L24- WBC Pathology III Flashcards
BL = (1):
- (aggressive/benign) neoplasm of (3) cells
- associated with (4) infection and (5) genetically
1- Burkitt Lymphoma 2- aggressive 3- B-cells 4- EBV 5- t(8;14)
list the three types of BL
- geographic area if applicable
- EBV association if applicable
- Sxs
(Burkitt Lymphoma)
- all present with rapidly growing tumor masses
- (1)/(2) mostly affect children / young adults
1) Endemic in parts of Africa: 100% EBV association, 50% with jaw / facial bone tumors (50-60%) and spreads to other sites (ovary, testis, kidney)
2) sporadically in other areas (non-endemic), 15% EBV association, abdominal masses in distal ileum / ilececal OR stomach +/- ascites
3) Immunodeficiency-Associated, mainly HIV pts, 25% EBV association, presents with Sxs related to underlying immunodeficiency
BL has a _______ appearance on histology
(Burkitt Lymphoma)
‘starry sky’ appearance, most from external sites
discuss BL prognosis
(Burkitt Lymphoma)
-although aggressive, good response to high dose chemotherapy
- most children / young adults can be cured
- older adults do a little worse
Hairy Cell Leukemia is a (common/rare) (fast/slow) growing neoplasm of (3) cells. It mainly affects (4- age, sex) group of people.
1- rare
2- slow / indolent
3- B-cells
4- older males (M:F, 4:1)
Hairy Cell leukemia mainly infiltrates (1) and (2)- indicate associated symptom. (3) is its hallmark characterization. Also note that (4) is rarely ever involved.
1- spleen (splenomegaly)
2- bone marrow (pancytopenia)
3- WBCs with fine, hair-like cytoplasmic projections
4- lymph nodes
Hairy Cell Leukemia diagnosis:
- (1) PB presentation
- (2) BM presentation
- (3) spleen appearance
1- round / kidney shaped nuclei, pale blue cytoplasm, thread/bleb-like extensions
2- infiltrates of small lymphocytes w/ abundant cytoplasm (fried egg appearance), enmeshed in reticulin
3- red pulp infiltration => beefy red appearance
Hairy Cell Leukemia is positive for the following CDs markers (indicate which ones are unique to it)
- CD20
- CD25
- ***CD11c
- ***CD103
Plasma Cell Neoplasms are cancers of (1) cells, that will release abundant amounts of (2). (3) and (4) are the key tumor markers for these neoplasms.
1- B-cells (matured into plasma cells)
2- Ig or Ig fragments (heavy, light chains)
3- M-proteins in blood
4- Bence Jones proteins (monoclonal λ, κ light chains)
name the Plasma Cell Neoplasm relating to:
- (1) IgG
- (2) IgM
- (3) heavy chains
- (4) light chains
- (5) other
1- Multiple Myeloma (multiple tumor sites) and Solitary Plasmacytoma (single tumor / bone site)
2- Waldenstrom Macroglobinemia
3- Heavy Chain Disease
4- primary amyloidosis (AL type)
5- MGUS, monoclonal gammopathy of undetermine significance
Multiple myeloma is a neoplasm of (1) cells located in (2). It commonly affects (3) age group and (4- sex, race) populations.
1- plasma cells
2- multiple bones
3- 65-70 y/o
4- males, African
Multiple Myeloma:
(1) is most frequent form
(2) is the other common form
IgG, 60%
IgA, 20%
Multiple Myeloma clinical presentation:
-(1) affect on bones, (2) is the appearance on X-Ray, (3) is the main effect as a result
-(4) is the main blood manifestation, leading to increased risk of (5)
1- pain –> destruction or fractures in axial skeleton (vertebrae > ribs > skull)
2- multiple lytic lesions
3- excess bone resorption –> hypercalcemia
4- cytopenias (b/c BM replacement)
5- infections (neutropenia + hypogammaglobulinemia)
Multiple Myeloma clinical presentation:
- (1) is the main organ affected secondary to (2)
- (3) is the other main sign seen in (4) organs
1- kidney –> renal insufficiency
2- hypercalcemia (Bence-Jones proteins + amyloid deposition)
3- organomegaly
4- spleen, liver, LNs, lung (late in disease course)
Mutiple Myeloma Dx:
-(1) is an absolute requirement seen in BM
(plus one of the following)
i) (2) is another possible requirement, suggested by the following signs: (3)
ii) (4) is another possible requirement, represented by (5) markers
1- clonal BM cells >10%
2- presence of organ/tissue impairment
3- hypercalcemia, renal insufficiency, anemia, bone lytic lesions
4- presence of malignancy via biomarker 5: >60% clonal plasma cells in BM, OR >1 focal lesion on MRI, OR involved:uninvolved serum free light chain >100
(1) is a sign of Mutiple Myeloma seen in urine, produced from (2)
1- M protein
2- IgG, IgA
