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