Term
| What are the different major options of stem cell transplant to treat hematological malignancies? |
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Definition
Principles include stem cell "rescue" of patient marrow following high dose chemotherapy
1) Autologous
- Stem cell "rescue" only
2) Allogeneic
- Myeloablative- stem cell rescue and GVL effects
- Non-myeloablative- GVL effect only (for older and/or compromised patients who cannot handle myeloablative) |
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Term
| How are HLA genotypes inherited and why/when is HLA matching necessary for stem cell transplantation |
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Definition
1) Human Leukocyte Antigens are inherited as complete haplotypes (HLA-A, -B, -C, -DR, -DQ, -DP), so kids have 25% shot of getting both parental alleles
2) In case of allogeneic transplants from unrelated donors, HLA matching must be performed to prevent GVHD.
**minor histocompatibility antigens may still differ and will determine degree of GVHD. |
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Term
| Where can donor stem cells be accessed and why might you choose one source over another? |
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Definition
Peripheral blood of HLA matched donor is prefereable
1) Bone marrow (superior inferior iliac spine)
- Slow and invasive
- Less risk of GVHD
2) Peripheral blood (mobilization required)
- Faster and less invasive
- Greater risk of GVHD
3) Umbilical chord
- If HLA matching donor cannot be found, donor immune cells are not yet mature and allow greater mismatch
- Slower and more chance of infection |
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Term
What are the major purposes of given conditioning regimens preceding stem cell transplant?
What are the different types and when are they indicated? |
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Definition
1) Need to eradicate tumor cells and, in allogeneic SCTs, induce immunosuppression to allow for engraftment
**engraftment measured by PMN > 0.5 X 10^9/l and Platelets >30 X 10^9/l**
2) Types
- Myeloablative- Bisulfan, cyclohosphamide, melphalan (all alkylating agents) and etoposide (Topo II inhibitor)
- Immunosuppressive/Non-myeloablative- Fludarabine (anti-metabolite), ATG, Alemtuzumab (anti-C52) |
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Term
| Which hematological disorders are mainly treated by Allogeneic vs. Autologous SCT? |
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Definition
1) Autologous
- MM, non-hodkins lymphoma, hodgkin's lymphoma, neuroblastoma, germ cell turmors, amyloidosis
2) Allogeneic
- AML, ALL, Myelodysplastic syndromes, myeloproliferative disorders, non-hodgkins, hodgkins, CML, CLL, aplastic anemia, sickle cell. |
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Term
Which of the following disorders should NOT be treated with autologous stem cell transplantation?
1. Hodgkin's lymphoma
2. Chronic lymphocytic leukemia
3. Non-hodgkin's lymphoma
4. Multiple myeloma
5. Neuroblastoma |
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Definition
| 2- CLL, CML, ALL, AML, myeloproliferative and myelodysplastic syndromes and aplastic/sickle cell anemias should be treated with allogeneic SCT |
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Term
| What are the primary complications of hematopoietic SCTs? |
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Definition
1) GVHD (handle with immunosuppresion).
- Affect skin, Gi tract and liver acutely, and mediated by minor HLA antigen mismatch.
- Tissue injury stimulates cytokine release and inflammation, which activates donor T-cells to attack recipient's cells
2) Infection
- Myelosuppression from conditioning and use of immunosuppression.
- Post transplant periods include
- pre-engraftment/1 month (bacterial, viral, reactivation of HSV, RSV and enteric)
- post-engraftment (to day 100, bacterial, viral and reactivation of CMV, RSV and enteric, EBV)
- late phase (encapsulated organisms because of functional asplenia, CMV, VZV, fungal) |
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Term
| What types of infections are patients receiving SCTs prone to and how is this affected by the time, post-transplant? |
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Definition
Immunosuppression and Myelosuppression do a trick. Need prophylaxis with Acyclovir, Cipro and Fluconazole.
1) pre-engraftment (1 month)
- bacterial, viral, and reactivation of HSV, RSV and enterics
2) post-engraftment (to day 100)
- bacterial, viral and reactivation of CMV, RSV and enteric, EBV
3) late phase (>100 days)
- encapsulated organisms because of functional asplenia, CMV, VZV, fungal |
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Term
Graft Vs leukemia effect seen in stem cell transplant is characteristic of:
1) Allogeneic transplantation
2) Autologous transplantation
3) Non-myeloablative chemotherapy
4) Radiation therapy |
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Definition
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Term
HLA system mediates donor and recipient immune responses. HLA loci are found on
1) Chromosome 5
2) Chromosome 16
3) Chromosome 6
4) Chromosome 9 |
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Definition
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Term
The following are all advantages of Peripheral Blood SCT EXCEPT:
1) Faster engraftment
2) Lower risk of GVHD chronically
3) Same outcome after SCT as bone marrow stem cell transplantation
4) Less invasive procedure than bone marrow SCT |
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Definition
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Term
Regarding Conditioning regimens which of the following is true:
1) Allows for engraftment of donor stem cell with appropriate immunosuppression
2) Increase tumour cells
3) Increase transfusion requirements
4) Is not myelosuppressive |
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Definition
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Term
Regarding Conditioning regimens which of the following is true:
1) Allows for engraftment of donor stem cell with appropriate immunosuppression
2) Increase tumour cells
3) Increase transfusion requirements
4) Is not myelosuppressive |
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Definition
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Term
Autologous stem cell transplantation is used for the following malignancies EXCEPT:
1) Multiple myeloma
2) Hodgkins lymphoma
3) Germ cell tumour
4) Acute leukemia
5) Neuroblastoma |
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Definition
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Term
In GVHD, the following are all true, EXCEPT:
1) Mediated by major HLA antigen mismatch
2) Patients receive Prophylaxis with immunosuppression
3) Acute GVHD affects liver, skin, GI tract
4) Risk of GVH is highest for the first 50 days when immunosuppresion is used |
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Definition
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Term
Apart from GVHD, infection is one of the most common causes of mortality after SCT. Which of the following is not seen post engraftment period?
