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238 Cards in this Set
- Front
- Back
|
what is bandemia assoc w/?
describe LN appearance in a pt w/ bandemia? |
neutrophilic leukocytosis (infxn)
nodes are firm, movable, tender, w/ erythema of overlying skin |
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if node is fixed, is it benign or malignant?
what makes it fixed? |
malignant
tissue desmoplasia --> fibrous tissue prodxn that affixes the LN to surrounding structures |
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what two things will flow cytometry tell you abt the BM aspirate?
|
type of cells (to place cell in a lineage)
stage of development (maturity) |
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what does genetic analysis tell us?
what is it useful for? |
translocations and chr abnormalities
use for: - classification - prognosis |
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If BM shows homogeneous popn of immature blast cells w/ normal granulocytes and diminished # of megakaryocytes and platelets, what is his most likely dx?
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acute lymphoblastic leukemia
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what are the B-cell markers?
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TdT
CD10 (CALLA) CD19, CD20 CD21 CD23 CD79a CD22 (used to confirm BCL rather than TCL lineage) |
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what is significant abt CD21?
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it is also an EBV receptor (on mature B-cells and follicular dendritic cells)
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what are the T-cell markers?
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CD1, CD3
CD4, CD8 CD5 (seen in a small subset of B-cells in CLL) |
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which marker is seen on progenitor cells and pluripotent hematopoietic stem cells only?
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CD34
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which marker is a pan-leukocyte marker?
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CD45 (LCA)
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what is the most likely location for a T-cell lymphoblastic leukemia?
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thymus (c/o as mediastinal mass)
precursor to TCL |
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if a blast is TdT(+), can it be a myeloid tumor?
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no
TdT is almost always seen in blasts only |
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how can you tell that a T-cell immature?
how would a mature T-cell look like? |
immature --> CD3, CD4, CD8
mature --> CD3 + CD4 OR CD8 (not both!!) |
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mature B-cells are going to have Ig in cytoplasm or on cell surface?
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in the cytoplasm
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most likely dx in a kid w/ mediatinal mass?
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lymphoBLASTIC leukemia/lymphoma
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are there any specific markers for follicular B-cell lymphoma?
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no, they are just CD19, CD20, CD10
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are there specific markers for mantle zone lymphoma?
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yes, CD5 and cyclin D are specific
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50 yo man c/o for evaluation of lymphocytosis that was detected on CBC obtained during routine PE. He feels well w/o complains, relatively active and no fatigue. Most likely dx?
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CLL
indolent course "rolling stone" dz discovered accidentally on routine CBC |
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what kind of B-cells are seen on flow cytometry in pts w/ CLL?
- smear appearance? - Ig /light chains? - surface markers? |
- monoclonal B-cells w/ atypical features and abundant cytoplasm
- weak/dim surface IgG/IgM w/ monoclonal expression of kappa (MC) light chains - CD19, CD20, CD10, CD23, CD5 (T-cell marker) |
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how are CLL B-cells different from normal reactive B-cells?
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CLL B-cells --> monoclonal
reactive B-cells --> polyclonal |
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what is the characteristic peripheral smear finding in pts w/ CLL?
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smudge cells --> released nucleus after cell has been crushed during smear preparation (cells are very fragile and spherocytic)
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what makes leukemia acute?
what makes leukemia chronic? |
immature cells
mature cells |
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once B-cells are released from their origin into circulating blood, and go to a LN but are never exposed to Ag -- what is it called?
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naive or virgin B-cell
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if a B-cell goes to a LN in the B-cell area outside of the follicle -- what is it called?
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mature peripheral B-cell
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if the B-cell stays outside of the follicle -- what is it called?
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parafollicular B-cell
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what kind of cells secrete IgG?
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plasma cells
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when a mother develops an aministic response Abs to her 2nd kid after giving birth to 1st kid -- what kind of Abs are these?
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anti-D Abs
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what maturity stages of B-cells do you look for in CLL?
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naive B-cells and post-germinal center memory B-cells
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which one leads to leukemia /lymphoma?
- B-cells - T-cells |
B-cells --> leukemia
T-cells --> lymphoma |
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what is the most prominent cytogenetic feature of CLL?
|
chromosomal abnormalities:
MC --> trisomy21, deletions of 13q12-14, 11q, 17p high-grade dz & worse prognosis --> deletions in 13q and 17p |
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can lymphomas have a normal wbc count?
