Lectures Flashcards
What is the hemoglobin curve called?
What tissue has the highest Hb saturation?
What tissue has the lowest Hb saturation?
If there is 100% saturation in the lungs, and 50% in the muscle, what percentage of oxygen was delivered?
What kind of shift helps dump oxygen in the muscle? This represents lower what?
Oxygen dissociation curve
Lungs
Muscle
50% (just subtract the difference)
Shift to the right, affinity
What means increased WBC count?
What means increased platelet count?
Describe the symptoms of anemia.
Describe the cause and symptoms of uncompensated anemia.
Leukocytosis
Thrombocytosis
Dyspnea, fatigue, malaise, pallor, tachycardia
Severe rapid blood loss, Hypotension, tachycardia, confusion, organ failure
Describe Warfarin, how it works, etc.
Used in rat poison, inhibits vitamin K, thus affecting 2, 7, 9, 10, it takes a few days for nonfunctional proteins to enter circulation, oral absorption (thus given as a pill), then it binds plasma proteins, thus hypoproteinemia makes it less effective, rapidly gets destroyed if it doesn’t bind plasma proteins.
Rifampin / barbiturates / phenytoin can shorten half life by increasing P450 enzymes, and so can alcoholism.
Describe B12 and its absorption.
Cobalamin has binding proteins removed by pepsin in stomach, then salivary gland haptocorrin binds, then it enters duodenum where pancreas enzymes remove haptocorrin, then intrinsic factor from the parietal cells in the fundus of the stomach bind, binds to IF receptors in the ileum, and it is moved to liver by transcobalamin 2.
Describe beta chain encoding and alpha chain encoding.
Describe the effect of removing these chains.
Beta chains are encoded by one gene on chromosome 11, whereas alpha chains are encoded by 2 genes on chromosome 16.
Removing alpha causes beta to aggregate together, and removing alpha causes beta to aggregate together into the beta tetramer called hemoglobin H, which destroys RBCs.
Describe the function of 2,3-BPG.
Describe the function and structure of hemoglobin F.
2,3-BPG binds to the beta subunit of deoxyhemoglobin, removing it from the equilibrium, preventing oxygen from binding, thus decreasing the amount of oxyhemoglobin to reach a new equilibrium.
Fetal hemoglobin belongs to fetuses and contains a gamma subunit instead of a beta subunit, meaning it binds oxygen more tightly than the adult hemoglobin of the mother and attracts more oxygen in the lower partial pressure environment of the womb, which shifts oxygen dissociation curve to the left for higher affinity.
What drains catchment areas?
Describe some of the common issues affecting lymph nodes.
Describe infections of the lymph nodes.
Afferent lymphatic vessels
Lymphadenitis can inflame lymph nodes, lymphadenopathy can make them grow, lymphangitis can inflame nearby lymphatic vessels, chronic issues tend to be painless whereas acute issues are painful by suddenly stretching the lymph node capsule.
Damaged afferent lymphatic vessels make you susceptible to lymph node infection, bacterial infections cause regional lymphadenopathy because bacteria are larger, whereas viruses can spread more easily and cause general lymphadenopathy.
Describe B12 deficiency and its symptoms.
B12 deficient patients may feel numbness or tingling due to demyelination of the dorsal column, and experience dementia or psychosis. Therefore, you should always check a demented patient for B12 deficiency, which may help you recover some function with supplementation.
Like folic acid deficiency, B12 deficiency causes beefy red tongue (glossitis), macrocytic anemia with high MCV, and hyper-segmented neutrophils. But you cannot treat a B12 deficient patient by giving them folic acid.
What two chemicals are required for DNA synthesis?
Describe iron administration.
Whose normal diet is insufficient to meet iron needs?
Vitamin B12, folate.
Pregnant women (2nd and 3rd trimester).
You can give it orally or parenterally, although oral is preferred. Furthermore, Fe2+ or ferrous iron is preferred because it is absorbed 3x greater than Fe3+ or ferric iron. We give ferrous sulfate pills (a type of salt) which is mostly absorbed in the upper small intestine right away. Stomach acidity helps keep it in the ferrous state, so antacids may reduce its bioavailability, and so will food, so we give it before meals.
Describe the different forms of Von Willebrand disease.
There are three types, type 1 is autosomal dominant and decreased VWF, it is the most common, but it is not very severe as it causes heavy menses and nosebleeds, and they lack petechiae because they have sufficient platelets. Type 2a is just like type 1 and is also autosomal dominant but is due to having a correct amount of a structurally abnormal VWF. Type 3 is the most severe but is rarer because it is autosomal recessive. They have very low WVF this factor 8 is destabilized and they have symptoms of hemophilia A.
Describe the ways of administering iron.
Describe the side effects.
Why would you give parenteral iron? Or if there is what? Name three preparations of parenteral iron.
Why are iron pills dangerous?
You want a steady supply of iron for hematopoiesis so you should give it several times per day. Large single doses have diminishing returns in how much reaches the blood because you saturate the uptake mechanisms, so spread it out. If they cannot tolerate oral iron due to malabsorption, give them parenteral iron in the form of sodium ferric gluconate, iron sorbitol, or iron sucrose.
Iron pills will give you heart burn and constipation.
They look red so kids will eat whole bottle and reach toxic levels.
Describe how you can activate factor 9.
Describe how thrombin works.
Describe the pathways of the coagulation cascade.
Factor 7 can activate factor 9 via the alternate pathway, or factor 11 can activate it. Thrombin can directly activate factor 9, or it can indirectly activate factor 9 by activating factor 11.
Thrombin initiates a clotting positive feedback loop by turning on factors 7, 9, 11, and 5.
There are three pathways, the intrinsic pathway (12, 11, 9, 8), the extrinsic pathway (7), and both feed into the common pathway (10, 5, 2, 1). Note that factor 2 is also called thrombin and factor 1 is also called fibrinogen. There is also a fourth pathway, called the alternate pathway, which connects factor 7 to factor 9.
Describe parvovirus B19.
Parvovirus B19 is a small ssRNA virus for whom young children are a reservoir. It is cytotoxic to erythroid progenitor cells and turns them into giant pro-normoblasts with nuclear inclusions that are visible in the bone marrow. It can cause pure red blood cell aplasia and therefore aplastic anemia. It can cause ‘fifth’ disease in school age children which gives them a slapped cheek rash and a reticulated (lake like) rash on their body.
Describe the four cutaneous porphyrias.
Describe porphyria cutanea tarda.
Describe erythropoietic protoporphyria.
They are porphyria cutanea tarda, erythropoietic protoporphyria, congenital erythropoietic porphyria, and x-linked protoporphyria. These only cause skin lesions and do not cause neurological symptoms.
Porphyria cutanea tarda later is the most common porphyria and presents later in life. It results from a uroporphyrinogen decarboxylase deficiency, and is associated with hepatitis C.
Erythropoietic protoporphyria is due to a deficiency in ferrochelatase, which is the last enzyme in the pathway in the mitochondria, and can be precipitated by lead poisoning.
Describe how you prepare for a transfusion.
Describe how blood bag usage is tracked.
When can you violate the FDA regulations?
Any hospitalized patient who is suspected of needing blood undergone typing and screening. Typing means finding their blood type (antigens on the cells) and screening means looking for antibodies in the blood. If an antibody is found, you must then figure out what it is targeting.
The transfusion rate describes the percentage of patients who receive blood, and the utilization rate describes the number of blood bags per patient.
You can violate the the FDA regulations when it is medically necessary, but you must write them a letter explaining why.
Describe the progression of hematopoiesis up until birth.
At the beginning of gestation, the yolk sac produces blood. A few months in, the liver and spleen begin to produce blood. By the fourth month of gestation, the bone marrow will begin to produce blood cells and is the primary source of blood by birth.
Describe fresh frozen plasma.
What will plasma from an A person contain?
Who can an O type person receive plasma from? Why?
Who can an A person get plasma from? What about a B person?
