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112 Cards in this Set
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what is polycythemia
what are the types of polycythemia |
Increased RBC. Erythrocytosis, incerased Hb, Hct
1. Relative: Hct is increased bc plasma is decreased (seen in dehydration) 2. Absolute -Primary: EPO LOW -Secondary: EPO HIGH. plasma normal, RBC mass increases |
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what is the cause of relative polycythemia
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its when there is increase in HCT bc there is a decrease in plasma
dehydration, diarrhea, cholera, hanta virus |
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what is the normal breakdown of HCT and Plasma
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HCT- 45%
PLasma 55% |
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what is the Hct and Plasma in:
1. normal 2. secondary Erythrocytosis 3. Polycythemia vera 4. Relative polycythemia |
Normal: Hct, 45% PLasma 55%
2. Secondary Erythrocytosis: Hct high, Plasma normal 3. Polycythemia Vera: incerased Hct, Increased Plasma 4. Relative Polycythemia: Hct normal/high (NO increase in RBC mass), PLasma decerased |
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what is Gaisböck’s syndrome
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stress indices polycythemia
associated with HTN, obesity, smoking, stress/anxiety |
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what is
1. Polycythemia Vera 2. Absolute secondary Polycythemia **what is EPO level and what are the blood cells doing |
1. Polycythemia Vera- "vera" means true. RBC mass, Hct, and PLasma, PMN, platelets all increase. EPO is low. Jak2 Mutation. Myeloproliforative disorder
2. Absolute Secondary Polycythemia- EPO is high at appropriate times ex- high altitude, cyanotic heart disease, pulm disease OR inappropiate bc of renal cell carcinoma, Hepatocellular Ca, cerebellar hemangioblastoma |
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what type of polycythemia is due to JAK2 mutation. characterize it
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Polycythemia vera (absolute primary polycythemia)
myeloproliforative disorder EPO is LOW RBC mass, blood volume, PMN, plate ALL INCREASED (myeloid stem cells proliforate) |
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what is the polycythemia that is EPO independent but occurs bc the BM proliforates in the Absence of GF signal
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Polycythemia Vera
*EPO is low, JAK2 mutation makes it myeloproliforative *absolute primary polycythemia **RBC mass, plasma, PMN, plate all are increased (myeloid stem cells proliforate) |
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waht is the classification of polycythemia that occurs when the body is hypoxic and so the kidney sends out more EPO (as is normal)
what if you have polycythemia that has EPO high and you cant figure out why they would have high levels as their O2 is great! |
absolute SECCONDARY polycythemia
**recall the primary is "vera" and EPO is low **can also be a paraneoplastic syndrome- RO renal cell cancer |
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what is a paraneoplastic syndrome associated with renal cell carcinoma
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absoulte secondary polycythemia- renal cell carcinoma sends out lots of EPO
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what does hemorrhagic diatheses, what does diathesis mean
what are the 3 categories of bleeding diatheses |
makes you more prone or susceptible
1. Increased fragility of BV 2. platelet deficit 3. derangments of coagulation |
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what are the lab tests for bleeding disorders, what categories of disease can make you bleed
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PT, PTT, Platelet count ALWAYS
can also do, bleeding time, platelet studies, vWF **fragile BV *platelet problem *coagulation problem |
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if PTT is long whats the culprit
what about PT what about BOTH |
PTT- 12, 11, 9, 8
PT- TF, 7 Both: 10,5,2 |
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what test is long when the following is deficient
TF 2 5 7 8 9 10 11 12 |
PTT- 12,11,9,8
PT- TF, 7 BOTH: 10,5,2 TF 2- BOTH 5- BOTH 7- PT 8- PTT 9- PTT 10- BOTH 11- PTT 12- PTT |
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PTT stands for...
PT stands for |
PTT- thromboplastin time (11, 12, 8,9) intrinsic
PT- prothrombin time (TF, 7) extrinsic |
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what measures the extrinsic and common path PTT or PT
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PT- prothrombin time (TF, 7)
used to monitor coumadin |
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what measures intrinsic path
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PTT- 12,11,8,9
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what kinds of things can make you bleed bc the vessel wall is abnormal?
what is the PT- PTT and platelet |
vasculitis, bad collagen, defect in vessel, amyloid
PT, PTT, Platelet count all are normal |
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what are hte causes and consequences of vasculitis
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Cause: pathogens (meningiococcemia, rickettsia, DIC, infective endocarditis)
|
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meningiococcemia and rickettsia cause what?
