = protein wasting) Elderly- collagen atrophy |
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another name for Hereditary hemorrhagic telangiectasia
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Osler-Weber-Rendu disease
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what is the inheritance of Hereditary hemorrhagic telangiectasia
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Autosomal dominant
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clinical presentation of Hereditary hemorrhagic telangiectasia
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mucosal bleeds
epistaxis GI bleeds (20% life threatening) AV malformations of brain, lung, liver |
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where do you typically see systemic amyloidosis and what is it associated with
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around the skin of the eyes
Primaryl AL multiple myeloma |
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what is Cerebral amyloid angiopathy (CAA)
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Aβ amyloid accumulation in cerebral vessels
2nd most common cause of non-traumatic cereberal hemorrhage |
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strict definition of thrombocytopenia
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platelet count < 150,000/mm3
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clinical manefestations of thrombocytopenia
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Petechiae, ecchymoses, purpura
mucosal bleeding intracrainial bleeding |
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causes of thrombocytopenia
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‐ Decreased production
‐ Decreased platelet survival ‐ Sequestration ‐ Dilutional |
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causes of decreased platelet production
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Drugs (alcohol, thiazides, chemo)
virus (HIV, EBV) malnutrition (B12, folate) Myelophthisic disorders |
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causes of decreased platelet survival
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DIC, TTP, HUS
immune complex induced thrombi allo/autoantibodies hemangiomas prosthetic heart valves Malig HTN |
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what are the two thrombocytopenias caused by alloantibodies
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neonatal (maternal Ab's that cross the placenta)
post transfusion |
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what is the cause of primary thrombocytopenia due to autoantibodies
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Idiopathic immune
thrombocytopenic purpura (ITP) |
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what is the cause of seconday thrombocytopenia due to autoantibodies
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drug (heparin, sulfas, quinadine)
HIV, CMV, EBV autoimmune (SLE) CLL |
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What is idiopathic thrombocytopenic purpura
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• IgG autoantibodies target eptitopes on platelet GPs
Platelets opsonized ‐spleen phagocytizes; destroys platelets megakaryocytes may also be damaged in the bone marrow |
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what are the morphalogical features features of ITP
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Hemorrhages : petechiae, purpura, etc.
Spleen normal in size Increased, immature megakaryocytes; atypical morphology megathrombocytes on periph blood smear |
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clinical features of chronic ITP
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Females <40 most common
Petechiae, ecchymoses follow minor or no trauma easy bruising; epistaxis, bleeding gums Risk for intracranial hemorrhages |
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clinical features of acute ITP
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childhood disease
purpura, mucosal bleeding ‐ Abrupt onset 2 weeks after viral illness 80% self limited |
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treatment of ITP
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Corticosteroids
Intravenous immunoglobulin Anti‐CD 20 (Rutuximab) splenectomy |
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lab findings of ITP
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norm PT and aPTT
dec platelet inc atypical megakaryocytes anti-platelet Abs |
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what is type I HIT
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heparin induced thrombocytopenia
unfractionated heparin rapid onset and most common platelets aggregate, but do not clot |
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what is Type II HIT
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5-14 days after initial treatment
Ab to factor IV-heparin complex intravascular thrombi form life threatening (look for a 50% reduction in platelet count) |
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why does HIV cause thrombocytopenia
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infection of megakaryocytes
molecular mimicry- gp120 cross reacts with gpIIb-IIIa |
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how do you get microangiopathic hemolytic anemia in TTP and HUS
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platelet aggrigations form microvascular hyaline thrombi that cause shear stress on RBCs
RBCs form helmet cells or schistocytes and are removed by the spleen |
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what causes Thrombotic thrombocytopenic purpura (TTP)
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a deficiency in ADAMTS 13 which is a vWF cleaving protease
genetic or autoantibody |
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clinical features of TTP
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"FARTN"
Fever Anemia Renal Thrombocytopenia Neurological defects |
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what is the only difference in clinical features for HUS as compared to TTP
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No Neurological defects
you just "FART" |
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Treatment of TTP and HUS
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HUS- supportive
TTP- Plasmaphoresis (fatal without) |
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what are the most common clotting factor deficiencies
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VIII and vWF
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which clotting factor deficiencies are X-linked
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VIII and IX
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what is the only clotting factor deficiency that is autosomal dominant
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vWF
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what clotting factors arent produced with a Vit K deficiency
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VII
IX X II |
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why does DIC cause bleeding
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it consumes all of the clotting factors and activates fibrinolysis
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what is a genetic deficiency in clotting factor VIII called
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X-linked Hemophilia A
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where is Factor VIII produced
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Liver
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where is vWF synthesized and what is its function
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by endothelial cells
binds platelets to collagen via Id-IX enables platelet aggrigation via IIb-IIIa extends the life of VIII |
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what is the clinical presentation of vW disease
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‐ Spontaneous bleeding from mucous membranes
‐ Menorrhagia ‐ Excessive bleeding from wounds ‐ Normal platelet count ‐ Prolonged bleeding time |
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features of Type I vW disease
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AD inherit
decreased vWF NORMAL PTT |
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features of Type 2 vW disease
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defects in vWF
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features of type 3 vW disease
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AR inherit
PROLONGED PTT |
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treatment for vW disease
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• DDAVP (desmopressin) for Type 1
• Cryoprecipitate, 1 unit/10 kg bid • Avoid aspirin |
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what is the most common, serious, hereditary bleeding disorder
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Hemophilia A (VIII)
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who is typically affected by Hemophilia A
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Males
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what develops in 15 - 30% of Hemophilia A pts
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anti-factor VIII antibodies
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what are the clinical features of Hemophilia A
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• Massive hemorrhage following trauma
• “Spontaneous” hemorrhage; easy bruising • Hemorrhage into joints (hemarthrosis) with joint deformity • Prone to CNS hemorrhage • No petechiae |
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what are the lab tests for Hemophilia A
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• Platelet count: normal
• PT: normal • PTT: PROLONGED • Factor assays performed for factor VIII activity |
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how do you treat Hemophilia A
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Infusion of factor VIII concentrates
FEIBA (factor 8 inhib bypass agent) |
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what clotting factor is dificient in Hemophilia B
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IX
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what is another name for Hemophilia B
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Christmas disease
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what group of individuals have a high carrier rate for factor XI deficiencies
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Ashkenazi Jews
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what cytokines are released from monocytes to activate the endothelium in response to endo/exotoxins (DIC)
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TNF
IL-1 |
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causes of DIC
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Sepsis
seperation of the placenta amniotic fluid embolism malignancy Major trauma snake bite, vasculitis, liver disease, hemolytic transfusion reaction heat stroke |
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what lab values are inc during DIC
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FDP and D-dimer
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what is Waterhouse‐Friderichsen syndrome
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effect of DIC
‐ bilateral adrenal hemorrhage, adrenal failure and death ‐ associated with meningiococcemia |
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treatment of DIC
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remove cause
treat with anti & pro coagulants |
