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67 Cards in this Set
- Front
- Back
Define polycythema and types, relative vs. absolute, secondary vs primary.
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Erythrocytosis: increased [RBC], Hb & Hct
Types: -Relative: plasma volume decrease: dehydration, diuretics, cholera, henta "four corner's flu" virus,; stress polycythemia/ gaisbock's synrome unkown etiology w/ htn, obesity, stress & smoking -Absolute --Primary: low Erythropoietin; -----hereditary = mutated receptor -----polycythemia vera: RBC mass increased w/ plasma volume expansion --2ndry: high Erythropoietin, RBC mass increased w/o plasma volume expansion |
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What are the causes of relative polycythemia?
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relative polycythemia: reduction in plasma volume increases Hct, Hb
dehydration, diuretics, cholera/diarrhea, henta "Four Corners Flu" virus else Gaisbock's "stress polycythemia" syndrome: unknown link from htn, obesity, smoking, & stress to reduced volume polycythemia |
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Compare polycythemia vera with absolute secondary polycythemia taking note of erythropoietin level, changes in other blood cells.
What are the causes of each? |
Polycythemia Vera:
Clonal proliferation of myeloid stem cells from JAK2 mutation Erythropoietin will be low RBC mass, blood volume, neutrophils & platelets all increased Absolute 2ndry Polycythemia: appropriate or tumor probably renal carcinoma Increased EPO w/ increased Hct (w/o blood volume expansion) |
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RBC mass, blood volume, neutrophils & platelets all increased
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Polycythemia Vera:
Clonal proliferation of myeloid stem cells from JAK2 mutation Erythropoietin will be low |
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2ndry Absolute Polycythemia
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Increased Erythropoietin
Appropriate when: Living at high altitude Cyanotic Heart Disease Pulmonary Disease Inappropriate:Paraneoplastic >>Renal Cell Carcinoma<< Hepatocellular CA Cerebellar Hemangioblastoma |
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What are the causes and consequences of vasculitis
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Bleeding Disorder with Norma PT, PTT, & Platelet Count
causes: organisms that infect endothelium/invade walls of blood vessels: meningiococcemia rickettsia other infections, DIC, infective endocarditis (subungual hemorhages) Immune complex deposition: SLE, RA Hypersnsitivity Rxn to Rx: Leukoclastic Henoch-Schonlein Purpura-- IgA Complex Depsotition following URI |
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Any time there is bleeding, what tests should you run?
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PT, PTT and platelet count
Prothrombin Time: extrinsic pathway. 7, V X prothromin, fibrinogen or circulating anticoagulant. Should be ~12 seconds. used to monitor coumadin therapy. Patrial Thromboplastin Time: Intrinsic Pathway: 12, XI, 9, 8, XV21 or circulating anticoagulant; <40s |
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Bleeding Disorder with Normal PT, aPTT, and Platelet Count
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Caused by Vessel Wall Abnormalities
Common Vasculitis, Impaired collagenous support, Hereditary/acquired defects, Amyloid |
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Meningitis + Rash
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Meningococemia
rash is bleeding disorder from vasculitis |
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subungual hemorrhage
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sign of infective endocarditis
you are seeing emboli of infectiou smaterial |
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Rickettsia and Bleeding
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Infects endothelium causing hemorrhages
Normal PT, Normal PTT, Normal Platelet Count |
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Infective Endocarditis and Bleeding
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Infects endothelium causing hemorrhages
Normal PT, Normal PTT, Normal Platelet Count |
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Meningococcemia and Bleeding
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Infects endothelium causing hemorrhages
Normal PT, Normal PTT, Normal Platelet Count |
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RA and Bleeding
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Vasculitis from Immune Complex Deposition
Normal PT, Normal PTT, Normal Platelet Count |
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SLE and Bleeding
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Vasculitis from Immune Complex Deposition
Normal PT, Normal PTT, Normal Platelet Count |
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Henoch Schonlein Purpra
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Normal PT, Normal PTT, Normal Platelet Count
IgA Complement Deposition Induced Vasculitis following URI Purpuric Rash Colicky Abdominal Pain Polyarthralgia Acute Glomerulonephritis |
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Purpuric Rash
Colicky Abdominal Pain Polyarthralgia Acute Glomerulonephritis |
Henoch Schonlein Purpra
IgA Complement Deposition Induced Vasculitis following URI Normal PT, Normal PTT, Normal Platelet Count |
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Impaired Collagenous Support
[Not an Objective] |
Scurvy- Vitamin C Deficiency