Who can an AB person get plasma from?
Fresh frozen plasma is frozen within 8 hours and can be stored for one year. It contains the cofactors and clotting factors necessary to treat a Coumadin overdose.
Anti-B antibodies.
Anyone, because foreign antibodies have nothing to attack.
(A or AB), (B or AB).
AB (because these donors don’t have any antibodies against A or B in the recipient).
Describe the pathways and factors are measured by PTT.
Describe the pathways and factors measured by PT.
What can activate factor 11?
Describe the role of thrombin.
PTT measures all intrinsic and common factors (12, 11, 9, 8, 10, 5, 2, 1).
PT measures all extrinsic and common factors (7, 10, 5, 2, 1).
Factor 12 and thrombin can activate factor 11.
Thrombin activates platelets by giving their surface a negatively charge surface and by releasing calcium. It also triggers a positive feedback loop by activating factors 11, 9, 7, and 5.
Describe VWF.
Von Willebrand factor exists as a very large multimer which is highly thrombogenic and must be cleaved by ADAMTS-13 before becoming less thrombogenic. VWF is stored in Weibel-Palade bodies in endothelial cells as well as alpha granules in platelets. It can also be found in the subendothelium and and plasma. VWF can bind collagen in the subendothelium as well as the GP 1b and GP IIb-IIIa receptors on platelets, and therefore sticks platelets to each other as well as collagen in the vessel wall. It also stabilizes factor 8 which would otherwise be rapidly destroyed.
Describe beta thalassemia.
Beta chains are absent so alpha chains aggregate as insoluble clumps, which damages RBCs. The spleen then destroys them via hemolysis and causes Jaundice. This also forms hypochromic microcytes causing anemia. This hemolysis increases EPO release, triggering hypoproliferation that that expands the bone marrow and can break bones. This can even cause extramedullary hematopoiesis in the liver and spleen. It will also cause hepcidin to decrease which increases the iron in the plasma, causing iron overload known as secondary hemochromatosis.
Describe a universal RBC donor.
Describe a universal plasma donor.
Describe cryoprecipitate.
Describe leukocyte reduction.
The type O blood type is a universal RBC donor because their cells lack antigens.
Type AB is a universal plasma donor because their plasma lacks antibodies against A or B antigens.
Cryoprecipitate is the miracle product for hemophiliacs because it contains factors VIII, VWF, and fibrinogen.
Leukocyte reduction means you remove leukocytes by irradiating blood products to prevent the spread of CMV into immunocompromised patients, or transfusion reactions to leukocyte antigens. This does not kill RBCs or platelets because they lack nuclei.
Describe intravascular hemolysis.
What term means lots of Hb leaked into the blood?
What term means lots of Hb leaked into the urine?
What means hemosiderin leaked into the urine?
Intravascular hemolysis occurs inside of the vessel. When hemoglobin leaks out, haptoglobin binds it and gets depleted in the blood. The free hemoglobin gets oxidized to methemoglobin.
Hemoglobinemia.
Hemoglobinuria.
Hemosiderinuria.
What rickettsia causes typhus? What spreads it? What war did this impact?
What else causes typhus? These all have what kind of rash?
Rickettsia prowazekii, lice, WW1 (because a guy named Prowazecki got typhus in WW1)
Rickettsia typhi, Black measles rash (same as Rocky Mountain spotted fever)
What PBS cells appear in G6PD deficiency? What special stain shows what bodies? What are these made of?
How do bite cells form?
What lab can confirm the diagnosis of G6PD deficiency? This disease is a type of _______ hemolytic anemia. Does it cause chronic hemolysis? Why? Thus what two symptoms do they lack?
Bite cells, crystal violet stain, Heinz bodies, denatured hemoglobin chains
Spleen phagocytes remove the chunk containing a Heinz body
G6PD levels, episodic, no, exposure to the stressor is periodic, splenomegaly / gallstones
For hemoglobin binding affinity, what word do you avoid using?
What type of Hb has a weaker affinity?
What type of Hb has a stronger affinity?
What relaxes Hb even more?
BPG binds what type of hemoglobin? So initially, BPG immediately removes what from the equation? So what decreases to equilibrate with the scarcer deoxyhemoglobin?
High
Tensed
Relaxed
Binding of even more O2
Deoxyhemoglobin, deoxyhemoglobin, oxyhemoglobin
What is plasma mostly made of?
What three proteins are in plasma?
How many RBCs are per cubic mm?
How many platelets are per cubic mm?
How many leukocytes / WBC?
Water
Albumin, antibodies, fibrinogen
4-6 million RBCs
120-300 thousand
5-10 thousand
What acute porphyria is AD? Mutation to what enzyme? Can also show lesions where?
What acute porphyria results from ALA dehydratase deficiency? What element can precipitate an attack? Why?
Hereditary coproporphyrinogen (cops are dominant), coproporphyrinogen oxidase, skin
Delta aminolevulinic acid dehydratase deficiency porphyria, lead, by inhibiting ALA dehydratase
Viral hemorrhagic fevers are caused by what four families?
Name two filovirus diseases.
Name two flavivirus diseases.
Many of the manifestations result from what two things? They cause ______ to synthesize what factor? This triggers what? It elevates _______, reduces the number of _______, and therefore causes severe what?
What cells will be consumed?
Filoviruses, flaviviruses, arenaviruses, bunyaviruses
Marburg / Ebola (Filo is ME)
Yellow fever / dengue (the flavor of the yellow den)
Direct viral tissue damage and host immune response, macrophages, cell surface tissue factor, coagulation cascade, D-dimer, platelets, bleeding
Leukocytes
What generalized lymphadenopathy has a pathogen from livestock? What dairy can transmit it?
What cells ingest it? It is transported to where? It forms _____ with _______ infiltrates in tissues. It causes what specific type of fever? Where does the localized disease cause pain?
What is the most sensitive culture for brucellosis? Why does PCR not work that well? What unique appearance will occur in the liver?
Brucellosis, unpasteurized dairy product
Macrophages, lymph nodes, granulomas, inflammatory, undulent fever, joints
Bone marrow, lack of standardization, snowflake calcification
What are the progenitor cells for both blood lineages?
What two major lineages are there? What stem cell corresponds to each lineage?
What cells arise from the myeloid lineage?
What are the only cells arising from the lymphoid lineage?
Hematopoietic stem cell
Myeloid, lymphoid, early progenitor with myeloid potential, early progenitor with lymphoid potential
Neutrophils, macrophages / monocytes, eosinophils, basophils, platelets, erythrocytes
B cells, T cells, NK cells
What does PTT stand for? You will activate what factor in a test tube? This activates what two pathways? Measures the time it takes to form what?
What factors does PTT measure?
What does PT stand for? You add what to activate factor 7? This triggers what two pathways? Measures time to form what?
What factors does PT measure?
Partial thromboplastin time, factor XII, intrinsic and common, clot
12, 11, 9, 8, 10, 5, 2, 1
Prothrombin time, tissue factor, extrinsic and common pathways, clot
7, 10, 5, 2, 1
How are hemophilia A and B inherited? What is missing in each?
What does severity depend upon? Less than what percentage causes severe bleeding? What level causes moderate bleeding? What level causes mild bleeding?
Patients present with easy what? And massive what? Why may they need knee replacements? This may also lead to what physical defect?
X linked recessive (due to a deficiency), factor 8 (A) and factor 9 (B)
Factor levels
1%, up to 5%, over 5%
Bruising, bleeding, repeated bleeding in their weight bearing joints, joint deformities
What is slightly less severe than beta thalassemia major?
It causes moderate what? What is the beta chain tetramer called? If young, they may also have what tetramer of the gamma chain?
What two conditions are less severe? What kinds of cells will appear? They have what, but are not dependent on what? These can appear similar to what anemia?
Beta thalassemia minor patient may have what compensatory hemoglobin form?
