DIT Heme Flashcards
Hemophilia symptoms?
Macrophemorhage: bleeding into joints. Easy bruising. High PTT (b/c intrinsic pathway)
VIII is for accelerating 9’s activation of X
VI is for activation of X
Tx: recomb VIII in hemophilia A
Vit K deficiency is what?
Clotting factors 2, 7, 9 and 10, C and S
It is from gut bacteria (newborns)
Warfarin
End stage liver disease can look like it
What is factor V leiden?
V is accelerating factor (inhib by C and S normally) but it is MUTATED so resistant to activated protein C
most common kind
What is a prothrombin gene mutation causing?
Untranslated region causes more production of prothrombin and more clots
It is gene 20210 (like beverly hills!)
What is result of antithrombin deficiency? What is the cause?
No effect on PT, PTT or thrombin time, but can’t break down clots well. HEPARIN WON’T WORK B/c thats the mechanism of heparin (antithrombin activation)
Caused by inherited deficiency OR nephrotic syndrome as antithrombin is lost in urine
What tissues use malate shuttle and what uses G3P shuttle?
32 ATP are from mAlate shuttle
Heart and liver (a in malate is for extra ATP)
G3P is in muscle for 30 atp
Heparin action
toxicity
Antidote?
Increases antithrombin activity (less Xa and thrombin)
Short half life
Tox: osteoporosis, Bleeding, HIT
HIT is heparin induced thrombocytopenia: heparin binds platelet factor 4, autoantibody for heparin PF4 complex activates the platelets and thrombosis and thrombocytopenia!
What do you use if patient has HIT but still needs anticoag?
Thrombin inhibitors (same part of coag cascade, but different mechanism). After you stop heparin, of course
Bivalirudin
Argatroban
What does warfarin inhibit?
Synthesis of gamma carboxylation of Vit K dependent clotting factors II VII IX X and C and S
What does antithrombin act on?
Decreases action of IIa (thrombin) and Xa
What are the thrombolytics? What is the mechanism?
What do you give for toxicity? (KNOW THAT)
Alteplase, urokinase, and streptokinase
Activate PLASMIN which breaks down fibrin clots
STEMI if no cath lab
Acute Stroke if early on
AMINOCAPROIC ACID! KNOW THAT
What causes potter syndrome?
Failure of devo of ureteric bud
Life span of RBC? High yield factoid.
120 days (4 months)
Basophilic stippling looks like what? is caused by what?
Purple dots in RBC.
Lead is the LEADING cause of basophilic stippling. Remember that.
Thalassemia, anemia of chronic disease and alcohol can also do it
Echinocytes look like what? caused by what?
Regular spikes are caused by uremia/renal failure
Acanthocytes
Spur cells. Spikey but irregular.
Liver disease and abetalipoproteinemia (states of cholesterol dysregulation)
Spherocyte caused by what?
hereditary spherocytosis or autoimmune hemolysis
Schistocytes are caused by what? What do they look like?
Helmet cell seen in: DIC, TTP/HUS (pathologic clotting causing cells to be forced through the fibrin and they get sheared), traumatic hemolysis (heart valve prosthesis)
Target cells caused by what? mnemonic alert
THAL
Thalassemia
Hemoglobin C disease
Asplenia
Liver disease
What are howell-jolly bodies? When do you see them?
Basophilic nuclear remnant in RBC and normall removed by splenic macrophage, but if a splenic, you still have them.
Big purple spot in cell
Bit cell and heinz bodies?
G6PD.
No HMP shunt, no reduced glutathione, so oxidized hemoglobin makes heizn bodies.
Heinz bodies stain with methylene blue
Teardrop bells when?
Myelofibrosis b/c squeezing cells out of fibrotic bone marrow and they get malformed
Ringed sideroblasts look like what? What do you consider?
Blue dots surrounding nucleus in RBC. Consider: LEAD POISNOING, or other stuff.
Happens when heme synth disorder and iron granules surround the nucleus or RBC
What is erythroblastosis fettles? Symptoms?
Maternal anti Rh
Give anti Rh at 28 weeks, after trauma, and within 3 days delivery
Anemia from hemolysis
jaundice and kernicterus
Hydrops fetalis (fetal edema)
Intrauterine death
Where is fetal blood from Mnemonic alert
What are hemoglobin subunits?
Young Liver Synthesizes Blood
Yolk sac
Liver
Spleen
Bone marrow (long bones of kids, axial bones of adult)
Alpha always, gamma goes, becomes beta
What is rate limiting step of heme synthesis?
delta ALA synthase
What are symptoms for acute intermittent porphyria?
