Anemic/Clotting Disorders Flashcards
General pathophysiology of each major anemia and clotting type covered in BiB
How do Thalassemias lead to anemia?
LOW RBC COUNT via inefficient erythropoesis and increased hemolysis of defective RBCs containing excess globins (alpha, beta, gamma tetramers).
Where are red blood cells hemolyzed?
Predominately in the spleen (liver and bone marrow also)!
Where is the spleen located? What kind of pain can be associated with it? (2)
LUQ, anterior to stomach. Abdominal pain (stomach). Upper shoulder (upon deep breath)
What can cause hepatosplenomegaly?
Increased RBC hemolysis. Increased production of RBCs (extramedullary hematopoesis?).
When a RBC is lysed, released hemoglobin becomes heme. What does heme break down to? What can that lead to clinically?
Indirect Bilirubin –> Jaundice, scleral icterus, gallstones
Iron –> 2ndary Hemochromatosis
What stimulates extramedullary hematopoesis?
Low RBC’s will lead to low O2 delivery. This will make the body think it’s in a hypoxic state, releasing EPO and stimulating extramedullary hematopoesis.
How can intravascular hemolysis affect urine?
Hematuria, hemoglobinuria, dark red urine.
Between intravascular and extravascular hemolysis, which will affect urine?
Intravascular Hemolysis
In what type of vasculature does HgbS commonly cause vaso-occlusion? What could that lead to?
Cerebral –> Stroke
Respiratory –> Acute Chest Syndrom (ACS)
Renal –> hematuria, proteinuria
Penile –> Priapism
Extremities–> Dactylis
What risks do chronic blood transfusions carry?
2ndary hemochromatosis or allogeneic immune response
What is the purpose of a (Direct) Coomb’s Test?
A test that is used to detect hemolytic antibodies or complement proteins already bound to RBCs.
What is an indirect Coomb’s test?
A test for detecting unbound anti-RBC antibodies in the serum.
How would haptoglobin levels be affected by intravascular hemolysis? extravascular hemolysis?
intravascular –> low haptoglobin levels (more free Hgb in blood to bind)
extravascular –> somewhat normal haptoglobin levels (less free Hgb in blood to bind)
What type of hemolysis (extra, intra?) will Sickle Cell Disease lead to?
Extravascular hemolysis. HbS will be eaten up by spleen.
What enzymatic reaction does aminolevulinate dehydratase catalyze?
aminolevulinic acid to porphobilinogen
Bloody Diarrhea is indicative of what type of virus?
E.coli, shiga toxin.
What is the function of ADAMTS13?
To cleave and inactivate vWF.
What is a major difference between HUS and TTP?
TTP can have arterial blockage to the brain as well as the kidneys, causing neurologic symptoms. HUS has arterial blockage primarily in the kidney, no neurologic symptoms.
What abnormality will a peripheral smear show you in immune thrombocytopenic purpura (ITP)?
decreased platelet count
Patients with a splenectomy or splenic dysfunction are susceptible to infection because…?
Decreased production of IgM and memory B-cells.
parvovirus B19
How can sickle cell affect the spleen?
Spleen can become dysfunctional.
What long-term prophylactic is usually given after a patient receives Heparin for suspected embolism?
Warfarin
What is a big indication in which you should you suspect lead poisoning?
A person’s WORKPLACE/LIVING SITUATION is mentioned. Do they work/live where lead/metal exposure is a risk factor?
What are good distinguishers of Paroxysmal Nocturnal Hemoglobinuria? (3)
Anemia, Venous Thrombosis, and PANCYTOPENIA!
Stillbirths can be attributed to which clotting disorder?
Antiphospholipid Syndrome.