Alpha thalassemia (hemoglobin H disease)
Hemolytic anemia, hemoglobin H, hemoglobin barts
Beta thalassemia minor / alpha thalassemia minor (alpha thalassemia trait), microcytic hypochromic cells, anemia, transfusions, iron deficiency anemia
HbA2 (because the body makes delta chains instead of beta chains)
What is the mnemonic for microcytic anemia?
Parvovirus can cause what three fetal complications? What does it not result in?
What do you want to control in a parvovirus B19 infection? How can you fix fetal anemia?
Where does malaria often occur? What three pathogens often cause malaria? What is the specific vector?
TAILS (thalassemia, anemia of chronic disease, iron deficiency anemia, lead poisoning, sideroblastic anemia)
Micro-mouse has tails
Miscarriage, death, fetal hydrops, does not result in neurological issues
Fever, intrauterine blood transfusion
Africa / Asia / Latin America, plasmodium falciparum / plasmodium vivax / plasmodium ovale, female anopheles mosquito
Where is the blood factory? What type of bone marrow? What other name does this tissue have?
What means blood cells are made outside of the bone marrow? What two organs are involved? What condition causes this?
What clear cells fill up bone marrow?
In the bone marrow, ____ cell colonies are surrounded by ______ cells such as _____ called nurse cells.
Bone marrow, red bone marrow, medulla ossium rubra
Extramedullary hematopoiesis, liver and spleen, severe anemia
Adipocytes
Red, stromal, macrophages
What is the first thing you look for in a PBS?
What two things do you look for in RBCs?
What index best measures the color of a RBC?
Myeloblasts and metamyelocytes are not what?
When does bone marrow begin producing blood? When do hematopoietic stem cells migrate to the bone marrow?
A reference cell (mature lymphocyte)
Color / size
Mean Corpuscular Hemoglobin Concentration
Terminally differentiated
Month 4, month 4
What is a typical MCV value?
What are high MCV RBCs called?
What are low MCV RBCs called?
Normal RBCs are the same size as what?
What cells are smaller than this?
What disease classically results in microcytes?
80-100 femptoliters
Macrocytes
Microcytes
The nucleus of a small lymphocyte
Microcytes
Iron deficiency anemia
What two specific thalassemia have a similar presentation?
What thalassemia is lethal? Is there a form of beta thalassemia as lethal?
Which (survivable) thalassemia is most severe? When does it become symptomatic?
They require regular what? Marrow expansion creates deformities to what? What forms spikes on an X Ray? Beta major can also expand what two organs? You can measure for what compensatory hemoglobin?
Beta thalassemia minor / alpha thalassemia trait (alpha thalassemia minor)
Alpha thalassemia hydrops fetalis, no
Beta thalassemia major, infancy
Transfusions, bones, cranium, spleen / liver, fetal hemoglobin
What causes babesiosis? What is its vector? What is its reservoir? This it is similar to what disease? What disease has a similar location? Where in the world is it most common?
What surgery increases risk? Why? What patients are most at risk? What microbiological stage identifies babesia? This has symptoms similar to what disease? Most infections are what? What type of unique fever?
It infects what cells? Thus severe cases can cause what? What two drugs can treat it?
Severely ill patients may require what procedure?
Babesia, deer tick, white footed mouse (babies are small like mice), Lyme disease, Lyme disease, U.S. and europe
Splenectomy, cannot filter blood, immunocompromised, merozoite form (tetrad structure), malaria, subclinical, fever spikes
RBCs, anemia, clindamycin and quinine
Transfusion
What strange metric indicates levels of RBC production?
What does myeloid represent? What does erythroid represent?
What is a normal M/E ratio? If the ratio is 1:1, what does this mean?
What are the three prongs of trilineage hematopoiesis?
What does each prong mean?
Myeloid to erythroid ratio (M/E ratio)
Granulocytes and their precursors, erythrocytes and their precursors
2-3/1, the body is making too many RBCs
Erythroid / myelocytic / megakaryocytic
Erythroid = RBC Myelocytic = WBC Megakaryocytic = platelets
What blood cell has a C shaped band?
When is it seen?
A blast should never be found where?
It may contain red lines called what?
What is the safest place to perform a bone marrow aspirate?
Band cell
Bacterial infection (Bandteria)
In the peripheral blood
Auer rods
Iliac crest
What two organs synthesize heme? It starts and ends in what organelle? But where do some intermediate steps occur? What cells lack mitochondria?
What final enzyme turns protoporphyrin 9 into heme? What element does this require?
Therefore, heme is just iron added to what?
Bone marrow and liver, mitochondria, cytosol, erythrocytes
Ferrochelatase, iron
Protoporphyrin 9
What is one broad cause of anemia?
This causes a low what? The body cannot do what?
What is another broad cause of anemia?
This causes high what? The body tries to do what?
Impaired production / hypoproliferative
Low reticulocyte index (under 2%), compensate for anemia (you will have a low RI even though the bone marrow becomes hypercellular)
Destruction of cells / blood loss
High reticulocyte index (over 2%), compensate for anemia
What does HS stand for? These mutations occur in what proteins?
What pattern of inheritance is this? HS causes what major symptom?
What two things does this, like all hemolytic anemias, cause?
What will the PBS show? What metric is increased? Why? Splenomegaly prevents it from filtering out what?
What test can measure HS? But what technique is better? What virus can cause aplastic crisis? Why do they feel bad?
What can treat an aplastic crisis?
Hereditary spherocytosis
Ankyrin / spectrin / band 3, band 4.2 (as the RBC surface proteins will make RBCs look like spheres)
Autosomal dominant, hemolytic anemia
Jaundice, splenomegaly
Spherocytosis, MCHC, cell is smaller so density of Hb is higher, Howell-Jolly bodies
Osmotic fragility test, quantitative flow cytometry, parvovirus B19, their anemia prevents them from compensating for decreased RBC production due to the virus
Blood transfusion
What mutation can increase clotting? What specific type of mutation? This makes factor 5 resistant to what? Thus they often form what?
What mutation can increase prothrombin levels? This also increases the risk of what?
Both the Leiden and prothrombin mutation heterozygotes have what effect? What if they are homozygous?
Rare deficiencies in _____, _____, or ______ can also prevent the inhibition of clotting.
What is the most common clotting cascade mutation?
Factor V Leiden mutation, point mutation (arginine to glutamine), protein C, deep vein thromboses
G20210A, deep vein thrombosis
Slight increased risk of DVT, severe risk of thrombosis
Antithrombin III, protein C, protein S
Factor V Leiden mutation
What virus causes the owl’s eye inclusion? It is what type of virus, just like what?
What are most cases?
What type of mononucleosis?
How is this lymphadenopathy different from that of EBV?
What is the most common congenital viral infection in the U.S.? What % have symptoms at birth? What is the most common neurological sequela?
It will cause hemorrhage of what? This can lead to what? What antiviral is ineffective?
Cytomegalovirus, heroesvirus, Epstein Barr virus
Asymptomatic
CMV mononucleosis
Not limited to the cervical area (because it is generalized)
Congenital CMV, 10%, sensorineural hearing loss
Retina, blindness, acyclovir
To measure hemoglobin, you ___ the cells, free ______ is oxidized to ________, which binds with ______ ions to form _______, and you measure its absorbance with what wavelength?
Lyse, hemoglobin, methemoglobin, cyanide, cyanomethemoglobin, 540 nm
Name four iron studies.
What measures the iron-transferrin complex? What kind of disease decreases this number?
What measures the maximum possible ability to carry iron? How does iron deficiency anemia affect this number? Why?
What is a normal transferrin saturation? How does iron deficiency anemia affect this? Why?
What two results are highly specific for iron deficiency anemia?
Serum iron, TIBC (total iron binding capacity), % saturation, ferritin
Serum iron, iron deficiency anemia
TIBC (measures the empty car), increases this number, more of the transferrin is unbound
33%, lowers this, less transferrin is bound to iron
High TIBC (high transferrin) with a low saturation
A ton of reticulocytes in your blood will correct what? These alone may increase what index?