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bleeding due to vasculitis (PT, PTT, platelets are normal)
**DIC and infective endocarditis can also cause bleeding bc of BV abnormalities |
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when might you see a subengual hemmorhage/? what might be another clinical manifestation ocaused by the same thing
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infective endocarditis
**they are splinter hemmorhages in the nail bed **bleeding bc of BV disorder (vasculitis) **can also get petechiae in the legs and other places |
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what does meningiococcemia look like
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get a rash
*vasculitis that causes hemmorages |
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tell me about immune/inflammatory vasculitis
causes |
immune complex deposition in BV
IC from: 1. RA, SLE 2. hypersensitivity to drug, aka leukocytoclastic vasculitis 3. Unknown: henoch-schonlein purpura |
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whats leukocytoclastic vasculitis
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vasculitis associated with hypersensitvities to drugs
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whats Henoch-Schonlein Purpura
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-palpable purpura from unknown AG that makes complexes and causes vasculitis
IgA complex deposits in BV Seen aafter URI -rash, colicky pain, polyarthralgia, glomerulonephritis |
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yo man with previous URI in in the hospital with fever, symmetric polyarthritis, rash, abd pain, and hemeuria
waht can be the cause |
Vasculitis caused by IgA complex deposition
Henosch Schonelin |
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The patient, a 54 y/o man with long history of
alcohol abuse, cirrhosis of the liver and pancreatitis was noted to have a purpuric macular eruption of the legs. • Purpura were nontender, 2‐3mm and associated with hair follicles. Hairs were twisted into corkscrews. • His gums were swollen with areas of hemorrhage. • Fecal occult blood was positive. • A nutritional deficiency is suspected. Diagnosis? |
Alcoholosm associated with thiamine, gums assoc with scurvy- vit C
**purpura with hair follicles, loss of vascular support. *the collagen is bad |
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a kid is suspected to be abused bc she AWLAYS is bruised, when the doc takes a look her fingers are hyper mobile, what is a better explaination for the bruising
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Ehler Danlos
*also will have stretchy skin *bruise bc BV are danaged easily *skeletal deformities **loss of collagenous support |
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what 4 conditions are associated with BV damage bc there is a loss of collagenous support
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1. Scurvy
2. Ehlers Danlos- collagen 1/3 defective so decreased vascular support --> fragility and easy trauma 3. Cushings: lots of corticosteroid, protein washing and loss of oerivascular support 4. OLD!!! **keep in mind this type of bleeding (caused by BV fragility) will ahve NORMAL labs |
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what is the purpura seen in the eldery as a result of normal aging/atrophy
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batemen, actinic
**BV fragility leads to hemorrhage, normal labs |
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Herediatary Hemmorhagic Telangiectasia
AKA inheritance Morphology Clinical |
1. called osler weber rendu
2. AD inheritance 3. tortuous thin walled vessels seen in mouth and under nails 4. Clinical- mucosal bleeds (nose, GI) petechiea on mouth, hands, organs |
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a kid comes in with frequent epitaxis that is NOT due to digital trauma. You note the kid has petechiea in the mouth. The dad also had frequent nose bleeds. what is the problem
(18 y/o male. Suffered from repeated episodes of epistaxis. Telangiectasias first noticed by a second physician consulted for anemia. Patient’s father had a milder, undiagnosed form of this disease.) |
heriditary telangiectasia (AD inheritance)
also called osler weber rendu **tortuous thin walled BV in mucosa. lots of hemmorages (BV reason for hemorrhare- normal labs) |
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A 67 y/o female has multiple telangiectasias on
her hands, arms, legs, lips and nasal mucosa. • She has had numerous episodes of epistaxis and GI bleeding since her mid‐20s. • Telangiectasias are seen endoscopically in her stomach and colon. • Her maternal family history is strong for similar lesions dx? inheritance |
herideitary telangiectasia- osler weber randeu
AD inheritanec |
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what are the types. locations and causes of vascular amyloid
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1. AL- multiple mylemoa (monoclonal plasma cells)
2. Cerebral Amyloid Angiopathy. ab amyloid accumulation, common in AD. build up in cerebral and cortical vessels |
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pinch purpura around the eye, nasal bridge is associated with what
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amyloid (congo red stain amyloid)
**see periorbital bleeding in systemic amyloidosis |
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can platelet problems cause bleeding
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you bet!
