Notes in LE031 TMAs-SLD

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Published	10/29/2024	microangiopathy = disease of {{c1::small blood vessels}}thrombotic microangiopathy (TMA) = thrombosis {{c1::of small vessels }}
Published	10/29/2024	{{c1::thrombotic microangiopathies}}, characterized by:microangiopathic hemolytic anemia consumptive thrombocytopeniamicrothrombieneurologic dysf…
Published	10/29/2024	microangiopathic hemolytic anemia (test and cause):{{c1::non-immune hemolysis indicated by negative Coombs test (direct antiglobulin test)results from…
Published	10/29/2024	schistocytes are indicative of {{c1::microangiopathic hemolytic anemia (intravascular shearing/fragmentation of RBCs)}}, which can be caused by {{c1::…
Published	10/29/2024	RBC categorization by size:microcytic (x4):{{c1::iron deficiencyanemia of inflammationthalassemiasideroblastic anemia}}normocytic (x4):{{c2::anemia of…
Published	10/29/2024	anemia categorization based on reticulocyte response (production index threshold of {{c1::2 - BM prod sufficient # reticulocytes AKA premature RBC in …
Published	10/29/2024	which categories of anemia could be suggested based upon reticulocyte count or RBC size in blood smears (so x2 for each) :iron deficiency: {{c1::hypop…
Published	10/29/2024	intravascular RBC breakdown in blood vessels (x4):{{c1::very high LDH (destroyed RBCs release lactate dehydrogenase)very low haptoglobin (destroyed RB…
Published	10/29/2024	intravascular RBC destruction of blood vessels indicated by very high LDH, very low haptoglobin, high reticulocyte count, high indirect bilirubin coul…
Published	10/29/2024	extravascular RBC destruction in spleen indicated by high LDH, low haptoglobin, high reticulocyte count, very high indirect bilirubin suggests (x4 dis…
Published	10/29/2024	non-immune hemolytic anemia (x6):{{c1::hereditary spherocytosisG6PD deficiencysickle cell diseasethalassemiathrombotic microangiopathydisseminated int…
Published	10/29/2024	DAT: positive; IgG+, C3- would be associated with what anemia?
Published	10/29/2024	DAT: positive; IgG-, C3+ would be associated with what anemia?
Published	10/29/2024	blood smear containing RBC spherocytes would be associated with what anemia?
Published	10/29/2024	blood smear containing RBC agglutination(clumps of RBCs) would be associated with what anemia?
Published	10/29/2024	medications, autoimmune disease, and lymphoma could cause which type of anemia?
Published	10/29/2024	epstein-barr virus, mycoplasma, lymphoma could cause which type of anemia?
Published	10/29/2024	what is on your differential? what's the first lab you order? what if that first lab indicates anemia? indicates thrombocytopenia? then what lab…
Published	10/29/2024	what do these lab test results indicate?
Published	10/29/2024	what do you see on blood smear? what could be diagnosis?
Published	10/29/2024	Schistocyte is indicative/marker of {{c1::RBC fragmentation }} due to:{{c1::MAHA (microangiopathic hemolytic anemia) - from direct trauma to RBCs…
Published	10/29/2024	what are the 3 possible contributors to microvascular thrombosis (that leads to RBC hemolysis in thrombotic microangiopathy)?
Published	10/29/2024	what are the 3 major causes of TMA (thrombotic microangiopathy)?
Published	10/29/2024	{{c1:: TTP (thrombotic thrombocytopenic purpura)}} is characterized by:feverMAHA (microangiopathic hemolytic anemia)thrombocytopeniaacute kidney …
Published	10/29/2024	TTP is caused by {{c1::formation of excessive platelet-vWF plugs}}
Published	10/29/2024	primary hemostasis and formation of platelet plugs:normally - {{c1::vWF}} unravel and bind to {{c1::platelets}}, tether them to {{c1::subendothelial c…
Published	10/29/2024	normal platelet-vWF plug formation:1) vWF are released as {{c1::ultra-large vWF (ULvWF) multimers}}2) {{c1::ADAMTS13 (metalloproteinase)}} is enzyme t…
Published	10/29/2024	what are the consequences of microvascular thrombosis (caused by excessive vWF-platelet plug formation from UL vWF) in TTP? (x3){{c1::thrombosis --&gt…
Published	10/29/2024	Clinical presentation of {{c1::TTP}}Lab test to order is {{c1::ADAMTS13}} and next stage of management/treatment is {{c1::plasmapheresis}}
Published	10/29/2024	PLASMIC score for TTP - probability that {{c1::ADAMTS13}} activity <10%
Published	10/29/2024	Lab results of MAHA and consumptive thrombocytopenia:confirming non-immune mediated hemolysis:{{c1::reticulocytosis (}}high {{c1::reticulocyte count)}…
Published	10/29/2024	Altogether thrombocytopenia, hemolytic anemia, schistocytes in blood smear --> indicate TTP until proven otherwise i.e., if abnormal levels of&nbsp…
Published	10/29/2024	TTP management via (x3){{c1::plasma exchange - removes anti-ADAMTS13 antibody to replace ADAMTS13 enzyme in blood which is not inhibited (**cornerston…
Published	10/29/2024	indicates what disease and what should be treatment?
