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IBI Test 3 > B3.052 Hematopoietic Stem Cell Transplant > Flashcards

B3.052 Hematopoietic Stem Cell Transplant Flashcards

1
Q

4 major causes of pancytopenia

A 
hematopoietic stem cell injury
clonal hematopoietic cell mutation
myelophthisis
defective maturation
enhanced peripheral destruction
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2
Q

disorders associated with hematopoietic stem cell injury

A 
aplastic anemia (idiopathic, immune mediated, secondary to drugs, toxins, etc)
Fanconi anemia
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3
Q

disorders associated with clonal hematopoietic cell mutation

A

acute leukemia
myelodysplasia
paroxysmal nocturnal hemoglobinuria

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4
Q

disorders associated with melophthisis

A

metastatic cancer
granulomatous disorders, TB
lymphoma
myelofibrosis

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5
Q

disorders associated with defective maturation

A

megaloblastic anemias

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6
Q

disorders associated with enhanced peripheral destruction

A

hypersplenism
autoimmune disorders
hemophagocytic lymphohistiocytosis

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7
Q

affected cell in myelodysplastic syndrome

A

myeloid stem cell

all cell lines affected, clonal hematopoiesis

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8
Q

kinetics of myelodysplastic syndrome

A

ineffective hematopoiesis (apoptosis of maturing cells in marrow)

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9
Q

what types of gene mutations are associated with MDS?

A

more than 90% of patients have mutations
more than 40 genes
commonly DNA methylation proteins
5q and 7q deletions common

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10
Q

what are some prognostic variables of MDS?

A 
cytogenetics
bone marrow blast %
Hbg conc
platelet count
neutrophil count
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11
Q

treatment for MDS

A

only definitive therapy is allogenic stem cell transplantation

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12
Q

what are the overarching goals of HCT?

A

restore normal hematopoiesis in bone marrow failure syndromes
replace diseased marrow with healthy marrow
rescue after marrow ablative treatments
correcting genetic diseases
establishes a graft-versus-leukemia (tumor) effect

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13
Q

totipotent stem cells

A

cell can develop into complete organism
unlimited capacity
found in early embryos

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14
Q

pluripotent stem cells

A

can form any of >200 cell types
located in undifferentiated inner cell mass of the blastocyst
found in early embryos

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15
Q

multipotent stem cells

A

committed cell that can form other tissues

located in fetal tissue, cord blood and adult somatic tissue

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16
Q

what are 4 factors associated with identification of stem cells

A

TPO - self renewal
SCF - proliferation/differentiation
TGFB- cell cycle dormancy
Ang-1 - cell cycle dormancy

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17
Q

what are the 5 major steps in stem cell transplant?

A
  1. collection
  2. processing
  3. cryopreservation
  4. chemotherapy
  5. infusion
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18
Q

what is autologous stem cell transplant and when is it used?

A

patients own stem cells
a technique to give high dose chemo
no immunological barriers

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19
Q

cons to autologous transplant

A

“tumor contamination”

not useful for bone marrow failure syndromes

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20
Q

what factors plays a dominant role in donor selection?

A

HLA Typing

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21
Q

what is HLA?

A

distinguishes self from non self
human leukocyte antigen
cell surface glycoproteins encoded on chromosome 6
inherited as haplotypes (1 in 4 chance a sibling will be identical)

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22
Q

how is HLA typing done?

A

serology w antigen specific anti sera

DNA code in cell’s nucleus

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23
Q

what is a novel immunologic condition that arises in marrow transplantation?

A

rejection is bidirectional
-graft rejection
-graft versus host disease (GVHD)
tolerance develops, immunosuppression not lifelong

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24
Q

what are components of small cell graft

A 
facilitating cells
NK cells
dendritic cells
T and B lymphocytes
stem cells, progenitors
25
Q

T lymphocyte functions in the patients

A

GVL

GVHD

26
Q

essential factors necessary for GVHD to occur

A

immunologically competent donor graft
histo-incompatilbility between donor and host
immunologically incompetent host

27
Q

how is GVHD related to graft versus malignancy effect?

A

lower incidence of leukemic relapse of patients who get GVHD
higher relapse in syngeneic vs allogenic BMT
high relapse rates in T cell depleted BMT
cytogenetic remission induced after post BMT relapse of CML by infusion of donor leukocytes

28
Q

which disorders have a high sens to graft vs tumor effects?

A

CML
CLL
low grade NHL
mantle cell NHL

29
Q

which disorders have a medium sens to graft vs tumor effects?

A

AML
intermediate grade NHL
HL
MM

30
Q

which disorders have a low sens to graft vs tumor effects?