What is cause?
What is tx?
6 P’s of acute intermittent porphyria
Porphobilinogen deaminase Painful abdomen (neuropathic) Port wine urine Polyneuropathy Psych disturbances Precipitated by booze, drugs, starvation
Tx: heme and glucose to inhibit delta ALA synthase (rate limiting step upstream)
What is associated with porphyria cutanea tarda?
What is the cause?
Uroporphyrinogen decarboxylase (easy to remember b/c tea colored Urine in uroporphyrinogen decarb)
Think homeless man: big beard (hypertrichosis), sunburn (photosensitive), hepatitis c, LFTs, boozer
What enzymes does lead effect?
Tx?
2 steps. ALA dehydrogenase and Ferrochelatase (last step). Last step problem causes accumulation of protoporphyrin
EDTA or succimer
Lead poisoning symptoms. mnemonic
Findings?
Mental retardation
LEAD
Lead Lines on gingival or metaphase of long bones
Encephlaopathy and erythrocyte basophilic stippling
Abdominal colic and sideroblastic Anemia
Drops of wrist and foot and Dimeracaprol and EDTA are first line treatment
Also renal failure and retardation.
Succimer is used for chelation in kids b/c SUCks to be kid who eats lead
Tumors that release EPO? mnemonic alert
Potentailly Really High HEMatocrit
Pheo
RCC
HCC
Hemangioma
Tx of sideroblastic anemia?
Iron levels?
sideroblasts in mitochondria
B/c ala synthase is slow
Give B6 b/c cofactor for ala synthase
High serum iron High ferritin (both b/c iron is just piling up)
B12 vs folate deficiency? which is seen with boozers?
What do you see for homocysteine and MMA?
Booze can cause folate deficiency (think B12 is from meat, lots of alcoholics like meat)
Homocystein is increased for both
Methylmolonic acid is only increased in B12 deficiency
What is orotic acuduria?
Deficiency in UMP synthase, so pyrimidine synthesis issue.
You see megaloblastic anemia not responsive to folate or b12
Tx is UMP
orotic acid in urine
megaloblastic anemia
No hyperammonemia (how you differentiate with ornithine trancarbamylase deficiency)
Nonmegaloblastic macrocytic anemia is caused by what?
DNA synth is impared
Liver disease; alcoholism, reticulocytosis, drugs (50FU, zidovudine, hydroxyurea)
Mechanism for anemia of chronic disease?
Findings in blood and iron levels?
IL-6 causes hepcidin from liver to stop ferroportin and there is also activation of macrophage to take in iron
Low serum iron, low transferrin, high ferritin.
Starts as normocytic anemia, can progress to microcytic anemia
Bone marrow filled with adipocytes? What is the dx?
Aplastic anemia
Nonhemolytic normocytic anemias are what?
Anemia of chronic disease
Aplastic anemia (drugs, virus, fanconi (DNA repair defect), idiopathic)
Chronic kidney disease: no EPO
Thalassemia flash cards
make them
What does haptoglobin do? How does measuring it help with anything?
Haptoglobin binds up free hemoglobin in blood.
If hemolysis, haptoglobin has been bound up and levels decrease in intravascular
What is MCHC in hereditary spherocytosis?
RDW?
Higher b/c less volume but same hemoglobin, so higher concentration
Red sell distribution width is wider
What is a pyruvate kinase deficiency doing?
Autosomal recessive defecgt in pyruvate kinase (can’t make it from PEP), so you get red cell lysis and HEMOLYTIC anemia in newborn
What happens with deficiency of CD55 and CD59?
DAF is gone so don’t turn off complement of RBCs
Paroxysmal nocturanl hemoglobunira.
Hams test is red cell lysis at low pH b/c that activates complement
Kidney issue with sickle cell?
Renal papillary necrosis (also seen in heterozygotes)
What are warm agglutinins?
Cold
WARM is GGGreat.
IgG. SLE, CLL or with drugs like methyldopa
Coombs+
Cold is IgM with mycoplasma or EBV or CLL (again)
What is a coombs test?
What is indirect coombs?
Direct:
You add anti-human antibody antibodies to see if there is antibody bound to RBCs and then RBCs agglutinate
indirect is adding patients serum to normal RBCs to see if they will agglutinate when Coombs reagent is added to see if blood transfusion is safe
What is one cause of pigmented gallstones?
Hemolysis
What is Hams test?
Adding acid to RBCs to see if it activates complement and destroys cells b/c no CD 55/59 AKA DAF