platelet deficit or dysfunction |
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what is thrombocytopenia
what is the count to be considered thrombocytopenic when do you have spontaneous bleed when do you ahve bleed with injury what is the normal fx of plateles |
- bleeding bc of decreased platelets
- platelets <150,000 is thrombocytopenia - <30,000 spontaneous bleeding - 30-50,000 post traumatic bleeding - PT and PTT are normal PETECNIAE Normal platelets Fx in primary hemastasis (platelet plug formatin) and as Phospholipid platform for coagulation |
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what is hypersplenism and dilutional thrombocytopenia
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Hypersplenism- when the spleen sequesters lots of platelets
Dilutional- when platelets are diluted by a MASSIVE transfuation |
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what are the clinical manifestations of thrombocytopenia
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Petechiae, ecchymosis, purpura
Mucosal bleeds Intracranial bleed |
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what are some cauess of thrombocytopenia
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decreased production
decreased survival sequestration (hypersplenism) dilutional (massive transfusion) |
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why might platelet production be decreased? whats in the ddx
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1. drug indiced- EtOH, thiazides
2. Infection (HIV) of megakaryocytes ddx includes pancytopenia pancytopenia is a decrease in: platelt, RBC, PMN |
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what can cause pancytopenia
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1. aplastic anemia (no RBC, PMN, plate)
2. myelopthisic disorders: BM is replaced (seen in cancer mets, leukemia/lymphoma, myeloproliforative disease, granuloma) 3. Ineffective magakaryocites: B12/folate defect 4. drug indiced |
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ok so production of platelets can be low bc of drugs, aplastic anemia, viral infection of megakaryocytes. what can cause decreased survival of platelets
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LOTS more things
1. Microangiopathic hemolytic anemia (DIC, TTP, HUS) 2. Giant Hemangiomas 3. Heart valves (prosthetic) 4. HTN 5. Sepsis IMMUNE MEDIATED 1. AB:AG complex 2. Antiplatelet AB |
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what is microangiopathic hemolytic anemia
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it includes DIC, TTP, and HUS and is a category od diseases that decrease platelet survival through mechanical (non immune) mechanism
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what are the mechanical causes of decreased platelet survival
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1. Microangiopathic Hemolytic Anemia (DIC, TTP, HUS)
2. Giant hemangiomas 3. prosthetic heart valves 4. Malignant HTN 5. sepsis |
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what are some immune mediated reasons platelet survival can be decreased
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1. immune complexes circulation can cause platelets to form thrombi
2. Antiplatelet AB |
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pts with SLE get thrombocytopenia why
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1 type II reaction against BV
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A 24 y/o P1G2 patient with uneventful pregnancy
and labor gives birth to a term infant, Apgars 9, 10 at 2 and 5 minutes. Neonatal exam of the infant demonstrates extensive petechiae and scattered purpura and ecchymoses. What lab tests would you order? |
petechiae and purpura indicated bleeding get PTT, platelets and PT for ALL BLEEDERS
PT normal PTT normal Platelet LOW **can be caused by mom AB attacking baby platelets- neonatal thrombocytopenia |
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what is the pathogenesis of neonatal thrombocytopenia
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Maternal AB against HPA (human platelet AG) on the baby that were inherited from dad
Ab crosses placenta to baby Immune mediated platelet destruction by alloAB **can affect FIRST preg and others. |
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what are the causes of secondary autoimmune thrombocytopenia
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1. drugs- heparin, quinidine, sulfa
2. Infections- HIV, CMV, mono 3. Autoimmune disease- SLE (type II HS rxn) 4. Chronic Lymphocytic Leukemia |
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whats going on in primary idiopathic thrombocytopenia (IPT)
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IgG targets non specific epitopes on platelets (perhaps AB to GPIIBIIIa)
splenectomy will work as tx bc you are removing the source of platelet removal |
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campare acute ITP (idiopathic thrombocyto) and chronic ITP
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ACUTE: common, childhood, M=W, self limited!
