Week 4 Cancers Flashcards
Acute Myelogenous Leukemia
20%(WHO) of bone marrow is bone marrow myelobasts
M0
Undifferentiated myeloid leukemia
M1
Acute myeloid leukemia w/o differentiation
M2
Acute myeloid leukemia with differentiation
M3
Acute promyelocytic leukemia which can be readily cured t(15;17) commonly see auer rods
M4
acute myelomonocytic leukemia
M5
acute monocytic leukemia
M6
Acute erythroleukemia
M7
acute megakaryocytic leukemia
Favorable cytogenetics
t(8;21) t(16;16) t(15;17) and auer rods
Remission
less than 5% blasts after 14 days
Chronic Lymphocytic Leukemia
Disorder of B cells but CD5+(t cell antigen), CD20+ and TdT(lymphoblasts) negative “CD23+” , tx with patience. treat sx. infection is problem Adults only
Hairy Cell leukemia
Hairy cells, splenomegaly, no lymphadenopathy, pancytopenia. Is a chronic B cell proliferative disorder. often >40 Male. Chicken wire bone marrow. TRAP+ CD20+ CD5- Tx. chemo, good prognosis Adults only
Prolymphocytic Leukemia
prolymphocytes, splenomegaly w/o lymphadenopathy, rare and aggressive. very high wBC, Low Hgb and platelets. dark dense chromatin Adults only
Large granulated Lymphocyte leukemia
Large granulated lymphocytes. “T cell” leukemia. Neutropenia- suceptible to infections. Long survival. Adults only
Chronic Myeloid Leukemia
neutrophilic leukocytosis. basophillia, phildelphia chromosome t(9;22) bcr-abl tyrosine kinase, three phases. Slow onset, fever, fatigue, full abdomen, night sweats
tx. imatinib
CML Chronic phase
stable counts, easily controlled, half go on to accelerated phase, half go onto blast crisis.
CML Accelerated phase
unstable counts, blast crisis within 6-12 months
CML Blast crisis
-becomes acute leukemia, migh mortality, blasts over 20%. can get a lymphoid problem as well
Chronic myeloproliferative disorders
Chronic myeloid leukemia, Polycthemia vera, Essential thrombocytopenia, Myelofibrosis occur only in adults. Long clinical course, increased WBC with left shift, may evolve into acute leukemia
Polycythemia vera
High RBC (sludgy blood) can get infarcts downstream diferent from secondary polycythemia, thrombosis and hemorrhage, Jak-2 kinase mutation in mostly all. Dx. high red cell mass, normal O2 sats. can have splenomegaly, thrombocytosis, increased WBC, Leukocyte alkaline phosphatase, increased B12. Marrow full of eryth. precursors. tx. phlembotomy, myelosuppressive drugs. Death from thromb. or hemmor.
JAK-STAT pathway
Signal binds cytokine receptor which has JAK bound. JAK Phos. receptor, STAT binds P. JAK phosphorylates STAT, STATS dimerize and become transcription factors.
Essential Thrombocytopenia
form of Chronic myeloproliferative disorder. Very high platelet count. Young women. Thrombosis and hemorrhage. Dx. platelets> 600,000. Hgb<13 or RBC normal. No marrow fibrosis, no other reason for thrombocytosis.
Experience bleeding and clotting.
Sx. pupura(red dots in skin from hem.), bruising, pallor, tachycardia, big spleen. tx. platelet pheresis, asprin
Chronic Myelofibrosis
form of Chronic myeloproliferative disorder Marrow full of myeloid cells, extramedulllary hematopoiesis- huge spleen. “teardrop red cells” from fibrotic marrow. Sx. LUQ fullness, weakness, fatigue. tx. supprotive. 3-5 year survival.
Imatinib
BCR-ABL tyrosine kinase inhibitor which causes apoptosis. Hugely effective. Not effective against blast phase disease. Taken for life. Treats CML with t(9,22)
Lymphoma
Malignancy of hematopoietic cells, starts in lymph nodes, can spread to blood or nodes. Mostly B and T cells. present with lymphadenopathy. Lymphoma lymph node- said to have fish flesh texture
Benign Follicular hyperplasia
large irregular follicles, mixture of cells in germinal centers, tingible body macrophages, B cell response to some immune stimulus.
Benign interFollicular hyperplasia
Expanded areas between follicles, mixture of cells, partial effacement, T-cell response to some immune stimulus, invade fat in the node.
Non-Hodgkin Lymphoma
Malignant proliferation of lymphoid cells. (blasts or mature) skips throughout body in unpredictable pattern. many subtypes, most are B cell. Sx. -painless, firm lymphadenopathy, extranodal manifestations, B sx. weight loss, night sweats, fever B=bad.