A

ALL

high grade NHL

31
Q

3 primary sources of stem cells

A

BM
peripheral blood (at conc of approx. 1% found in BM)
umbilical cord blood

32
Q

advantages to BM collection

A

typically only one collection
usually enough cells collected
low T cell content

33
Q

disadvantages to BM collection

A 
not readily available
surgical procedure
risks to donor
costly
high risk of tumor cell contamination
restrictive HLA-matching requirements
34
Q

agents that mobilize stem cells to peripheral blood

A

filgrastim
sargramostin
plerixafor
chemo (autologous donations only)

35
Q

advantages of using PBSC

A 
less complex procedure
stem cell count adequate
higher progenitor cell content
low risk of tumor cell contamination
fastest engraftment time
36
Q

disadvantages of using PBSC

A 
requires catheter placement and medical therapy to stimulate cell production
limited availability
can require multiple collections
high T cell content
HLA matching restrictive
high risk for chronic GVHD
37
Q

advantages of using UCB

A 
non invasive/ no risk
readily available and no donor attrition
high number of minority donors
less stringent HLA matching
decreased viral transmission
decreased rate of GVHD
robust graft versus leukemia effects
38
Q

disadvantages of using UCB

A 
specialized training for collection
size limitations of unit
no donor lymphocyte infusion
theoretical genetic disease transmission
theoretical concern for maternal contamination (GVHD)
slowest engraftment time
increased risk of graft failure
39
Q

components of the preparative regimen

A

radiation
high doses of chemo
targeted agents

40
Q

how is graft rejection prevented?

A

eradicate host immune system (T cells)

immunosuppressive component of conditioning regiment

41
Q

how is GVHD prevented?

A

suppress donor immune system and minimize recognition of host cells as foreign
immunosuppressive medications starting before stem cell infusion and typically continues at least 6 months past HCT

42
Q

what is the tradeoff between prophylaxis and other outcomes?

A 
aggressive prophylaxis:
-less GVHD
-MORE infection
-MORE relapse
minimal prophylaxis:
-MORE GVHD
-less infection
-less relapse
43
Q

things that can cause acute toxicities after HCT

A

thrombotic microangiopathy
GVHD
infections

44
Q

symptoms of acute toxicities

A 
mucositis
nausea/vomiting diarrhea
pulmonary
sinusoidal obstructive syndrome
iron overload
drug toxicity
hemorrhagic cystitis
drug toxicity
45
Q

what is SOS

A

sinusoidal obstructive syndrome

obliteration of small intrahepatic central venules

46
Q

pathophys of SOS

A 
injury to:
-sinusoidal endothelial cells
-hepatocytes
-stellate cells
in the venules causes:
-microthrombosis
-fibrin deposition
-ischemia
-fibrinogenesis
systemically causes:
-portal hypertension
-hepatorenal syndrome
-multi organ failure
47
Q

clinical manifestations of SOS

A

hyperbilirubinemia
ascites
weight gain
hepatomegaly

48
Q

what is TMA

A

generalized endothelial dysfunction resulting from chemo and other triggers
causes microangiopathic hemolytic anemia and platelet consumption
thrombosis and fibrin deposition in microcirculation

49
Q

risk factors for TMA

A

therapy: TBA, calcineurin inhibitos, sirolimus
GVHD
infections: aspergillus, CMV, adenovirus

50
Q

clinical features of TMA

A

microangiopathic hemolytic anemia + increased LDH or other markers of hemolysis
thrombocytopenia or increase platelet transfusion requirements
renal dysfunction or neurological abnormalities
PRES= posterior reversible encephalopathy syndrome

51
Q

diagnosis and management of TMA

A

discontinue calcineurin inhibitor

plasma exchange

52
Q

phase 1 of HCT infectious disease path

A

day 0 to 15045 (engraftment)

carrier breakdown and neutropenia

53
Q

phase 2 of HCT infectious disease path

A

day 15-45 to 100
impaired cellular and humoral immunity
NK cells followed by start of CD8+ T cell recovery

54
Q

phase 3 of HCT infectious disease path

A

day 100 to 365+
impaired cellular and humoral immunity
slowly B cell and CD4+ T cell recovery and diversification of cells

55
Q

why can CMV infection occur post HCT?

A

reactivation of latent virus or newly acquired virus from donor graft or blood transfusions
common in allogenic but rare in autologous

56
Q

how is CMV prevented?

A

allogenic HCT recipients are monitored with plasma CMV PCR assays at least weekly through day 100 after HCT
treated preemptively if values exceed threshold

57
Q

pathophys of chronic GVHD

A

less well understood than acute GVHD
late expression of alloreactivity
dysfunctional immune reconstitution

58
Q

symptoms of chronic GVHD

A 
dry eyes
oral lesions
nail dystrophy
skin sclerosis
deep sclerosis
bronchiolitis obliterans
loss of bile ducts
fasciitis
skin ulcers
59
Q

what are Pseufo-Pelger Huet cells

A

bilobed neutrophils frequently seen in myelodysplastic syndrome

IBI Test 3 (19 decks)

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