WBC 2 Flashcards
Precursor or primitive B and T cell neoplasms are called what
Acute lymphoblastic leukemia (ALLs)
-B-ALL and T-ALL
Most ALLs are what type?
manifest as what?
B-ALLs
-acute childhood leukemias
T-ALLs tend to present in who?
as what?
Adolescent males
-Thymic “lymphomas”
What is the most common cancer of children
ALL
Epidemiology of ALLs
- Most younger than 15 . . .peak at age 3
- 3x as common in whites than blacks
- slightly more in boys
- Hispanics have highest frequency
How should follicles normally be in the cortex?
nicely arranged
What are the 3 broad types of lymphomas
- NHL
- HL
- plasma cell neoplasia
2/3 of NHL and virtually all Hodgkin lymphomas present as what?
other 1/3 of NHL?
enlarged non-tender lymph nodes (often >2cm)
-symptoms related to involvement of or in extranodal sites (skin, stomach, brain)
General clinical presentations: Lymphocytic leukemias?
Plasma cell neoplasia - multiple myeloma?
Hodgkin Disease?
- Bone Marrow suppression
- Lytic lesions of bone/secretion of light chains or entire immunoglobulins
- Fever
- M3
- Aggressive course
- DIC
Acute Promyelocytic Leukemia (APL)
2 year old comes in lethargic and fever . . thinking maybe infections. . you run a CBC with platelet count and it is 10,000 (low)
- it is Not infection; doesn’t suppress platelet count
- Child has Acute lymphocytic leukemia until proven otherwise
Chronic leukemia and platelet counts
do NOT tend to be low
in HL and NHL . . is platelet count at presentation clinically useful
usually not
What tumors tend to produce cyclic fevers
B cell
What are the neoplasms of immature B and T cells
B ALL and T ALL
What are the neoplasms of MATURE B cells
- Burkitt lymphoma
- DLBCL
- Extranodal Margin Zone Lymphoma
- Follicular Lymphoma
- hairy Cell leukemia
- Mantle Cell lymphoma
- Multiple myeloma/solitary plasmacytoma
- SLL/CLL
What are the Neoplasms of MATURE T or NK cells
- Adult T cell leukemia/lymphoma
- peripheral T cell lymphoma, unspecified
- Anaplastic Large cell lymphoma
- Extra-nodal NK/T cell lymphoma
- Mycosis fungoides/Sezary syndrome
- Large/granular Lymphocytic leukemia (NK cell lineage)
What are the Classic subtypes of Hodgkin disease?
What is the one Non classic subtype?
- nodular sclerosis
- mixed cellularity
- lymphocyte-rich
- lymphocyte-depleted
Lymphocyte predominant
Reactive and hyperplastic cells in the background of Hodgkin disease that aren’t neoplastic but elaborate all kinds of cytokines leading to fever
Reed-Sternburg cells
The most common site of primary extranodal lymphoma is where?
Almost all of these are what?
what is next most frequent site?
GI tract
NHL
Skin
When a NHL occurs only in skin, it is called what?
cutaneous lymphoma
What is a common tissue manifestation of ALL
testicle in boys
age and presentation of B ALL
- children
- Pancytopenia
- Aggressive
Age and presentation of T ALL
- Adolescent males
- thymic masses
- aggressive
Big cells
alot of cytoplasm
alot of nuclei
- Peripheral T cell lymphoma
- B cells don’t have alot of cytoplasm or alot of nuclei
- very little cytoplasm . . High N:C ratio
- chromatin nicely dispursed
- nucleoli and no granules
- These are blasts in peripheral blood
- Don’t know if its B or T cell
- if it’s a 3 year old then more than likely B ALL
Approximately 90% of ALLs have numerical or structural chromosomal changes . . most common being what?
hyperdiploidy
Pre B markers
- TdT
- CD10
- CD19
- CD22
Onset of ALL
Abrupt stormy onset within days to a few weeks of first symptoms
symptoms of ALL related to what?
depression of marrow function
- fatigue due to anemia
- fever reflecting infections secondary to neutropenia
- bleeding due to thrombocytopenia
Mass effects of ALL
- Bone pain from marrow expansion and infiltration of subperiosteum
- Generalized lymphadenopathy, splenomegaly, and hepatomegaly
- Testicular enlargement
- in T ALL complications related to compression of large vessels and airways in mediastinum
CNS manifestations of ALL
headache, vomiting, nerve palsies resulting from meningeal spread . . all more common in ALL in contrast to AML
Favorable prognostic factors for ALL?
- Age 2-10
- low WBC count
- Hyperdiploidy
- Trisomies 4, 7, and 10
- t(12;21)
Unfavorable prognostic factors for ALL
- under age 2
- in adolescent or adult
- peripheral blasts >100,000
- t(9;22) “philadelphia chromosome” . . often BCR-ABL fusion
Auer rods
AML
Peripheral B cell neoplasma are typically what age?
What are the 2 that sometimes are found in children?
