HEME/ONC/ENDO part 2

Question 1

IRON DEFICIENCY ANEMIA

• Diagnosis either by labs or trial of iron replacement therapy
o Oral:
 _____ 325 mg qd providing 180mg of iron of which approximately 10mg are absorbed (more in a severely deficient state); on an empty stomach
 Most appropriate….with food and go slow
 Appropriate response: return of hematocrit to level halfway to normal within 3 weeks with full return within 2 months.
 Iron therapy should then continue for another 3-6 months.
• Failure to respond:
o Non compliance: nausea and constipation
o Extended release formulation better tolerated
o Poor absorption; add ascorbic acid 250mg as patient may be achlorhydric.
o Incorrect diagnosis
• _____
o Indicate for those intolerant to oral iron, are refractory to oral iron, GI disease (usually IBD), and continued blood loss that can’t be corrected
o Only advocated for use in persistent anemia after reasonable course of oral therapy
o Possibility of anaphylaxis; lower incidence with sodium ferric gluconate
o Coat the iron in protective carbohydrate shells
o Current preparations are safe and can be infused in less than 5 minutes
o Doses up to 510mg by IV bolus over 15 mins
o Most patients need 1-1.5g which corrects for iron deficit and replenishes stores

Answer 1

Ferrous sulfate

Parenteral Iron

Question 2

ANEMIA OF CHRONIC DISEASE

• In most cases, no treatment is necessary
• _____ (epoetin alfa)
o Effective for kidney failure, and anemia related to cancer or inflammatory disorders and HGB less than 10 g/dL
o Requires adequate intensity of _____ to be effective in dialysis
o Expensive so should only be used in transfusion dependent or highly responsive candidates (quality of life improved by hematologic response) (i.e.: maintaining HGB between 10-12g/dL

Answer 2

Recombinant erythropoietin

dialysis

Question 3

THALASSEMIA

• Mild Thalassemia (alpha trait or Beta minor)
o No Treatment
• Hemoglobin H
o _____ supplementation; avoid medicinal iron and oxidative drugs(sulfonamides)
• Severe Thalassemia
o Regular _____
o _____ supplementation
o Splenectomy
o _____ (subq infusion of deferoxamine)
o Allogenic _____ transplantation
 Beta thalassemia Major; only curative tx

Answer 3

Folate
transfusions
Folate
Iron chelation
bone

Question 4

VITAMIN B12 DEFICIENCY PERNICIOUS ANEMIA

• Parenteral therapy ___
o IM/subq doses of 100mcg-1000mcg q day x 1 week; q week x 1 month and then monthly for life
o Oral cobalamin may be used since 1% of dose is absorbed 1mg/day indefinitely (once correction of the deficiency has occurred)
o Neurologic symptoms: long term parenteral therapy; _____ therapy as well since may have concomitant deficiency,
o Immediate improvement in their sense of well being
o Hypokalemia may complicate first several days of therapy
o Reticulocytosis in first 5-7 days; clinical picture normalizes in 2 months
o CNS symptoms resolve if > 6 months in duration but become permanent if not treated promptly
o Rare transfusion of RBC; _____ to prevent heart failure (develops slowly and the plasma volume is increased).

Answer 4

B12
folic acid
diuretics

Question 5

FOLIC ACID DEFICIENCY

• _____ replacement of 1mg/d
o Response similar to what is see in Vitamin B12 deficiency
o Rapid improvement and sense of well-being within 5-7 days
o Correction of hematologic abnormalities in 2 months
o Large doses may correct symptoms of B12 deficiency except for the neurologic damage

Answer 5

Folic Acid

Question 6

G6PD deficiency

• None but avoidance of _____ (ex. drugs that cause oxidative stressors)

Answer 6

stressors

Question 7

POLYCYTHEMIA VERA

· 1st line –> _____
o 500ml weekly (one unit) until hematocrit is <45%); then maintain by repeated phleb PRN
§ Requirement for phleb should gradually _____
§ Avoid _____ & it may thwart efforts of phleb/low iron diet
§ Maintaining HCT at <45% decreases incidence of thrombotic complication
· 2nd line –> _____ (Myelosuppressive Therapy)
o If phleb not practical or high phlebotomy requirement, thrombocytosis, intractable pruritus
· Adjunct Therapy –>
o Low dose _____ = reduce risk of thrombosis & not increase bleed risk
o Allopurinol = hyperuricemia (300mg/d PO)
o Diphenhydramine or other H1 antagonist = pruritus

