2nd Exam: Hematopathology Flashcards
Pain after drinking alcohol, think:
Hodgkin Disease
Auer rods, think this:
AML
Translocation t(8;14), think:
Burkitt’s Lymphoma
Lymphadenopathy may be due to:
reactive ( define) or neoplastic conditions
Ex’s of reactive conditions leading to lymphadenopathy:
infections, ai, malignant (non-lymphoid, lymphoid–lymphoma)
Infections that can lead to lymphadenopathy:
infectious mono, cat scratch disease, bacterial lymphadenitis, tubeculous lymphadenitits
Ai disease that can lead to lymphadenopathy:
RA
Ex’s of malignancies that can lead to lymphadenopathy:
non-lymphoid, lymphoid-lymphoma
Ex of non-lymphoid malignancy that can lead to lymphadenopathy:
metastatic carcinoma
T-cell zone of normal lymph node:
Parafollicular cortex
B-cell zone of normal lymph node:
Lymphoid follicles
Symptoms of lymphadenopathies depends on:
Node location, central vs. peripheral
Palpable nodes:
peripheral
Central nodes:
sup vena cava syndrome, obstruction of urethra or bowels
Cancer that begins in lymphoid cells of immune system
lymphoma
Lymphoma typically starts here:
lymph nodes (enlarged) or extra nodal lymphoid tissue (masses)
2 basic types of lymphoma:
hodgkin’s, non-h
Types of non-Hodgkin’s lymphomas:
indolent, aggressive
Define effacement:
normal structure replaced by tumor cells
2 types of leukemia:
lymphoid, myeloid
Malignant progressive, bone marrow produces inc # of immature or abnormal wbcs:
Leukemia
Effects of BM infiltration:
pain, weakness, shortness of breath, fatigue, CHF, neutropenia, anemia, thrombocytopenia, inc infection , bleeding, petechiae (bleeding into skin)
Clinical presentation of Hematologic Malignancies:
malaise, fatigue, w8 loss, fever, organ/ bm infiltration, lymphadenopathy, acute or chronic
Low grade hematologic malignancy:
mature appearing cells, low rate of proliferation, chronic, longer life expectancy untreated
High grade hematologic malignancy:
immature/ primitive cells, high mitotic rate, rapidly progressive, onset w/in wks, fast death (couple years) wo tx, may cure w aggressive therapy
TF? High grade hematologic malignancy is immunologically primitive.
F. (Difference between immunologically primitive and primitive)
Most common acute leukemia in children:
ALL
Classification is in regards to:
lineage, molecular chara, shape, immunophenotye, Cx syndromes, stage of differentiation
TF? lymphomas and lymphoid leukemias are classified into 1 classification system.
T
primitive marker:
CD34
5 lymphoid lineages:
precursor neoplasm, mature t-cell and b-cell neoplasm, Hodgkin’s lymphoma, post-transplant lymphoproliferative disorders
4 categories of neoplastic proliferations of WBc’s:
lymphoid lineage, histiocytic and dentdritic cell neoplasms (rare), hematopoeitic/ myeloid disorders, acute leuk’s of ambiguous origin
Types of precursor lymphoid neoplasms:
b-/ t-cell lymphoblastic leukemia/lymphoma
Blasts are associated w acute/ chronic.
acute
Markers of T-cell lineage:
CD1-8
markers for B-cell lineage:
CD10+
Marker shared by both T and B-cell lineages:
CD34, primitive marker
myelodysplastic vs. myeloproliferative:
inneffective vs effective hematopoiesis
Myeloproliferative neoplasms such as CML, high or low grade?
low
Types of hematopoeitic/ myeloid disorders:
myeloroliferateive neoplasms, myelodysplastic syndromes, myelodysplastic/myeloproliferative neoplasms, AML and related precursor neoplasms
myelodysplastic/myeloproliferative neoplasms, ineffective or effectve hematopoeisis?
both? (check)
ex of myeloproliferative neoplasm:
CML
87% of lymphomas are:
non-Hodgkin’s
Almost 50% of leukemias are:
CLL (60+ men,)
There are more cases of AML than:
ALL + CML
Non-Hodgkin’s lymphomas can be broken into these 2 branches:
B cell and T cell neoplasms
B cell neoplasms:
CLL/ small lymphocytic lymphoma, Diffuse large cell lymphoma, Burkitt’s lymphoma
Ex of T cell neoplasm:
mycosis fungoides, cutaneous lymphoid, mushroom like appearance
CD for T lymphs:
3, 4, 5, 8
CD for B lymphs and surface Ig:
19, 20
CD for most granulocytes:
13
CD for granulocytes, R-S cells:
15
CD for stem cells, pleuripotent:
34
CD for all wbc’s:
45
neoplastic cell in Hodgkins:
Reed Sternberg cells
Do low grade lymphomas divide?
no
Burkitt’s lymphoma:
Kids, endemic in Africa, extranodal, begins in man or max, esp in Africa, involves colon, adrenal, kidney, ovary, aggressive, high grade, poor prognosis, tx w chemo
Tx for Burkitt’s lymphoma:
chemo
TF? Burkitt’s lymphoma is a primitive B cell neoplasm.