CHRONIC: males, less than 40, petechia/ecchymoses w/o truauma, hx of nose bleed, increased risk for intracranial hemorrhage, normal spleen, EXCLUDE SECONDARY CAUSE of bleedign. Tx with immunosuppression (cortocosteroids, IVIg, anti CD20). common to relapse |
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whats the BM and clinical presentation of idiopathic thrombodytopenia (primary)
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BM: increased megakaryocytes, they may be immature, they are atypical
Petechiae large thrombocytes seen on peripmeral smear. Abrupt onet after viral infection. self limited. pupura, mocosal bleeding. Tx with IV Ig, steroids Spleen is normal in size (compared to how huge it is in spherocytosis nad chronic) |
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what does it mean when you see megathrombocytes
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that there are young platelets
can be ITP |
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A 27 year old woman presents with petechial
rash over the legs and epistaxis. • She had delivered of two babies uneventfully and had no other hospitalizations. • Three weeks before presentation she suffered an influenza‐like illness that lasted 5 days. She recovered and then became fatigued 4 days ago at which time she noticed the “rash”. • She took no medication • Petechiae and ecchymoses were noted. |
Influenza tells us its AB mediated- ACUTE PRIMARY IDIOPATHIC thrombocyto
petechiae, test for PT PTT and platelet (ALL bleeders get this) PT, PTT normal PLatelts low Immune mediated primary idiopathic test for HIV, SLE, adn BM to RO other cause. exclude causes of secondary ITP |
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Child brought by mother after pickup from day
care where she feared that he had been abused. • His only abnormalities were superficial bruises. • Platelet count was 1000/μL (normal >150,000). • Increased megakaryocytes were present in his otherwise normal bone marrow aspiration. • Past history is treatment for sinusitis, conjunctivitis and bronchitis 2 weeks earlier. • What is the diagnosis? • Prognosis? How does prognosis compare with previous case. |
platelets- low
Hx of infection-- AB mediated destruction of platelets Likely to get over it (often self limited), tx with corticosteroids and IVIg |
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what are the labs in ITP
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PT, PTT normal
platelet decreased BM megakaryocytes increased Antiplatelet AB present EXCLUDE causes of secondary ITP |
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what is the pathogenesis of HIT type II
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Heparin Indiced thrombocyto
heparin is given and the body makes AB to heparin, the AB activates platelets adn forms intravascular thrombi, all of these thrombi are life threatening |
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what are the clinical features of HIT type II
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heparin indiced thrombocyto
-onset 5-14 days after heparin is given - 50% decrease in platelets - AB to hepatin is found - AB activates platelets and we get thrombi formed, this clot fomration is why our platelet count is low |
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what is type I HIT
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most common, seen with unfractionated heparin
platelets aggregate but dont form clots :) |
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A 67 y/o man was admitted with diagnosis of
deep vein thrombosis and pulmonary embolus. He c/o SOB, chest pain, and hemoptysis. • Diagnosis of PE was established by V‐Q scan. • Patient was started on unfractionated heparin, 6000 units q4h after loading dose of 20,000 units. He was continued on heparin and oozing was noted from venipuncture sites on the 9th day What is the cause of thrombocytopenia what additional labs are needed what is the treatment |
heparin, get PT, PTT, platelets as baseline before therapy
9th day means its type II HIT platelets are normal on admission but 10,000 on day 9 PTT/PT prolonged on day 9 HIT type II Labs: BM? Tx: STOP heparin |
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what is the most common blood problem in HIV, whats the mech
|
thrombocytopenia
HIV infects megakaryocytes and so decreased production increased destruction bc of immune dysregulation Auto AB to gpIIbIIIa cross react with HIV gp120 |