Answer 7

phlebotomy

decrease
medicinal iron
Hydroxyurea
Aspirin

Question 8

SICKLE CELL ANEMIA

TREATMENT
àALLOGENEIC HEMATOPOIETIC _____ TRANSPLANTATION
- Before the onset of significant end-organ damage
- Can cure more than 80% of children with sickle cell anemia who have suitable HLA-matched donors à get a stem cell from someone else with same genetic makeup and can cure these kids
- Reasonably good quality of life
- Investigational in adults

àMEDS
- Disease modulators – _____
o Reduces the frequency of painful crises in patients whose quality of life is disrupted by frequent vaso-occlusive pain episodes (three or more per year)
o Increases hemoglobin F levels epigenetically
o Potent chemotherapeutic agent with potent effects on the bone marrow
- Omega-3 fatty acid supplements may also reduce vaso-occlusive episodes and reduce transfusion needs
- Monoclonal antibody (_____) reduces vaso-occlusive episodes by 50% by blocking P selectin and disrupts the adverse interactions of platelets, red blood cells, and leukocytes with the endothelial wall
- L-glutamine has been shown to favorably modulate sickle pain crises and acute chest syndrome
- ACEI recommended in patients with microalbuminuria

àSUPPORTIVE CARE = THE MAINSTAY OF TREATMENT

• _____ supplementation of 1 mg orally daily
• Transfusions for aplastic or hemolytic crises
• Precipitating factors should be identified and infections treated if present
• Keep patient well _____; generous analgesics and supply oxygen if hypoxic
• Pneumococcal vaccination

àFOR ACUTE VASO-OCCLUSIVE CRISIS – DO _____
- Especially in the presence of priapism, significant pain, acute chest syndrome, or stroke
o Long term transfusion therapy reduces risk of stroke in children
o Phenotypically matched transfused red blood cells are recommended to reduce the risk of red blood cell alloimmunization à use matched red cells

Answer 8

STEM CELL
HYDROXYUREA 
CRIZANLIZUMAB-TMCA
Folic acid
hydrated
EXCHANGE TRANSFUSIONS

Question 9

AUTOIMMUNE HEMOLYTIC ANEMIA

• _____ 1-2 mg/kg/d ¬– we have autoantibodies being produced so suppress immune system with prednisone
• _____ –> may cure the disorder (often successful)
• Rapid hemolysis à requires plasmapheresis – remove all the autoantibodies from plasma
• Treatment with monoclonal antibodies (_____), danazol (modified testosterone), immunosuppressive agents (cyclophosphamide, vincristine, azathioprine, etc.), and high dose intravenous immune globin
• Transfusion under guidance of hematologist (difficult to cross-match due to autoantibody)

Answer 9

Prednisone
Splenectomy
Rituximab

Question 10

APLASTIC ANEMIA

• Supportive treatment –> erythropoietic or myeloid growth factors or both **giving them something to produce more RBCs or WBCs
• Transfusions of _____ and platelets
• _____ as needed for infections
• Severe aplastic anemia –> median survival without treatment is 3 months (only 20% of patients survive a year)
  o < 40 y/o: allogeneic _____ transplant in HLA matched relative; may benefit from non-relatives if no benefit from immunosuppression **can do with non-relative donor if immunosuppression (prednisone) and no benefit
  o > 40 y/o: immunosuppression with equine antithyocyte globulin (ATG) + _____; may add eltrombopag (thrombopoietin mimetic) + steroids to avoid ATG infusion reaction and serum sickness
• Summary: do supportive treatment, do transfusion if needed, but if we cannot correct the issue –> immunosuppression is the choice if over 40 or under 40 who have no matched relatives (can go outside family line)

Answer 10

RBCs
antibiotics
bone marrow
cyclosporine