Increased homocysteine leads to increased what and what?
A defect to what causes macrocytic anemia?
What kind of anemia has a globin chain defect?
What kind of anemia has IL6 activation?
Hemolytic anemia, MCV
Atherosclerosis and clotting
DNA synthesis
Thalassemia
Anemia of chronic disease
What is the RBC expiration date? What is the platelet expiration date? Where does blood flow in the US? How regulated is it?
Who can’t give blood based on travel?
Or if you have been what?
You then screen for what?
What gets lots of extra processing? What does not get much extra processing?
42 days, 5 days, central US to the coast, extremely regulated
Traveled to malaria endemic area in a year
Incarcerated more than 72 hours
Viral markers
Plasma, red blood cells
What is a big risk of heparin? For severe bleeding, what is the antidote? This binds to the _____ charged _______ groups. This only works for what type of heparin?
What should stop treatment immediately? Never do what?
Bleeding, protamine sulfate, negatively, sulfate, large MW heparin
Signs of thrombocytopenia (platelet drop below 50%), never give them heparin ever again
What lab should you always order for bleeding? This will also let you check for what? Or insufficient what?
What two tests assess coagulation issues?
If either is increased, perform what study? What does this involve?
How will you know if the patient is factor deficient? What can you infer if mixing does not result in improvement?
Why may a patient with hemophilia develop factor inhibitors? What disease can naturally produce inhibitors of these factors? What are these inhibitors often composed of?
CBC, anemia, platelets
PT (INR) and PTT
Mixing study, mix patients sample with normal plasma
Mixing will correct PT and PTT, the patient has a factor inhibitor
Antibodies against medically administered recombinant factors, lupus, autoantibodies
What does ITP mean? What is the best sign of this? What will a PBS show? What will a BMB show are still normal?
This is always a diagnosis of what? Why?
They will have what visible symptom? Bleeding from where? Platelet count below what can cause what two bleeds?
Children often have what type of ITP? Why? Why is this not as dangerous? What can reduce this immune response? What organ often destroys platelets? Thus what surgery can help treat acute ITP?
Immune thrombocytopenic purpura, decreased platelet count, larger platelets (platelets swell due to damage from antibodies), megakaryocytes (these are normal because the antibodies are only targeting platelets in the blood)
Exclusion (as are most idiopathic autoimmune disorders), many things can cause thrombocytopenia
Petechiae, nose and gums, 5,000, subarachnoid or intraparenchymal hemorrhage
Acute, they get sick which triggers autoimmunity, self limiting, steroids, spleen, splenectomy
What is the major non-dietary source of iron?
What molecule stores most iron? Which cells have it? What two areas in particular?
______ in the liver consume old ______, turn their _______ into amino acids and capture heme. This heme is then harvested for what two things?
The porphyrin becomes what? The iron becomes stored where? Or it may be released into what carrier?
Recycling by the body
Ferritin, all cells, bone marrow and liver
Macrophages, erythrocytes, globin, iron and porphyrin
Bilirubin, ferritin or hemosiderin, transferrin
What transporter removes iron from the cell? Once in the blood stream, what must be done to free iron? It binds what transporter protein?
It can then be stored where, or utilized where?
What cell utilizes it in the bone marrow? What allows this cell to uptake the ferric/transferrin complex?
What liver hormone blocks ferroportin? This causes iron to be lost how?
Ferroportin, oxidized to the ferric state, transferrin
In the liver, bone marrow
Erythroid precursor, receptor
Hepcidin, as ferritin sloughed off in the intestine
What type of heparin has a more predictable kinetic profile? Which one is most common?
Because this lacks the added chain, what does not not attract well? But what can still bind regardless of the extended tail chain? Thus low MW heparin binds what more than what?
What is the smallest of low MW heparin? It is just the what?
Heparin is not absorbed where? Must be given how? Unfractionated heparin has a half life dependent on what? It this good?
If someone has a PE, give them _______ for immediate inhibition of ________, and then wean them to ______ anticoagulants.
Low molecular weight heparin, enoxaparin (low MW)
Thrombin (II), factor X, factor X more than factor II
Fondaparinux, core pentasaccharide
GI tract, parenterally, dose, no (very hard to use)
Heparin, coagulation, oral
Vitamin B12 and folic acid are both required for what? Folic acid becomes what specific form? It carries what group? It adds a methyl to what group to form what amino acid?
What is the left over folic acid version called? What is this form essential for? It specifically turns what into what?
DNA synthesis, methyltetrahydrofolate, methyl group, homocysteine, methionine (thus folate deficiency causes a homocysteine buildup)
Tetrahydrofolate (lacking the methyl group), DNA synthesis, deoxyuracilmonophosphate to deoxythymidinemonophosphate (you need TETra to make the THYmidine)
Drugs that do what can increase the effectiveness of warfarin? Why? What deficiency also helps warfarin be more effective?
What does warfarin toxicity cause? What is the ideal INR target for warfarin? What INR can be risk of bleeding? Give them what immediately? How long may this take to work? So what can you give them that works immediately?
Warfarin can also cause what major issue? What kind of drug has a half life of months? How do you solve an overdose?
Drugs that displace warfarin from the plasma proteins, increased concentration of free warfarin, vitamin K deficiency
Bleeding, 2 to 3, greater than 5, vitamin K (to generate more normal coagulation factors), 48 hours, plasma transfusion
Birth defects, superwarfarin, regular vitamin K
What indicates a normal beta chain allele? What means no beta chains are made at all? What means you have some beta chains? These are collectively called what?
What are the possible genotypes for beta thalassemia minor?
What are the possible genotypes for beta thalassemia major?
B, B0, B+, beta thal alleles
- B, B+
- B, B0 (All have at least one normal copy)
- B+, B+
- B+, B0
- B0, B0 (No normal copies at all)
What anemia is due to too few RBCs being produced?
This will have a reticulocyte index below what?
This may be due to deficiencies of what nutrients?
What does severe blood loss count as?
Hypoproliferative anemia
2%
Iron deficiency, folate deficiency
Destructive / hemolytic anemia
All anemias cause ______ bone marrow, with what single exception?
Would iron deficiency anemia increase RI? Why? So what index is decreased?
What anemia can cause neurological issues? What anemia can cause SOB and neurological issues? What from history tells you that something is not sickle cell?
What other anemia-related disease can cause SOB and neurological issues?
Hypercellular, aplastic anemia
No, even though bone marrow is hypercellular, more reticulocytes don’t make it into the blood, reticulocyte index
B12 deficiency anemias, sickle cell anemia, have not been to the hospital before
TTP (thrombotic thrombocytopenic purpora)
X linked sideroblastic anemia with ataxia has what deficiency gene?
What kinds of sideroblastic anemias can you get later in life? Clonal issues are due to what syndromes? This increases risk of what blood disease?
Porphyrias will have elevated ________ levels, whereas sideroblastic anemias have normal ________ levels because they target different ______.
A normal neutrophil has how many nuclear segments? Myelodysplastic syndromes May cause how many segments? And the cytoplasm may lack what? Normal erythroid precursor cells are what shape? What shape may be dysplastic precursors? The nuclear segments may also not be what?
ABCB7 Gene (cleanup in aisle B7)
Acquired, myelodysplastic syndromes, acute myeloid leukemia
Protoporphyrin IX, protoporphyrin IX, pathways
About 3, 2, granules, perfectly round, abnormal shapes, connected
The ferritin metric measures what? How does iron deficiency anemia affect this? Why?
What is STFR? How does iron deficiency anemia affect this? Why?
How does iron deficiency anemia affect hepcidin? Why?
The body stores of iron, decreases, less iron exists to be stored
The receptor that allows the erythroid precursor to bind the iron-transferrin complex, increases, less STFR is bound to iron so it floats away from the cell and accumulates in the plasma
It decreases it, no need to inhibit iron absorption if iron levels are already low
What is the most common cause of iron deficiency anemia? Over what time period? What group of women can have this?