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what causes thrombocytopenia bc of thrombotic microangiopathies
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TTP, HUS
CLinical: thrombocytopenia, intravascular thrombi, microangiopathic hemolysis |
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what is the pathogenesis of microangiopathic hemolytic anemias
what are clinical, lab, and morphological findings |
1. endo damage--> vWF released and makes platelets stick
2. platelets are tied up and so we get thrombocytopenia, the platelet aggregations for intravascular thrombi 3. mechanical lysis of RBC leads to microangiopathic hemolytic anemia TTP, and HUS see schistocytes |
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what is seen on smear that makes you think HUS and TTP (microangiopathis hemolytic anemai)
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schistocytes (decapitated RBC)
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what are schistocytes
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decapitated RBC seen in microangiopathic hemolytic anemia (HUS, TTP)
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what ar ehte 2 trpes of thrombotic microangiopathies
what are some common features |
o Thrombotic Thrombocytopenic Purpura (TTP)
o Hemolytic Uremic Syndrome (HUS) FEATURES oHyaline thrombi in any blood vessels in the body oShould see fragmentation of RBC’s (schistocytes) on peripheral smear due to hemolysis causes elevated bilirubin oMacrocytes with polychromasia oIncreased unconjugated bilirubin Decreased haptoglobin oEndothelial activation of von Willebrands factor |
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how are fragmented cells (schistocytes) formed in HUS nad TTP
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decapitated by fibrin
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waht is TTP
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Thrombotic Thrombocytopenic Purpura
Deficit in ADAMTS 13 (protease for vWF) sx: FAT RN goes crazy --> Fever Anemia (schistocytes/hemolysis) THrombocytopenia Renal Failure Neurologic signs FATAL if you dont do plasmaphoresis |
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thrombotic thrompocytopenic purpura are seen when?
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Following febrile illness. Its when ADAMT 13, the vWF protease, is broken and we get lots of pletelet thrombi form in vasculature. there are also schistocytes formed.
FAT RN Fever, Anemia (hemolysis/schistocytes) Thrombocytopenia, Renal failure, neurologic Needs plasmaphoresis to take out the clots |
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This patient was seen for menometrorrhagia.
She was anemic and underwent a diagnostic D&C at which time leiomyomata were discovered. Patient recovered uneventfully. • The next day, she suddenly developed a severe headache and became unable to speak although she understood simple commands. A right facial droop was noted. Lumbar puncture yielded xanthochromic, hemorrhagic CSF. She was febrile with blood pressure 150/80. • Petechial hemorrhages and multiple splinter hemorrhages were present. • CT of the head showed a tiny hemorrhage of the right parietal lobe and infarctions of the left frontal lobe and right cerebellar cortex UA: protein, 0-5 hyaline casts, RBC, WBC CBC: low Hb, platelets low, RBC morph- schistocytes, reticulocytosis 1 day later PT, PTT, fibrinogen- normal, ro DIC CHemistry- renal disease, mm degradation (AST, LDH, CPK) Whats the Dx Whats the treatment |
HA, speach problems- Neurologic (FAT RN)
Xanthochromic- blood, protein Hemorrhages- low platelets UA- indicated renal disease (FAT RN) Thrombotic Thrombocytopenic Purpura (TTP) (Fever, anemia, thrombocytopenia, renal, neurologic, schistocytes) Treatment: START ASAP, can be life threatening. Plasmaphoresis to take out vWF complexes TTP- ADAMT 13 (vWFprotease) is deficient so we get lots of platelet thrombi |
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what is the clinical feature of HUS and cause
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1 E coli O157H7- shiga toxin is toxic to endo
2. Sx: bloody diarrhea, acute gastroenteritis, then renal failure 3. NO AB!!! it makes it worse 4. Renal Failure predominated |
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in HUS and TTP we have renal failure, what is a good wat to tell them apart
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TTP has neufrologic signs
HUS- some E coli with shiga toxin |
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who gets HUS commonly?
