Hematologic Malignancies Flashcards
(51 cards)
1
Q
AML clinical presentation
A
- BM failure
- leukostasis
- coagulopathy *APL
- extramedullary: leukemia cutis, gingival hypertrophy, chloromas
- metabolic: low glucose (artificial), hypokalemia (renal damage)
2
Q
ALL clinical presentation
A
- BM failure
- ?leukostasis
- ?coagulopathy
- extramedullary: LN, splenomegaly, mediastinal mass, CNS relapse, testes
- metabolic: tumor lysis syndrome (renal failure)
3
Q
myeloid markers
A
- myeloperoxidase, lysozyme
4
Q
lymphoid markers
A
- TdT
5
Q
AML: diagnosis
A
- Auer rods, large immature blasts, increased cytoplasm
6
Q
AML: favorable cytogenetics
A
- t(8,21), inv(16), t(15,17)
- NPM-1, CEBPA
7
Q
AML: unfavorable cytogenetics
A
- 11q23 trans, del 5, del 7
- FLT-3
8
Q
APL
A
- AML-M3
- leukopenia, DIC, bilobed nucleus, primary granules, Auer rods
- t(15,17) OR PML-RAR product confirms diagnosis
- treat: ATRA, ATO
- APLDS: leukocytosis, fever, pulm infiltrates; treated with anthracycline
9
Q
AML therapy
A
- 7 and 3: cytarabine and anthracycline
- hypomethylating agent in older patients
10
Q
ALL: favorable cytogenetics
A
- t(12,21), hyperploidy
11
Q
ALL: unfavorable cytogenetics
A
- t(9,22), t(4,11), 11q23 trans, hypoploidy
12
Q
ALL therapy
A
- vincristine
- glucocorticoid
- anthracycline
- L-asparaginase
- cytarabine
- cyclophosphamide
- methotrexate
- tyrosine kinase in Ph+
13
Q
B cell markers
A
- CD10, CD19, CD20
14
Q
T cell markers
A
- CD2, CD3, CD4, CD5, CD8
15
Q
myeloid markers
A
- CD13, CD33, CD117
16
Q
most common indolent NHL
A
- follicular lymphoma
17
Q
most common aggressive NHL
A
- diffuse large B cell lymphoma
18
Q
Ann Arbor staging
A
- I: single LN or extralymphatic site
- II: 2+ LN groups on same side
- III: 2+ LN groups on both sides
- IV: extranodal disease, diffuse non-lymphoid spread, BM
- A: asymptomatic
- B: unexplained fever, night sweats, >10% wt loss
- M: bulky mediastinal mass
- E: involvement of extranodal site
- spleen counts as LN
19
Q
treatment paradox
A
- indolent NHL slow but not cured
- aggressive NHL fast but can be cured
20
Q
NHL is NOT treated with ____.
A
- surgery
21
Q
NHL chemo
A
- R-CHOP (aggressive)
- rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone
22
Q
HIV associated lymphoma
A
- aggressive B-cell NHL
- treat with HAART
23
Q
HL features
A
- progressive adenopathy (usually neck/mediastinum)
- EtOH intolerance
- associated w/ EBV (50% of cases)
- bimodal age distribution
24
Q
HL diagnosis
A
- Reed Sternberg cell w/ CD30 and CD15 with lymphocytes and eosinophils
25
Reed Sternberg cell
- IgH rearrangement
| - monoclonal
26
Classical HL
- nodular sclerosis
- mixed cellularity
- B-lymphocyte rich
- lymphocyte depleted
27
Non-classical HL
- nodular lymphocyte predominance (CD20)
28
HL primary chemo
- ABVD
- adriamycin (cardiotoxic)
- bleomycin (pulmonary toxic)
- vinblastine (neurotoxic/hematotoxic)
- dacarbazine (hemotoxic)
29
HL other chemo
- MOPP (nitrogen mustard)
- radiation
- brentuximab
- immune checkpoint inhibitor
30
CLL smear morphology
- overabundance of mature B cells with smudge cells
31
CLL immunophenotype
- CD19, CD20, CD23, CD5, monoclonal Bcell, low density sIg and CD20 expression
32
SLL
- CLL in lymph node
33
Simplified Rai staging system
- low: lymphocytosis in blood /marrow
- intermediate: lymphadenopathy and hepatosplenomegaly
- high: anemia and thrombocytopenia
34
CLL prognostic molecular factors
- good = mutated
| - bad = unmutated, CD38, ZAP20, 11q del, 17p del
35
