Krafts IHO Week 4 Flashcards
1
Q
sudden onset, adults or children, rapidly fatal without tx, composed of immature cells (blasts)
A
acute leukemia
2
Q
slow onset, occurs only in adults, longer course, composed of mature cells
A
chronic leukemia
3
Q
malignant proliferation of immature myeloid or lymphoid cells in the bone marrow
A
acute leukemia
4
Q
Causes of acute leukemia
A
clonal expansion, maturation failure
5
Q
Badness of Acute Leukemia
A
crowd out normal cells, inhibit normal cell function, infiltrate other organs
6
Q
Onset of Acute Leukemia
A
within days
7
Q
Symptoms of Bone Marrow Failure in A Leukemia
A
fatigue, infections, bleeding (skin petichiae, nose bleeds won’t stop, etc)
8
Q
CBC in Acute Leukemia features
A
blasts/immature cells, leukocytosis, anemia, thrombocytopenia
9
Q
malignant proliferation of myeloid blasts in blood marrow, 20% cutoff for dx of blasts of WBC, many subtypes, bad prognosis
A
Acute Myeloid Leukemia
10
Q
M0-3
A
neutrophillic series (myeloblasts, promyelocytes, etc)
11
Q
M4/5
A
involve monocytic series (monoblasts etc)
12
Q
M6
A
erythroid series (erythroblasts)
13
Q
M7
A
megakaryocytic series (megakaryoblasts)
14
Q
Where do you see myeloperoxidase (MPO)
A
Neutrophil, use to see if the blasts are neutrophilic
15
Q
What does non-specific esterase stain?
A
Monocytes
16
Q
lots of myeloblasts, bland blasts, MPO negative, NEED MARKERS
A
AML-M0
17
Q
lots of myeloblasts, no maturation (just blasts but some have MPO and auer rods)
A
AML-M1
18
Q
lots of promyelocytes, faggot cells, DIC, t(15;17) in all cases
A
AML-M3 (acute promyelocyte leukemia)
19
Q
neutrophilic leukocytosis, basophilia, philadelphia chromosome, three phases
A
chronic myeloid leukemia
20
Q
high WBC, left shift neutrophilia, basophilia, low hemoglobin, high platelet count, low LAP
A
CML
21
Q
s/sx: slow onset, fever, fatigue, night sweats, abdominal fullness, big spleen and big liver
A
CML
22
Q
Accelerated Phase of CML
A
50%, unstable counts, blast crisis within 6-12 months
23
Q
Blast Crisis of CML
A
50%, acute leukemia with high mortality
24
Q
high RBC, thrombosis and hemorrhage, Jak-2 mutation
A
Polycythemia Vera
25
Primary Polycythemia Vera
intrinsic myeloid cell problem
26
Secondary Polycythemia Vera
dt increased EPO
27
s/sx: HA, pruritis, dizziness, thrombosis, infarction, big spleen and liver, plethora (flushing)
Polycythemia Vera
28
Treatment of Polycythemia Vera
phlebotomy, if needed myelosuppressive drugs
29
Prognosis of Polycythemia Vera
9-14 years, death of thrombosis or hemorrhage, leukemic transformation in some patients
30
Very high platelet count in blood, in young women, dx of exclusion, throbosis and hemorrhage
Essential Thrombocythemia
31
Platelets of >600,000, Hgb
Essential Thrombocythemia
32
s/sx: bleeding, thrombosis, purpura, bruising, pallor, tachycardia, biggish spleen
Essential Thrombocythemia
33
Essential Thrombocythemia Treatment
platelet pheresis, myelosuppressive drugs, apsirin
34
Essential Thrombocythemia Prognosis
5-8 years, death from thrombosis or hemorrhage, leukemic transformation in some patients
35
panmeylosis then marrow fibrosis, extramedullary hematopoiesis, teardrop red cells
chronic myelofibrosis
36
s/sx: LUQ fullness, weakness, fatigue, palpitations, huge speen, pallor, tachycarida
chronic myelofibrosis
37
Treatment of chronic myelofibrosis
supportive, maybe myelosuppressive drugs (early on)
38
Prognosis of chronic myelofibrosis
3-5 years, death from marrow failur, leukemic transformation in some patients
39
monoclonal plasma cell proliferation, monoclonal gammopathy, decreased normal immunoglobulins, osteolytic lesions
