Heme/Onc Flashcards

Question

B12 deficiency anemia

Answer 1

pernicious anemia- autoantibodies attack gastric parietal cells in the stomach so that the body can't make IF inadequate B12 intake (vegetarian) Note: folate insufficiency develops a lot more quickly than B12 deficiency Resection of the ileum Bacterial overgrowth Diphyllobothrium latum infection (fish tapeworm) ``` H and P peripheral neuropathy paresthesias ataxia loss of vibration sense dementia ``` labs: megaloblastic anemia decreased B12 levels high serum MMA and homocysteine (intermediate byproducts that build up with B12 is insufficient) Schilling test (out-dated) Treatment: - IM B12 - dietary B12 supplements - Intranasal B12

Answer 2

Anemia in patients with chronic inflammatory states DM defect in iron mobilization and utilization total body iron content is normal serum iron levels may be low Treatment: treat the underlying disorder supplemental erythropoietin to hgb 11 or 12

Answer 3

pancytopenia from bone marrow failure (leuopenia and thrombocytopenia as well) ``` Causes: radiation drugs toxins viral infection (EBV, HIV, parvovirus) SCD- parvo B19 aplastic crisis idiopathic congenital ``` H and P anemia (weakness, pallor, fatigue) leukopenia (persistent infections) thrombocytopenia (poor clotting, easy bruising, petechiae) Labs: pancytopenia hypocellularity IH: fatty infiltration, with aplastic anemia Treatment: address underlying cause bone marrow transplant worse the older you get

Answer 4

hemolytic anemia | DIC, TTP, HUS

Answer 5

abetalipoproteinemia

Answer 6

uremia, hemolysis

Answer 7

G6PD deficiency | it's macrophages that bite the Heinz bodies

Answer 8

lead poisoning

Answer 9

lead poisoning

Answer 10

megaloblastic anemia

Answer 11

G6PD deficiency

Answer 12

anemia due to defects in the heme synthesis pathway ``` May be inherited (genetic) or acquired (alcohol abuse, INH, lead poison, zinc tox, copperdeficiency) ``` High RDW MCV low, hypochromic sideroblasts are normal nucleated RBC precursors with visible iron granules in their cytoplasm, found in the bone marrow Ringed sideroblasts, which have iron granules surrounding the nucleus, are NOT normal. ``` Treatment: Supplement vitamin B6 (pyridoxine) Address underlying cause, stop alcohol Transfusions Erythropoietin Deferoxamine or phlebotomy if patient is iron-overloaded ``` myelodysplasia with ringed sideroblasts also exists and can progress to acute leukemia

Answer 13

alpha thalessemia- defect in alpha globin more prevalent in patients of African and Asian descent alpha thalessemia minima: 1 mutated allele, asymptomatic alpha thal minor or trait: 2 mutated alleles, asymptomatic ``` HbH disease: 3 mutated alleles a ton of beta globin made of 4 beta globin molecules microcytosis chronic hemolysis ``` Hemoglobin Bart's: 4 mutated alleles, incompatible with life made of 4 gamma globins hydrops fetalis (leads to death)

Answer 14

defect in beta globin mediterranean populations beta thal minior: - decreased amounts of beta- globin - minimal anemia - increased HbA2, made of alpha globin and gamma globin Beta thal major: no beta globins at all HbA2 and HbF more severe anemia target cells: central darkening, surrounded by pallor, then more darkening on the outside

Answer 15

iron overload due to transfusions: iron chelator (deferoxamine) for iron overload rule out thalessemia before starting iron supplements on an anemic patient. Rule out can be done with hemoglobin electrophoresis