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yes
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where does CLL tend to show up?
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EVERYWHERE
("rolling stone" dz --> in BM, LNs, spleen) |
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what is the definition of CML?
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CML is a neoplasm of pluripotent hematopoietic stem cells --> leads to preferential differentiation of granulocytic progenitors
CML is 1 of 4 chronic myeloproliferative disorders |
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what is CML assoc w/?
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Philadelphia chromosome that leads to a BCR-ABL fusion gene w/ tyrosine kinase activity
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what kind of cells proliferate in CML?
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granulocytic precursors
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what are the 4 myeloprliferative disorders?
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1. CML (chronic myelogenous leukemai)
2. polycythemia vera 3. essential thrombocytosis 4. myelofibrosis w/ myeloid metaplasia |
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what does myelofibrosis lead to?
|
neoplastic extramedullary hematopoiesis
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are fibroblasts affected in CML?
why or why not? |
no, fibroblasts are not affected
b/c they do not have the Ph chromosome |
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is terminal differentiation affected in CML?
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no (will still see mature neutrophils)
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what are the 3 lineages that multipotent stem cell can give rise to?
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monocytic
granulocytic megakaryocytic |
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In a pt w/ chronic myeloproliferative dx, if there are any cytogenic, molecular, and/or hematopoietic GF response abnormalities they are most likely found in which cells?
|
in all 3 hematopoietic cell lines, but not in non-hematopoietic cells
(molecular and cytogenetic abnormalities have no effect on BM fibroblasts) |
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where does the initial manifestation of lymphomas most likely to occur?
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in lymphoid tissue, then BM and peripheral blood
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do lymphomas necessarily have to originate in spleen and thymus?
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no, they can originate from any lymphoid tissue
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where do leukemias originate?
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originate in BM, and then secondarily seed other tissues (LNs, splee, liver)
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where does nodal NHL arise?
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in LNs
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where does extra-nodal NHL arise?
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specialized groups of lymphoid cells, MALT, mucosa of lungs and spleen
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what are the (3) characteristics of chronic myeloproliferative disorders?
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1. terminal differentiation is relatively unaffected
2. BM hypercellularity, inc hematopoiesis, and elevated cell counts in peripheral blood 3. neoplastic cell infiltration suppresses residual normal progenitor cells in the BM |
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are cells in CLL germinal center-derived, parafollicular, or sinusoidal?
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parafollicular
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multiple myeloma (MM) and plasmacytoma are dz of what kinds of cells?
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plasma cells
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54 yo pt w/ wt loss, generalized rubbery adenopathy (esp the lump in right groin), and splenomegaly. What dx do you consider?
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leukemia
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CMV and Bartonella infxns will present w/ lymphadenopathy in which areas?
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axillary and inguinal
(localized to extremities) |
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EBV and infections mononucleosis will present w/ hyperplasia of which region in the LN?
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paracortical area
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what cancers would you consider in a pt w/ inguinal lymphadenopathy (3)?
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1. SCC of penis, vulva, anus
2. malignant melanoma of LE 3. ca of uterus |
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how would LNs look in a pt w/ leukemia?
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diffuse effacement of LN architecture d/t infiltration of large lymphocytes w/ eccentric nucleus and deeply basophilic cytoplasm
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what is the correct test to order in a pt w/ lymphoma (2)?
|
LN biopsy
BM biopsy |
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what dz is assoc w/ BCR-ABL?
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CML
(BCR-ABL is the Ph chr, has tyrosine kinase activity) |
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what is FMC-7 marker for?
what are the other markers for this dz? |
prolymphocytic /hairy cell leukemia
CD11c --> adhesion molecule CD25 --> IL receptor CD103 |
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BCL6 translocation is seen w/ what dz?
- what chromosome is it on? -what does it encode? |
diffuse large B-cell lymphoma
- BCL6 is on chr #3 - BCL6 encodes a zinc-finger TF that regulates devt of germinal center B-cells |
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which transolcation involves the BCL2 gene and what dz is it seen w/?
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BCL2 involves t(14:18) translocation
seen in diffuse large B-cell lymphoma |
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what viruses are assoc w/ diffuse large B-cell lymphomas?