CLL chemo
- fludarabine/chlorambucil = oral
- rituximab, alemtuzomab
- B-cell signaling: ibrutinib
36
MGUS
- serum Ig <3, <10% plasma cells in bm, small M spike
- NO: anemia, renal dysfunction, lytic bone lesions
- good prognosis, assoc. w/ autoimmune disease
37
multiple myeloma
- large M spike, >10% plasma cells in bm, serum Ig >3, end organ involvement
- diagnose: UPEP, SPEP, skeletal survey, bm biopsy/aspirate, quantitative Ig
- Rouleaux formation on smear
38
multiple myeloma: CRAB
- hypercalcemia
- renal dysfunction
- anemia
- lytic bone lesions
39
multiple myeloma treatment
- chemo: melphalan and cyclohosphamide
- steroids: prednisone, dexamethasone
- imids: thalidomide, lenalidimide
- proteasome inhibitor: bortezomib
- HSCT
40
myeloproliferative neoplasms: definition and types
* excessive production of terminally differentiated fully functional cell
- CML
- polycythemia vera
- essential thrombocytopenia
- primary myelofibrosis
41
CML
- chronic myeloid leukemia (MPD): granulocytes
- clinical: triphasic course (chronic, accelerated, blast); splenomegaly, high wbc, high platelet, excess cytokine
- affected tk: BCR-ABL1 fusion gene (phil) t(9,22)
- leukemia risk: 100%
- survival: >90% 5 year with tki rx
- therapy: tki, allogenic HSCT, IFN alpha
42
PV
- polycythemia vera (MPD): red cells
- clinical: splenomegaly, high rbc, high platelet, facial plethora, vasomotor, low serum epo
- affected tk: JAK2, MPL, CALR
- leukemia risk: 5-20%
- survival: 9-12 yrs with rx
- therapy: phlebotomy, aspirin, hydroxyurea, IFN alpha, JAK2 inhibitor
43
ET
- essential thrombocytopenia (MPD): platelets
- clinical: high sustained platelets, enlarged MK in bm, asymptomatic, vasomotor, thrombosis/hemorrhage
- affected tk: JAK2 (50%), MPL, CALR
- leukemia risk: <5%
- survival: near normal
- therapy: hydroxyurea, aspirin, observation
44
PMF
- primary myelofibrosis (MPD): MK -> bm fibroblasts
- clinical: MK proliferation w/ fibrosis, variable counts, high LDH, hepatosplenomegaly*, anemia w/ tear drops, extramedullary hematopoiesis
- affected tk: JAK2 (50%), MPL, CALR
- leukemia risk: 5-20%
- survival: poor (<3 years)
- therapy: supportive, plenectomy, chemo (mephalan, hydroxyurea, busulfan), JAK2 inhibitor, allogenic HSCT (only cure)
45
myelodysplastic syndrome general features
- cytopenia w/ hypercellular BM, dysplasia, ineffective hematopoiesis
- abnormally increased cell death in BM
- macrocytosis and thrombocytopenia
- Pelger-Huet cells: bilobed nucleus in neutrophils
46
MDS cytogenic abnormalities
- chr5 del
- chr7 del
- trisomy 8
- 11q23 abnormalities
47
MDS bone marrow
- granulocytes: hypersegmented, hyposegmented, hypogranular, dysmorphic granules
- erythroid: binucleate precursors, nuclear budding, ringed sideroblasts
- MK: hyposegmented, hypersegmented
48
MDS classification
- peripheral blood cytopenias
- BM morphology (dysplasia)
- BM blasts
- IPSS scoring: bm blast %, bm karyotype, #/degree cytopenia, patient age
49
MDS treatment
- control symptoms, minimize toxicity, decrease progression to AML, allo HSCT if young
50
5q-syndrome
- MDS but different treatment
- megakaryocytes w/ hypolobulated nuclei, refractory macrocytic anemia, norm or high platelet
- treat: lenalidomide (favorable outcome)
51
Cancer IHCs
- carcinomas
- breast
- prostate
- lymphoma
- melanoma
- neuroendocrine
- germ cell
- soft tissue/sarcoma
- CK, EMA
- ER, PR
- PSA
- leukocyte common Ag (CD45)
- S-100
- chromogranin, synaptophysin, NSE
- betaHCG, AFP
- vimentin