Lecture 7 (Hem/oncology)-Exam 4 Flashcards
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- What is MCV? What is small and large?
- What is MCHC? What is hypo/hyperchromic?
- WHat is MCH?
- What is RDW?
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HEMOCHROMATOSIS
* What is it?
* What are risk factors?
* What are s/s?
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- Iron Overload
- Risk factors: severe hemoglobinopathies, hematological malignancies, sideroblastic anemias, & multiple RBC transfusions
- Signs/symptoms: lethargy, hepatomegaly, hepatic cirrhosis, arthropathy/arthritis, diabetes mellitus, heart disease, hypogonadism
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HEMOCHROMATOSIS
* What are the labs?
* What is the imaging/diagnostics?
* What is the txt?
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- Labs: serum ferritin level significantly elevated, transferrin saturation elevated, and liver enzymes elevated
- Imaging/diagnostics: MRI/MRE of liver and liver biopsy
- Treatment: iron chelation agents (ie IV deferoxamine or PO deferasirox) & supportive treatment of damaged organs
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THALASSEMIAS
* Defect in what?
* What is beta thalassemia? Who is it mc in?
* What is alpha thalassemia? Who is it mc in?
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Defect in the alpha or beta chains of adult hemoglobin
* Beta thalassemia – defect of beta globin chains; low beta chains-> African, Mediterranean descent MC
* Alpha thalassemia - defect of alpha globin chains; low alpha chains->Middle East, North Africa, Southeast Asia descent MC
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THALASSEMIAS
* Imbalance of what?
* Decreased what?
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- Imbalance of alpha and beta chain production
- Decreased RBC survival and short RBC lifespan
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Hemoglobin:
* What is it?
* Normal hemoglobin made up of what? What does it do?
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- Oxygen carrying component of RBC
- Normal hemoglobin made of 2 alpha and 2 beta globin chains
- Each globin chain binds oxygen molecule
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Beta thalassemia
* What are the chains like?
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- Two normal alpha chains
- Partial (B+) or complete (B) beta globin deficiency
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BETA THALASSEMIA
* What mutation?
* Dominant or recessive?
* Reduced or absent what?
* Increased or unmatched what? What does it cause?
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Point mutation on chromosome 11
Autosomal recessive
* Two mutated genes required to cause severe disease
Reduced or absent beta globin chain synthesis
Increased, unmatched alpha chains
* Clump together forming inclusion bodies
* Damage RBC cell membranes
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BETA THALASSEMIA
* What does deficiency of beta globin chains lead to?
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Increased free alpha globin chains
* Clump together and form inclusion bodies
* Damage RBC cell membrane causing
* Hemolysis – RBC destroyed in bone marrow
* Extravascular hemolysis – RBC destroyed in spleen
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Beta thalassemia
* Hemolysis leads to what?
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- Hemoglobin spillage directly into plasma
- Hypoxia
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Beta thalassemia-> hemolysis
* Hemoglobin spillage directly into plasma. Heme gets recycled to where (2)
* Hypoxia increases what? What is enlarged?
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Beta thalassemia-hemolysis
* What is ineffective?
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Ineffective erythropoiesis, chronic anemia, hypoxia cause increased iron absorption from duodenum
* Worsens hemochromatosis
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Hemochromatosis
* What are the different areas?
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- Cardiac (arrhythmias, pericarditis)
- Liver (cirrhosis)
- Endocrine (growth retardation, thyroid disorders, diabetes mellitus)
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Beta thalassemia:
* Minor: What is the genetic mutation and what are the symptoms?
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Beta Thalassemia
* Intermedia: what is the genetic mutation? What are the sx?
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Beta Thalassemia
* Major: what is the genetic mutation? What are the sx?
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transfusion dependent beta thalassemia
* What are the indications for transfusion? (3)
* What is the timing?
* What are the goals (3)?
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Iron chelation therapy (ICT)
* Prevents accumulation of what?
* Remove what? (2)
* Initiation? What are the indications (ferritin levels and liver iron concentration)?
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Iron absorption
* How is heme iron absorbed?
* How is non heme iron absorbed?
* Once absorbed Fe2 is stored by what?
* Once needed-what happens?
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What are the typical sxs of anemia? What are the iron deficiency sxs?
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Iron deficiency anemia - treatment
* What is first line?
* What is second line?
* What is for unstable patients?
First-line:
* oral iron replacement (stable patients)
Second-line:
* parenteral iron replacement (stable patients)
Packed RBC transfusion (unstable patients)
* Reserved for severe anemia (< 7 gm/dL) with symptoms
* Menstruating woman
* Rectal bleeding
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Oral iron supplements: General considerations
* Different amounts of what?
* Variable what?
* Best absorption when?
* Avoid taking with what?
* What is common about the treatment?
* Different amounts of elemental iron with each iron salt
* Variable absorption – improved with acidic environment (Vit C?)
* Best absorption when taken on empty stomach – **most patients cannot tolerate**
* **Slow-release, enteric-coated tablets generally not effective**
* Avoid taking with medications that decrease absorption
* **Treatment failure common** – noncompliance due to intolerance
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# Iron replacement PO
Avoid taking with medications that decrease absorption? (3)
* Al, Mg, Ca containing antacids
* Antihistamines
* Proton pump inhibitors
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Ferrous sulfate:
* What is the dose?