1) Mucositis
2) CMV viremia
3) Acute GVHD
4) Bacterial infection
5) Candida |
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Definition
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Term
| What are the basics of the ABO and Rh blood group systems? |
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Definition
1) ABO are carbohydrate antigens with antibodies that develop by 4 months
(Type "AB" has no ABO antibodies and can receive from anyone, but donate to NOONE)
2) Rh system (Da antigen)- Rh+ in 85% of individuals
- Moms who are Rh (-) make IgG against Rh and can cause hemolysis in Rh (+) fetus
**gotta give Rh (-) moms Rh immune globulin (passive anti-D)** |
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Term
| What types of pre-transfusion testing must be done on donors and recipients before transfusions take place? |
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Definition
1) Donor
- medical h/x and PE
- ABO, Rh type and serum antibodies
- Infectious disease testing
2) Recipient
- ABO and Rh typing, with Antibody screen (Rh, Kell, Kidd, Duffy< Ss) using indirect Coomb's test.
3) Patients with negative antibody screens can get "abbreviated" crossmatching screens, but those with positive screens need 30 minute inducbation to confirm IgG antibody and ABO compatibility. |
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Term
| What is the utility of the Direct Anti-globulin Test (DAT) in the case of transfusion screening? |
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Definition
| Direct Coomb's detects IgG or C3b bound to patient's RBCs and should be ordered if HEMOLYSIS is suspected (check for autoimmune hemolytic anemia) |
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Term
What type of blood product tests should be ordered in each of the following conditions?
1) Patient who may need transfusion but it is not expected
2) Surgical planning
3) Hemorrhage |
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Definition
1) Type and Screen
- Get patient ABO, Rh and antibody screen, but not crossmatching (can be ready in 10-15 minutes)
2) Type and Cross
- Specified # of units also crossmatched and reserved for 72 h
3) Uncrossmatched, Group O RBC |
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Term
Which blood products are indicated in each of the following scenarios?
1) Symptomatic anemia with Hct 19%
2) Platelet count of 9,000/ uL
3) PT/INR of 1.9
4) Surgical patient with active bleeding and Fibrinogen <100 mg/dl
5) Short term intravascular volume expansion during hemodialysis or plasmapheresis |
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Definition
1) Packed RBCs (1 g/dl Hb and 3% Hct per unit)
- containing RBC and preservative anticoagulant (ADSOL). Platelets and WBC are nonfunctional and small amount of plasma present.
If, Hct <21%, gotta give it. If 21-24%, use judgment and consider cardiopulmonary disease. If 25-30%, ONLY indicated with MI or infarction
2) Prophylactic whole blood platelets (4-5 units for adults and 1/10kg in kids) to prevent spontaneous bleeding. If whole blood fails, try apheresis
- each unit raise count 5-10K/ul
3) Coagulopathy with INR >1.5 needs FFP (no viable RBCs, WBCs or platelets).
- Give 15-20 ml/Kg body weight.
4) Cryoprecipitate (6 unit dose, each raising 5-10 mg/dl in adult)
5) Albumin and Plasma Protein fraction (virally inactivated colloid volume expander) |
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Term
What are the main contraindications for using pre-operative autologous donation (PAD)?
What is generally used instead? |
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Definition
1) Bacteremia or severe cardiopulmonary disease
2) Perioperative autologous collection or Directed donation |
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Term
| What are the most common acute indications to stop a blood transfusion? |
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Definition
1) Acute hemolysis (gave wrong blood) - diuresis and DIC therapy
2) Febrile (HLA antibodies to donor WBCs)- antipyretics
3) Allergic (antibodies to plasma proteins)- antihistamines
4) Transfusion related circulatory overload- slow down and diuresis
5) Anaphylaxis (antibodies to IgA or plasma contents)- give Epi |
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Term
| What are the most common transfusion-associated infections to be on the lookout for? |
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Definition
1) Hepatitis C causes 95% of post-transfusion hepatitis
2) CMV is clinically significant risk in immunosuppressed
3) Bacteria most common with platelets (must be kept at room temp)
HIV, Hep B, WNV and Syphilis are possible but RARE |
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Term
When might you give each of the following blood products to patients?
1) Whole Blood
2) Packed RBCs (1 unit for 1g Hb and 3% Hct)
3) Whole blood platelets (4-5 units for adult and 1/10kg in kid)
4) Fresh frozen plasma (14-20 ml/kg)
5) Cryoprecipitate
6) Albumin and Plasma protein fraction |
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Definition
1) rarely available, because must be made in components
2) Symptomatic anemia (Hct <21 % or between 21-24% with cardiopulmonary disease)
- RBC with preservative anti-coagulant, non functional WBCs and Platelet
3) TP with <10,000/ul (prophylaxis for spontaneous bleed)
- 10-50K may need it if invasive procedures
- 50-100k with coagulopathy or bleeding at critical sites
4) Coagulopathy with PT INR>1.5 or partial thromboplastin time of 8-10 s above normal in patient with active bleeding or surgery.
- NOT volume expander (use Albumin and PPF)
5) Fibrinogen deficiency <100 mg/dl
6) Short term intravascular volume expansion in liver disease, plasmapheresis, bypass or hemodialysis. |
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