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EBV (in AIDS and immunosuppressed pts)
HHV-8 (in body-cavity based large cell lymphomas of pts w/ advanced HIV infxns and HIV(-) elderly) |
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is splenomegally assoc w/ CLL?
how does splenomegaly present in a pt? |
yes, it is MC finding
splenomegaly present as early satiety |
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what are "B symptoms" usually seen w/ (5)?
|
1. Hodgkin lymphoma
2. CLL 3. malaria 4. disseminated histoplasmosis 5. miliary TB |
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do CLL lymphocytes undergo apoptosis? are they sensitive to chemo?
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no, they do not undergo apoptosis
they are not sensitive to chemo b/c they are not actively dividing (they just live forever) |
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what are the "B symptoms"?
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fever
night sweats unexplained wt loss >10% of total body wt |
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why do pts w/ CLL present w/ hypogammaglobulinemia?
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b/c they have a lot of mature, but not terminally mature (plasma cells, memory cells) cells, which have not been exposed to Ag and therefore do not secrete Ig
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what kind of infxns are pts w/ CLL prone to?
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1. viral infxns (herpes, VZV)
2. infxns w/ encapsulated orgs |
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what kind of cells do you look for in ALL?
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lymphoBlasts
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what will you see on the peripheral smear of a pt w/ CML?
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EVERYTHING
it will look like BM w/ mature and immature cells look for lots of granulocytic cells |
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why is retulocyte count high in pts w/ CLL?
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reticulocytes are indicative of hemolysis b/c there are AI Abs against rbcs in CLL pts
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what will CLL rbcs look like? platelets?
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rbcs and normocytic and normochromic
platelets are normal |
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what is Richter syndrome?
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B-cells de-differentiate (stop living forever and start to divide very aggressively)
presents as a rapidly-enlarging mass in spleen or LN indicates transformation to diffuse large B-cell lymphoma |
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If circulating cells are primarily neutrophils, metamyelocytes (have a dent), myelocytes, promyelocytes -- what is the most likely dx?
what are those cells collectively called? |
dx --> CML
they are all granulocytes |
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what is the diagnostic characteristic of AML?
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>20% myeloid blasts in the BM
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what is low LAP score indicative of?
when is it high? |
LAP (leukocyte alkaline phosphatase) is a stain for normal wbcs that are undergoing normal differentiation
Low LAP score is indicative of CML b/c neoplastic cells are not developing properly LAP score is high in a leukemoid rxn (infxn) |
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what are the 3 stages in normal progression of CML?
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1. stable (chronic) phase
2. accelerated phase 3. acute leukemia w/ blast crisis |
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what dz can progress to AML?
|
all chronic myeloproliferative dx:
1. CML 2. polycythemia vera 3. essential thrombocytosis 4. primary myelofibrosis |
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what is the MC symptom assoc w/ myeloproliferative dz?
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pancytopenia
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what are MC chr abnormalities seen in pts w/ myelodysplastic syndromes?
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trisomy8, 5q deletion
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what is bi-cytopenia?
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decrease in platelet and rbc #s
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what is assoc w/ toxic granulations and Dohle bodies?
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leukemoid rxn
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What are Auer rods and what condition are they seen in?
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Auer rods are immature myeloblasts made of pink crystallized granules
seen in AML |
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how do you distinguish b/in CLL and AML on the peripheral smear?
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CLL --> smudge cells
AML --> large blasts w/ Auer rods in the cytoplasm |
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what is leukemia hiatus and which dz is it assoc w/?
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leukemia hiatus --> gap in normal maturation of cells: there are mature and immature cells, but no intermediates; eventually immature cells spill over into the peripheral blood
assoc w/ AML |
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how do you know when CML has converted to AML (reached blast crisis)?
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there are >20% blasts in BM, which is indicative of AML
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normally, CML converts into AML; however, in ~5-10% of cases it converts to something else. What is that dz and what is the mechanism of conversion?
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in 5-10% of cases CML converts into ALL (instead of AML)
this is b/c some of the early cells convert into lymphoBlasts --> leads to ALL |
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44 yo has persistent dx that shows leukoytosis w/ predominance of mature neutrophils, few myelocytes, metamyelocytes, and promyelocytes. Meyloblasts are not IDd. BM shows similar pattern of hyperplastic granulocytic cells showing maturation. What is the dx?
