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)
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2
Q

ALL clinical presentation

A
  • BM failure
  • ?leukostasis
  • ?coagulopathy
  • extramedullary: LN, splenomegaly, mediastinal mass, CNS relapse, testes
  • metabolic: tumor lysis syndrome (renal failure)
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3
Q

myeloid markers

A
  • myeloperoxidase, lysozyme
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4
Q

lymphoid markers

A
  • TdT
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5
Q

AML: diagnosis

A
  • Auer rods, large immature blasts, increased cytoplasm
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6
Q

AML: favorable cytogenetics

A
  • t(8,21), inv(16), t(15,17)

- NPM-1, CEBPA

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7
Q

AML: unfavorable cytogenetics

A
  • 11q23 trans, del 5, del 7

- FLT-3

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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
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9
Q

AML therapy

A
  • 7 and 3: cytarabine and anthracycline

- hypomethylating agent in older patients

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10
Q

ALL: favorable cytogenetics

A
  • t(12,21), hyperploidy
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11
Q

ALL: unfavorable cytogenetics

A
  • t(9,22), t(4,11), 11q23 trans, hypoploidy
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12
Q

ALL therapy

A
  • vincristine
  • glucocorticoid
  • anthracycline
  • L-asparaginase
  • cytarabine
  • cyclophosphamide
  • methotrexate
  • tyrosine kinase in Ph+
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13
Q

B cell markers

A
  • CD10, CD19, CD20
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14
Q

T cell markers

A
  • CD2, CD3, CD4, CD5, CD8
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15
Q

myeloid markers

A
  • CD13, CD33, CD117
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16
Q

most common indolent NHL

A
  • follicular lymphoma
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17
Q

most common aggressive NHL

A
  • diffuse large B cell lymphoma
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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
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19
Q

treatment paradox

A
  • indolent NHL slow but not cured

- aggressive NHL fast but can be cured

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20
Q

NHL is NOT treated with ____.

A
  • surgery
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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