Ehlers-Danlos Syndrome: Inheritied defects of Tyle 1 & Collagen: Joint laxity with easy bruising Cushing Syndrome: Corticosteroids cause protein wasting, loss of perivascular support Normal Collagenous Atrophy in the Elderly |
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Hereditary Hemorrhagic Telangiectasia
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AKA Osler Weber Rendau Disease
Tortuous Thin Walled Vessels Visible in Mouth, Under Nails No cappillary: A-->V Results in Mucosal Bleeds: Epistaxis, life threatening GI AVM in brain, lung, liver Will have iron deficiency anemia |
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Osler Weber Rendu Disease
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AKA Hereditary Hemorrhagic Telangiectasia
Tortuous Thin Walled Vessels Visible in Mouth, Under Nails No cappillary: A-->V Results in Mucosal Bleeds: Epistaxis, life threatening GI AVM in brain, lung, liver Will have iron deficiency anemia |
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Vascular Amyloid
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Systemic Amyloidosis: any organ usuallly skin of eyes; mostly primary AL from monocloncal plasma cells (multiple myeloma)
Bleeding with Normal PT, PTT and Platelet Count |
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Racoon Eyes
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Periorbital Bleeding, Think:
AL Type Systemic Amyloidosis from monoclonal plamsa cells (multiple myeloma) Bleeding with Normal PT, PTT and Platelet Count |
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Pinch Purpura
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Think:
AL Type Systemic Amyloidosis from monoclonal plamsa cells (multiple myeloma) Bleeding with Normal PT, PTT and Platelet Count |
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Cerebral Amyloid Angiopathy
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A-Beta amyloid accumulation in cerebral meningeal and cortical vessels
Family Tendency, may cause hemorrhage Bleeding with Normal PT, PTT and Platelet Count |
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Thrombocytopenia
Clinical Manifestations |
Plateletts <150k, Normal PT, Normal PTT\
Manifestations: Petechia, Spontanous BBleeding |
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PT Normal
PTT Normal Platelets 200k |
This is not a problem,
Platelets are within normal range |
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PT Normal
PTT Normal Platelets 100k |
Thrombocytopenia
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Hyperspenism Thrombocytopenia
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Splenic Sequestration
Normallly a reserve for 1/3 of platlets any splenomegally may cause thrombocytopenia Tx: splenectomy |
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Dilutional Thrombocytopenia
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Mass transfusion
eg 10 units you have essentially washed out all of their blood |
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Reduced Platelet Production
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Rx: ETOH, Thiazides
HIV Infxn of Megakaryocytes Pancytopenia: platelets, RBC's, Neutrophils all Down --aplastic anemia --myelophthisic disorders: bone marrow replaced by metastatic cancer, leukeima, lymphoma, myeloproliferatve diseases, granulomas --myelodysplasia:B12 folate deficiency, --Rx: CA chemo, ETOH |
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Platelets, RBC's, Neutrophils all Down
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Pancytopenia.
Causes: --aplastic anemia --myelophthisic disorders: bone marrow replaced by metastatic cancer, leukeima, lymphoma, myeloproliferatve diseases, granulomas --myelodysplasia:B12 folate deficiency, --Rx: CA chemo, ETOH |
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Thrombocytopenia in SLE
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Can have anti-platelet antibodies, type 2 hypersensitivity usually vs HPA Human Platelet Antigen membrane glycoproteins
Platelets Used up vs Immune Complex Deposition induced vasculitis |
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Neonatal Thrombocytopenia
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Maternal Antibodies vs Fathers HPA Human Platelet Antigen cross placenta, even in first pregnancy
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Autoantibody Causes of Thrombocytopenia
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2ndry Causes:
Rx-associated: >>Heprain<< Quinidine, Sulfonamides Infections: HIV, CMV, EBV Autoimmune: SLE Chronic Lymphocytic Leukemia Primary Cause: ITP Idiopathic Immune Thrombocytopenia Purpura Not a Dx until all other causes have been ruled out |
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ITP
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Idiopathic Immune Thrombocytopenia Purpura
Dx of Exclusion: Rx (heparin), Infectious, SLE, CLL IgG binds platelets, splenic phagocytosis, Spleen is normal Bone Marrow has increased megakaryocytes [Tx: do not give platlets, remove spleen] Chronic: insidious course Acute: childhood, abrupt onset 2 weeks after viral illness, mostly self limited, else progress to chornic |
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Bleeding
Normal PT Normal PT Thrombocytopenia BM: Increased Megakaryocytes |
ITP:Idiopathic Immune Thrombocytopenia Purpura
Dx of Exclusion: Rx (heparin), Infectious, SLE, CLL IgG binds platelets, splenic phagocytosis, Spleen is normal [Tx: do not give platlets, remove spleen] Chronic: insidious course Acute: childhood, abrupt onset 2 weeks after viral illness, mostly self limited, else progress to chornic |