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CML
(granulocytic hyperplasia) |
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which particular type of granulocyte is particularly elevated in CML?
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basophils
(eosonophils too) |
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what is interesting about the granulocytic cells in pts w/ CML?
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these are mixed granules (both basophilic and eosinophilic)
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which cells does Hodgkin lymphoma come from?
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germinal center or post-germinal center B-cells (this is what gives it a unique immunophenotype)
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RS cells are diagnostic of what dx? Is finding that cells alone enough to make dx?
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Hodgkin lymphoma
need to make sure that RS cell is present against a reactive non-neoplastic background of lymphocytes, neutrophils, plasma cells, eosinophils, and macrophages |
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what is the only subtype of HL that is more common in women?
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nodular sclerosing
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which HL subtype is MC in immunosuppressed pts?
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lymphocyte depletion
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which HL subtype is the rarest?
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lymphocyte predominance
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which HL subtype is not assoc w/ EBV?
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lymphocyte predominance
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which HL subtype occurs mostly in younger pts and has frequent mediastinal involvement?
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nodular sclerosing
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which HL subtype normally does not involve the mediastinum?
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lymphocyte predominanace
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which virus has a pathognomic role in HL? what is the transforming step?
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EBV
cells express LMP-1 (latent membrane protein-1) which is encoded by EBV genome and has transforming activity |
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which HL subtype is CD20+, CD15-, CD30-, EBV-?
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lymphocyte predominance
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which HL subtype is CD30+, CD15+, EBV-?
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nodular sclerosing
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which HL subtype is CD30+, CD15+, EBV+?
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mixed cellularity
lymphocyte rich lymphocyte depletion |
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which cytokines are implicated in pathogenesis of nodular sclerosing HL?
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TGF-beta
IL-5 |
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which cytokine is implicated in pathogenesis of mixed cellularity HL?
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IL-5
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do RS cells possess B-cell markers?
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no
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what is the only type of RS cell that expresses CD20?
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L&H variant
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what is the role of NF-kB in HL pathogenesis?
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activated my EBV LMP-1, it's a TF that promotes B-cell survival and proliferation
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what is the most important prognostic variable in HL?
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stage (tumor burden)
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does HL invade Waldeyer's ring, mesenteric nodes, or extranodal sites?
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no
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what are the complications of HL therapy?
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1. increased risk for developing other hematologic ca (MDS, AML, NHL)
2. development of solid ca of lung, breast, stomach, skin, soft tissues |
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what is the pattern of LN spread in HL?
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localized to a single axial group of nodes (cervical, mediastinal, para-aortic); spread is orderly and contiguous
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is there generalized lymphadenopathy w/ HL?
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no
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describe an RS cell
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two mirror-image nuclei surrounded by a clear halo
CD15+, CD30+ |
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CD4 helper T-cells that home to the skin are seen in which dz that leads to exfoliative erythroderma?
|
Sezary syndrome
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what is the difference b/in mycoses fungioides and Sezary syndrome?
|
circulating tumor cells are seen in 25% of cases of mycoses fungioides
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61 yo male c/o high total protein and low albumin, distended abdomen, hyperuricemia, hyper-Ca, high Cr w/ signs of renal failure, and normal LAP. Bone density is greatly decreased. X-rays are pending. Most likely dx?
what do you expect to see on the X-rays? |
multiple myeloma (MM)
X-rays --> multiple "punched out" lytic lesions in the bones and skull |
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what leads to hyper-Ca and bone destruction in MM?
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upregulation of RANKL in neoplastic cells --> osteoclast-activating factor, induces bone breakdown
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what makes dysplastic cells significant in MM?
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they are derived from the same B-cell clone, so they make the same Ig (seen as the "M spike" on plasmaphoresis)
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33 yo woman c/o low-grade fevers, night sweats and generalized malaise for past 6 mo. Non-tender cervical and supraclavirular lympadenopathy. You see RS cells on her smear, w/ 2 lobes and abundant cytoplasm, surrounded by reactive lymphocytes, marcophages, and eosinophils.
What is the dx? |
Hodgkin lymphoma, nodular sclerosing type
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65 yo man w/ adv lymphoma and rapidly growing ileal mass (extra-nodal but lymphoid), generalized adenopathy, peripheral blood and BM spread shows one residual germ center remains, diffuse neoplastic infiltrate in the mantle zone. Cells in the germ center are not neoplastic. What is the dx?
|
mantle cell lymphoma
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what are the markers for mantle cell lymphoma and what is the translocation?