is there a genetic predisposition |
HUS- E coli, shiga toxin
Common in kids and old Genetics: compliment regulation HUS- blood diarrhea, gastroenteritis, then its RENAL FAILURE **in HUS the shiga toxin damages endo so vWF is released and we get lots of intravascular thrombi formed, this is what clots up the glomerulus and leads to renal failure |
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After eating potato salad, a 71 y/o man
develops bloody diarrhea. In a few days, he is in acute renal failure with thrombocytopenia, and hemolytic anemia. His PT, PTT, are normal. He dies of myocardial infarction. Hyaline microthrombosis is noted in multiple organs including the renal glomeruli. Extensive petechiae are present in the epicardium. |
HUS- potato salad indicated E coli O157H7
MI- bc of thrombi Shiga toxin damages BV and so vWF makes clots. |
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what are some causes of HUS besides E coli with shiga
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radiation
drugs **will still get irreversible kidney damage |
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how are the microangiopathies treated
PPT HUS |
PPT- plasmaphoresis ASAP
HUS- NO AB!!! supportive |
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inheritance of clotting factors
X linked AD AR |
x- 8 9
dom- vWF recessive- ALL OTHERS **genetic deficit usually one factor affected **if more than 1 factor is affected its usually acquired (liver damage, vit K-7 9 10 2 protein C deficit, DIC) |
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what factors are affected with vit K deficit
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7 9 10 2 protein C
**coumadin can cause vit K deficit **in acquired deficits like this several factors are affected! if genetic usually a single factor other acquired: liver disease DIC |
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what are the causes of clotting factor deficits (4)
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1. Vit K deficit. 7 9 10 2 protein c (coumadin induced)
2. Liver disease 3. Genetic X linked- 8 9 AD- vWF Recessive- all others 4. DIC |
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what coagulation factor travels with vWF
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8, it extends the life of 8
**when vWF is deficient (AD) it can look like an 8 deficit (8 deficit is hemophilia A) |
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vonWillebrain Disease
1. Inheritance 2. Clinical features 3. labs 4. treatment |
1. autosomal dom
2. Clinical: spontaneous bleed from mucosa, excess blood from wounds, heavy menses, 3. LABS: NORMAL platelet count, PROLONGED bleed time 4. Tx: desmopressin, cryoprecipitate, NO aspirn |
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what is type I vWF diseae
what about type 3 |
1. most common, AD inheritance, mild bleeding. vWF decreased but PTT is normal. its a partial deficit, one allele is normal
3. SEVERE- autosomal recessive. its TOTALLY different acts as factor 8 deficit (vWF binds 8 to extend t 1/2) PTT is prolonged |
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This 5 year old boy was seen for a marked
tendency for bruising of the arms and legs. • He had an episode of epistaxis at age 3 that required hospitalization 1 unit transfusion with packed cells. • Family history: The mother, maternal grandfather and maternal greatgrandmother had episodes of abnormal bleeding‐epistaxis, easy bruising and menorrhagia. The mother had required 10 units of RBCs at delivery The family history suggests what pattern of inheritance? • Does the pattern of clinical bleeding suggest an abnormality of primary or secondary hemostasis? • What additional lab tests are indicated? • If the patient requires surgical intervention, what would be the treatment of choice for a bleeding complication |
inheritance suggests AD bc its in all generations
vWF disease, type I superficial bleeds, primary hemostasis PT, PTT, platelet, bleed time (expect bleed time to be long) Tx with desmopressin |
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what are the characteristics of clotting factor deficiencies (except vWF, its a bit different)
|
1. large ecchymoses/hematoma
2. hemarthrosis 3. prolonged bleeding 4. GI GU bleed **deep bleeds (recall vWF deficit was superficial mucosal bleeds) |
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An individual with hemophilia A is unable to
solidify the platelet plug formed following endothelial damage; this leads to abnormal bleeding. This condition is caused by a defect in the coagulation cascade. Which of the following elements of the coagulation cascade is defective in persons with hemophilia A? A) Factors II, VII, IX, and X B) Factor VIII C) Factor IX D) Lack of protein C E) Mutations in antithrombin III |
factor 8
A) Factors II, VII, IX, and X. VIT K B) Factor VIII- Hemophilia A C) Factor IX- hemophilia B "christmas disease" D) Lack of protein C- Thrombosis E) Mutations in antithrombin III- Thrombosis |
