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52 Cards in this Set

  • Front
  • Back
Cyclin D1 (t11:14)
Mantle cell Lymphoma
IPI
Age >60, LDH elevated, Advanced Stage (III or IV), Zubrod
BCL2 staining
indicates BCL-2 t(14:18)
Follicular lymphoma
Ki-67
measures nuclear proliferation
High in Burkitts (Highly aggressive), low in Follicular (Low grade)
Waldenstrom's Macroglobulinemia ID
Cold Agglutination Rouleaux formation (RBC agglutination)
Raynaud's syndrome
Nephrotic Syndrome, CHF, Skin problems
CLL
"smudge" cells, virgin B-cells (now known to come from memory B-cells too)
BM replaced neoplastic B-cells -> hypogammaglobulinemia, Normocytic Anemia (50%), Thrombocytopenia (40%)
5X10^9/L cloncal CLL B-cells
CD5+, CD20+ (dim), CD23+, sIg (dim)
13q-
deletion has favorable prognosis
17p-
adverse
Mantle Cell
CD20+, neg for other markers
Low Grade NHL
"CaLL Waldenstrom's for Marge's Hairy Follicles"
Hair Cell CD
CD20+, CD10- (CALLA), CD5- (B1-B-cells, also T-cell), CD25+ (IL-2R)
IMPORTANT CD11c+, CD103+ (ITGAE - Hairy cell only one w/ - only on T-cells)
CLL CDs
CD5+ (B1-B-cell), CD20+, CD19+, CD103-, CD11c-
Reed Sternberg Cell CD
CD 30+ (TNFRSF8 - on active but not resting T- and B- cells), CD15+ (on neutrophils - mediates phago and chemotaxis) , CD45- (on hematopoetic cells except RBC and plasma), CD20-
Sweet's Syndrome
neutrophilic dermatosis
see in MDS and AML
M4 & M5
extramedullary leukemia
bad prognosis
bad prognosis chromosomes
t(9:22) (BCR-ABL aka Phil), t(4:11), 5q-, 7q(-), FLT3-ITD
good prognosis chromosomes
Hyperdiploidy, t(12:22) TEL-AML1
Burkitt's translocation
CMYC next to Ig promoter
t(8:14)
AML M3 genes
DIC
t(15:17), PML-RARalpha fusion protein
Philadelphia gene
t(8:21) -FLT3-ITD better
worse is11q23
AML M3 treatment
all trans retinoic acid
ALL classification in adults
most common
L1 = small lymphoblasts, L2 large lymphoblasts, L3 Burkitt type w/ vacuoles
pre-B cell is most common
ALL treatment
L-Aspariginase: only approved use is for ALL
Ph1(+) = philadelphia chromosome -> TKI
AML treatment
All-trans retinoic acid (ATR) and arsenic: AML M3 ONLY
Complete remission -> start on consolidation therapy -> Maintenance therapy
Sunitinib: (tyrosine-kinase inhibitor)
CML treatment
Imatinib (a TKI, aka Gleevec)
Allogenic BMT in younger pt (if match) = curative in 70-80%
Blast transformation: Lympoid: treat as for ALL + dasatinib
Myelod: treat as for AML + dasatinib
JAK2 mutation
Essential thrombocythemia and PRV and AMMM
renders hemopoietic cells more sensitive to TPO and EPO
Internal deletion of Chromosome 4
HES (Hypereosinophilic syndrome)
can have a constitutively Tyrosine Kinase
Macrocytic anemia w/ normal B12 & Folic acid
Refractory anemia
5q- Syndromes
MDS
responds to lenalidomide
CML (general)
40-60 yo Expansion of pluripotential stem cell -> lympoid or myeloid lineage
WBC 50-200,000/μL
anemia (normocytic to macrocytic), Ph+ t(9:22), BCR-ABL (Tyr-Kinase), low LAP
CML Stages
Chronic ↑ Granulocytes in Marrow
Accel (3yrs) basophilia, thrombocytosis, progressive anemia, weird RBC shapes (aniso/poikilocytosis)
Blast Crisis: >30% blasts (myeloid or lymphoid) rapid ↑blasts (TdT +)
CML Clinical
Hepatosplenomegaly, general lymphadenopathy, myeloblasts w/o Auer rods
WBC 50,000-200,000/μL, Thrombocytosis or thrombocytopenia, BM hypercellular
CLL
>60 yo, General lymphadenopathy, ↑ immune hemolytic anemia (Warm IgG & Cold IgM)
WBC 15,000-200,000 lymphoblasts <10%, Neutropenia, SMUDGE CELL
Normocytic anemia or Thrombocytopenia