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markers --> CD5, cyclinD/IgH juxtaposition
translocation --> t(11:14) |
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what is the role of cyclin D in cell cycle?
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regulates G1-S progression of cell cycle
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what the MC NHL in the US?
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follicular lymphoma
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what are the clinical s/s (2) and prognosis of mantle cell lymphoma?
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1. generalized lymphadenopathy
2. "B symptoms" prognosis is poor |
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which lymphoma is aka MALToma?
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marginal zone lymphoma
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where do marginal zone lymphomas occus?
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strong tendency to occur at sites of chronic immune inflam rxns (salivary glands, thyroid, stomach)
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what are the 2 features that make marginal zone lymphoma difficult to diagnose?
|
1. remains localized to the site of origin for long periods of time
2. predominant cell popn resembles normal marginal zone B-cell |
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what is the pathogenic mech of MALTomas?
|
stepwise progression from polyclonal immune resp (infxn resp) to monoclonal neoplasm
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what are the genetic abnormalities seen in marginal zone lymphomas?
- translocations? - overexpressions? |
translocations --> t(11:18) and t(1:14)
overexpressions --> BCL10 /IgH fusion gene |
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what does marginal zone lymphoma eventually transform to?
|
diffuse large B-cell lymphoma
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which lymphoma has multifocal mucosal involvement of small bowel and colon, and what is the name for such rxn?
|
mantle cell lymphona
rxn --> lymphomatoid polyposis |
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hair-like cytoplasmic projections are characteristic of?
|
hairy cell leukemia
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what are the patterns in:
- Burkitt lymphoma - SLL/CLL - ALL - follicular lymphoma |
- Burkitt lymphoma --> "starry sky"
- SLL/CLL --> diffuse - ALL --> diffuse - follicular lymphoma --> nodular |
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what is the t(14:18) translocation assoc w/?
|
follicular lymphoma
diffuse large B-cell lymphoma |
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what is the t (9:22) translocation assoc w/?
|
CML
|
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what is the t(15:17) translocation assoc w/?
|
acule promyelocytic leukemia
|
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what is the treatment for acute promyelocytic leukemia and how is curative?
|
all-trans retinoic acid
fusion protein d/t translocation blocks myeloid cell differentiation; tx leads to differentiation of cells, which clear from the marrow and allow for resumption of normal hematopoiesis |
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what are the translocations assoc w/ Burkitt lymphoma?
|
t(8:14)
t(2:8) t(8:22) |
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what is the t(11:14) translocation assoc w/?
|
mantle cell lymphoma
|
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what is the t(11:18) translocation assoc w/?
|
marginal zone lymphoma
|
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which 3 lymphomas present w/ "starry sky" appearance?
|
Burkitt lymphoma
lymphoblastic lymphoma |
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which dx are PAS(+)?
|
ALL
|
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68 yo man has noted presence of several lumps on the right side of his neck for the past 5 mo. On PE he has firm, non-tender movable LNs palpable on R cervical posterior region; there is no splenohepatomegaly. Labs: normal wbc and platelets, very slightly low Hb. Cervical LN bx shows numerous crowded follicles composed of small, relative monomorphic cleaved lymphocytic cells w/ varying # of larger cells; few mitotic figures. Most likely dx?
|
follicular lymphoma
|
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what differentiates follicular lymphoma from follicular hyperplasia?
|
cells in lymphoma are monomorphic
|
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what gene is involved in follicular lymphoma?
|
BCL2
t(14:18) translocation |
|
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45 yo man noted appearance of his face over 7 mo, skin is thickened and reddened. Punch biopsy shows infiltration by neoplastic T-cells. What is the dx?
|
mycosis fungioides
|
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what is the marker for adult T-cell leukemia?
