Notes in 06 Hemolytic Anemias

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Published	12/08/2023	{{c2::Thrombotic thrombocytopenic purpura (TTP)}} is due to inhibition or deficiency of the {{c1::ADAMTS13 (vWF metalloprotease)}} enzyme
Published	12/08/2023	What demographic is associated with thrombotic thrombocytopenic purpura? {{c1::Adult females}}
Published	12/08/2023	The pentad of classical symptoms seen in {{c6::thrombotic thrombocytopenic purpura (TTP)}} may be remembered using the mnemonic FAT RN: F: {{c1::Fever…
Published	12/08/2023	What is the treatment (3) for thrombotic thrombocytopenic purpura (TTP)? {{c1::plasmapheresis}}, {{c2::corticosteroids}}, {{c3::rituximab}}
Published	12/08/2023	{{c1::Disseminated intravascular coagulation (DIC)}} occurs due to pathologic activation of the coagulation cascade
Published	12/08/2023	Disseminated intravascular coagulation (DIC) is characterized by widespread {{c1::microthrombi}} formation, resulting in ischemia and infarction
Published	12/08/2023	In disseminated intravascular coagulation, consumption of platelets and factors results in {{c1::bleeding}}
Published	12/08/2023	What type of red blood cell is associated with disseminated intravascular coagulation? {{c1::Schistocytes (due to microangiopathic hemolytic anemia)}}
Published	12/08/2023	Pyruvate kinase deficiency presents as hemolytic anemia {{c1::without::with or without}} Heinz bodies
Published	12/08/2023	Anemia is classified as {{c1::normocytic}} when the MCV is between {{c2::80}} to {{c2::100}} μm3
Published	12/08/2023	A normal reticulocyte count is between {{c1::1}} to {{c1::2}}%
Published	12/08/2023	Some of the hemoglobin in the blood due to intravascular hemolysis is returned to the spleen by {{c1::haptoglobin}} molecules
Published	12/08/2023	Normocytic anemia with {{c2::intra}}-vascular hemolysis is associated with {{c1::decreased}} serum haptoglobin
Published	12/08/2023	Days after an episode of {{c2::intra}}-vascular hemolysis, {{c1::hemosiderin}} may be seen in the urine
Published	12/08/2023	What enzyme may be elevated in hemolytic anemia (both extra- and intravascular)? {{c1::Lactate dehydrogenase (LDH)}}
Published	12/08/2023	Extra- and intravascular hemolysis may present with increased {{c1::urobilinogen}} in the urine, which results from increased conjugated bilirubin
Published	12/08/2023	Does extravascular hemolysis present with hemoglobinuria and/or hemosiderinuria? {{c1::No}}
Published	12/08/2023	What is the treatment for severe or refractory hereditary spherocytosis? {{c1::Splenectomy}}
Published	12/08/2023	What are the three main conditions which precipitate sickling in sickle cell anemia? {{c1::Hypoxemia}}, {{c2::dehydration}}, and {{c3::acidosis}}
Published	12/08/2023	Sickle cell anemia is treated with {{c1::hydroxyurea}} and hydration
Published	12/08/2023	Hemoglobin C disease is primarily characterized by {{c1::extravascular}} hemolysis
Published	12/08/2023	{{c2::Paroxysmal nocturnal hemoglobinuria (PNH)}} is caused by an acquired defect in {{c1::hematopoeitic}} stem cells, resulting in absent {{c3::GPI}}
Published	12/08/2023	Paroxysmal nocturnal hemoglobinuria is primarily characterized by {{c1::intravascular}} hemolysis
Published	12/08/2023	{{c1::Paroxysmal nocturnal hemoglobinuria}} may be confirmed via {{c3::flow cytometry}}, which detects a lack of CD-{{c2::55}} and CD-{{c2::59}}
Published	12/08/2023	What is the mode of inheritance of G6PD deficiency? {{c1::X-linked recessive}}
Published	04/17/2024	G6PD deficiency renders RBCs susceptible to {{c1::oxidative}} stress
Published	12/08/2023	In G6PD deficiency, Heinz bodies are removed from RBCs by splenic macrophages, resulting in formation of {{c1::bite}} cells
Published	12/08/2023	What is the most common enzymatic disorder of RBCs? {{c1::G6PD deficiency}}
Published	12/08/2023	The {{c1::direct}} Coombs test adds {{c2::anti-human globulin (Coombs reagent)}} to a sample of a patient's {{c3::RBCs}}
Published	12/08/2023	The {{c1::direct}} Coombs test answers the question: "Are the patient's RBCs already bound by immunoglobulin (or complement)?"
