Lymphadenopathy Flashcards
Lymphadenopathy
Lymph nodes may come enlarged lymphadenpathy
in both reactive and neoplastic processes
Reactive lymphadenopathy is more common in children than adults. The differential diagnosis for lymphadenopathy in adults includes neoplastic processes such as lymphoma and metastatic tumor, more commonly than children
Reactive lymphadenopathy
Infectious mononucleous, cat scratch, etc
Autoimmune
Histologic patterns- follicular hyperplasia (B cell expansions), Paracortical hyperplasia (T cell expansion), sinus histiocytosis (kinda looks like fibrosis of paracortex)
Neoplastic lymphadenopathy- general principles of lymphoid neoplasia
Lymphoid leukemias are predominantely blood and bone marrow based
Lymphomas are predominantely tissue based (lymph nodes ans spleen and liver)
Lymphomas can involve the blood sometimes
Lymphoid neoplasms
Follicular lymphoma, mantle cell lymphoma, diffuse large B cell lymphoma, burkit lymphoma, double hit lymphoma, hodgkins lymphoma, extranodal marginal zone lymphoma, anaplastic large cell lymphoma, peripheral T cell lymphoma, extranodal NK/T cell lymphoma
Lymphomas
Non-hodgkin lymphomas- Mature B cell lymphomas (follicular lymphoma, mantle cell lymphoma, diffuse large B cell lymphoma, burkitt, extranodal marginal zone lymphoma, double hit lymphoma
Burkitt lymphoma
neoplastic proliferation of intermediate sized B cells (CD 10+, CD 19 +, CD20+)
Associated with EBV
Extranodal mass in young kids and adults
African form in jaw, sporadic in abdomen
Driven by translocations involving Cmyc and immunoglobulin loci t 8–>14
Can also be t (2,8) or t(8,22)
3 variants endemic (EBV- associated), Sporatic, and immunosuppression-related (EBV associated),
Starry sky
VERY AGGRESSIVE
Diffuse large B-cell lymphoma
Diverse chromosomal rearrangement Neoplastic proliferation of large B cell (CD 19, CD20+) Grow diffusely in sheets Most common form of Non-hodgkin lymphoma Clinically aggressive Sporadic or from a follicular lymphoma Affects all ages, but most common in adults, often appears as a rapidly growing extranodal mass/ lymphadenopathy Starry ski VERY AGGRESSIVE
Extranodal marginal zone lymphoma (MALToma)
Neoplastic proliferation of small B cells (CD20+, CD19+, Cd 5 ad 10 –) that expands the marginal zone
Nipple on a breast, the breast is the expanded marginal zone
Associated with chronic inflammatory states such as Hashimotos thyroiditis, sjorgren syndrome, H pylori
Margin is formed by post geminal zone B cells, usually in mucosal sites (Gastric Maltoma as a result of h pylori)
t(11,18) is the most common abnormality in gastric, MALT1-IAP2 fusion gene, tend to be localized
INDOLENT
Follicular lymphoma
neoplastic proliferation of small B cells (CD 10 +, CD19 +, CD 20+)
driven by t(14–>18) creating BCL2-IgH fusion gene or less commonly BCL6 rearrangements
BCL2 on chromosome 18 to the Ig heavy chain locus on chromosome 14, results in Bcl2 an apoptosis inhibitor
Treatment ivolves chemotherapy or anti CD20 Ab(rituximab)
REtroperitoneal involvement, slow growth but can transform to Diffuse large B cell lymphoma. Generalized lymphoma (diruption of normal lymphnode arcitecture, lack of tingible body macrophages, bcl2 expression, monoclonality)
Mantle cell lymphoma
neoplastic proliferation of small B cells. Mantle cell lymphoma (CD 5 +, CD19+, CD20+)
Older males in the GI tract, moderately aggressive
Driven by t(11,14) creating cyclin D-IgH fusion gene
Cyclin D goes on Igh and gets overexpressed
Mature T/NK cell lymphomas
Peripheral T cell lymphoma, Extranodal NK/T-cell lymphoma, Anaplastic large cell lymphoma
Peripheral T cell lymphoma, unspecified
Helper T cell, no specific chromosomal abnormality
Mainly older adults, presents with lymphadenopathy
AGGRESSIVE
Anaplastic Large cell lymphoma
Helper T cell, rearrangements of ALK gene
Children an young adults usually with lymph node and soft tissue disease, Aggressive
CD4 T cell neoplasm (tyrosine kinase receptor (t2,5)
Extranodal NK/T cell lymphoma
NK cell (common) or cytotoxic T cell (rarely)
EBV associated
Adults with destructive extranodal masses, Sinonasl, aggressive
Adult tumor, nasal mass with necrosis, EBV associated, Asia, sounth america, aggressive
Hodgkins lymphomas
Classical and nodulat lympmhocyte predominate Hodgkin lymphoma (NLPHL
Classical hodgkins lymphom vs Nodular lymphocyte predominaate hodgkin lymphona
Classica: younger kids, mediastinal/cervical, REED STERNBER owel cells in the background of reactive inflammatory cells (eosinophils, neutrophils), lacunar cells nodules and sclerosis
Immunophenotype(CD 15, CD30), B cells, contiguous, predictable spread, moderately aggressive
NLPHL: cervical, axillary middle aged kids, nodular collections of popcorn cells with lymphocytes, CD20, CD45, Frequent relapses, INDOLENT
Lymphoma treatment classes
Topoisomerase inhibitors (block enzyme topoisomerase, include etoposide and athacyclines) Vinca Alkaloids (anitmicrotuvule agents, vincristine/vinblastine) Alkylating agents (attaches Alkyl group to DNA, cyclophosphamide, melphalan)
Diffuse large B cell lymphoma treatment
Most common form of non hodgkin lymphoma
CHOP therapy+ rituximab (infection, leukopenia, organ toxicites
Rituximab is anti CD20 so it kills B cells via ADCC
If relapsed 2 options : autologous transplant or CAR-t cell therapy
Cytokine Release syndrome
a potentially lethal complication of CAR-T cell infusion
CRS, non Ag specific toxicity that occurs– hypotension, renal failure, shock, and death
Symptoms are related to systemic inflammation secondary to elevated levels of pro inflammatory cytokines
high grade fever to death and organ failure
IL6 is the central mediator
Treat with Tocilizumab mAb against IL6
hodkin Lymphoma treatment
ABVD treatment (adriamycin, bleomycin, vinblastine, dacarbazine
Relapsed patients: Antibodu drug conjugate (brentuximab), Immunotherapy (pembrolizumab or nivolumab)
PD1 inhibition
Allogeneic transplant
Double hit lymphoma
when follicular lymphoma becomes diffuse large B cell lymphoma
Myc rearrangement and BCL2 BCL6 rearrangement
Aggressive
Resemble diffuse B cell lymphoma or burkitt or both