F. not primitive, but it is a B cell neoplasm
Lymphoma belt:
wet, hot, malaria, infected with EBV, immunocompromised
most highly proliferative human tumor:
Burkitt’s lymphoma
30% of all childhood lymphomas in US:
Burkitt’s
Area affected in most US cases of Burkitt’s lymphoma:
abdomen
Burkitt’s lymphoma, intra or extranodal?
extranodal
TF? Burkitt’s lymphoma never begins in the lymph nodes.
F. rarely
Pathology of Burkitt’s lymphoma:
B cell origin (not primitive, CD 19, 20), small-intermediate size lymphos, monotonous pop, round nuclei, prominent nucleoli, high mitotic rate, starry sky patter w macs, apoptosis of tumor cells
pale area of starry sky pattern of Burkitt’s Lymphoma slide:
benign macrophages
Causes of Burkitt’s lymphoma:
Latent EBV infection, translocation, t(8;14) (ebv related to mono, too) How does malaria play a role?)
Cases of Burkitt’s Lymphoma are more commonoly assoc w latent EBV infection in U.S. or Africa?
Africa
Translocation resulting in Burkitt’s lymphoma involves:
c-myc (8q24) and IgH gene (14q32), overexpression of c-myc
c-myc:
regulates proliferation (of what, the B cells?), oncogene
What is translocated in Burkitt’s lymphoma?
c-myc from q arm of #8 + p and rest of q from #14
Genes effected in translocation that leads to Burkitt’s lymphoma:
H chain gene (antibody H chains?, H chain enhancer?) and c-myc
Most lymphomas and leukemias are assoc w:
molecular/ chromosomal abnormalities
2 moa for lymphomas and leukemia:
activate oncogenes, lose tsg’s
How are oncogenes activiated or tsg’s lost, leading to lymphomas and leukemias?
variety of mechs
Etiologic and pathogenic factors in leukemias and lymphomas:
molecular abnormalities, translocations, oncogenes, inherited genes, viruses, infection, chronic antigenic stimulation, immune dysfunction, environmental agents, chemo, radiation
Virus that makes lymphs divide more frequently:
EBV
Cx man of Hodgkin’s Disease:
Pel-Ebstein fever, night sweats, pruritis, painless cervical and mediastinal lymphadenopathy, except after alcohol
Pel-Ebstein fever:
cyclically fever, usually 1-2wks
pruritis:
itchy skin wo rash
Lymphadenopathy:
abnormal number, size or consistency
early presentation of Hodgkins:
neck lymphadenopathy
Spread of Hodgkin’s:
to adjacent ln groups, orderly, begins in 1 ln, splenic involvement late in development, progression makes staging imp in prognosis, because it has to spread to the spleen via adjacent tissues
High grade Hodgkin’s:
includes BM and liver
Types of Hodgkin’s
nodular sclerosis and mixed cellular types
Nodular sclerosis type Hodgkin’s:
75%, usually young women, cervical, mediastinal nodes, dx in Stage I-II usually, reactive, eosinophilic lymphos, lacunar cells, rarely R-S cells, birefringent bands of collagen dividing node into nodules
Mixed cellular type Hodgkin’s
25%, men over 50, dx in Stage III-IV usually, many R-S cells and lymphocytes, eosinophils, inflammatory cells in background
Lymphadenopathy and splenomegaly w multiple nodules is assoc w:
Hodgkin’s, splenomegaly is also assoc with AML
Malignant cell of Hodgkin’s
Reed-Stenberg cell
R-S cell is more abundant in this type of Hodgkin’s:
mixed cellular type
TF? R-S cells are always diagnostic of Hodgkin’s.