Multiple myeloma
40
M-spike (monoclonal band), (60% IgG, 20% IgA, IgD or IgE rare, never IgM), Bence-Jones in protein in urine, decreased normal Ig
Multiple Myeloma
41
Blood-anemia and rouleaux, Marrow-plasma cells and amyloid
Multiple Myeloma
42
Lymphoplasmacytoid lymphoma, IgM, hyperviscosity syndrome
Waldenstrom macroglobulinemia
43
small M spike with no myeloma sx, occasionally transforms into myeloma
Monoclonal gammopathy of undetermined significance (MGUS)
44
Signs of malignant plasma cells
do not differentiate into plasma cells, continue to proliferate and accumulate in marrow, produce large amounts of immunoglobulins, normal death of cells doesn't occur, crowds out other cells-rbc precursors; suppress antibody synthesis by normal plasma cells
45
s/sx: 80% present with bone pain (low back/pelvis/ribs), bruising or bleeding from decreased platelets, infx from decreased levels of normal immunoglobulins, hypercalcemia, renal failure, hyperviscosity syndrome
Multiple Myeloma?
46
Classic Triad of Multiple Myeloma
anemia, bone pain, renal failure
47
Dx Criteria for Multiple Myeloma
1. bone marrow with >20% plasma cells OR 2. plasmacytoma w/monoclonal protein in serum >3g/dl or monoclonal protein in urine lytic lesions 3. usual clinical features of myeloma 4. exclude connective tissue ds, chronic infx, carcinoma, lymphoma and leukemia
48
Treatment of Multiple Myeloma
dexamethasone, melphalan (alkyalating agent), cyclophosphamide (alkylating agent)
49
HPSC from peripheral blood and growth factors are given after transplantation, safe in 1-2% death rate, issue with contamination of the autologous graft by meyloma cells
Autologous Peripheral Blood Stem Cell Transplant (PBSC)
50
antiangiogenic agent (first used with advanced and refractory myeloma)
Thalidomide
51
newer antiangiogenic agent; maintenance after transplantation signficantly prolonged progression-free and event-free survival among patients with multiple myeloma
Lenalidomide (multiple myeloma)
52
proteasome inhibitor
Bortezomib (multiple myeloma)
53
Prognosis of Multiple Myeloma with Conventional Therapy vs intensive therapy under age 45
3-4 years vs 50% ten year survival
54
Cause of death with multiple myeloma
marrow replacement pancytopenia, renal failure, sepsis, acute leukemia, other chronic illness unrelated
55
malignant proliferation of lymphocytes in blood, bone marrow; several disorders; occur only in adults; long course that is indolent but incurable
Chronic Lymphoproliferative Disorders
56
small, mature lymphocytes (=to small lymphocytic lymphoma), B-cell, but CD5+, long inexorable course
Chronic Lymphocytic Leukemia
57
older (>40), asymptomatic at first, later organ filtration, infection may occur due to hypogammaglobulinemia
Chronic Lymphocytic Leukemia
58
Positive for B-cell antigens (CD20) and a T-cell antigen (CD5), but negative for TdT
Chronic Lymphocytic Leukemia
59
Treatment of CLL
conservative, reduce organomegaly, infection
60
Prognosis of CLL
counts, adenopathy, bone marrow pattern, mean survival 9 years, death usually from infection
61
hairy cells, splenomegaly without lymphadenopathy, pancytopenia, TRAP +
Hairy Cell Leukemia
62
older (>40), M:F is 5:1, big spleen but no lymphadenopathy, usually pancytopenia and always monocytopenia (always)
Hairy Cell Leukemia
63
TRAP + ctyochemistry and immunophenotype is positive for B-cell antigens and negative for CD5
Hairy Cell Leukemia
64
Treatment and Prognosis of Hairy Cell Leukemia
Chemotherapy, usually 10+ years
65