Published	10/29/2024	NOTE this TTP content is all acquired TTP (aTTP) which constitutes 90% of TTP cases vs congenital TTP
Published	10/29/2024
Published	10/29/2024	what is hemolytic uremic syndrome characterized by? (x3)
Published	10/29/2024	mechanism of action of hemolytic uremic syndrome (HUS) - 3 steps:{{c1::bacterial exotoxins bind capillary endotheliumcell uptake of exotoxin induces e…
Published	10/29/2024	in HUS (hemolytic uremic syndrome):{{c1::shiga toxin-producing E. Coli (STEC) O157:H7}} causes direct damage to {{c1::renal}} arterioles and capillari…
Published	10/29/2024	symptoms/timeline of E. coli O157:H7 infection:3 days after ingestion (undercooked food, unpasteurized milk/products) --> (x4) {{c1::diarrhea, abdo…
Published	10/29/2024	HUS treatment (x2){{c1::supportive care: RBC transfusion, hydration IV fluidsNO antibiotics - they don't help and may worsen disease}}
Published	10/29/2024	order {{c1::bacteria culture test for E. coli}}, diagnose with {{c1::HUS}}, treat with {{c1::RBC transfusion}} and {{c1::IV fluids}}
Published	10/29/2024	if patient with hemolytic anemia, renal damage, thrombocytopenia doesn't get better with plasma exchange, indicates that it is not {{c1::TTP}} but rat…
Published	10/29/2024	usually {{c1::acquired TTP (not congenital)}} presents in adults vs {{c1::HUS}} can present in children
Published	10/29/2024	in patients with {{c1::HUS}}, ADAMTS13 activity >10% aka normal vs in {{c1::TTP}} ADAMTS13 activity <10%
Published	10/29/2024	{{c1::atypical HUS (aHUS)}} patient might have hemolytic anemia, renal damage, thrombocytosis BUT does not have diarrhea (rules out {{c1::classical HU…
Published	10/29/2024	aHUS pathogenesis = complement-mediated endothelial cell injurynormally:1. {{c1::complement}} goes to pathogens and forms {{c1::MAC (membrane-attack c…
Published	10/29/2024	aHUS treatment:{{c1::eculizumab - monoclonal ab against C5 (terminal complement protein that is essential to MAC formation) by inhibiting C5 convertas…
Published	10/29/2024	distinct clinical features of:TTP {{c1::CNS}} abnormalities {{c1::responds}} to plasma exchange therapy{{c1::low <10%}} activity levels o…
Published	10/29/2024	pathogenesis of TTP{{c1::autoimmune ADAMTS13 inhibitor}} -> {{c1::reduced ADAMTS13 activity (cleaving UL vWF multimers)}} -> {{c1::excess/persis…
Published	10/29/2024	diagnosed with {{c1::aHUS}}, treatment with {{c1::eculizumab}}, see improvement seen via {{c1::platelet}} count and {{c1::renal}} function
Published	10/29/2024	MAHA with abnormal coagulation tests = {{c1::disseminated intravascular coagulation (DIC) }}
Published	10/29/2024
Published	10/29/2024	disseminated intravascular coagulation (DIC) is due to {{c1::abnormal activation of coagulation cascade}} + {{c1::hyperfibrinolysis}}
Published	10/29/2024	laboratory features/results for DIC:abnormal {{c1::coagulation tests: PT (>13s), PTT (>35s), IRN}}high {{c1::D-dimer (formed due to fibrinolysis…
Published	10/29/2024	disseminated intravascular coagulation (DIC) treatment (x3):{{c1::supportive care - keep fibrinogen above 150treat underlying disease (e.g., infection…
Published	10/29/2024	diagnosis is {{c1::DIC}}, treat the underlying {{c1::sepsis}} + supportive care with prophylactic {{c1::anticoagulation}} and {{c1::blood products to …
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