Low grade non-hodgkin’s lymphoma
older patients, indolent (not dividing fast) small mature cells, non destructive. 4 types: small Lymphocytic lymphoma, follicular lymphoma, mycosis fungoides
High grade non-hodgkin’s lymphoma
Children, aggressive, big ugly cells, destructive. 3 types: large cell lymphoma, lymphoblastic lymphoma, Burkitt, lymphoma
Small Lymphocytic Lymphoma
type of NHL Low grade. Small mature lymphocytes, same as CLL, B-cell lesion, but CD5+. long course. RIchter transformation= bad
Marginal zone Lymphoma (MALT)
type of NHL Low grade Mucosa associated lymphoid tissue. a bunch of lymphomas with marginal zone pattern. MALT is common type-salivary glands, lymphoid tissue Goes away with antibiotics to H. Pylori.
Mantle Cell Lymphoma
“CD5+, CD23-, Cyclin D1 +” type of NHL Low grade “t(11;14)” Mantle zone pattern, small angulated lymphocytes, translocation leads to cyclin D1 and IgH to be by each other, 14=H. allows passage through checkpoints to be faster. aggressive. CD20 and CD5 positive
Follicular Lymphoma
type of NHL Low grade. Follicular pattern. “t(14,18)” cells immortal. small cleaved cell, mixed or large cell. grade1-3. fat in lymph nodes. Butt cells=clefted nuclei of lymphocytes. Early therapy does not improve survival. Treat when forced to with rituximab
Mycosis fungoides
type of NHL Low grade. messed up lion face pic. skin lesions appear as excema-> mushroom growth on skin. blood involvement, T- cell immunophenotype. Sezary cells- look like little brains
diffuse-Large cell Lymphoma
type of NHL high grade. Large B cells, extranodal involvement, rapid growth, bad prognosis.
Lymphoblastic Lymphoma
type of NHL high grade. B and T types. Same as Acute Lymphoblastic leukemia. “T”-Lymphoblastic lymphoma seen in “t”een, male with mediastinal mass.
Burkitt Lymphoma
type of NHL high grade. Endemic(near mandible) vs Nonendemic(abdomen). typically child with fast growing mass. “starry sky pattern” “t(8,14) c-myc and IgH. can involve blood- burkitt’s leukemia. Blue cytoplasam and punched out vacuoles.
Adult T-cell Leukemia/ Lymphoma
Japan/ carribean. HTLV-1 virus. skin lesions, hypercalcemia, aggressive. Not common, hard to treat. flowery blood cells.
Hodgkin Lymphoma
Younger patients, good prognosis. CD30+ Contigious sprad. 5 subtypes. “Reed-Sternberg cell” (owl’s eyes). a huge multinucleated cell with big nucleoli. peaks in males 20-30, and later in life. Spreads node to node. different subtypes
Nodular lymphocyte Hodgkin Lymphoma
asymptomatic young male, good prognosis, B cell origin, popcorn cells, most common subtype, lacunar cells. CD20+
Mixed cellularity Hodgkin Lymphoma
Worse prognosis, usually disseminated at presentation. Classic reed sternberg cells.
Lymphocyte rich Hodgkin Lymphoma
Uncommon, usually localized, popcorn cells.
Lymphocyte depletion Hodgkin Lymphoma
Rare, often disseminated, classic reed sternberg cells. , collagen or reticulin background.
Chronic Lymphocytic Leukemia Staging
- Rai system
- Rai 0: lymphocytosis –can just monitor
- Rai 1: lymphadenopathy
- Rai 2: splenomegaly
- Rai 3: HgB < 11 g/dL
- Rai 4: platelets < 100K
CLL curative modality
Mostly just watch them, but best way to cure is allogenic transplant
Staging of Lymphomas
Stage1: localized disease, single node or organ
Stage 2: two or more node regions on same side of diaphragm
Stage3: two or more lymph node regions above and below diaphragm
Stage 4: widespread disease, multiple organs, with or without lymph node involvement.
Lymphoma staging scan
PET. Lymphomas love me sum shugga
ECOG SCORE
1 point for each max=5= worst age>60 PS2-4? Stage 3-4 LDH elevated Extra nodal (stage 4)
NHL treatment
CHOP!, R-CHOP for B cell lymphomas- R=rituxumab
Indolent B cell Lymphomas
Follicular, CLL/SLL, Marginal zone, MALT, LPL or wldenstrom
Aggressive B cell lymphomas
DLBCL, Mantle cell
Highly aggressive B cell lymphomas
Burkett.=EBV
CHOP
Treats CD20+ (bcell lymphomas) do not treat T cell lymphomas, No CD 20