What virus is it assoc w? |
CD25
HTLV-1 |
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what is the nuclear appearance in adult T-cell leukemia?
|
multilobar "clover leaf" or flower appearance
|
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59 yo man w/ hx of chronic erythema and scaling of the skin; Generalized scaling on face, abdomen, back, butt, and extremities. w/ palmoplantar hyperkeratosis. What markers are assoc w/ this condition?
|
CD2, CD3, CD5
CD4 no TdT |
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what is the prognosis for adult T-cell leukemia?
|
extremely aggressive that is refractory to tx in most cases
|
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what are the 3 most common causes of neutropenia (agranulocytosis)?
|
1. inadequate or ineffective granulopoiesis
2. accelerated removal /destruction 3. drug-induced (most signif) |
|
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what is the use of G-CSF (granulocyte colony-stimulating factor) in neutropenia?
what produces G-CSF? |
decrease duration and severity of neutrophil nadir d/t chemo drugs
marrow stromal elements make G-CSF |
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where are Dohle bodies located, what are they composed of, and what do they accompany?
|
Dohle bodies are cytoplasmic patches of ER seen in polymorphonuclear leukocytosis (seen together w/ toxic granulations)
|
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what is basophilic leukocytosis indicative of?
|
underlying myeloproliferative dz (such as CML)
|
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when do you see lymphocytosis?
|
viral infxns
Bordetella pertussis infxn |
|
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what is the effect of glucocorticoid administration on demargination and extravasation of neutrophils?
|
increased demargination
decreased extravasation |
|
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what 2 things can neoplastic leukocytosis be confused w/?
|
1. acute childhood viral infxns
2. severe inflam states (can lead to myelogenous leukemia) |
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what are the cells assoc w/:
- follicular hyperplasia - paracortical hyperplasia - sinus histiocytosis |
- follicular hyperplasia --> B-cells
- paracortical hyperplasia --> T-cells - sinus histiocytosis --> macrophages |
|
|
in follicular hyperplasia, which B-cells are in the dark /light zones?
|
dark zone --> centroblasts
light zone --> centrocytes |
|
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what features are used to differnetiate follicular hyperplasia from follicular lymphoma (4)?
|
follicular hyperplasia has:
1. preservation of LN architecture 2. parked variation in shape and size of lymphoid nodules 3. freq mitotic figures, phagocytic macrophages 4. recognizable light and dark zones |
|
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what clinical picture accompanies sinus histiocytosis?
|
epithelial ca
|
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Philadelphia chr occurs in which 3 diseases?
|
1. ALL
2. AML 3. CML |
|
|
which lymphomas are assoc w/ EBV genome?
|
Butkitt lymphoma
Hodgkin lymphoma diffuse large B-cell lymphoma NK-cell lymphoma |
|
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what is assoc w/ HHV8?
|
diffuse large B-cell lymphoma
|
|
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what is H. pylori assoc w/?
|
gastric marginal cell lymphoma (MALToma)
|
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why are all lymphoid neoplasms monoclonal?
|
b/c they derive from a single transformed cell
|
|
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which lymphomas derive from germinal center B-cells?
|
1. Burkitt lymphoma
2. diffuse large B-cell lymphoma 3. follicular lymphoma |
|
|
which lymphomas derive from naive B-cells?
|
1. mantle cell lymphoma
2. SLL/CLL |
|
|
which lymyphoma derives from post-germinal center B-cell?
|
diffuse large B-cell lymphoma
|
|
|
which lymphoma derives from post-germinal center memory B-cell?
|
1. marginal zone lymphoma
2. hairy cell leukemia 2. SLL/CLL |
|
|
what type of cells comprise ALL (acute lymphoBLASTic leukimia?
|
immature pre-B or pre-T lymphoBLASTS
|
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which dz presents as a mediastinal mass in adolescent boys?
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pre-T ALL
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what are the morphological differences b/in pre-B and pre-T lymphoblasts?
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there are none
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other than BM, spleen and liver, what other 2 organs are involved in ALL?
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testicles and CNS
(aka "sanctuary sites" d/t blood-testes barrier and BBB) |
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is ALL associated w/ Philadelphia chromosome?
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yes
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what are the 4 mehanisms of pathogenesis assoc w/ ALL (translocations /other genetic aberrations)?
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1. hyperdiploidy
2. t(12:21) 3. t(9:22) --> Ph chomosome 4. translocation of MLL gene |
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which T-cell marker is seen on B-cells in CLL?
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CD5
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which lymphoma presents w/ BCL2 overexpression and what does that lead to?
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follicular lymphoma
BCL2 prevents apoptosis and promotes tumor cell survival |
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is follicular lymphoma curable w/ conventional chemo?