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Englarged platelets
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Young Platelets: platelets are bine gused up faster
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Increased Megakaryocytes
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ITP:Idiopathic Immune Thrombocytopenia Purpura
Dx of Exclusion: Rx (heparin), Infectious, SLE, CLL IgG binds platelets, splenic phagocytosis, Spleen is normal [Tx: do not give platlets, remove spleen] Chronic: insidious course Acute: childhood, abrupt onset 2 weeks after viral illness, mostly self limited, else progress to chornic |
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HIT type II
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Heparin Induced Thrombocytopenia
5% of unfractionated heparin exposed pts Antibody to heparin-platelet factor 4 complex activates platelets --> life threatening intravascular thrombi Heparin must be discontinued PT: prolonged PTT: prolonged |
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PT: Prolonged
PTT: Prolonged Platelets: Rapid Drop Off 2nd week of Rx Therapy |
HIT type II
Heparin Induced Thrombocytopenia 5% of unfractionated heparin exposed pts Antibody to heparin-platelet factor 4 complex activates platelets --> life threatening intravascular thrombi Heparin must be discontinued PT: prolonged PTT: prolonged |
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Most Common Hematologic Abnormality in HIV
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HIV-associated thrombocytopenia
HIV infxn of megakaryocytes Immune destrx: anti-HIVgp120 cross react with gpIIb-IIIa |
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Thrombotic Microangiopathies
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TTP: Thrombocytic Thrombocytopenic Purpura
HUS: Hemolytic Uremic Syndrome Overlap Endothelial injury leads to vWF complexes and platelet aggregation forming microvascular thrombi & hyalin thrombi. mechanical lysis of RBC's into shistocytes Platelets are down Unconjugated Bilirubin is UP Haptoglobin is Down TTP differentiated by absence of vWF cleaving protease |
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Platelets are Down
Unconjugated Bilirubin is Up Haptoglobin is Down Schistocytes are present |
TTP: Thrombocytic Thrombocytopenic Purpura
HUS: Hemolytic Uremic Syndrome Overlap Endothelial injury leads to vWF complexes and platelet aggregation forming microvascular thrombi & hyalin thrombi. mechanical lysis of RBC's into shistocytes TTP differentiated by absence of vWF cleaving protease ADAMTS 13 |
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TTP
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Endothelial injury leads to vWF complexes and platelet aggregation forming microvascular thrombi & hyalin thrombi. mechanical lysis of RBC's into shistocytes
Platelets are Down Unconjugated Bilirubin is Up Haptoglobin is Down Schistocytes are present Absence of vWF cleaving protease ADAMTS 13 (present in HUS) Perfect Storm Pt: HIV+ Female 2 weeks into an infxn w/ classic pentad: Fever, Microangiopathic Hemolytic Anemai, Thrombocytopenia, Renal Failure, Transient Neurologic Defects Rapidly fatal: plasmapheresis to remove vWF |
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Fever
Schistocytes Petecheiae Proteinuria/Hemuria Neurologic Sx |
Classic Pentad for TTP
Endothelial injury leads to vWF complexes and platelet aggregation forming microvascular thrombi & hyalin thrombi. mechanical lysis of RBC's into shistocytes Rapidly Fatal: plasmapheresis to remove vWF |
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Epidemic HUS
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Mostly assoc with Shiga Toxin (vertoxin) of E Coli O157H7 in children & elderly with genetic predisposition;
Complement deregulation leads to Thrombocytic Microangiopathies: Endothelial injury leads to vWF complexes and platelet aggregation forming microvascular thrombi & hyalin thrombi. mechanical lysis of RBC's into shistocytes; like TTP without vWF protease deficiency Sx of TTP w/o Neurologic Invovlement: Fever Schistocytes Petecheiae Proteinuria/Hemuria (hyaline thrombi in glomeruli) Do not treat with antibiotics! |
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ADAMTS 13
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vWF cleaving protease
Normal in HUS, Deficiency results in TTP: Endothelial injury leads to vWF complexes and platelet aggregation forming microvascular thrombi & hyalin thrombi. mechanical lysis of RBC's into shistocytes |
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Diarrhea
Fever Schistocytes Petecheiae Proteinuria/Hemuria |
HUS: Mostly assoc with Shiga Toxin (vertoxin) of E Coli O157H7 in children & elderly with genetic predisposition;
Complement deregulation leads to Thrombocytic Microangiopathies: Endothelial injury leads to vWF complexes and platelet aggregation forming microvascular thrombi & hyalin thrombi. mechanical lysis of RBC's into shistocytes; like TTP without vWF protease deficiency |
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Deficiency of multiple clotting factors
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Generally Liver Failure
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Most common clotting factor deficiencies
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Factor 8 x linked
vWF autosomal dominant |
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X linked clotting factors
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8 & 9
