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179 Cards in this Set

  • Front
  • Back
what is "philia"
reactive increase in #
what is caused by neutrophilia
bacterial sepsis
what is caused by lymphocytosis
viral, immune
what is caused by eosinophilia
allergy/parasites
what is "penia"
decreased #
neutropenia, lymphocytopenia, eosinopenia, and pancytopenia are seen in what
drugs, viral infections, radiation, chemotherapy, etc.
what are the characteristics of B lymphocytes
- part of humoral immune sys. - live for yrs -5-15% lymphocytes
what roles do B cells play
-stimulate change to plasma cells - make specific antibodies (1st IgM then IgG)
What are the characterisitics of T lymphocytes
- triggered by antigens from macrophages - cell mediated immunity - 70% in peripheral B - selected in thymus - 2/3 helper; 1/3 cytotoxic
What is the role of helper t cells
-recognize antigen and self HLA on presentng cell
what is the role of the cytotoxic t cell
binds w/ anitgen and HLA of target cell
what are natural killer cells
- subsets of CD8's - kill by adhesion to target cell or antibody dependent cell mediated toxicity
what caused lymphocytes to increase
- infections - lymphatic leukemia CLL, ALL, NHL - physiological - thyrotoxicosis - hypopituitarism - some carcinomas - myasthenia
what causes lymphocytes to decrease
- inflammation - corticosteroids - immune deficiency - some cancers - CT diseases
what is a CD3
mature T cell
what is a CD4
helper t cell
what is a CD5
T and B subset
what is a CD8
killer t cell
what is a CD10
precursor b (seen in ALL)
what is avCD 14+
monocyte
what is a CD19
B cell
what is a CD34
stem cell
what is a CD56
natural killer cell
where did the name "hodgkin's" lymphoma come from
Thomas Hodgkin- English pathologist who described the disease in 1832
what are the essentials of dx of hodgkin's lymphoma
- B cell lymphoma - painless lymphadenopathy - constitutional symptoms may/may not be present - pathological dx by lymph node bx
what is the epidemiology of hodgkin's lymphoma
-biomodal age distribution= 1 peak age 15-40; 2nd peak >50-60 male/female - 20,000 cases annually in N.A./Europe - higher in higher in socioeconomic - lifetime risk 1/250-1/300 in N.A.
what is the A & P of hodgkin's lymphoma
-group of cancers characterized by Reed Sternberg cells in appropriate reactive cellular background - several subtypes: 1. lymphocyte predominance 2. nodular sclerosis 3. mixed cellularity 4. lymphocyte depletion
what are the important features of hodgkin's lymphoma
- tendency to arise w/in single lymph node and spread in orderly fashion to continuous lymph nodes - widespread hematogenous dissemination only late in course
what is the etiology of hodgkin's lymphoma
exact etiology remains unclear
what increases the risk of hodgkin's lymphoma
EBV increases 3 fold
what is the causiologse/pathophysiology of hodgkin's lymphoma
-cells are unable to manufacture intact antibodies - Hodgkin/Reed Sternberg cell avoids self destruction - no consistent translocation noted
what are the % of classic hodgkin's lymphoma
nodular sclerosis 60-80%; lymphocyte rich 5%; mixed cellularity 15-30%; lymphocyte depleted <1%
what is the predominant hodgkin's lymphoma
nodular lymphocyte 5%
What are the characteristics of nodular sclerosing HL
-most common subtype - composed of large tumor nodules w/ scattered lacuna of classical Reed-Sternberg cells in a background of reactive lymphocytes, eosinophils, and plasma cells w/ varying degrees of collagen fibrosis/sclerosis
what are the characteristics of mixed cellularity HL
- common subtype - composed of numerous classic Reed-Sternberg cells mixed w/ numerous inflammatory cells w/out sclerosis
what are the characteristics of lymphocyte rich or lymphocytic predominance HL
-rare subtype - most favorable prognosis
what are the characteristics of lymphocyte depleted HL
-rare subtype - large #'s of often pleomorphic Reed-Sternberg cells w/ only few reactive lymphocytes
what are the symptoms of HL
- majority asymptomatic - painless lymphadenopathy (mass): painless, firm, rubbery common in neck - const. symptoms- fever, weight loss, drenching night sweats 25-30% - Pel Ebstein fever - generalized puritis - pain in lymph node after alcohol ingestion is an unusual symptom
what is Pel Ebstein fever
-relapsing, high grade can reach 105-106 for 7-10 days - fever spikes abrupt in onset and resolution
what are the signs/physical exams signs of HL