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no
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what is the translocation associated w/ follicular lymphoma?
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t(14:18)
(w/ BCL2 overexpression) |
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what does diffuse large B-cell lymphoma do to the LNs?
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obliterates the underlying architecture
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body cavity-based large B-cell lymphomas are assoc w/ which virus?
what kinds of pts get it? where do lymphomas arise from? |
HHV8
in HIV pts and HIV(-) elderly arise from pleural and ascitic effusions |
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other than HHV8, what other virus is assoc w/ diffuse large B-cell lymphoma?
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EBV
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how does diffuse large B-cell lymphoma present?
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as a rapidly enlarging symptomatic mass at a single nodal or extra-nodal site
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what is the Burkitt lymphoma appearance on peripheral smear and why?
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"starry sky" appearance
apoptotic cells are devoured by pale macrophages |
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what gene is Burkitt lymphoma assoc w/ and on what chromosome is it?
what are the translocations? |
c-MYC, chr #8
t(8:14) t(2:8) t(8:22) |
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what are African Burkitt lymphoma tumors are infected with and where are they most likely to present?
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EBV
present in the jaw |
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where does non-endemic Burkitt lymphoma most likely to present?
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as ileocecal or peritoneal mass
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what cells comprise the Burkitt lymphoma?
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relatively mature B-cells
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are high mitotic index and apoptopic cell death typical for Burkitt lymphoma?
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yes
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is Burkitt lymphoma curable?
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yes, though aggressive it is treatable w/ short-term high-dose chemo (many pts are cured)
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what is the name for the free light chains excreted in the urine?
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Bence-Jones protein
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what kidney disorder is assoc w/ MM?
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amyloidosis
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MM is a lesion of that kind of cells?
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terminally differentiated B-cells
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what is the MC malignant gammopathy?
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MM
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what do MM tumors secrete?
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free light chains
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what leads to the bony destruction in pts w/MM (which cytokines are implicated)?
which cyclins are in involved? |
cytokines --> RANKL and MIP1-alpha
osteoclast activating factor that leads to "punched out" lytic lesions in long bones, spine, skull etc cyclin D1 and D3 |
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what is the pattern of tumor infiltration in pts w/ MM?
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cells diffusely infiltrate as sheet-like masses that completely replace normal elements
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what is the pathogenesis of Tamm-Horsfall protein in MM?
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involved in formation of protein casts in DCT and collecting ducts (together w/ Ig light chains and albumin)
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do glomerular and peritubular hyaline deposits stain w/ Congo red (stain used for amyloid deposition detection)?
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no
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which light chains are most common in MM kidney?
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kappa light chains
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rank Ig produced in MM in order of frequency
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1. IgG --> MC
2. IgA 3. free light chains 4. IgD, IgE 5. IgM --> rare, assoc w/ Waldenstom macroglobulinemia 6. non-secreting MM type |
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does MM interfere w/ immunity?
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yes, it suppresses humoral immunity
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what are the systemic effects of hyper-Ca assoc w/ MM (4)?
|
renal --> renal dz, polyuria, amyloidosis
neuro --> confusion, weakness, lethargy GI --> bloating, constipation immuno --> recurrent bact infxns |
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what causes hyperviscosity syndrome?
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aka Waldenstom macroglobulinemia
d/t excessive IgM prodxn and aggregation |
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when BM becomes involved in MM, what kind of anemia does it produce?
|
normochromic, normocytic
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what are the 2 MCC of death in pts w/ MM?
|
renal failure
infxns |
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what is plasmacytoma and where does it occur?
|
solitary myeloma lesion, occurs in the same spaces as MM --> bone or soft tissue
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what are the extraoseous lesion locations for plasmacytoma (3)?
|
1. lungs
2. oronasopharynx 3. nasal sinuses |
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what is MGUS?
what is the clinical course and progression rate to MM? |
MGUS --> monoclonal gammopathy of uncertain significance
M-proteins in sera, no BJ proteinuria 1% of pts annually progress to MM |
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what is lymphoplasmacytic lymphoma?
|
B-cell neoplasm of older adults that secretes monoclonal IgM in amts sufficient enough to cause hyperviscosity syndrome (Waldenstom macroglobulinemia)
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what is the mechanism of hemolysis in pts w/ lymphoplasmacytic lymphoma?