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Autosomal dominant clotting factor deficiencies
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vWF
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autosomal recessive clotting factor deficiencies
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all except 8, 9 & vWF
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Vitamin K deficiency & clotting
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deficiencies of 7, 9, X, 2 & protein C
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Factor 8 in Circulation
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Small "Labile" molecule
very short halflife normally circulates bound to vWF to extend halflife |
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vWF disease
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synthesized by endothelium, circulates as multimer, stabalizes factor 8 in circulation, favors paletlet aggregation vai IIb-IIIa, interacts with glycoprotein Ib-IX in subendothelium
3 Types, only 2 of significance Type 1: autosomal dominant KO leaves you with one working copy; mildly excessive bleeding, normal PTT; Tx = desmopressin increases vWF prodxn Type 3: autosomal recessive, extremely low vWF, unable to stabalize 8. Acts like a Factor 8 deficiency, prolonged PTT, hemarthrosis; Tx = crypoprecipitate PT & Platelet count normal Bleeding time prolonged |
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PT normal
PTT normal Platelet Count normal Bleeding Time Prolonged |
vWF diesease
Type 1: autosomal dominant KO leaves you with one working copy; mildly excessive bleeding, normal PTT; Tx = desmopressin increases vWF prodxn Type 3: autosomal recessive, extremely low vWF, unable to stabalize 8. Acts like a Factor 8 deficiency, prolonged PTT, hemarthrosis; Tx = crypoprecipitate |
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large ecchymoses/hematomas
hemarthrosis bleed into weight bearing joints, GU, GI |
clotting factor deficiencies
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Hemophilia A
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most common serious hereditary bleeding disorder, X linked
of those with the most common serious form (<1% factor activity) 1/4 will develop anti-factor 8 antibodies large ecchymoses/hematomas hemarthrosis bleed into weight bearing joints, GU, GI ~2x/mo Key lab is prolonged PTT |
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Prolonged PTT
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Coagulation Deficiency
Most Likely Hemophilia A, Factor 8 Deficiency Else Factor 8 Deficiency Ashkenazi Jews = AR Factor XI deficiency |
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What is the most common serious autosomal factor deficiency?
What re the labs? |
AR Factor XI deficiency
Common in Ashkenazi Jews Prolonged PTT |
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Christmas Disease
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Hemophilia B
Factor 9 Deficiency X linked recessive PTT prolonged |
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Ashkenazi Jew Bleeding
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AR Factor XI deficiency
Prolonged PTT |
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DIC causes
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consumptive coagulopathy: systemic acitvation of coagulation pathways leads to thrombi throughout microcirculation
Causes: --tissue factor or thromboplastic substances enter circulation; 50% released from placenta --Sepsis, Meningococcemia: LPS, endotoxins induce IL1, TNF & activate endothlium --Noninfx inflmmatory disease eg SLE --Thermal/Crush Injuries: Heat Stroke, MVA -Malignancy -Major trauma esp involving brain --Misc: snake bite, vasculitis, liver disease, hemolytic transfusion reaction |
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DIC
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Disseminated Intravascular Coagulation
Death Is Comming consumptive coagulopathy: systemic acitvation of coagulation pathways leads to thrombi throughout microcirculation Widespread fibrin deposition in microciculation-->Ischemia and infarcts, esp in high blood flow areas: brain (Sheehan postpartum pituitary necrosis), heart, kidneys, adrenals (Menningococeemia --> Waterhouse-Friderichsen Syndrome) Fibrin Strands: microangiopathic hemolytic anemia Bleding diathesis: depletion of coagulation factors, increased fibrionlysis Labs: FDP (fibrin degredation products) and D-Dimer increased PT, PTT prolonged, Platelets decreased |
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PT Prolonged
PTT Prolonged Platelets Low FDP up D-Dimer up |
Disseminated Intravascular Coagulation
consumptive coagulopathy: systemic acitvation of coagulation pathways leads to thrombi throughout microcirculation Widespread fibrin deposition in microciculation-->Ischemia and infarcts, esp in high blood flow areas: brain (Sheehan postpartum pituitary necrosis), heart, kidneys, adrenals (Menningococeemia --> Waterhouse-Friderichsen Syndrome) Fibrin Strands: microangiopathic hemolytic anemia Bleding diathesis: depletion of coagulation factors, increased fibrionlysis |
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Waterhouse Friderichsen Syndrome
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Acute Hemorrhagic Necrosis of Adrenal Glands 2ndry to DIC induced by meningococcemia
Widespread fibrin deposition in microciculation-->Ischemia and infarcts, esp in high blood flow areas: brain (Sheehan postpartum pituitary necrosis), heart, kidneys, adrenals Labs: FDP (fibrin degredation products) and D-Dimer increased PT, PTT prolonged, Platelets decreased |