-cervical adenopathy 80-90% - nontender lymphadenopathy in the neck, supraclavicular area, and axilla - mediastinal adenopathy >50% @ dx - T cell immune def. even in early stage - prone to infection: HZ/shingles in 1/4 pt; fungal/mycobacterial - immune defect may persist after lymphoma cured
what are the D dx of HL
-NHL - TB lymphadenitis (scrofula) - cat scratch - metastatic cancer - drug induced pseudolymphoma (phenytoin)
what is sclerosis
disease where abnormal collections of chronic infammatory calls (granulomas) form as nodules in mutliple granulomas (of non caseating type) most often appear in lungs/lymph nodes but any organ can be affected
what diagnostic studies are done for HL
-CBC - ESR - serum chemistry - lactate dehydrogenase level LDL - immunophenotyping CD30/CD15 - lymph node histopathology - chest radiography CXR PA and Lat - CT-neck thorax abdomen pelvis - PET - Gallium scan
what is seen in the lymph node histopathology of HL
most cells are normal lymphoid, plasma cells, monocytes, and eosinophils - Reed sternberg cells-pathognomonic finding
what are the decisions and dx of HL based on
staging
What are the stages of the Ann Arbor nomenclature
Stage I- 1 lymph node region involved Stage 2- 2 node areas on 1 side of diaphragm Stage 3- lymph regions involved on both sides of diaphragm Stage 4- disseminated w/ bone marrow or liver involvement
what are the "destinations" of HL
Stage A- lack of const. symptoms; Stage B- 10% weight loss over 6 mo., fever, night sweats (B symptoms) Stage X- bulky dz Stage E- extranodal dz
what is the acute Tx plan for HL
comb. chemo for most pt including all stage IIIB and IV stages
What are the drugs used for HL
MOPP-Mechlorethamine, Oncovin, Procarbazine, Prednisone
ABVD- Adriamycin, Bleomycin,Vinblastine, Dacarbazine
BEACOPP- Bleomycin, Etoposide, Doxorubicin (adriamycin), Cyclophosphamide, Vincristine (onocovin), Procarbazine, Prednisone
What is the chronic Tx plan for HL
-if relapse after radiotherapy= salvage chemo - comb. chemo/radiotherapy for bulky mediastinal masses - chem now tested for earlier stages of dz
who has excellent prognosis in HL
IA IIA dz treated by radiotherapy 10 yr survival rates >80%
who has a 5 year survival rate for HL
disseminated dz- IIIB IV 5 yr survival rates of 50-60%
who has poor prognosis for HL
pt who are older, have bulky dz, lymphocyte depletion HL, or mixed cellularity HL
what is the prognosis for pt w/ recurrent dz after initial radiotherapy
may still be cureable w/ chemotherapy
what does a high dose chemo w/ autologous stem cell transplantation for relapse offer
35-50% chance of cure if dz is still chemo sensitive
what are the complications of HL
- secondary malignancies (leukemia 1st 10 yrs/ solid tumors over time) - leukemia - lung/breast cancer - hypothyroidism -constrictive pericarditis - infertility - heart failure
when is leukemia a complication for HL
if pt receives alkalating agents/ comb. chemo/XRT
when is lung/breast cancer a complication for HL
if pt receives XRT to chest, especially high in smokers
when is hypothyroidism a complication for HL
after irradiation of meck
when is constrictive pericarditis a complication for HL
after radiotherapy to mediastinum
when is infertility a complication for HL
after using akylating agents
when is heart failure a complication for HL
after adriamycin Tx
What are the essentials of Dx for NHL
-fever, anemia, infections - marked lymphadenopathy - no Reed sternberg sells or eosinophilia - large group different lymphomas
what is the clinical aspect of NHL lymphomas
low, intermediate, high grade
what are the cell types of NHL
B, T, histocyte, other
what is the histology found in NHL
follicular and diffuse
What is the A & P of NHL
-solid tumors of immune system
what is the important feature of NHL
tendency to arise w/in single lymph node area and spread in orderly fashion to contiguous lymph nodes - widespread hematologous dissemination only late in course
what is the epidemiology of NHL
- increased incidence/mortality in 1990's - since 1970- incidence doubled - common, 4th rank heme cancer - 63000 cases -US 2007/19000 deaths
what is the lifetime risk of develpoment of NHL
1/46 men 1/56 women more freq. in elderly 3:1 men : women
what ages are present in NHL
any age; median 55-75
what is the etiology of NHL
of most NHL-unknown
who is at higher rish of NHL
ppl: exposed to chemicals, pesticides, solvents; EPV, family history of NHL, HIV