What means hidden blood loss? Where does this occur? What two things can cause occult bleeding?
You may have impaired iron _____, or even have _______, which increased what regulatory hormone? This is associated with what specific type of anemia?
Blood loss, long term, women who menstruate
Occur bleeding, GI tract, gastric ulcer / colon cancer
Absorption, inflammation, hepcidin, anemia of chronic disease
What must you decrease in sickle cell?
What condition helps protect sickle cell patients?
What is the goal of emergent exchange transfusion?
How do sickle cell SB+ patients perform? Why? So what level is higher?
How to sickle cell SC patients perform? Why?
HbS
Hereditary persistence of fetal hemoglobin
Reduce HbS to a level of about 30 / 40%
Quite well (because the B+ beta thalassemia minor allele at least makes some normal HbB instead of HbS, thus less HbA is sequestered), HbA is higher
Quite well (because this HbC replaces HbS, thus less HbA is sequestered)
Sickle cells have what clustering? What happens to them? What is the life span of a sickle cell? They also have lots of _____, which forces out _____ and _____.
Sickle cells do what to vessels? This causes what crisis? What does this feel like? What drugs can help this?
Name five triggers of a sickle cell crisis.
Band3 clustering, hemolysis, 10-20 days, calcium, potassium, water
They stick to them, vaso-occlusive crisis, very painful, pain drugs
Hypoxia, dehydration, acidosis, infections, pregnancy
What kind of hormone stimulates neutrophils? What is the drug name?
What can you attach to it, and why? What do you call this modified drug?
Use this in patients undergoing high dose ____ or _____ chemotherapy. This also stimulates transplanted ______ to produce what?
It may also treat what congenital disease? Or what type of anemia? This will all reduce the risk of what? What is another name for pegfilgrastim? What does ANC mean?
Granulocyte colony stimulating factor, filgrastim (GRAnulocyte STIMulate)
Polyethylene glycol, extend the half life, pegfilgrastim
Cancer, HIV, bone marrow, neutrophils
Congenital neutropenia, aplastic anemia, infection, neulasta (NEUtrophils LAST longer), absolute neutrophil count
What other diseases may cause ITP?
Only consider a _____ if they ______ after prior treatment attempts.
What anti CD20 drug helps treat ITP?
What platelet count means patients can safely undergo surgery?
What does HIT mean? How long after does this occur? This somehow creates what antibody? It also damages what? This leads to very severe what? Stop all what?
Lupus, HIV, (as anything affecting the immune system could cause IMMUNE thrombocytopenia)
Splenectomy, remiss
Rituxan (it TUX away the immune system)
50,000
Heparin induced thrombocytopenia, 5-10 days after dose is given, IgG, endothelial cells, clotting, heparin
What is toxic in the free form? Why?
Iron in the body may be bound to what? Where?
Name two functional locations for iron.
Name two storage forms of iron. Why is ferritin a better form of storage?
How much iron is found in the functional state? What about the storage form?
Iron, it creates free radicals
Transferrin, circulation
Hemoglobin / myoglobin
Ferritin / hemosiderin, because iron stored in hemosiderin cannot be used again
2/3, 1/3
What two pathogens are associated with innate immune cell infection?
What cell does each infect?
What type of cell is infectious?
What serves as a reservoir for ehrlichia? What do its symptoms feel like? Without what? What kills it very well?
Ehrlichia/anaplasma
Ehrlichia (phagocytes), anaplasma (neutrophils)
Dense core cells
Deer, Rocky Mountain spotted fever (without the rash), doxycycline
What viral pathogen can cause regional and general adenopathy? It is what type of virus? What is this infection called?
What triad of symptoms does it cause? What percentage of adults are seropositive? It infects what cells in what tissue?
What other severe side effect can latency cause?
What do you see in a PBS?
What lymph nodes are commonly associated with mono? Why? Why do we ask infected adolescents to avoid contact sports? What antibody persists for life? Which one disappears?
Epstein Barr Virus, herpesvirus, Infectious mononucleosis
Fever / pharyngitis / cervical lymphadenopathy, 90%, B cells, lymphoid tissue
Hodgkin and non-Hodgkin lymphoma, nasopharyngeal carcinoma
Abnormal lymphocytes with large and irregular nuclei and tons of cytoplasm
Cervical lymph nodes, transmitted by kissing, splenomegaly leads to splenic rupture, IgG, IgM
What food contains folic acid? How long does it take to deplete folic acid stores? What about B12 stores?
Who often has decreased folic acid intake? Who else has increased requirements for folic acid? What disease causes increased folic acid requirements? So supplement them with what?
Name one difference between B12 deficient patients and folic acid deficient patients.
Green leafy vegetables, a few weeks, a few years
Alcoholics (they don’t eat vegetables), pregnant women, sickle cell disease, folic acid
Neuropsychiatric abnormalities for B12 deficiency
Extravascular hemolysis means cells are destroyed where? What cells? What grows in size?
Both intravascular and extravascular hemolysis can cause _______ and _______.
What chain does sickle cell affect? What genotype does the normal beta chain have? What genotype do sickle cell patients have? This is what specific type of mutation?
Sickle cell beta chains lack what property? When is this fine? But what causes sickle cell hemoglobins to aggregate? What kind of cell does this form?
Liver and spleen, phagocytes, liver and spleen (primarily the spleen)
Anemia, jaundice
Beta chain, BB, SS, glutamic acid to valine point mutation at position 6 (wear a GluV when using a sickle)
Solubility, high oxygen concentrations, low oxygen concentrations, sickle cell
Does whole blood have harmful effects? Risk of what two things?
When do people develop an ABO blood type? By when do people make antibodies against foreign blood antigens? Where do we think they come from?
What are the genotypes for each blood type?
What type of RBC can be given to anyone? Does this work for plasma? Because plasma has different what?
Platelets require what for survival? Thus they must be in what type of bag?
Yes, too much volume for the CV system can kill the patient, or risk for infection
Birth, 6 months, gut microbiome
OO (type O)
AA / AO (type A)
BB / BO (type B)
AB (type AB)
O type, no, antigens
Oxygen, gas permeable bag
What are the three components of the CBC?
What data help you understand anemia?
What tells you how many erythrocytes are in your blood?
Why could this be misleading?
What measurement is much more informative?
WBC, RBC, platelets
RBC indices
RBC count
Anemic people may have normal RBC count
How much hemoglobin is present
What stem cell can create endless growth in the bone marrow? This causes what disease? There is no room for what cell type in the bone marrow? Causing what other symptom?
What signal creates more RBCs?
What signal creates more monocytes / neutrophils?
What signal creates more platelets?
Hematopoietic stem cells, leukemia, megakaryocytes, bleeding
Low oxygen
Infections
Blood loss / injury
What are the two components of the RBC membrane?
What is the lattice called? What two things comprise it?
What does actin do?
What does ankyrin do?
This spectrin mesh gives the RBC what property? So what does an absence of spectrin cause to happen? What cell does the RBC become?
Lipid bilateral, spectrin
Spectrin, alpha / beta chains
Actin binds the alpha subunit to band 4.1
Ankyrin binds the beta subunit to band 4.2
Flexibility, the RBC breaks apart, spherocyte
What is the classic histological finding of cerebral malaria? How fatal is it when treated and when untreated? What history must you obtain for malaria?
What is the standard diagnostic tool for Malaria? Will Malaria always be detectable? Why? So repeat a blood smear how often?
P. Falciparum will have thin _____, absence of ______, and a ______ shaped gametocyte.
P vivax and P ovale have _____ rings, ______ in the blood, and ______ shaped gametocytes. What dots are also visible?