Answer 16

``` H and P sickle cell pain episodes infection hypoxia trauma bone pain/infarction: fish vertebrae (middle infarcts, so the vertebrae get narrow in the center) ``` osteonecrosis of the femoral head or humoral head hair on end on skull xray chest pain stroke painful swelling of hands and feet ``` dyspnea priapism splenomegaly jaundice leg ulcers ``` ``` labs: anemia sickling HbS increased HbF ``` ``` Treatment: hydration oxygen analgesics folate longterm management with hydroxyurea, increased HbF avoid triggers, stay healthy ``` ``` Treatment: pneumococcal vaccine especially if asplenic HiB Meningococcal vaccine hep B vaccine annual flu vaccine ``` prophylactic penicillin until age 5 Transfusions Stem cell transplant Gene therapy Complications: - chronic anemia - pulmonary htn because of occlusion of pulmonary vasculature - heart failure - aplastic crisis - acute chest syndrome (cp, hypoxemia, pulm infiltrates) - stroke - osteonecrosis - autosplenectomy vaccinate against Hib, pneumococcus, meningococcus, influenza, hep B - increased risk of infection - salmonella osteomyelitis - strep pneumo - h, influenzae - meningococcus - klebsiella

Answer 17

acute leukemia

Answer 18

salmonella

Answer 19

increased cortisol levels chemotherapy radiation lymphoma

Answer 20

Collagen-vascular disease Atopic diseases (allergies, asthma, Churg-strauss, allergic bronchopulmonary aspergillosis) Neoplasm Adrenal insufficiency (Addison disease) Drugs (NSAIDs, penicillins, cephalosporins) Acute interstitial nephritis Parasites (strongyloides, ascaris- loffler eosinophilic pneumonitis) other causes include HIV, hyper IgE syndrome, coccidiomycosis, etc)

Answer 21

viral infections: viral hepatitis, HIV, EBV certain drugs chemotherapy aplastic anemia H and P recurrent infections Treatment: G-CSF GM-CSF Steroids

Answer 22

admit the patient for observation culture blood, urine, CSF broad-spectrum abx narrow down abx after you've isolated an organism

Answer 23

mediated by IgE antibodies, cross-link antibodies on mast cells mechanism: degranulation of mast cells hives, pruritis, bronchospasm, oropharyngeal angioedema, allergic rhinitis, asthma, anaphylaxis ``` Treatment: antihistamines leukotriene inhibitors bronchodilators corticosteroids ```

Answer 24

mediated by IgM and IgG antibodies directed towards self cells bind to specific antigens, and activates the complement cascade examples: - drug- induced hemolytic anemia - immune hemolytic anemia - hemolytic disease of the newborn - immune thrombocytopenia - acute rheumatic fever - Goodpasture syndrome - Bullous pemphigoid - Pemphigus vulgaris - Graves disease - Myesthenia gravis Treat: anti-inflammatories immunosuppressive agents plasmapheresis

Answer 25

mediated by IgM and IgG immune complexes against soluble antigens (soluble in the blood) The immune complexes deposit in tissues, and there they initiate complement cascade Examples: Arthus reaction serum sickness glomerulonephritis Treatment: anti-inflammatories

Answer 26

T cells and macrophages delayed-type hypersensitivity mechanism: T cells present antigens to macrophages examples: Transplant rejection, contact dermatitis, PPD testing Treatment: steroids, immunosuppressants

Answer 27

``` severe type 1 hypersensitivity MCC drugs also, insect stings latex eggs, nuts, seafood ``` H and P si/sx 5-50 minutes after exposure ``` tingling in the skin pruritis chest tightness angioedema (can't breath, can't swallow) syncope tachycardia wheezing urticaria hypotension ``` ``` treatment: ABCs, intubate if necessary stop the offending agent epinephrine H1/H2 blockers, helps with itching but not airway bronchodilators steroids (maybe) IV fluids if hypotension because you are trying to fill up the vascular space that opens up 2/2 vasodilation ```

Answer 28

``` drugs infections aplastic anemia folate and B12 deficiency alcohol, toxic to the bone marrow ``` bone marrow will show reduced or abnormal megakaryocytes Treatment: stop the offending agent, initiate bone marrow transplant if needed

Answer 29

``` Heparin, abciximab carbamazepine, phenytoin, valproate cimetidine acyclovir, rifampin sulfonamides (eg sulfasalazine, TMP-SMX) procainamide, quinidine quinine, gold compounds ```