is there any hereditary pattern of NHL
no
what do the types of NHL reflect
development of stages of lymphocytes - each type lymphoma arrested at certain stage of dev. & transformed into a malignant cell
what % of NHL originate from B cells or T null cells
85% B 15% T null
What are the characteristics of indolent low grade NHL
life expectancy in yrs if untreated - 85-90% present in Stage III or IV -incurable
what are the characteristics of aggressive high grade NHL
life expectancy in weeks if untreated - potentially cureable
what are the symptoms of NHL
lymphadenopathy - const. symptoms 20%- fever, night sweats, weight loss, B symptoms more common in high grade lymphomas
what are the signs/phy. exam signs of NHL
-cytopenias - hepatosplenomegaly - lymphadenopathy - hematogenous spread of dz (no predictable pattern) - classic lymphoma - extranodal primary - waldeyer's ring
what are the characteristics of lymphadenopathy in NHL
may fluctuate or spontaneously remit especially in low gade lymphomas
what are the characteristics of hematogenous spread of dz in NHl
no predictable pattern
what are the characteristics of classic lymphoma
arises in lymph node or bone marrow
what are the characteristics of extranodal primary in NHL
more common in high grade lymphoma
what are the characteristics of waldeyer's ring in NHL
frequent in GI lymphomas
what are the D dx of NHL
- hodgkin's dz - metastatic cancer - infectious mononucleosis - cat scratch - sarcoidosis - drug induced pseudolymphoma
what are the diagnostic studies for NHL
-chromosome changes - CBC, cehmistries, urinalysis - CT chest abdomen pelvis - bone marrow bx/aspirate - LP - PBS - cerebrospinal fluid cytology - serum LDH
what is a Lp done for in NHL
-AIDS lymphoma - t cell lymphoblastic lymphoma - high grade lymphoma w/ positive marrow
what will cerebrospinal fluid cytology show in NHL
malignant cells in some high grade lymphomas w/ meningeal involvement
what is serum LDH useful for in NHL
prognostic marker/ incorporated into risk stratification of Tx
what are teh imaging tests done in NHl
CT abdomen chest pelvis and chest x-ray- mediastinal mass in lymphoblastic lymphomas and others
what are the types of B cell lymphoma
-90% - percurser b cell lymphoblastic lymphoma - small lymphocytic lymphoma/ chronic lymphocytic leukemia marginal zone lymphomas- MALT - hairy cell leukemia - follicular lymphoma -matle cell lymphoma - diffuse large b cell lymphoma - burkitt's lymphoma
what are the marginal zone lymphomas
nodular, extranodal, splenic
what are the t cell lymphoma types
- anaplastic large cell lymphoma- peripheral t cel lymphoma - mycosis fungoides
what is decision making and dx based on for NHL
staging- same as HL
what is the acute Tx plan for NHL
-chemo w/ ackylating agents - fludarabine - rituximab - short course chemo - chemo w/ CHOP - rituximab + chop
what are teh alkylating agents used w/ chemo in NHL
- chlorambucil .6-1 mg/kg q 3 weeks - comb. chemo w/ cyclophosphamide, vincristine, and prednisone CVP
what may produce quivalent result w/ chemo + alkylating agents in some pt w/ NHL
fludarabine
what is rituximab and how is useful in NHL pt
-monoclonal antibody directed against b cell surface antigen CD 20 - effective as salvage therapy for relapsed low grade b cell lymphomas/ may improve outcome when added to initial chemo
what is short course chemo and how is it used in NHL
3 courses of cyclophospamides, doxorubicin (hydroxydaunomycin;adriamycin) , vincristin (oncovin), prednisone CHOP plus localize radiation for localized intermediate grade lymphomas
how should chemo with chop be used in NHL
6-8 cycles for more advanced intermediate grade lymphomas
how should rituximab + CHOP be used in NHL
for elder pt. w/ diffue large cell lymphomas
what is burkitt's lymphoma
- african variety jaw tumor strongly linked to EBV- US 50% EBV infection
how does burkitt's present
many present as abdominal mass
what is the burkitt's lymphoma growth pattern
most rapidly growing human tumor
what abnormalities are seen in burkitt's
chromosome abnormality: c myc oncogene linked to one of immunoglobulin genes
how is burkitt's treated
w/ multidrug regimen similar to pediatric leukemia/lymphoma regimens
what are mycosis fungoides
most common form of cutaneous t cell lymphoma
what does mycosis fungoides affect
typically skin may progress internally over time
is mycosis fungoides a fungal disease
NO!!
