Lymphadenopathy

Question 1

Lymphadenopathy

Answer 1

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

Question 2

Reactive lymphadenopathy

Answer 2

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

Question 3

Lymphoid neoplasms

Answer 3

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

Question 4

Lymphomas

Answer 4

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

Question 5

Burkitt lymphoma

Answer 5

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

Question 6

Diffuse large B-cell lymphoma

Answer 6

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

Question 7

Extranodal marginal zone lymphoma (MALToma)

Answer 7

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

Question 8

Follicular lymphoma

Answer 8

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)

Question 9

Mantle cell lymphoma

Answer 9

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

Question 10

Mature T/NK cell lymphomas

Answer 10

Peripheral T cell lymphoma, Extranodal NK/T-cell lymphoma, Anaplastic large cell lymphoma

Question 11

Peripheral T cell lymphoma, unspecified

Answer 11

Helper T cell, no specific chromosomal abnormality
Mainly older adults, presents with lymphadenopathy
AGGRESSIVE

Question 12

Anaplastic Large cell lymphoma

Answer 12

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)

Question 13

Extranodal NK/T cell lymphoma

Answer 13

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

Question 14

Hodgkins lymphomas

Answer 14

Classical and nodulat lympmhocyte predominate Hodgkin lymphoma (NLPHL

Question 15

Classical hodgkins lymphom vs Nodular lymphocyte predominaate hodgkin lymphona

Answer 15

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

Question 16

Lymphoma treatment classes

Answer 16

Topoisomerase inhibitors (block enzyme topoisomerase, include etoposide and athacyclines)
Vinca Alkaloids (anitmicrotuvule agents, vincristine/vinblastine)
Alkylating agents (attaches Alkyl group to DNA, cyclophosphamide, melphalan)

Question 17

Diffuse large B cell lymphoma treatment

Answer 17

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

Question 18

Cytokine Release syndrome

Answer 18

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

Question 19

hodkin Lymphoma treatment

Answer 19

ABVD treatment (adriamycin, bleomycin, vinblastine, dacarbazine

Relapsed patients: Antibodu drug conjugate (brentuximab), Immunotherapy (pembrolizumab or nivolumab)

PD1 inhibition

Allogeneic transplant

Question 20

Double hit lymphoma

Answer 20

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