Answer 30

splenomegaly normal one marrow biopsy Splenectomy can be curitive

Answer 31

Thrombosis plus thrombocytopenia (sudden decrease in platelet count by at least 50%) - heparin forms complexes with platelet factor 4 - antibodies against the complex cause platelet activation and aggregation (hypercoagulable thrombopenic state) - platelets are removed from circulation Labs: - serotonin release assay (gold standard, but expensive) - immunoassay (ELISA) detects platelets - heparin- induced platelet aggregation assay (specific, but not sensitive) Treatment: stop heparin, get labs to confirm the diagnosis anticoagulate with direct thrombin inhibitor like argatroban until PLT gets about 100,000 or so Then switch to warfarin for 3 months, or direct thrombin inhibitor These patients should avoid heparin

Answer 32

anti-platelet antibodies | platelet count

Answer 33

thrombotic thrombocytic purpura and hemolytic uremic syndrome deficiency of metalloprotease, leading to excessive platelet aggregation associated with E.COli O157:H7 HUS triad: hemolysis, uremia, thrombocytopenia ``` TTP pentad: hemolysis, uremia, thrombocytopenia neurologic sequelae fever ``` Fever, Anemia, Thrombocytopenia, Renal, Neuro symptoms (FAT RN) all features do not have to be present at once Treatment: steroids plasmapheresis FFP

Answer 34

antiphospholipids against specific phospholipids, increasing risk of thrombocytopenia and hypercoagulability seen in pregnancy lupus other autoimmune diseases

Answer 35

sequelae of eclampsia/pre-eclampsia ``` HELLP: Hemolysis Elevated Liver enzymes Low Platelets (Hypertension) ``` Treat by inducing labor, and delivering the baby

Answer 36

autosomal dominant deficiency of vWF and sometimes factor 8 MC inherited bleeding disorder vWF is released into local circulation from endothelial cells 1. It helps platelets adhere to vessel walls and crosslink with one another 2. it stabilizes factor VIII This is important for formation of fibrin clot Labs: increased PTT due to factor 8 dysfunction increased BT decreasd RISTOCETIN COFACTOR activity decreased vWF antigen normal platelet count, platelets are normal too, they just won't work H and P: easy bruising mucosal bleeding menorrhagia Tx: Desmopressin (induces vWF secretion), first-line for acute bleeding Cryoprecipitate or factor VIII concentrates for severe or refractory bleeding OCP for menorrhagia avoid aspirin and other platelet inhibitors

Answer 37

vWF factor 8 these remain at normal levels even when there is liver failure

Answer 38

10,9,7,2,c,s poor intake (green leafy vegetables) malabsorption eradication of gut flora that produce vitamin K ``` H and P: easy bruising mucosal bleeding melena hematuria delayed clot formation macrohemorrhage ``` Labs: increased PT, INR Treatment: PO or IM vit K FFP for acute bleeding RF: ESRD

Answer 39

``` H and P uncontrolled bleeding that is spontaneous, following minimal trauma, after surgery hemarthrosis IM bleeding GI and GU bleeding ``` This is a macrohemorrhage, fibrin clot issue Labs: increasd PTT normal PT normal BT ``` Treatment: Hemophilia A- give factor VIII Hemophilia B- give factor IX Desmopressin can induce factor VIII release Transfusions may be needed ``` Complications: hemophilia can lead to death from severe uncontrolled bleeding

Answer 40

excess clotting leads to hypocoagulability ``` STOP Making Thrombi Sepsis Trauma OB complications Pancreaitis Malignancy Transfusions ``` ``` H and P uncontrolled bleeding hemoptysis jaundice distal cyanosis hypotension especially with sepsis ``` ``` Labs: decreased platelets increased bleeding time increased PT increased PTT everything is messed up decreasd fibrinogen (all being used up) increased D-dimer ``` Schistocytes Treatment: treat the underlying cause if platelets are diminished, replace platelets If PT and PTT are increased, give FFP or cryoprecipitate +/- heparin

Answer 41

due to coag cascade defects Factor V Leiden mutation (most common) Antithrombin deficiency Protein C deficiency Protein S deficiency Prothrombin gene mutation (prothrombin G20219A) Screen if patient has recurrent DVT or fhx of DVT anticoagulate for life only if they've had thrombotic event(s)

Answer 42

DDAVP for severe bleeding, cryoprecipitate or factor VIII concentrates OCPs avoid aspirin and other platelet inhibitors

Answer 43

factor V Leiden mutation

Answer 44

1. secure the airway, give oxygen 2. give IV fluids 3. Give vasopressors (NE is choice) 4. Colloid can help bulk up intravascular volume 5. If the pressors aren't working, consider adrenal insufficiency and glucocorticoids as treatment 6. Broad spectrum antibiotics initially. Avoid antibiotics before culturing to avoid false negative cultures 7. glycemic control between 140 and 180. If you gave glucocorticoids or the patient has DM, consider giving insulin

Answer 45