* What are the adverse effects?
* What are the CI?
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IV iron replacement
* What are the indications? (4)
* Various formulas exist for determining what?
* Specific administration recommendations vary by what?
* _ efficacy
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IV iron replacement: LMW iron dextran
* What is the disadvange and benefit?
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Anemia of chronic disease (inflammation):
* What will chronic renal disease have?
* What will chronic inflammation have? What are different causes of chronic inflammation.
* What will be elevated and low?
* What is the txt?
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Erythropoietin
* How does erytrohopietin work?
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Recombinant erythropoietin (EPO)
* What are Erythropoietin stimulating agents (ESAs)? What are two examples?
* What is the indication?
Erythropoietin stimulating agents (ESAs) are recombinant EPO produced pharmacologically via recombinant DNA technology
* epoetin alfa (Procrit)
* darbepoetin (Aranesp)
Indication: anemia from CKD or malignancies with hemoglobin < 10 g/dL
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EPO dosing:
* CKD:
* Chemo related anemia:
* Surgery associated transfusion reduction: ⭐️
* CKD: 50 to 100 units/kg IV or SC **three days/week**
* Chemo-related anemia: Start with 40,000 units subcutaneously every week or 150 units/kg/dose subcutaneously three times weekly
* **Surgery-associated transfusion reduction: 300 units/kg/dose subcutaneously daily for 15 days before surgery**
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Dosing of darbepoetin:
* Chronic kidney disease-associated anemia
* Chemo-related anemia:
JUST KNOW THIS ONE WAS MARKETED FOR A WEEK
* Chronic kidney disease-associated anemia: Start with 0.45 mcg/kg IV or subcutaneously weekly.
* Chemo-related anemia: Start with 2.25 mcg/kg/dose subcutaneously every week or 500 mcg subcutaneously every three weeks
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Recombinant erythropoietin (EPO)
* What are the adverse effects?
* Risk of thrombotic events – increased blood viscosity
* Increased risk of tumor progression – not indicated for myelosuppressive chemotherapy if cure is anticipated outcome
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Recombinant erythropoietin (EPO)
* What are the goals (4)
* Lowest dose to reduce RBC transfusions
* **Hb level should be no greater than 11g/dL**
* **Monitor Hb at least weekly**
* Consider addition of iron if no effect
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Fill in for normocytic anemias
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Sickle cell anemia
* What type of disease?
* Most common with what population?
* What results in sickle hemoglobin?
* Autosomal recessive
* Most common with African heritage (1:400 African American births)
* Substitution of valine for glutamic acid on the beta-hemoglobin chain resulting in sickle hemoglobin (HbS)
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* Sickle cell trait (SCT) –
* Sickle cell SS –
* Sickle cell SC –
* How is diagnosed?
* Sickle cell trait (SCT) – one normal, one sickle gene (HbA, HbS)
* Sickle cell SS – two sickle genes (homozygous); more severe (HbS, HbS)
* Sickle cell SC – one HbS, one HbSC; less severe
* Diagnosed at birth or via hemoglobin electrophoresis
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Newborn screening
* Screening for what?
* What is not required
* Screening for sickle cell mandated in all states
* Thalassemia is not core
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Sickle Cell Anemia
* What does HbS cells carry like?
* What causes the HbS cells to sickle?
* What can sickle cells do after?
* What is repeat sickle?
* HbS cells carry oxygen just like HbA
* Deoxygenation, dehydration, acidosis cause HbS cells to sickle
* Sickled cells can return to normal once reoxygenated
* Repeat sickle – unsickle results in damage and premature destruction
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Repeat sickle – unsickle results in damage and premature destruction
* What are the examples of damage?
Anemia
Hyperbilirubinemia (hemolysis)
* Jaundice
* Scleral icterus
* Gallstones
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What is the normal hemoglobin throughout the years?
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Sickle cell anemia
* What do sickled cells not do well!
* What leads to pain?
* Sickled cells do not move through capillaries well
* vaso-occlusion -> hypoxia -> tissue ischemia ->PAIN
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Sickle cell anemia: Vaso-occlusion
* MC where?
* What bones?
* What happens in spleen?
* CNS?
* Lungs?
* kidneys?
* Penis?
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Sickle cell anemia: Effects on the spleen
* What happens to cause spleen sequestration?
Spleen sequestration (mild to severe)
* RBCs trapped in spleen
* Medical emergency
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Sickle cell anemia: Effects on the spleen
* How does spleen infaract happen?
* What is there an increase risk of?
Infarction – scarring and fibrosis causing auto-splenectomy (aka: functional asplenia)
* Increased risk of infection with encapsulated organs
* S. pneumonia, H. influenza, N. meningitis, Salmonella spp
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What is the treatment strategies of effects on the spleen from sickle cell anemia?
* Penicillin or amoxicillin prophylaxis for children < 5 years
* 23-valent pneumococcal vaccine after age 2 years
* Close monitoring for patients with fever
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VOC treatment
* Where is treatment for mild?