F. in the right env
Genome found in some R-S cells:
EBV, esp mixed cellular type
Cell origin of R-S cells:
B cells, but does not express typical B cell markers, CD15 (granulocyte marker) and CD30 (activation marker), used to calsify lymphoma as Hodgkins
Cell origin of Hodgkin’s cells:
B cells
granulocyte marker:
CD15
activation marker:
CD30
Subtype of R-S cell:
Lacunar cells, nodular sclerosis
How to differentiate bw nodular sclerosis type and mixed cellular type:
lacunar cells: nodular sclerosis, mixed: more R-S cells, EBV found in R-S cells
Hodgkin’s cells:
binucleated, bilobated, mirror image, prominent nucleoli, owl-like appearance
B-cell that does not mark w typical B cell markers, think:
Hodgkin’s
Large, clear cells in distinct space, think:
nodular sclerosis type Hodgkin’s
Major difference between Hodgkin’s disease and non-Hodgkin’s Lymphoma:
Hodgkin’s does not skip to distant nodes
System used to stage Hodgkin’s Disease:
Ann Arbor System
Stage I Hodgkin’s:
one ln or one ln group, 100% 5y survival
Stage II Hodgkin’s:
2+ ln, same side of diaphragm, 87% 5y survival
Stage III Hodgkin’s:
lns on both sides of diaphragm, 71% 5y survival
Stage IV Hodgkin’s:
disseminated, non-nodal organ involvement, 45% 5y survival
Stage I or II Hodgkins recurrence rate after 20 years:
less than 20%
Oral mani of lymphomas:
involve palate, tonsils, Waldeyers ring (circle of lymphoid tissue in back of throat)
neck nodes are indicative of what tumor type?
all types
Oral lymphomas are usually of __ cell origin.
B
Oral lymphomas:
older pts, usually secondary lymphoma, complication of chemo (low platelets, WBC count), infections likely (ie herpes zoster) the
Complication of chemotherapy tx for oral lymphomas:
low platelets, WBC count, ulcers, fungal infections, herpes
Oral lymphomas, 1’ or 2’?
either, usually 2’
Pt w lymphoma is more likely to get this infection:
herpes zoster, oral manifestation
Pharyngeal tonsil is aka:
adenoid tonsil
Interrupted circle of protective lymphoid tissue:
Waldeyer’s Ring
Location of Pharyngeal tonsils:
upper midline in nasopharynx
Location of tubal tonsils:
near opening of auditory tube
Location of palatine tonsils:
sides of oropharynx
Location of lingual tonsils:
under mucosa of pos 3rd of tongue
Tonsil that are part of Waldeyer’s Ring:
Pharyngeal, tubal, palatine, lingual
Block in WBC differentiation/ maturation:
Acute leukemia
Many immature WBCs (CD34+, primitive) in BM can lead to:
suppression of normal hematopoeisis, anemia, thrombocytopenia
Acute leukemia can spread to:
liver, spleen, ln, various organs inc. meningeal infilatration
Meningeal infiltration is seen in:
Acute leukemia, children
BM aspiration is done here:
posterior superior iliac crest, through cortical bone, into marrow space bw spongy bone
Normal BM is 50%:
fat
Leukemic BM is:
hypercellular, much less fat, diffusely infiltrated, full of blasts
Heterogenous group of acute/ aggressive myeloid malignancies:
AML
AML:
wide age range, uncommon in kids, onset w/in wks-months, fatigue, fever, skin palor, swollen, bleeding gums (hypertropy, neoplastic cell infilatration), petechiae, hepatosplenomegaly
splenomegaly is assoc with:
Hodgkin’s and AML, CLL
% remission from AML w chemo:
60%, only 15% disease free after 5y
Pathologic findings of AML w granulocytic maturation:
inc WBC in peripheral blood, many blasts, auer rods, round nuclei and azurophilic granules, reduced platelets (under 100,00), more than 30% blasts in BM, normally 3%, positive myeloperoxidase staining (enzyme in primary granules)
Enzyme found in primary granules of AML w granulocytic maturation:
myeloperoxidase
of blasts normally in peripheral blood:
none
Most common leukemia in US:
CLL
CLL:
men 60+, often no symptoms at dx (leukemic cells in peripheral blood), often involve lymph nodes (lymphoma), indolent/ painless course
Pathology of CLL:
inc WBC in peripheral blood, 5,000+ abnormal (small, smooshed, broken) lymphos in peripheral blood that look normal, smudge cells indicate increased cell fragility, inc lymphos in BM, aggregates or clusters, mark with CD 19 and 20 (B cells) and CD 5 (T cell marker)
How to determine type of leukemia:
CD 5, a T cell marker
BM of CLL pt:
hypercellular, no fat, almost all lymphos
When to tx CLL:
lymphadenopathy, hepatosplenomegaly, anemia, low platelets
Px of CLL:
good: 10-15 surival
TF? There is no lymphadenopathy with Burkitt’s lymphoma:
F. right? since it CAN start in lymph nods but usually does not (check)