Most common cause overall of lymphadenopathy
Benign reaction to infection
66
Most common malignant cause of lymphadenopathy
Metastatic Carcinoma
67
large, irregular follicles, mixture of cells in germinal centers, tangible body macrophages, B-cell response to some immune stimulus
Follicular Hyperplasia
68
expanded area between follicles, mixture of cells, partial effacement, T-cell response to some immune stimulus
Interfollicular Hyperplasia
69
malignant proliferation of lymphoid cells (blasts or mature cells) in lymph nodes, skips around, many subtypes, most are B cell
Non-Hodgkin Lymphoma
70
Painless, firm lymphadenopathy, extranodal manifestions, 'B' symptoms: weight loss, night sweats, fever
Non-Hodgkin Lymphoma
71
older patients, indolent (incurable), small mature cells, non-destructive
Low Grade Non Hodgkin Lymphoma
72
children, sometimes, aggressive (curable?), big ugly cells, destructive
High Grade Non Hodgkin Lymphoma
73
Name the 4 Types of Low Grade Non Hodgkin Lymphoma
small lymphocytic lymphoma, malt lymphoma, follicular lymphoma, mycosis fungoides
74
Name the 4 Types of High Grade Non Hodgkin Lymphoma
large cell lymphoma, lymphoblastic lymphoma, Burkitt lymphoma
75
small mature lymphocytes, same thing as CLL, B-cell lesion, but CD5+ (weird), long course; death from infection
Small Lymphocytic Lymphoma
76
actually a bunch of lymphomas, marginal zone pattern, malt lymphoma, helicobacter pylori
Marginal Zone Lymphoma
77
mantle zone pattern, small angulated lymphocytes, t(11;14)-cyclin D1 and IgH, more aggressive
Mantle Zone Lymphoma
78
follicular pattern (later diffuse), small cleaved cell, mixed or large cell, Grade 1, 2, or 3, t(14;18)-IgH and bcl-2
Follicular Lymphoma
79
Stage 1 (single node), Stage 2 (2 or more nodes on same side of diaphragm) Prognosis Follicular Lymphoma
90% for 5 years
80
Stage 3 (lymph nodes on both side of the diaphragm) and Stage 4 (diffuse extranodal involvement)
40% for 5 years
81
Skin lesions, blood involvement, cerebriform lymphocytes, T-cell immunophenotype
Mycosis Fungoides/ Sezary Syndrome
82
large B cells, extranodal involvement, grows rapidly, bad prognosis
Diffuse Large-Cell Lymphoma
83
two types: B and T, lymphoblasts in diffuse pattern, same as ALL, T-lymphoblastic lymphoma often in teenage male with mediastinal mass
Lymphoblastic Lymphoma
84
child with fast-growing, extranodal mass, starry-sky pattern, t(8;14)=c-myc and IgH, occasionally involves blood
Burkitt Lymphoma
85
Japan/Carribean basin, HTLV-1, skin lesions, hypercalcemia, very aggressive
Adulte T-cell Leukemia/Lymphoma
86
Younger patients, good prognosis, contiguous spread, five subtypes, Reed-Sternberg cell
Hodgkin Lymphoma
87
Types Classical Hodgkin Lymphoma
Nodular sclerosis, lymphocyte rich, mixed cellularity, lymphocyte depletion
88
asymptomatic young male with cervical lymphadenopathy, good prognosis (early stage), B-cell origin, popcorn cells
Nodular L-P Hodgkin Lymphoma
89
most common subtype, good prognosis (early stage), Lacunar cells
Nodular Sclerosis Hodgkin Lymphoma
90
worse prognosis, usually disseminated at presentation, classic Reed-Sternberg cells, mixture of background cells
Mixed Cellularity Hodgkin Lymphoma
91
uncommon, usually localized at presentation, popcorn cells
Lymphocyte-Rich Hodgkin Lymphoma
92
rare, often disseminated at presentation, classic Reed-Sternberg cells, collagen or reticulin background
Lymphocyte Depletion Hodgkin Lymphoma
93
Treatment and Prognosis of Hodgkin Lymphoma
surgery, chemo, radiation, prognosis depends on stage, danger: second malignancies