``` chloroquine primaquine quinine atovaquone/proguanil mefloquine ```

Answer 46

Infectious mononucleosis Downey cells Teenagers and young adults get this infection 90-95% of adults are seropositive symptoms appear 2-5 weeks after infection ``` H and P: 3-6 months of fatigue sore throat malaise lymphadenopathy- posterior primarily splenomegaly fever tonsillar exudates ``` can have comorbid strep throat ``` Labs: positive heterophil abs positive Epstein-Barr serology elevated LFTs hemolytic anemia thrombocytopenia increased lymphocytes ``` Tx: supportive care self- limited reassurance there is no antiviral medication available NSAIDs or acetaminophen for fever, sore throat, malaise Encourage rest and plenty of fluids Return to sport (risk of splenic rupture) -may return gradually to noncontact sports 3 weeks after symptom onset -may return gradually to contact sports 4 weeks after symptom onset Steroids only helpful if impending airway compromise due to enlarged tonsils or if life- threatening sequelae develop (fulminant liver failure, hemolytic anemia, thrombocytopenia) ``` Complications: splenic rupture aplastic anemia DIC HUS-TTP ```

Answer 47

38.390 RR>20 12,00010% bandemia

Answer 48

``` fever, myalgias, fatigue HA sore throat rash lymphadenopathy mucosal ulcers persistent high fevers ```

Answer 49

several years without symptoms while the virus is destroying lymphocytes

Answer 50

``` opportunistic infections AIDS-defining illnesses weight loss night sweats neurological changes recurrent/unusual infections ```

Answer 51

CD410% of baseline H and P: chronic diarrhea weakness fever Tx: HIV meds and supportive care, antiretroviral therapy, appetite stimulants (megestrol)

Answer 52

CD4 may be over 200 intellectual decline, ataxia, apathy due to virus. Treat with antiretroviral therapy diagnosis: cerebral atrophy on CT or MRI

Answer 53

streptococcus pneumoniae haemophilus influenzae nocardia severe symptoms: productive cough high fever hypoxia diagnosis: sputum gram stain, lobar consolidation on CR, diffuse, nodular infiltrates on CXR Treatment: pathogen specific

Answer 54

an AIDS- defining illness | CD4

Answer 55

CD4 35 mmHg

Answer 56

also AIDS- defining CD4

Answer 57

AIDS- defining H and : HA, confusion, fever, focal neurological symptoms, seizures Dx:multiple ring- enhancing lesions on CT or MRI Tx: pyrimethamine + sulfadiazine+ leucovorin or pyrimethamine + clindamycin

Answer 58

a demyelinating disease | CD4

Answer 59

99% sensitive for HIV antibodies If positive, then repeat ELISA is performed Preliminary positive= both screening tests were positive Confirm with specific western blot to screen out false positive

Answer 60

tracks disease progression clinical improvement during HIV treatment- how is HIV affecting the immune system?

Answer 61

measures viral replication in the blood PCR- amount of virus in a sample of blood how useful is the antiretroviral regimen may show evidence of acute infection prior to ab response

Answer 62

emtricitabine+ tenofovir+raltegravir