Published	12/08/2023	The {{c1::indirect}} Coombs test adds {{c2::donor blood}} and {{c3::anti-Ig antibody (Coombs reagent)}} to the patient's {{c4::serum}}
Published	12/08/2023	The {{c1::indirect}} Coombs test answers the question: "Does the patient have antibodies in their serum?"
Published	12/08/2023	Microangiopathic hemolytic anemia (MAHA) is primarily characterized by {{c1::intravascular}} hemolysis
Published	12/08/2023	{{c3::Macroangiopathic hemolytic}} anemia may be seen with prosthetic {{c1::heart valves}} and aortic {{c2::stenosis}}
Published	12/08/2023	What pathological RBC is associated with micro- and macroangiopathic hemolytic anemia? {{c1::Schistocytes ("helmet cells")}}
Published	12/08/2023	Which type of gallstone is associated with extravascular hemolysis? {{c1::Bilirubin (pigment) stones}}
Published	12/08/2023	{{c2::Hemolysis}} is associated with dark urine due to increased urine {{c1::urobilinogen}}
Published	12/08/2023	{{c1::Kernicterus}} is the deposition of bilirubin in the brain, especially basal ganglia, causing neurologic deficits and possibly death
Published	12/08/2023	What glycolysis enzyme deficiency causes hemolytic anemia in a newborn?{{c1::Pyruvate kinase deficiency}}
Published	12/08/2023	When the "Shiga-like" toxin of Enterohemorrhagic E. Coli (EHEC) enters systemic circulation, what occurs?{{c1::Hemolytic uremic syndrome}}
Published	12/08/2023	What demographic is hemolytic uremic syndrome (HUS) most common in?{{c1::Children < 10 y.o.::age}}
Published	12/08/2023	Hemolytic disease of the newborn is also known as {{c1::Erythroblastosis fetalis}}
Published	12/08/2023	4 day old baby with total bilirubin level 21 → {{c1::phototherapy::next step}}
Published	12/08/2023	Heinz bodies and bite cells{{c1::G6PD deficiency::condition}}
Published	12/08/2023	{{c3::Hereditary spherocytosis}} will show {{c1::decreased}} mean fluorescence of RBCs in {{c2::eosin 5-maleimide (EMA) binding}} test
Published	12/08/2023	What test is used for Rh typing and antibody screening? {{c1::Indirect coombs test}}
Published	12/08/2023	(+) Coombs test is suggestive of {{c1::autoimmune hemolytic anemia::condition}}
Published	12/08/2023	Warm agglutinin = {{c1::IgG::IgM/IgG}} autoimmune hemolytic anemiaCold agglutinin = {{c1::IgM::IgM/IgG}} autoimmune hemolytic anemia
Published	12/08/2023	{{c1::DIC}} is when the patient has uncontrolled bleeding and clotting simultaneously
Published	12/08/2023	G6PDH deficiency results in lysis of RBCs because of {{c1::ROS buildup}}
Published	12/08/2023	Hemolytic anemia presents with {{c1::increased}} reticulocyte count
Published	12/08/2023	Some of the heme in the blood due to intravascular hemolysis is returned to the liver by {{c1::hemopexin}} mol…
Published	12/08/2023	Both warm and cold AIHA can be treated with {{c1::rituximab}}
Published	12/08/2023	Steroids and splenectomy are only effective at treating {{c1::IgG}}-mediated hemolytic anemia
Published	12/08/2023	Mitapivat is a new drug that can treat {{c1::pyruvate kinase deficiency}}
Published	12/08/2023	What pathologic RBC is seen with storage of packed RBCs?{{c1::Echinocyte ("burr cell")}}
Published	12/08/2023	Acute hemolytic transfusion reaction occurs within {{c1::24}} hours of transfusion
Published	12/08/2023	Iron from hemoglobin is stored as hemosiderin in {{c1::proximal tubule cells::location of nephron}} which can lead to hemosiderinuria
Published	12/08/2023	Raynaud’s phenomenon is seen in {{c1::cold::warm/cold}} autoimmune hemolytic anemia
Published	12/09/2023	Sickle cell anemia is the result of a polymorphism where a {{c1::glutamate (polar acidic)::amino acid}} is replaced by a {{c2::valine (nonpo…
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