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Factor 8.9 deficit
1. Clinical 2. Inheritance 3. Labs |
8- hemophilia A **more common**
9, hemophilia B 1. Clinical: hemarthrosis, deep hematomas, "deep bleed", spontaneous hemorrhage, NO PETECHIAE 2. Inheritance: x linked recessive 3. LABS: PTT long in both. B will also have abnormal facoor 9 assay |
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wht is christmas disease
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hemophilia B, factor 9
|
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what is a factor 8 inhibitos
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anti factor 8 AB that develops in some pts with hemophilia A
x linked inheritance: seen in males with no fx |
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if 20-30 times a year you have spontaneous bleeds and deep hematomas with NO petechiae what can be the problem
|
hemophilia A or B
**no petechiea bc platelets are normal |
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in what disease can you see a pseudotumor 2 to hematoma
|
hemophilia, this gets DEEP bleeds and no petechais (nothing wrong with platelts)
can also get cystic remodling of glenohumeral joint (bone cyst bc of a hematoma in the shoulder) |
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tell me about hemarthrosis seen in hemophilia a and b
|
deep bleed into joints
cyctic deformity, hemosiderin makes it dark- lack of blood |
|
This 7 day old boy is seen for evaluation for a
bleeding disorder. • He was the product of a normal delivery to a healthy 22 year old primagravida. On the second day after delivery, he was circumcised and bled for 4 days, but received no specific treatment. • A maternal uncle was diagnosed with hemophilia A at age 18 months when a fall produced hematoma of cheek and eye. The uncle’s record shows a PTT of 88 sec (<42) and Factor VIII activity of <1%. • Patient received factor concentrates and was discharged. He continued to have frequent episodes of bleeding and required factor VIII concentrates twice a month. • Patient’s lab PT 12 seconds (10‐12) PTT 111 seconds (<42) • Factor VIII activity <1% • Mother‐Factor VIII activity 19% |
PTT is long in hemophilia
Platelet and PT are normal |
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how is hemophilia A treated
|
recombinant factor 8 is given prophylactically, not just when you start to bleed
|
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A 49 y/o male was seen for large hematoma
over anterior surface of tibia where he had been accidentally struck. • Past history: Hemorrhoidectomy, required 5 unit blood transfusion. • GI bleeding 2 yrs. later due to duodenal ulcer; ‐ gastrectomy, 11 units blood transfused. • GI bleeding due to ulcer at operative site; further surgery with 20 units transfused. • Bleeds profusely from dental extractions. Younger brother diagnosed with factor IX deficiency. ‐ History of recurrent GI bleeding with surgery; multiple transfusions. • One brother with no history of bleeding • One sister with excessive bleeding following nasal polypectomy that did not require transfusions. what do you think the labs are (PT, PTT, Platelet) what is the most likely dx what is the inheritance whats the confirmatory test |
PT, platelet normal
PTT long most likely: factor 8, 8 is more common than 9 X linked recessive (both 9 and 8) Confirm with factor 8.9 assay |
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whats the difference in clinical presentation btwn factor 8 adn 9 hemophilia
|
nothng
8 and 9 are BOTH x linked recessive |
|
Hemophliaa B
1. deficit 2. another name 3. inheritance 4. lab |
1 9
2. christmas disease 3. x linked recessive 4. long PTT, 9 assay abnormal |
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what ist he most common serious autosomal factor deficit?
wat are the labs |
factor 11 (vWF is a little more common)
common in ashkenazi jews PTT long, |
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A 65 year old woman bled for 10 days after a
dental extraction. One sibling died from postop hemorrhage; there is a history of bleeding in a number of relatives. PTT is markedly prolonged and bleeding time w/in normal limits. Diagnosis? a. factor VIII deficiency b. factor XI deficiency c. factor XII deficiency d. factor IX deficiency e. von Willebrand’s disease |
factor 11
Female so not 8 or 9 Bleed time normal so not vWF |
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an Ashkenazi Jewi has increased PTT, what is a common autosomal coagulation factor deficit they might have
|
11
|
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tell me about DIC
|
thrombo-hemorrhagic disorder
**systemic activation of coagulation leads to thrombi throuought microcitculation **fibrinolysis is activated, d dimer is high **common as a secondary complication of lots of diseases |
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what are 2 mechs that trigger DIC (systemic activation of coagulation cascade)
|