|
1. BM infiltration causes anemias
2. exacerbation is by AI hemolysis d/t cold agglutinins of the IgM type |
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what are the symptoms of Waldenstom macroglobulinemia?
|
1. visual impairment (tortuosity, distention and AV nicking of retinal veins)
2. neuro problems --> headaches, dizziness, deafness d/t sluggish blood flow 3. bleeding problems --> d/t interference w/ platelet fxn and coag factor consumption 4. cryoglobulinemia --> precipitation of macroglobulins at 4C that leads to Raynaud syndrome and cold utricaria |
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what is the prognosis for lymphoplasmacytic lymphoma?
|
incurable progressive dz w/ median survival = 4 yrs
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how do you treat the symptoms of lymphoplasmacytic lymphoma?
|
plasmaphoresis
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does mantle cell lymphoma involve BM and spleen?
|
not usually
|
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does mantle cell lymphoma present w/ B symptoms?
|
yes
|
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what do pts die from w/ mantle cell lymphoma?
|
death is d/t complications of organomegaly d/t tumor infiltration
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hypermutated Ig genes in hairy cell leukemia suggests what kind of cell origin?
|
post-germinal center memory B-cell
|
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does hairy cell leukemia present w/ lymphadenopathy?
|
no
|
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what kind of infxns are seen in hairy cell leukemia and what contributes to that?
|
high incidence of atypical mycobacterial infxns
d/t monocytopenia |
|
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what is a prognosis for hairy cell leukemia?
|
indolent course
good prognosis long-lasting remission in most pts |
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where does unspecified peripheral T-cell lymphoma tend to proliferate?
|
small vessel proliferation
|
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what is the phenotype of unspecified peripheral T-cell lymphoma, by definition?
|
mature T-cell phenotype
|
|
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what are the symptoms of unspecified peripheral T-cell lymphoma?
|
generalized lymphadenopathy
eosinophilia pruritus fever wt loss |
|
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what are the surface markers for unspecified peripheral T-cell lymphoma?
|
TdT(-), CD1(-)
CD2(+), CD3(+) T-cell Ag (+) |
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what dz is ALK mutation assoc w/?
what is the fxn of new fusion protein? |
anaplastic large cell lymphoma
leads to constitutively active form of tyrosine kinase (chr #2) |
|
|
which anaplastic large cell lymphoma has better prognosis --> ALK(+) or ALK(-)?
|
AKL(+) tumors have better prognosis
|
|
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what marker does adult T-cell leukemia /lymphoma express on its surface?
|
CD4
|
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what are the clinical findings assoc w/ adult T-cell leukemia /lymphoma (4)?
|
1. skin involvement
2. generalized lymphadenopathy and splenomegaly 3. lymphocytosis 4. hyper-Ca |
|
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what is the prognosis in adult T-cell leukemia /lymphoma?
that is the median survival? |
dz is extremely aggressive and refractory to tx in most cases
median survival = 8 mo |
|
|
describe the nuclei of the circulating neoplastic cells of mycosis fungioides?
what type of cell is that? |
cerebriform nuclei
CD4(+) cells |
|
|
what is the manifestation of Sezary syndrome?
|
exfoliative erythroderma
|
|
|
what is significant abt the cytoplasm of cells in large granular lymphocytic leukemia?
|
it contains scattered coarse azurophilic granules
|
|
|
describe BM involvement in large granular lymphocytic leukemia?
|
BM involvement is usually focal and w/o displacement of normal hematopoietic cells
|
|
|
in large granular lymphocytic leukemia, what are the markers expressed by:
- T-cells? - NK-cells? |
T-cells --> CD3, CD8
NK-cells --> CD16, CD56 |
|
|
what is Felty syndrome triad?
what is it assoc w/? |
Felty syndrome triad:
1. RA 2. splenomegaly 3. neutropenia assoc w/ large granular lymphocytic leukemia |
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|
what is a name for a destructive midline mass involving nasopharynx and skin + testes?
|
extranodal NK /T-cell lymphoma
|
|
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where does tumor infiltration occur in extranodal NK /T-cell lymphoma and what is the complication of such invasion?
|
small vessels
leads to ischemic necrosis |
|
|
which infxn is assoc w/ extranodal NK /T-cell lymphoma?
|
EBV
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