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Hematology > Hematologic Malignancies > Flashcards

Hematologic Malignancies Flashcards

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
Q

Reed Sternberg cell

A
  • IgH rearrangement

- monoclonal

26
Q

Classical HL

A
  • nodular sclerosis
  • mixed cellularity
  • B-lymphocyte rich
  • lymphocyte depleted
27
Q

Non-classical HL

A
  • nodular lymphocyte predominance (CD20)
28
Q

HL primary chemo

A
  • ABVD
  • adriamycin (cardiotoxic)
  • bleomycin (pulmonary toxic)
  • vinblastine (neurotoxic/hematotoxic)
  • dacarbazine (hemotoxic)
29
Q

HL other chemo

A
  • MOPP (nitrogen mustard)
  • radiation
  • brentuximab
  • immune checkpoint inhibitor
30
Q

CLL smear morphology

A
  • overabundance of mature B cells with smudge cells
31
Q

CLL immunophenotype

A
  • CD19, CD20, CD23, CD5, monoclonal Bcell, low density sIg and CD20 expression
32
Q

SLL

A
  • CLL in lymph node
33
Q

Simplified Rai staging system

A
  • low: lymphocytosis in blood /marrow
  • intermediate: lymphadenopathy and hepatosplenomegaly
  • high: anemia and thrombocytopenia
34
Q

CLL prognostic molecular factors

A
  • good = mutated

- bad = unmutated, CD38, ZAP20, 11q del, 17p del

35
Q

CLL chemo

A
  • fludarabine/chlorambucil = oral
  • rituximab, alemtuzomab
  • B-cell signaling: ibrutinib
36
Q

MGUS

A
  • 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
Q

multiple myeloma

A
  • 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
Q

multiple myeloma: CRAB

A
  • hypercalcemia
  • renal dysfunction
  • anemia
  • lytic bone lesions
39
Q

multiple myeloma treatment

A
  • chemo: melphalan and cyclohosphamide
  • steroids: prednisone, dexamethasone
  • imids: thalidomide, lenalidimide
  • proteasome inhibitor: bortezomib
  • HSCT
40
Q

myeloproliferative neoplasms: definition and types

A
  • excessive production of terminally differentiated fully functional cell
  • CML
  • polycythemia vera
  • essential thrombocytopenia
  • primary myelofibrosis
41
Q

CML

A
  • 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
Q

PV

A
  • 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
Q

ET

A
  • 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
Q

PMF

A
  • 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
Q

myelodysplastic syndrome general features

A
  • cytopenia w/ hypercellular BM, dysplasia, ineffective hematopoiesis
  • abnormally increased cell death in BM
  • macrocytosis and thrombocytopenia
  • Pelger-Huet cells: bilobed nucleus in neutrophils
46
Q

MDS cytogenic abnormalities

A
  • chr5 del
  • chr7 del
  • trisomy 8
  • 11q23 abnormalities
47
Q

MDS bone marrow

A
  • granulocytes: hypersegmented, hyposegmented, hypogranular, dysmorphic granules
  • erythroid: binucleate precursors, nuclear budding, ringed sideroblasts
  • MK: hyposegmented, hypersegmented
48
Q

MDS classification

A
  • peripheral blood cytopenias
  • BM morphology (dysplasia)
  • BM blasts
  • IPSS scoring: bm blast %, bm karyotype, #/degree cytopenia, patient age
49
Q

MDS treatment

A
  • control symptoms, minimize toxicity, decrease progression to AML, allo HSCT if young
50
Q

5q-syndrome

A
  • MDS but different treatment
  • megakaryocytes w/ hypolobulated nuclei, refractory macrocytic anemia, norm or high platelet
  • treat: lenalidomide (favorable outcome)
51
Q

Cancer IHCs

  • carcinomas
  • breast
  • prostate
  • lymphoma
  • melanoma
  • neuroendocrine
  • germ cell
  • soft tissue/sarcoma
A
  • CK, EMA
  • ER, PR
  • PSA
  • leukocyte common Ag (CD45)
  • S-100
  • chromogranin, synaptophysin, NSE
  • betaHCG, AFP
  • vimentin

Hematology (6 decks)

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