Sequestration of infected erythrocytes in microvessels (thua blocking your brain), 5-20% when treated, 100% when untreated, travel history
Giemsa stained blood smears (to see the parasite), no, symptoms appear before high enough blood concentration of parasite, every 12 hours
Rings, schizonts, banana
Thicker, schizonts, spherically, schuffner’s dots (the vivacious oval shuffled dots)
Sickle cell patients have what anemia? They have ________ due to increased RBC production. When does sickle cell first become apparent?
In whom do bones expand? You may have what kind of hematopoiesis? Sickle cells cause what organ to swell? This does what to the spleen? So what happens by adulthood?
The spleen helps fight what type of microorganisms? Thus what are sickle cell patients susceptible to?
Hemolytic anemia, reticulocytosis, 4-6 months
Children, extramedullary, spleen, kills it, it goes away
Encapsulated, encapsulated microorganisms,
What means various RBC sizes and volumes?
What means variations in RBC shape?
What do you first look for in a peripheral blood smear?
What next?
Anisocytosis
Poikilocytosis
Size of the RBCs
Color and shape of the RBCs
Sickle cell splenomegaly can cause what sudden crisis? This traps too much what where? This causes what fatal symptom? What can restore this volume?
What crisis means a sudden decrease in Hb for 2 weeks? What virus must infect what tissue?
Sickle cell can cause what chest complication? What symptoms does this cause? Look for what on a CXR? What specific transfusion can save them?
Splenic sequestration crisis, blood, in the spleen, hypovolemic shock, blood transfusion
Aplastic crisis, parvovirus B19, bone marrow
Acute chest syndrome, dyspnea, tachypnea, low O2, hypoxemia (vicious cycle), new pulmonary infiltrates, emergency exchange transfusion (replace all of their blood)
What does TTP mean? It is a type of what? What is another form of thrombotic microangiopathy?
These both damage RBCs and cause what? Therefore what count will be high? Low what level?
What pentad of symptoms exists for TTP? What enzyme is deficient? Large ________ multimers are more what?
What is the treatment? To replenish what?
Thrombotic thrombocytopenic purpura, thrombotic microangiopathy, HUS
Microangiopathic hemolytic anemia, reticulocyte count, haptoglobin
- Thrombocytopenia
- Hemolytic anemia
- Renal failure
- Fever
- Neurological symptoms
ADAMTS13, VWF, thrombogenic
Immediate plasma exchange, ADAMTS 13
What do rapid malaria tests look for? Acquired immunity is not what? Can you get infected again? But what is better with subsequent infections?
What two traits protect against Malaria? What can treat chloroquine resistant malaria? Or what else? Be careful with quinine for what reason?
What is the only treatment for severe malaria in the U.S.?
What is used for malarial prophylaxis?
Malaria specific antigens, sterilizing, yes, symptoms
Sickle cells trait / thalassemias, artemisinin (Artemis fowl saves you from malaria), quinine, it will accelerate the hypoglycemia associated with malarial infection some (Quinn is probably hypoglycemic)
Quinidine gluconate (of course American malaria drug would be full of glucose)
Chloroquine (just like chlorox wipes prevent countertop bacteria)
Define hemolysis.
Name two types of hemolysis.
Hemolysis causes an increase in _______ bilirubin, causing what symptom? What enzyme do RBCs also release?
What do you see on a PBS? What process will try to increase RBC production?
Destruction of a RBC before its normal lifespan
Intravascular hemolysis and extravascular hemolysis
Unconjugated (indirect), jaundice, lactate dehydrogenase (LDH)
Polychromatic reticulocytes, RBC fragments and schistocytes, erythropoiesis
What kind of lesion damages bone marrow?
What space occupying lesion results from an infection? What results from scarring? What else can cause a space occupying lesion?
Name a primary malignancy of the bone marrow? Malignancies can also do what? Cancer will often spread to the bones from what two locations?
Space occupying lesion
Granuloma, fibrosis, cancer
Lymphoma, metastasize from other organs, breasts / prostate
What dye turns reticulocytes blue?
By staining what molecule?
Reticulocytes are slightly larger than what?
Reticulocytes are normally what % of RBC count?
What anemias destroy RBCs?
What cell type reaches what % to compensate?
Methylene blue
Ribosomal RNA
RBCs
1%
Hemolytic anemia
Reticulocytes, 2% or higher
How will VWD affect platelet count? What is normal in all of them? What is normal in type 1 and 2a? What is longer in type 3?
What test can assess platelet aggregation? What must be present for aggregation to occur? Which type has no aggregation?
What drug causes VWF release from what cells? How is it administered?
Or what can give them sufficient factors?
Doesn’t affect it (it affects the factor but not the platelets that bind the factor), PT, PTT, PTT is longer (because intrinsic factor 8 is destabilized)
Ristocetin-induced platelet aggregation, VWF, VWD type 3
Desmopressin (presses down on the granules to release VWF), endothelial cells (weibel palade bodies), nasal spray
Plasma transfusion
What is the best way to make the specific diagnosis of PNH? You search for what two proteins on these cells?
What test measures for acidic serum? To check for susceptibility to what? What other lysis test can be used?
What type of drug can inhibit C5 from becoming C5a? What is it called? This prevents what system from killing cells?
Flow cytometry, CD59, CD55
Ham’s test (acidic ham with orange juice), cell lysis, sucrose lysis test
Monoclonal antibody, eculizumab, complement system
What process restores normal blood flow after injury?
What is the first step? What is the second step? What is formed here?
What is the third step? What happens here?
What is the fourth step? What happens here?
What vessel layer contains collagen? What endothelium organelles contain VWF?
What are inactive platelets shaped like?
Some coagulation factors depend upon what?
Hemostasis
Vasoconstriction, primary hemostasis (platelet plug is made)
Secondary hemostasis, fibrin strengthens the platelet plug
Fibrinolysis, clot resorption
Subendothelium, Weibel-Palade bodies
Discs
Vitamin K
How many main porphyrias are there? These are due to abnormal enzymes for what pathway?
What are the major two categories? With what symptoms?
What does a neurovisceral attack look like? What causes the neurotoxicity?
What tuberculosis medicine can induce acute porphyria? What poisoning, with what unique cytoplasmic appearance? What technique can let you measure the amount of each porphyrin?
To treat acute porphyrias, treat what? Then give them medicine for what?
8, heme synthesis
Acute porphyrias (neurovisceral attacks), cutaneous porphyrias (photosensitive skin lesions)
Abdominal pain with some neurological issue, a buildup of ALA
Isoniazid, lead positioning, basophilic stippling, HPLC
Underlying cause, pain
What are the three categories of quantitative platelet disorders?
What condition can trap too many platelets in the spleen?
What are the two classes of qualitative platelet disorders?
Name three inherited qualitative platelet disorders.
What can cause non-inherited defective platelets?
Decreased production, increased destruction, sequestration
Hypersplenism
Inherited, non-inherited
Bernard-Soulier, Glanzmann’s thromboasthenia, von willebrand disease
Aspirin
Pink tissue in a BMB indicates what? This may cause what?
Tumor cells in a BMB will cause what?
What disease causes a hypocellular BMB?
What cells are very huge on a peripheral blood smear? They have very open what, and visible what?
When should you see myeloblasts in a peripheral blood smear? What disease causes this?
Fibrosis / scar tissue, anemia
Anemia
Aplastic anemia
Myeloblasts, chromatin, nucleoli
Never, leukemia
What two major types of thalassemia exist?
What two types of beta thalassemia occur?
What four types of alpha thalassemia occur?
Beta thalassemia / alpha thalassemia
- Minor
- Major
- Silent carrier
- Alpha-thal-trait
- Hemoglobin H disease
- Hydrops fetalis
What two symptoms does Zika cause? What transmits it? What is detectable after viral infection?
What percent are symptomatic? Are symptoms severe? it can cause what type of adenopathy?
It can rarely trigger what neurological complication?
What is the most feared complication? What kind of PCR can identify Zika?
Measles like rash, conjunctivitis, mosquitos, Zika viral RNA
20%, no, generalized
Guilin-Barre syndrome
Congenital microcephaly
RT-PCR (to amplify cDNA from viral RNA)
Which porphyria presents with brown teeth? What swells? When is it spotted? How?
