Bone Marrow Failure
Acute
MM/MGUS
Chronic
100
Name 2 drug classes that can cause aplastic anemia
1. Methimazole and PTU
2. Beta lactams
3. sulfonamides
4. NSAIDS
5. Anti-convulsants
- Discontinuing the drug can improve AA, but it takes many weeks for the cell count to recover.
100
Name the two acute leukemias
1. AML
2. ALL
100
Name the CRAB criteria
C: hypercalcemia
R: REnal failure
A: Anemia
B bone disease
100
Name the three phases of CML
1. Chronic: how most patients present, responds well to therapy
2. Accelerated phase
3. Blast crisis: secondary to AML
200
What diagnostic test is required to diagnose idiopathic pure red cell aplasia?
A bone marrow biopsy! It will show a selective decrease in erythrocyte precursor.
This then will be treated with immunosuppressive meds. PRCA can especially be causes by Parvovirus B19, mostly in immunocompromised patients. the anemia resulting in healthy patients is often clinically insignificant.
200
ALL is most common in what age group?
Children and adolescents
ALL in children is often curable, but >19 is inferior despite adoption of the same types of treatment approaches
200
How do you identify and quantify M protein in the diagnosis of MM?
SPEP
UPEP
serum FLC (free light chain) testing
200
Prompt treatment upon diagnosis of CML is performed to avoid what?
Blast phase and subsequent AML
300
What is the only cure for Myelodysplastic syndrome?
Allogenic hematopoietic stem cell transplant, which is too toxic for most adults.
However, if these patients are at a high risk of developing acute myeloid leukemia, then hypomethylating agents have been found to be effective
300
Acute Promyelocytic Leukemia is associated with what chromosomal translocation?
t(9;22)(q34;q11) - Philadelphia chromosome
t(15;17)(q24;q21) - Retinoic Acid Receptor
t(8;14)(q24;q32) - c-myc and IGH genes
Retinoic Acid Receptor - Which is why patients were able to achieve cure with all trans retinoid acid
Philadelphia: CML, and rarely ALL, and even more rarely AML
300
Name the three characteristics defining MGUS
M Protein level less than 3g/dL
Clonal plasma cells compromising less than 10% of bone marrow cellularity
Absence of related signs and symptoms of end organ damage
300
What class of medications are used primarily to treat CML?
Tyrosine Kinase inhibitors
400
What gene is seen in almost 97% of all cases of Polycethemia vera?
JAK2 (JAK2 V617F)
- usually diagnosed with elevated hemoglobin levels during routine testing
- symptoms: headache, fatigue, erythromelalgia, pruritus, uncontrolled hypertension, thrombosis
400
Myeloblasts
Auer Rods: Fused lysosymes
AML, APML, myelodysplastic syndromes
400
Which drug class is prescribed in MM to prevent skeletal events?
Bisphosphonates, they improve survival
400
What three Tyrosine Kinase Inhibitors are FDA approved for the initial treatment of CML?
Name at least one for half points
Imatinib, Dasatinib, nilotinib
500
What is the treatment for essential thrombocythemia in patients > 60 years?
Hydroxyurea, to lower the platelet count and decrease risk of thrombotic complications. Most patient are asymptomatic and are discovered on routine testing.
500
What is the initial treatment for AML?
Induction: anthracycline (daunorubicin, doxorubicin, epirubicin, idarubicin) + cytarabine
Goal: Ablate the bone marrow, eliminating hematopoietic cells
Responders: Consolidation therapy
Fun fact: anthracyclines were derived from Streptomyces peucetius
500
What is the emergent treatment to treat hyperviscosity syndrome in WM?
Emergent plasmapheresis to remove excess IgM, and therapy to decrease IgM production simultaneously.
500
For patients with accelerated or blast phase CML, what other type of therapy can be considered
Allogeneic hematopoietic stem cell transplantation