1. Release of tissue factor or thromboplastic substances into circulation
-released from placenta, cancer, mucin, 2. Eidespread endo damage -sepsis - endotoxins (LPS) exotoxins activate endo also heat stroke |
|
DIC
1. clinical 2. lab 3. cause |
1. Clinical: thrombic/hemorrhage. ischemia, microangiopathic hemolytic anemia
2. Lab: FDP and d Dimer increased 3. Cause: sepsis, obstretics, cancer, trauma, snake bite, vasculitis, liver disease hemolytic transfusion reaction |
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what is one of th emost common causes of DIC
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obstretics (premature seperation of placenta, amniotic embolus, retained dead featus)
sepsis cacner, trauma, snake bite, vasculitis, liver disease, hemolytic transfusion reaction |
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when do you see Purpura fulminans
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DIC of meningiococcemia (N meningitidis)
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what is the pathogenesis of DIC
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massive tissue injury, sepsis, or endo injury all cause release of tissue factor
tissue factor leads to widespread microvascular thrombosis this leads to: 1. plasmin activation --> fibrinolysis --> icnreased d dimer --> bleeding 2. vascular occlusion --> ischemic tissue damage BLEEDING |
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what might be the reason someone has micothrombi in arterioles and capillaries of kidney, adrenal, brain, heart
waterhouse Friderichensen syndrome |
DIC
Waterhouse Friderichesen- BL hemorrhage of adrenals |
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what is chronic DIC due to
what is Acute DIC due to |
CHRONIC: cancer, recurrent DVT, no bleeding diathesis
ACUTE: lots of bleeding that wont clot, can lead to shock, acute renal failure, ARDS, circulatory failure |
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76 y/o female admitted with fever and vomiting.
She was aphasic from a previous CVA (stroke) and fed via a PEG tube. She had been treated by her caretaker daughter for a urinary tract infection. • She was cold and clammy on admission to the hospital. Her temperature was 97oF, BP 101/42, respirations 18 and heart rate 133. • She had no urinary output and remained oliguric despite IV fluids. Hemodialysis started. She experienced respiratory distress later on the day of admission and was placed on a ventilator. Acrocyanosis developed. • She continued to deteriorate and was pronounced dead of multiple organ system failure due to septic shock within 48 hours of hospitalization. Blood pH 7.05 (7.2‐7.4) • Coagulation tests revealed prolonged PT, PTT and decreased platelets • AST, ALT, BUN, Creatinine, Bilirubin, and CK were all elevated • WBC 28,000 5000‐10,000 /dL • UA showed heavy branched and budding yeast, 2+ bacteria, > 200 WBCs/hpf, 10‐12 RBCs/hpf What is the cause of her abnormal coagulation tests? What is her diagnosis? |
COagulation: acute DIC that progressed into septic shock
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This 15 y/o boy presented with fever, tachycardia,
and tachypnea that rapidly progressed to unconsciousness within hours. • He was admitted to intensive care unit in septic shock with a temperature of 40.2oC, pulse180/min. and BP 70/45 mmHg. PT was 90 seconds (~12 sec.). • Petechia and purpura developed and spread on his face, arms and legs. Hands and feet were cool and cyanotic. Neisseria meningitidis was subsequently confirmed by biopsy of skin lesions and blood culture. • What is the diagnosis? • What serious complication is associated with this disease? • He recovered but required amputation of both forefeet and seven fingers. With reconstructive surgery and orthopedic shoes, he is able to walk and use remaining fingers in a pincer grip. |
meningiococcemia
**can loose a finger or toe |
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what is it called when your adrenal glands die in meningiococcemia
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waterhouse friderichsen
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A 57 year old alcoholic male was admitted to the
hospital for non‐life threatening injuries sustained in a fall. He was known to have cirrhosis of the liver. • He developed a fever on his second hospital day and blood cultures were positive for S. aureus. He was placed on antibiotics. Shortly thereafter, oozing was noted from venipuncture sites and the wounds from his fall. Admission 2nd Day Reference PT 14 seconds 17 seconds (10‐12 seconds) PTT 39 seconds 43 seconds <42 Platelet count 90,000/mm3 35,000 >150,000 Confirmatory tests: Fibrinogen 100mg/dl 170‐410mg/dl • Septicemia Associated endothelial damage Macrophage activation: release of tissue factor • Liver unable to clear activated coagulation factors • Decreased production of coagulation factors FDP >100μg/ml <10 μg/m |
PT, PTT increase on 9th day adn plalet droped
fibrinogen increases and FDP dropped |