What protoporphyria is gain of function? What is its inheritance pattern?
Congenital erythropoietic porphyria, spleen, infancy, reddish urine color
X-linked protoporphyria, X-linked gain of function
What wavelength and compound are used to visualize hemoglobin?
What are immature erythrocytes called?
They also lack what?
What do they still contain?
Reticulocytes are what color?
They are released to circulate for how long?
Then where do they go?
540 nm, cyanomethemoglobin
Reticulocytes
Nuclei
RNA
Bluish
1 day (because they have to return to the bone marrow immediately)
Back to bone marrow
What stain can detect iron? What disease prevents bone marrow from staining for iron? Why?
What do we call erythroid precursors storing dusty iron?
What are the two major sources of iron loss?
Prussian blue, iron deficiency anemia, all of the iron stores have been used up already
Sideroblast
Sloughing off (intestinal cells) and menses (blood loss)
What kind of anemia results from inflammation? What cytokine causes hepcidin to release? Hepcidin blocks what transporter? This causes iron to be pushed into where?
What iron studies will anemia of chronic disease result in?
This may somehow also reduce the response to what hormone?
What type of PBS results would you see?
Where are there adequate iron stores?
Anemia of chronic disease, IL6, ferroportin, storage
Low serum iron
Decreased or normal TIBC (because body is not missing iron so it doesn’t increase free transferrin)
Increased ferritin
EPO
Normocytic / normochromic, and hyocytic / hypochromic
In the bone marrow
What chemical classically causes aplastic anemia? So always ask them what? Name some viral causes of pancytopenia.
Name one inherited cause of aplastic anemia. What inheritance pattern?
Or hypoplasia of what?
Most patients with pancytopenia have what cause?
What two options may treat pancytopenia?
Benzene, where they work, EBV / CMV / rubella
Fanconi anemia (Batman made Falconi anemic), autosomal recessive (loss of function)
Kidneys (due to less EPO being produced by failing kidneys)
Idiopathic
Bone marrow transplant, immunosuppression (to treat an idiopathic autoimmune etiology)
What is recombinant interleukin 11 called (both names)? This binds what receptor? This stimulates what process, which in turn produces what? Give this to what kinds of patients?
What drug interacts with the thrombopoietin receptor (both names)? What kind of molecule is it? What gives it a longer half life? You only treat what disease with this?
What other drug (both names) stimulates the thrombopoietin receptor? It is a non-______ molecule.
Oprelvekin/neumega (new megakaryocytes) (Oprah is worth 11 billion dollars), IL-11 receptor, megakaryocytopoiesis, platelets, thrombopenic patients on chemotherapy
Romiplostim (Nplate), peptibody, two Fc fragments, idiopathic thrombocytopenic purpura (ITP) that is not responsive to corticosteroids (to reduce autoimmunity) or splenectomy
The Roman named Eltrom Bopag goes directly to the thrombopoietin receptor
Eltrombopag (promacta), non-peptide
Children with sickle cell develop _____ vessels in their brain, which clog with what? This can cause what stroke? The brain compensates by making new what? Why is this dangerous?
What sickle cell complication causes sustained erection? How do you treat this?
What specific bone infection does sickle cell cause?
What should you give to help with their splenic susceptibility to capsulated pathogens? What chemical helps by increasing HbF? Thus they form what strange tetramer? These don’t what?
Thick, clots, ischemic stroke, vessels, weak new vessels causing hemorrhagic stroke (sickle cell can kill you from a brain hemorrhage)
Priapism, exchange transfusion
Salmonella osteomyelitis
Prophylactic antibiotics, hydroxyurea (hydroxy gives them HIGH oxy), alpha/alpha/beta/gamma, aggregate
An anemic patient will have reduced what level?
What will increase the percentage of hematocrit without anemia?
For what is RDW a mathematical representation?
What value best measures hemoglobinization of RBCs?
What other value can be visualized on a blood smear based on cell size?
Hematocrit
Dehydration
Anisocytosis
MCHC - mean corpuscular hemoglobin concentration
MCV
Pregnant women have too much what?
This diluted what layer?
Therefore, what falsely appears to be too low?
What is hematocrit (RBC) also called?
What technique lets you visualize RBCs?
What stain is added to the smear?
Plasma
Hematocrit
Hematocrit
Packed cell volume
Peripheral blood smear
Giemsa Wright stain
What do platelets lack? What shape do inactive platelets have? They are fragments of what? They stay away from what? What is their lifespan? What hormone triggers their synthesis?
What two granules do they have?
What 6 things are stored within alpha granules?
What 4 things are stored within dense granules?
What factor is found in the platelet cytoplasm (outside of granules)? What does it do?
Nuclei, disc shaped, megakaryocytes, vessel wall, 7-10 days, thrombopoeitin
Alpha granules, dense granules
VWF, fibrinogen, factor V, factor XI, PDGF (platelet derives growth factor), PF4 (platelet factor 4)
ADP, calcium, epinephrine, serotonin
Factor XIII, binds fibrin that strengthens platelet plug
What is vitamin B12 called? It is used for the conversion of what to what?
More specifically, what version of cobalamin is required?
What specific version of cobalamin is used to convert homocysteine to methionine?
What two things will a B12 deficiency increase?
Whereas a folic acid deficiency will only increase what? It will not increase what?
Cobalamin, methylmalonyl Coenzyme A to succinyl coenzyme A
Adenosyl cobalamin
Methylcobalamin
Methylmalonic acid (methylmalonyl CoA) / homocysteine
Only homocysteine, methylmalonic acid (methylmalonyl CoA)
What does DIC mean? This is activation of what? Thus what two substances deposit in the body? Which one causes a positive feedback loop?
What term means the clotting uses up the platelets and factors? Thus there is low what? How does this affect PT and PTT? What is very high in the plasma?
What gets cleaved to d dimers? What gets split to fibrin split products?
Which metabolite is very specific to DIC? What is not specific to DIC? So what specific thing must you measure for DIC?
Disseminated intravascular coagulation, clotting cascade, thrombin / fibrin, thrombin
Consumptive coagulopathy, platelet count, increases them, fibrin split products (d dimers)
Fibrin-fibrin bands, fibrin monomers
D dimers (DD for DIC), fibrin split products, d dimers
What two reactants begin heme synthesis? They form what, with what enzyme?
Two of these then condense to form what? With what enzyme?
Four porphobilinogens then condense to form what? With what enzyme?
This then forms what? With what enzyme?
Coproporphyrinogen then forms what penultimate molecule? With what enzyme?
Succinyl CoA and glycine, ALA (aminolevulinic acid), ALA synthase
Porphobilinogen, porphobilinogen synthase
Uroporphyrinogen 3, uroporphyrinogen synthase
Coproporphyrinogen 3, uroporphyrinogen decarboxylase
Protoporphyrin 9, coproporphyrinogen oxidase
What do we use antiplatelet drugs for? What is the most common antiplatelet drug?
Name the four classes of antiplatelet drugs.
Aspirin inhibits what enzyme? Which prevents formation of what?
The P2Y purine receptors ultimately do what? Name four of the P2Y antagonists.
Which two are prodrugs? They require what for activation? Thus different patients may have what?
Prophylactic prevention of clots, aspirin
- Thromboxane A2 inhibitors
- P2Y purine receptor antagonists
- PAR (protease activates receptor) antagonists
- GP IIb/IIIa antagonists
You have the thromboxane, the fibrinogen binders, and the two P’s
Cyclooxygenase I, thromboxane A2
Turn on the fibrinogen receptors, clopidogrel / prasugrel / ticagrelor / cangrelor (PURe gruel = purine grel)
Clopidogrel / prasugrel, CYP2C19, different activation
What does DAT stand for? What does it test for? It may also test for a ______ reaction, or a ______ disease of the fetus.