Hypogammaglobinemia
AML
M0-M7, Auer rods in M2&M3, M3 (DIC - responds to all-trans Retinoic acid)
>20% blasts in marrow, extramedullary masses , low platelets
ALL
0-14yrs, Clonal pre-B cell (80%) or B, T-cell
CALLA+ (CD10+), TDT+, L1 (small lymphoblasts), L2 (Larger lymphoblasts), L3 (Burkitts)
Bcell: CNS & Tests metastasis, Tcell: Mediastinal or acute
WBC 10,000-100,0000, >20% blasts in PERIPH blood, normocytic anemia w/ thrombocytopenia, BM often replaced by blasts
40% CNS -> Cauda equina symptoms
Burkitt's Lymphoma
30% of children , Aggressive, EBV relation w/ t(8:14), "Starry Sky"
American: GI & Para-Aortic, African: Jaw
BM involvement, leukemic phase common
Diffuse Large B-cell Lymphoma
50% of adults w/ NHL (children too), B-cell derived from Germinal center -> SVC Symptoms
Localized disease w/ extranodal involvement (GI, Brain), EBV Assoc. w/ AIDS
Follicular lymphoma
B-cell from germinal center, t(14:18) -> overexpress BCL2 (anti-apoptosis)
General lymphadenopathy, BM involvement
Extranodal Marginal Zone Lymphoma
Assoc. w/ H.Pylori - Dervies from MALT (Maltoma - B-cells)
low grade
Mantle Cell Lymphoma
t(11:14), Cyclin D1
Painless lymphadenopathy and splenomegaly
Anaplastic T-cell
Ki-1 protein positive
Hairy Cell
NO lymphadenopathy, pancytopenia (hair-like projections)
TRAP+, CD11+, CD103+
B-cell leukemia
Adult T-cell Leukemia
WBC 10-50,000 (>20% blasts)
Normocytic anemia & thrombocytopenia (BM replaced by lymphoblasts)
Assoc w/ HTLV1
SKIN infiltration, Lytic bone lesions (hypercalcemia results)
CD4+, TdT -
MDS; Age, Signs, Blasts, Histology
50-80yo, Sweet's Syndrome (Neutrophilic dermatosis)
Normocytic to macrocytic anemia
Ringed sideroblasts, Myeloblasts <20%, HYPOSEGMENTED PMN (Pelger-Huet change)
hypercellular BM
5q - = good
7- = bad
Essential Thrombocytopenia
JAK2 (V617F) in 30-40%
Bleeding (usually GI), splenomegaly
Thrombocytosis (platelets >600,000) w/ abnormal morphology
Mild neutrophil leukocytosis, hypercellular BM w/ abnormal megakaryocytes
PRV
↑ RBC mass & Hb >20, ↓ EPO, Leukocytes >12,000
4H's (Hyperviscosity, Hypervolemia, Histaminia, Hyperuricemia
Pruritis after bathing, Thrombotic events, Splenomegaly
JAK2
AMMM
MASSIVE splenomegaly, Splenic infarcts
Normocytic anemia - teardrop cells, circulating, RBC precursors
Ph -, LAP score ↓ or normal
Early: Panhyperplasia -> Myelofibrosis/osteosclerosis
Multiple Myeloma
Hypercalcemia, ↑ESR, ↓platelets, Mott cell w/ Russell bodies (Excess Ig)
Flame cell, Normocytic anemia w/ Rouleaux
Lytic bone lesions, Fx, Hyperviscosity, Kidney problems
Lymphoblastic Lymphoma (Waldenstrom's Macroglobinemia)
Retinal hemorrhage, stroke (hyperviscosity), Lymphadenopathy, no lytic bone lesions, Raynaud's phenomenon, Dutcher body, IgM spike, BJ protein
no Renal disease/amyloidosis
ALL vs AML
Precursor B ALL: Blasts that express CD10 (CALLA), CD19 (follicular dendritic cells and B-cells, lost on plasma cells), and CD20 (standard on all B-cells), and CD22 (Sialic acid receptor)
AML - Blasts that express CD13 (Granulocytes & monocytes), CD14 (Innate immune – coreceptor for LPS), CD15 (neutrophil chemotaxis/phagocytosis), and CD33 (Cells of myeloid lineage)
MGUS
No BJ protein, small IgM spike
most common monoclonal gammopathy
Mycosis Fungoides & Sezary Syndrome
CD4 T-helper cells, 40-60yo
Skin rash spread to lymph nodes, lung, liver, spleen
groups of neoplastic cells in skin = Pautrier's microabcesses
Sezary = Mycosis Fungoides w/ leukemic phase (Circulating cells = Sezary cells)