Bone Marrow Failure

Question 1

What is bone marrow failure?

Answer 1

The failure of bone marrow to produce bone cells leading to pancytopenia. Leads to low red cells white cells and plateletts.

Question 2

What are the symptoms of anemia?

Answer 2

difficulty breathing, chest pain, fatigue

Question 3

Leukopenia symptoms

Answer 3

mpouth sores, infection, fever

Question 4

WHat are the causes of hypocellular bone marrow?

Answer 4

Aplastic Anemia as a reslut of Fancon’s anemia (inherited) or other aquired syndromes such as myelodisplastic syndromes

Question 5

What is aplastic anemia

Answer 5

failure to produce RBCs, WBCs, plateletts

Question 6

Fanconi’s anemia

Answer 6

becomes symptomatic at age 5. associated with bone marrow hypoplasia

Question 7

Other causes of aplastic anemia?

Answer 7

ionizing radiation, chemical agents, infections, pregnancy,PNH (RBC and WBC susceptible to complement lysis)

Question 8

Primary defect of aplastic anemia?

Answer 8

Absence of hematopoietic stem cells

Question 9

Hallmarks of aplastic anemia

Answer 9

Decreased retic count, hypocellular marrow, pancytopenia

Question 10

What is pure red cell aplasia

Answer 10

Characterized by a selective decrease in erythroid precursor cells. WBCs and plateletts unaffected

Question 11

How do you get a red cell aplasia?

Answer 11

Viral or bacterial infection, patients with hemolytic anemia may suddenly stop erythropoiesis, patients with thymoma- t cell mediated attack of erythroblasts or EPO

Question 12

WHat is a myelodysplastic syndrome?

Answer 12

primary, neoplastic stem cell disorder that tends to terminate in acute leukemia

Question 13

What does the bone marrow look like in a myelodysplastic syndrome>

Answer 13

Normocellular or hypercellular with evidence of qualitative abnormalities like ringed sideroblasts.

Question 14

What differntiates MDS from aplastic anemia?

Answer 14

Presence of a neoplastic clone

Question 15

Can MDS lead to acute leukemia?

Answer 15

Yup…if the percentage of bone marrow blasts rises to over 20% then it is acute leukemia.

Question 16

What is the method of mobilizing hematopoietic stem cells out of the bone marrow and into the peripheral blood for more easy access?

Answer 16

Apheresis

Question 17

What are the three HSC characteristics that make it possible to transplant them?

Answer 17

Their ability to regenerate in the marrow, their ability to find their way back to the marrow following to IV infusion, their ability to be frozen with no damage.

Question 18

What is autologous stem cell transplant?

Answer 18

take the stem cells out of a patient before hardcore chemo, reinfuse them later. Used mostly in the treatment of ppl with lymphoma and myeloma where intense chemo is needed.

Question 19

Allogenic SCT?

Answer 19

Comes from someone else

Question 20

How many HLA loci must match up to prevent graft vs host disease?

Answer 20

Three

Question 21

Advantage of allogenic transplant?

Answer 21

When the pt has a leukemia or lymphoma the graft vs. host disease effect can help prevent relapse of the tumor.

Question 22

Myeloablative therapy?

Answer 22

Chemo or irradiation of bone marrow prior to transplant.

Question 23

WHat drugs are used in myeloablative therapy?

Answer 23

cyclophosphamide and busulfan

Question 24

Why do myeloablative therapy>

Answer 24

has an immunosuppresive effect which prevents the transplant from being attacked by recipients immune system

Question 25

Non-myeloablative therapy?

Answer 25

Lower doses of chemo and radiation which are too low to eradicate all the marrow. Just enough therapy to keep the transplant from being rejected

Question 26

Complications of HSCT?

Answer 26

Infection- B/c the pts marrow must be destroyed prior to transplant which leaves the msusceptible to infection for a while
Patients lose aquired immunity so must be revaccinated after they are off immunosuppresive drugs

Question 27

Other complications

Answer 27

Veno-occlusive disease

mucositis- injury to mucosal lining of mouth and throat

Question 28

Treatment of acute GVHD?

Answer 28

Cyclosporine and methotrexate