What is the process of a DAT? What are the two possible results?
What does IAT stand for? What does it look for? You do this to prevent what?
What is the procedure for IAT?
Direct antiglobulin test, autoantibody attacking the patient’s own RBCs, transfusion reaction, hemolytic
Add patient’s RBCs to tube, add anti-globulin antibody, and of the patient’s RBCs are covered in auto-antibodies, the anti-globulin antibodies will agglutinate them together, positive / negative
Indirect antiglobulin test, antibodies in the plasma (hence it is indirect) that target all sorts of other potential antigens in the donor’s blood, transfusion reactions
Mix donor RBCs with recipient plasma to let any plasma antibodies attack them, and then add an anti-globulin antibody to agglutinate if positive result
What four factors are vitamin K dependent?
What two anticoagulation proteins are dependent upon vitamin K?
Name two drugs used for anticoagulation?
2, 7, 9, 10
Protein C, protein S
Warfarin, heparin
The female anopheles mosquito injects ______ into the host, which enter cells in the _____ and form a “_______”, which ruptures and enters the _______ cycle.
The immature trophozoite in the RBC is called what? It then develops into a ______ trophozoite, before forming a _______ that ruptures and infects other blood cells.
Or, the ring stage could form into a _______ that is indicative of plasmodium ______ if it is shaped like a what? Malaria feels like the what? What type of malaria involves the CNS? What medical condition causes black water fever?
Sporozoites, liver, schizont, erythrocyte
Ring stage, mature, schizont
Gametocyte, falciparum, banana, flu, cerebral malaria, hemoglobinuria
Immature blasts often have a large what? And ____ or open chromatin. Mature cells have smaller what? What condenses?
What word describes maturation of the cytoplasm without nuclear maturation? What are these cells called? Because they still have what?
B12 or folic acid deficient anemia causes what in the bone marrow? What happens to most megaloblasts in the bone marrow? Thus causing what symptom?
Nucleus, lacy, nuclei, chromatin
Asynchronous growth, megaloblasts, nuclei
Hypercellularity (even though it causes anemia, the DNA damage makes it grow too quickly), they die, anemia
What means you get a normal alpha chain? What means you deleted an alpha chain?
What is a healthy genotype for alpha chains?
What is the genotype for a silent alpha thalassemia carrier?
What are the two genotypes for the “alpha thalassemia trait” presentation?
How many copies must you lose for hemoglobin H disease? What about hydrops fetalis?
These fetuses lack what adult chains? So what aggregates instead? What is this aggregate called?
A, -
(A/A, A/A) (Four chains are wild-type)
(A/-, A/A) (Silent misses one chain)
(A/-, A/-) or (A/A, -/-)
Three, four (fatal)
Alpha, gamma chains, hemoglobin Bart
What drug can cause hapten-mediated immunohemolytic anemia? The ______ only attacks the RBC if what else is bound?
What drug can cause immune complex immunohemolytic anemia? In this case, what forms first? Then what happens next?
The third type involves a true what? What drug causes this? This drug induces the formation of what?
In which two scenarios will drug removal resolve the issue?
In which scenario will removal of the drug not resolve the issue? Why?
Penicillin, antibody, penicillin
Rifampin, immune complex of rifampin and antibody, this complex binds the RBC
“True anti-RBC antibody”, methyldopa, anti-RBC antibody
Hapten mediated / immune complex mediated
True anti RBC antibody, the anti RBC antibodies will always be in the body
Immature erythrocytes are called what?
Describe them.
Why are they blue?
What is the name for a single blue dot in a RBC?
What is the name for a bunch of blue dots in a RBC?
Immature erythrocytes still contain the entire what?
Reticulocytes
Anucleate, purplish / bluish cytoplasm, slightly larger than RBCs
They have already lost their nuclei but they still have some RNA left over
Howell Jolly body
Basophilic stippling
Nucleus
What infection causes severe swelling of the legs? What infects you?
What are the three pathogens? What transmits them?
What lymph nodes are most commonly infected? What causes pain? Diagnosis involves testing for what? Microscopically, these look like giant what?
Lymphatic filariasis, roundworm
Wuchereria bancrofti, brugia malaya, brugia timori, mosquitos
Inguinal lymph nodes, lymphadenopathy, antigens, worms
Name two types of dietary iron.
Which one is complexed? Which one is uncomplexed?
Non heme iron is in what state? What oxidation state does this mean? What must happen before it enters the enterocyte? Through what transporter?
Which iron can enter immediately?
What is the storage form of iron in what cell?
Heme iron, non heme iron
Heme iron, non heme iron
Ferric, plus 3, reduced to Fe2+, DMT1
Heme iron
Ferritin, enterocyte
What will a PBS of someone with iron deficiency anemia show?
What will be completely missing in bone marrow?
What will iron studies show for iron deficiency anemia?
What hemoglobin do normal adults have? What chains does it have? What percentage?
What other hemoglobin exists in adults? What chains does it have? What percentage?
Hypochromic microcytes, pencil shaped cells, lots of variation
Iron stores
Low serum iron, high TIBC, low saturation of transferrin, low ferritin
HbA, two alphas and two betas, 97%
BbA2, two alphas and two deltas, 3%
What allowed for safe blood transfusions? What technology allows you to remove only the blood component you need?
At first, ____ blood is taken. The potency means the _____ is of a sufficient level. They send some blood off for ____ testing. This whole blood is put into a what? What is the only reason to give whole blood? What can you give if you only want to increase pressure?
Give whole blood if there is what? Or conditions that reduce what capacity?
ABO typing, apheresis
Whole, hematocrit, viral, centrifuge, increase oxygen carrying capacity, saline
Uncontrolled bleeding, oxygen carrying capacity
Ask a patient about the _____ of the bleeding, and if it occurs ________ or if it was induced by what? Which type is more severe?
Ask what to determine if it is inherited? What results from small vessel bleeding? These look like what?
Larger bleeds create a what? What two terms describe this?
Deep tissue bleeding can cause pain where? What is this called? Or a collection of blood called what?
Duration, spontaneously, trauma, spontaneous
Family history, petechiae, red pin pricks on the skin
Bruise, purpura / ecchymosis
Joints, hemearthrosis, hematoma
A patient who needs how much blood per week likely has hemolysis? Thus what can indicate hemolysis?
What patients keep attacking blood with improper antigens? Why is this difficult to treat?
What can cause basophilic stippling? What unique pattern will a thalassemia patient have?
What has much lower Hb? What anemias can be acquired?
1 or 2 pints, transfusion requirement
Sickle cell patients, hard to find enough blood products that they won’t react to
Thalassemia, slightly low Hb with incredibly low MCV
Iron deficiency anemia, thalassemias
What anticoagulation protein can be bound or free? What binds the free form? It is referred to as a what? This means it is rapidly increases when?
C4bBP binds to free what? This depletes what? Leading to what state? What diseases can cause this triggering inflammation? Thus what protein mediates the hyper coagulation of inflammation?
What zymogen mediates fibrinolysis? What activates this? What other factor can do this? This turns plasminogen into what? Plasmin then does what? What are fibrin split products not quite the same as?
Protein S (may or may not be strapped), C4bBP (stick some C4 on the protein S), acute phase reactant, during inflammatory states
Free protein S, depletes free protein S, hypercoagulable state, cancers, C4bBP
Plasminogen, TPA (tissue plasminogen activator), factor XII, plasmin, degrades the clot, D-dimers
Neulasta will cause what symptom? What other side effect will a CBC reveal? When cleared, increased neutrophils make what organ swell?
What older granulocyte stimulating factor has been replaced? Why? What side effects does it have?
What kinds of growth factors create more platelets? What two classes are there?
Mild to moderate bone pain, granulocytosis, spleen
Sargramostim, severe side effects, flushing / hypotension / nausea / vomiting / dyspnea
Thrombopoeitic growth factors, those that target the interleukin-11 receptor, those that target the thrombopoietin receptor