describe the key feature of multiple myeloma on bone marrow biopsy
- multiple nuclei
- many vacuoles
- dec cytoplasm in comparison
describe the electrophoresis pattern of Igs seen in multiple myeloma
i) gain of one Ig
ii) gain of one light chain (λ, κ)
iii) loss of all other Igs
iv) loss of the other light chain (λ, κ)
Multiple Myeloma:
- (1) is the predictor of prognosis
- if untreated death result in (2)
- (3) is the most common treatment with a median survival rate of (4)
- younger patient may be candidates for (5), to yield a better prognosis
1- cytogentic abnormalities 2- 1 yr 3- Alkylating Agent chemotherapy (60% remission) 4- 4-7 yrs 5- (<50 y/o) bone marrow transplant
- 90% of NHL affect (1) cells
- only 10% affect (2) cells
1- B cells
2- T cells (and NK cells)
list some examples of T-cell lymphomas
(10% of all NHLs)
- peripheral T cell lymphoma
- anaplastic large cell lymphoma
- angioimmunoblastic T cell lymphoma
- extranodal T/NK cell lymphoma, nasal type
- subcutaneous panniculitis-like T cell lymphoma
- enteropathy associated T cell lymphoma
- hepatosplenic T cell lymphoma
Hodgkin’s Lymphoma:
- (small/large) tumors involving (2) cells which are considered (3)
- these cells are located in (4) and surrounded by (5)
1- large 2- Reed-Sternberg cells (RS cells) 3- altered germinal center B lymphocytes 4- germinal centers 5- numerous non-neoplastic inflammatory cells (lymphocytes, plasma cells, eosinophils)
Hodgkin’s Lymphoma:
- (1) are mainly involved with a (2) character and possibly pain associated with (3)
- systemic features, aka (4), include (5)
- (6) is the main cutaneous presentation
1- lymph nodes 2- painless rubbery enlargement 3- alcohol use 4- B-symptoms 5- fever, night sweats, weight loss 6- cuntaneous anergy = itching
Note- many other unusual presentations
describe the types of HL, according to phenotype / WHO
Classical (more common):
- CD15+. CD30+
- CD45-
Variant (less common):
- CD20+, CD45+
- CD15-, CD30-
list the types and subtypes on HL
Classical:
- nodular sclerosis, 65%
- mixed cellularity, 25%
- lymphocyte rich, very uncommon
- lymphcyte depleted, <5%
Variant:
-lymphocyte predominant, 5%
(1) is the most common type of HL, mainly affecting (2- age, sex) populations. It commonly involves (3) area of the body and is at Stage (4) at presentation.
1- Nodular Sclerosis (classical type)
2- young adults / adolescents, M=F
3- mediastinum +/- neck nodes
4- I, II
HL morphology:
- (small/large) nodules surrounded by (2)
- (3) type cells are most critical, called (4)
1- large nodules
2- thick fibrous collagen bands
3- Reed-Sternberg cells
4- lacunar RS cells
briefly describe mixed cellularity HL
(classical type)
-diffuse nodal effacement, frequent RS cells, often EBV+
-systemic Sxs and advanced stage presentation
briefly describe lymphocyte depleted HL
(classical type)
-abundant RS cells, very often EBV+, few lymphocytes and fibrosis in background
-elderly, HIV+, systemic sxs, advanced stage IV at diagnosis
briefly describe lymphocyte rich HL
(classical type)
-has nodular growth pattern, RS cell present (possibly mononuclear)
-lymphocytes, histiocytes (rarely eosinophils, neutrophils) in background
briefly describe lymphocyte predominant HL
(variant type)
-large nodules (few no collagen fibrosis), few RS cells
- age <35, low stage at presentation, good prognosis but tends to recur
- CD20+, CD45+, CD15-, CD30-
describe the (Ann Arbor) staging of HL
I- single lymph node (or single extra-lymphatic site/organ)
II- >2 LNs in region, same side of diaphragm (mediastinum)
III- LNs on both sides of diaphragm (possibly spleen)
IV- multiple / disseminated involvement of 1 or more extra-lymphatic organs / tissues
HL Tx and prognosis (what Tx, what is the biggest indicator of prognosis)
Tx: radiation +/- muti-agent chemotherapy (depends on stage)
Prognosis: depends on Stage
- 90% survival stage I/II
- 50% survival stage III/IV
- long-term survivors are prone to secondary cancers
HL:
- tumor cells are in (majority/minority)
- (2) describe spread / dissemination
- extranodal involvement (un-/common)
- most involvement of (4)
1- minority
2- node group –> spleen –> liver –> BM
3- uncommon
4- single axial node group
NHL:
- tumor cells are in (majority/minority)
- (2) describe spread / dissemination
- (3) extranodal involvement?
- most involvement of (4)
1- majority
2- no predictable pattern
3- common, >40%
4- multiple peripheral nodes