* What is the txt for moderate to severe?
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Acute txt of SC:
* What is the cause, txt, and notes for anemia, vaso-occlusive crisis, splenic sequestration?
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Acute txt of SC:
* What is the cause, txt, and notes for splenic auto infaraction, stoke and acute chest syndrome?
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What is the VOC prevention?
* How does it work?
* Approved for who?
* Can decrease what?
* hydroxyurea
* Increases HbF by stimulating HbF production->Exact mechanism unknown
* Approved for children and adolescents
* Can decrease vaso-occlusive episodes by up to 50%
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What are the adverse effects of Hydroxyurea? What is the monitoring?
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Fill in for the macrocytic anemias?
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Macrocytic anemias (MCV > 100𝜇𝑀3)
* What are the causes of vit b12 cobalamin?
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What is pernicious Vitamin B12 Deficiency?
* Vitamin B12 (cobalamin) is a precursor for what? What happens when there is a deficiency?
* What happens to blood cells?
* What happens to oral mucosa?
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Vitamin B12 Deficiency
* What lab levels are increased?
* What are they and what do they cause?
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What are the s/s of vit b12 deficiency?
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What are the labs you need to order for vitamin b12 diangosis?
* CBC
* Periferal blood smear
* Vitamin B12 and folate levels
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What will the cbc and peiferal blood smear show in a pt with vit b12 anemia?
* CBC – low hemoglobin / hematocrit, MCV > 100
* Periferal blood smear – large red blood cells
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Vitamin b12 and folate levels in vit b12 def
* What level is deficient, borderline?
* < 200 pg/mL = deficiency
* 200 to 300 pg/mL = borderline
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Consider checking methylmalonic acid (MMA) and homocysteine levels in vitamin b12 diagnosis
* What are the levels in b12 deficiency and folic acid deficiency?
* MMA and homocysteine **elevated** – B12 deficiency
* MMA normal, homocysteine elevated – folic acid deficiency
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Fill in for vitamin b12 txt
only drug and route
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Folic acid (vitamin B9) deficiency
* increase demand cause?
* Decrease intake casue?
* What are dectrease absorption causes?
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What are the drugs that cause folic acid (vit B9) deficiency?
* Phenytoin
* Trimethoprim
* Sulfasalazine
* Methotrexate
| Tri sulfing on meth, phen (phone) a friend
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Folic acid deficiency
* What is folic acid? What does a deficiency cause?
* What happens to Blood cells?
* What happens to the oral mucosa?
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Folic acid deficiency
* What are the homocysteine and methylmalonic acid levels? What do they cause?
* What happens to embyro?
Increased levels of homocysteine and normal methylmalonic acid
* Homocysteine = increased release of proinflammatory cytokines in vasculature and clumping of platelets (atherosclerosis, cardiovascular events)
Impaired closure of fetal neuropore
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Folic acid deficiency diagnosis
* What labs do you need to order?
* CBC
* Periferal blood smear
* Vitamin B12 and folate levels (serum)
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Folic acid deficiency diagnosis
* What does the cbc, periferal blood semear, vitamin b12 and folate levels show?
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Folic acid deficiency diagnosis
* What is the txt?
* Folic acid
* 1 to 5mg PO daily for 2-4 months
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Which of the following physical findings suggest pernicious anemia?
* Splenomegaly
* Petechiae
* Loss of vibratory sense
* Koilonychia
* Cheilitis
* Loss of vibratory sense
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Ferrous sulfate
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Autoimmune (Hemolytic anemia)
* What is first line and second line txt?
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Autoimmune (Hemolytic anemia)
* What might you remove and why?
Splenectomy
* 60 to 90% response rate; 30% relapse rate
* Response greater in those without underlying malignancies
* Vaccination against encapsulated organisms at least 14 days prior to splenectomy
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Rituximab:
* What is the MOA?
* What are the BBWs?
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Rituximab SE?
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Glucose-6-phosphate dehydrogenase (G6PD)deficiency
* What type of disease?
* Increases what?
* What does RBC hemolysis cause?
* What is damage?
* What does the perperial blood smear show?
* What does urobilin show?
* most patients are what?
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What does this show?
Glucose-6-phosphate dehydrogenase (G6PD)deficiency
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What are the G6PD triggers?
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Thrombocytopenia
* What is normal platelet count?
* What is thrombocytopenia?
* What is clinically significant?
* Risk of severe post-tramatic bleeding level?
* What is the level for spontaneous bleeding
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What are the causes of Thrombocytopenia
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Immune/Idiopathic Thrombocytopenic Purpura (ITP)
* What type of disease?
* Affects who?
* What do the labs show?
* How do you dx?
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ITP treatments
* What do you need to give to actively bleeding patients?
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ITP treatments
* What do you give a patient not bleeding?
* What is first line? (2)
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ITP treatments – second line
* Thrombopoietin-receptor agonists: What are the tyeps, approved for who?
* What are the adverse effects?
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ITP treatments – third line
* Immune modulators: what is the drug?
* What can you remove? What are the pros and cons?
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Pneumococcal 23 valent vaccination IM