Adults
- Burkitt lymphoma
- Marginal Zone Lymphoma
CLL and SLL differ only in degree of what?
degree of peripheral blood lymphocytosis
What is the diagnostic absolute lymphocyte count requirement for CLL
> 5000 per mm^3
What is the most common leukemia of adults in the western world?
CLL
median age at diagnosis of CLL?
gender?
- 60
- 2:1 male predominance
explain difference b/t CLL and SLL
- CLL has very high PB WC count . . may be higher than 20,000
- SLL just tends to have lymphadenopathy so you need to biopsy lymph nodes
- Hepatosplenomegaly
- Pancytopenia (50%)
- infections
- “dry tap”
Hairy cell leukemia
describe the typical lymph node appearance of CLL
architecture is obliterated and monomorphic . .can’t delineate anything
CLL is often called a pre leukemia. . most adults with it will die of something else because we can treat effectively and they aren’t aggressive . . what will most die from?
infection
If all you have was lymph node involvement. No lymphocytosis, no big white cell counts peripherally and you looked for immunophenotype and it comes up like CLL . . what do you call this?
SLL
Surface markers for CLL/SLL?
Hairy cell leukemia?
- IgM or IgM and IgD
- IgG
CLL/SLL Ig spike
A small monoclonal Ig “spike is present in blood of some patients
describe how CLL/SLL disrupts normal immune mechanism
- HYPOGAMMAGLOBULINEMIA is common and contributes to an increased susceptibility to infections particularly bacterial
- 10-15% of patients develop hemolytic anemia or thrombocytopenia due to AUTOANTIBODIES made by nonneoplastic B cells
If we see nucleoli in a lymphocyte, it is called what
Prolymphocyte
Smudge cells and scattered sperocytes
CLL
Proliferations centers . . pathognomonic for what?
CLL/SLL
sperocytes in CLL are caused by what?
making antibodies to RBC and causing autoimmune hemolytic anemia
Very aggressive phase that some CLL patients go into . .often an ALL like manifestations as in they will start producing blasts in peripheral blood or they can go into a diffuse large B cell lymphoma . . much more lymphadenopathy and organomegaly
Richter syndrome
What sites are almost always involved with the leukemic infiltrated of CLL
- Bone Marrow
- Splenic white and red pulp and hepatic portal tracts
prognosis with CLL transformation to diffuse large B cell lymphoma . . Richter Syndrome
less than 1 year
What is the most common for of INDOLENT NHL in the US?
Follicular Lymphoma
Epidemiology for follicular Lymphoma
- M=F
- Less common in Europe
- Rare in asians
2 principal cell types in follicular lymphoma
- Centrocytes
- Centroblasts
Describe the lymph node in follicular lymphoma
Obliteration of architecture . . follicles filling up entire compartment of lymph node
immunoperoxidase and tagged with antiBCL2 shoes follicle filled with cells tagged with BCL2
follicular lymphoma
PB in follicular lymphoma?
Bone marrow?
Spleen?
- lymphocytosis (less than 20,000) in about 10%
- paratrebecular lymphoid aggregates in 85%
- Splenic white pulp and hepatic portal triads
Clinical features of follicular lymphoma
- painless, generalized lymphadenopathy
- although incurable, is usually follows an indolent waxing and waning course
median survival for follicular lymphoma?
Does it improve by aggressive therapy?
7 to 9 years
-No
What is the usual approach to treating a symptomatic follicular lymphoma patient?
low dose chemo or anti-CD20 antibody
Follicular lymphoma and transformation?
- most go to DLBCL
- some go to tumors resembling Burkitt lymphoma emerge associated with translocations involving MYC
What are the special subtypes of DLBCL and what viruses?
- Immunodeficiency-associated Large B cell lymphoma (EBV)
- Primary Effusion Lymphoma (KSHV/HHV-8, in ALL cases)
What is the most common for of NHL?
DLBCL
Clinical presentation of DLBCL
- Aggressive
- rapidly fatal without treatment
- Usually a rapidly enlarging mass that can arise virtually anywhere
- WALDEYER ring involved frequently (posterior Pharynx)
- Primary of secondary involvement of the liver and spleen may result in large destructive masses
- Extranodal sites are common and BM involvement Uncommon in contrast to follicular lymphoma
Categories of Burkitt lymphoma? and what each involve?
- African (endemic): EBV and face or mandible
- Sporadic (nonendemic): viscera
- HIV (aggressive): viscera
Starry night morphology
Burkitt
- Low power wouldn’t see lymphoid follicles or germinal centers . .just diffuse infiltrate of cells
- Higher power: lots of mitosis and alot of nucleoli and some look like macrophages . . don’t have tingible bodies
- lacunar cells
- these cells stain for fat in cytoplasma
Burkitt lymphoma
Epidemiology of Burkitt
-mainly children or young adults
where do most Burkitt manifest
extranodal sites
Endemic: kidneys, ovaries, adrenal glands especially
SPoradic: ileocecum and peritoneum
Warburg effect . . fastest growing human tumor
Burkitt lymphoma
nature of Burkitt?
Aggressive
Burkitt response to intensive chemo
responds well
- most children and young adults can be cured
- outcome more guarded in older adults
