stuff 1 from saturday- lymphadenitis and non neoplasias Flashcards
common feature of all forms is activation of MOs and CD8s
hemophagocytic lymphohistiocytosis
how does hemophagocytis lymphohistiocytosis happen and the nick name
MOs eat up blood cell progenitors in marrow and formed elements in circulation. cytokine storm –> mediators suppress hematopoiesis and –> systemic inflammation occurs “shock like syndrome”
cytopenia, thrombocytopenias
macrophage activation syndrome
familial HLH, and most common trigger
familial hemophagocytic lymphohistiocytosis: CD8 and NK cells cant deploy cytotoxic granules–> acute febrile illness, organomegaly, hemophagocytosis of BM
MCT: EBV
a child with cancer
most common: acute lymphoblastic leukemia or acute lymphoblastic lymphoma
highly aggressive
an adult with cancer
Chronic Lymphocytic Leukemia or Small LL
most cases associated with the 14; 18 translo and overexpression of BCL2
follicular lymphoma: most common indolent lymphoma of adults
BCL6 associated, BCL2 in 1/3.
most common lymphoma of adults: diffuse Large b cell lymphoma, may arise from follicular lymphoma
strong ass w translos involving MYC
burkitt lymphoma, often latently infected by EBV
leukemias and NHLs more likely in adults
- diffuse large b cell lymphoma
- folliciular lymphoma
- hairy cell lymphoma
- mantle cell lymphoma
- Mult Myel/solitary plasmacytoma
- small lymphocytic lymphoma
- extranodal marginal zone lymphoma
- almost all T /NK cell neoplasms except for anaplastic large cell lymphoma
leukemias and NHLs most likely in children
BALLs and TALLs
anaplastic large cell lymphoma (cytotoxic T cells)
teenagers-20 yos
thymic lymphomas: TALLs
peake around age 3
ALLs
notch 1 more likey found in a
t cell neoplasm in children
a child with a lymphoma- most likely genetic mutations
PAX5
E2A
EBF
a child with lymphoma (ALLs) most likely translocations
t(12;21) involving ET6 and RUNX1
cd1
thymocytes and langerhans
tDt +, peroxidase +, positive for glycogen
lymphoBLASTs, myeloBLASTs, lymphoblasts
symptoms of the ALLs, and note t cell specific ones
symptoms related to reduced marrow- anemia, fever, neutropenia
symptoms related to mass effect: bone pain, LAD, splenomegaly, hepatomegaly, testicular enlargement.
in TALLs specifically: vessel compression (SVC syndrome) and meningeal spread (headaches)
factors inficating poor prognosis in ALLs
- age under 2 or presentation in adolescents/adulthood
- peripheral WBC above 100K
factors indicating a positive prognosis for ALLs
- diagnosed between 2-10
- low peripheral WBC count
- hyperdiploidy
- trisomy of chroms 4/7/10
- presence of t(12;21)
TRK mutations in ALLs
9;22 philly chrom translation. notable because most of the ALLs are transcription factor mutations.
starry sky appearance associated with what mutation and in what groups and in what kind of cells does this appear
8;14 translo in burkitts and ass w/70% adults and 30 of childhood NHLs, reactive histiocytosis
para-aortic nodes
american type burkitt lymphoma, mostly adults but also 30% of children w/NHL
Germinal B cell lymphomas
burkitt, diffuse, follicular
BCL-2 (-), CD5: how old is this person likely?
CLL: 60+ yo
BCL2+, CD5- : what is a hallmark mutation in this group?
14; 18 t, follicular lymphoma
pathonmemonic for CLL and SLL
PROLIFERATION CENTERS:
diffuse effaced LN by infiltrate with small lymphocytes, admixed with activated lymphocytes father in loose aggregates referred to as proliferation centers.
how can we determine B cell monoclonality? what does that indicate?
kappa and lambad light chains; indicates neoplasia
Most common deletions in CLL
11, triosmy 12, 13, 17
what kind of chromosomal translocations are present in CLL?
none, or almost. very rate.
ZAP 70 and Notch 1
CLL
MLLT mutations
histone methyltransferase = regulates gene expression. present in up to 90% of follicular lymphomas
seen in 90% of folliciular lymphomas
14;18 translo and MLL2 mutations
T cell immunodeficiency associated B lymphoma: ignition.
EBV
This is a large B cell lymphoma subtype.
BCL2 (-), BCL6+, royal lue cytoplasm containing clear cytoplasmic vacuoles. how old is the patient, probably?
children/young adults. burkitt’s
all forms are highly ass w/translos of MYC gene on chrom 8. what does this do?
upregulates enzymes required for the Warburg effect (tumor uses lactic acid and glycolysis to produce itself)
this is burkitt’s
a man in his 40s or 50s presents with painless lymphadenopathy and massive splenomegaly. Lymphoma cells are CD20+, but CD23-
mantle cell lymphoma
CD23- distingushies it from CLL/SLL, which is CD23+
CD23+
CLL/SLL
11;14 translocation
involves the IgH locus on chrom 14 and cyclin D on chrom 11: overexpression of cyclin D1
overexpression of cyclin D1
mantle cell lymphoma
reactive germinal centers with a nodular appearance. diffusely efface node. proliferations consist of homogenous populations of small lymphocytes with irregular to occasionally deeply clefted nuclear contous. no centroblasts or centrocytes
mantle cell lymphoma
the most likely of all NHL to spread
mantle cell lymphoma
big spleen, polyp like lesions in the gut. what is your concern?
the mantle cell lymphoma will spread.
patient has massive hepatosplenomegaly and complains that his gluten anti IFN medication isn’t working
he has mantle cell lymphoma, lymphomatoid polyposis, and was misdiagnosed with ulcerative collitis.
a continuum between reactive lymphoid hyperplasia and full blown lymphoma
marginal zone lymphoma
does not begin as a monoclonal neoplasm but a polyclonal one
marginal zone lymphoma
kidney shaped nucleus with bleb like extensions
hairy cell leukemia
massive splenomegaly with respiratory issues
hairy cell leukemia
doctor can’t seem to get a marrow sample from a patient whose primary sample returned CD11c +, CD25+, CD103+, annexin A1+
hairy cell leukemia often presents with a “dry tap”
90% SHOW activating point mutations with serine/threonine kinase BRAF
hairy cell leukemia
what two characteristics of hairy cell leukemia would you expect to find on patients 50% of the time?
massive splenomegaly (splenic sequestration) and pancytopenia
hepatic portal triads are often involved
hairy cell leukemia
increased incidence of monocytopenia caused by______ in the context of hairy cell leukemia
mycobacterium avium
TRAP
pathoma says hairy cell leukemia is positive for tartrate resistance acid phosphatase (TRAP)
seen best under a phase contrast microscope, and the marrow is involved by a diffuse interstitial infiltrate, and splenic red pulp is highly infiltrated, leading to obliteration of the white pulp
beefy red appearance
hairy cell leukemia