Lecture 2b Flashcards
Define aplastic anemia.
Failure of hematopoietic bone marrow due to suppression of injury to stem cells
What are the main causes of aplastic anemia?
Autoimmune supression
Benzene
Chemo
Hepatitis
Radiation exposure
Pregnancy
What are some symptoms of aplastic anemia?
Decreased WBC, RBC, and platelets
Leads to…
Infections
Anemia
Bruising, bleeding
Whats special about petechia and purpura?
They don’t blanch when pressure is put on it
Why are there no or very few reticulocytes in aplastic anemia?
The bone marrow isn’t functioning
What do you typically not see in aplastic anemia?
Hepatomegaly and spelnomegaly
What test do we use to check aplastic anemia?
Bone marrow biopsy
What are some treatments for aplastic anemia?
Remove underlying etiology
Red cell transfusion
Platelet transfusion
Multilineage: eltrombopag
Erthyropoietic: epoetin, darbepoetin
Myeloid: filgrastim, sargramostim
Bone marrow transplant
Triple therapy (immunosuppression, for those who can’t go through bone marrow transplant)
What is triple therapy?
Giving pts equine antithymocyte globulin(ATG), cyclosporine, and eltrombopag(promacta) for severe treatment of aplastic anemia
Steroids given along to reduce ATG side effects
How does Epoetin or Darbepoetin work?
EPO made with recombinate DNA that stimulates the erythroid precursors causing reticulocyte and RBC release
Rise in Hbg and Hct, dose-dependent (peaks 2-6weeks)
What is the different between Epoetin and Darbepoetin?
Darbepoetin has 3x the half life
Can be dosed weekly or Q 2 weeks
What are Epoetin and Darbepoetin indicated for?
Anemia from CKD
Chemotherapy
Myelodysplasia
What are the contraindications of Epoetin and Darbepoetin?
Allergy to drug
Uncontrolled HTN
Pure red cell aplasia after any EPO tx
What are some SE of Epoetin and Darbepoetin?
HTN
Thrombosis
Rash
Seizures
Pruritus
Fever
Edema
Dyspnea
Cough
Abdominal pain
What are the BBW for Epoetin and Darbepoetin?
Risk of death from thrombosis related issues
Increase Stoke in CKD with Hgb >11
Tumor progression of cancers
Increase risk of DVT after surgery
What do we need to monitor for patients on Epoetin and Darbepoetin?
Iron status
Hgb
BP
What is sideroblastic anemia?
Decrease of Hgb synthesis because of inability to make heme since it impairs the ability to incorporate iron into protoporhyrin IX(precursor to heme)
How is sideroblastic anemia inherited?
X-linked
Autosomal recessive
Mitochondrially inherited
How is sideroblastic anemia acquired?
Part of myelodysplastic syndrome
Alcoholism
Lead poisioning
Copper deficiency
Infection/inflammation
Medications: Isoniazid, Linezolid, Chloramphenicol
What other SE appears in sideroblastic anemia other than the normal anemic symptoms?
Palmar creases if Hgb <8-9 (also appears in iron deficient anemia)
May also see S/S with myelodysplastic syndrome
Why are reticulocytes decreased in sideroblastic anemia?
Pts have issue producing RBC
What shows up on a peripheral smear for sideroblastic anemia?
Basophilic stippling
Poikilocytosis
Anisocytosis
Polychromasia
What do we need to order before making a diagnosis of sideroblastic anemia?
Bone marrow aspirate
What does bone marrow aspirate show?
Erythroid hyperplasia: indicates effectiveness of erythropoiesis
Prussian blue stain: shows ringed sideroblasts and increase of iron stores
What are some treatments of sideroblastic anemia?
Transfusion
B6 (pyridoxine) or B1 (thiamine)
Stopping medication (isoniazid)
Genetic counseling
Phlebotomies, chelation (to prevent iron overload)
What is the main cause of anemia worldwide?
Iron deficient anemia
How much iron do we need to intake a day?
10-15mg
How much iron do we absorb?
10%
What is the role of ferroportin?
Iron transporter that release iron from cells
What dos Hepcidin do to ferroportin?
Promotes ferroportin breakdown which inhibits iron release
How much iron do we lose a day?
Lose 1 mg from skin/mucosa
So we absorb ~1mg and lose 1mg
What are some causes of iron deficiency anemia?
Deficient diet
Increased iron requirements
Chronic blood loss
Decreased absorption
Iron sequestration