how was mycosis fungoide first described
1st described skin tumors of severe case as mushroom like appearance
how is mycosis fungoides treated
electron beam radiation, UV light, or topical alkylating agents
what is adult t cell leukemia-lymphoma associated w/
HTLV 1 infection
where is adult t cell leukemia-lymphoma common
carribean southeastern us
what are the signs/symptoms/phy. exam signs of adult t cell leukemia-lymphoma
hepatosplenomegaly, leukocytosis, lymphadenopathy, skin involvement,lytic lesions of bone, hypercalcemia
what might adult t cell leukemia-lymphoma respond to
AZT and interferon
what is adult t cell leukemia-lymphoma
an oncogenic RNA retrovirus referred to as T cell leukemia and lymphoma- a demyleinating dz
what is AIDS lymphoma
agressive lymphomas of b cell origin - burkitts, burkitt's like and large cell immunoblastic
what is treatment like for AIDS lymphoma
often limited by immune compromise of pt
what is the prognosis of AIDS lymphoma
improved w/ HAART therapy
what is malt lymphoma
mucosa-associated lymphoid tissue lymphoma- chronic infection of stomach by helicobacter pylori
where is MALT loaclaized
stomach, indolent course
what is the prognosis for MALT
can be cured inmany cases by antibiotics against H. pylori
what is ocular adnexal lymphoma OAL
lymphoma affecting tissues surrounding the eye that may arise after chronic inflammation
what is the Tx of OAL
-antibiotic therapy against chlamydia - 1 pt treated w/ doxycycline 100 mg bid 3 weeks had complete remission for more than a yr, another pt had minimal remission for more than 18 months
what is the therapeutic procedure if a pt w/ lymphoma that is not bulky and asymptomatic
no initial therapy, some have spontaneous remission (most indolent lymphomas are disseminated at dx)
what is the therapeutic procedure for special forms of lymphoma (burkitt's, lymphoblastic, mantle)
require individualized therapy
what is the therapeutic procedure for clincally aggressive low grade lymphomas
allogenic transplantation
what is the therapeutic procedure for pt w/ limited indolent dz
localized radiation therapy
what is the prognosis for pt w/ indolent lymphomas
median survival 6-8 years dz ultimately becomes refractory to chemo
what is the prognosis for pt w/ relapse after initial therapy
if lymphoma is still partiallyt sensitive to chemo autologous transplantation- 50% chance long term salvage
what is the international prognostic index
widely used to categorize pt w/ intermediate grade lymphoma into risk groups
what are dz related complications
- cytopenia secondary to bone marrow infiltration - bleeding secondary to thrombocytopenia, DIC - cardiac secondary to large pericardial effusion - respiratory secondary to pleural effusion - SVC syndrome secondary to vertebral metastases - neurologic secondary to primary CNS lymphoma - GI obstruction, perforation, bleeding in pt w/ GI lymphoma (or caused by chemo) - pain secondary to tumor invasion - leukocytosis (lymphocytosis) in leukemia phase of dz
what is superior vena cava syndrome SVCS or SVCO (obstruction)
result of direct obstriction of superior vena cava by malignancies such as compression of vessel wall
what are the signs/ symptoms of SVCS
shortness of breath most common, followed by face/arm swelling
what is multiple myeloma also know as
plasma cell myeloma or kahler's dz (plasma cell malignancy, b-cells)
How common is MM
less common than NHL
how deadly is MM
more deadly that NHL
what is the avg life expectancy of ppl w/ MM
30-36 mo.; some pt develop indolent form and live for 10+ yrs
Is MM cureable
potentially w/ high dose chemo (bone marrow or stem cell transplantation)
what is MM
dz of malignant b lymphocytes
what are the patterns of signs/symptoms of MM
variable approx. 20% pt have no signs/symptoms
what is the etiology of MM
unknown
what are the suggested predisposing factors of MM
viral infection w/ HHV 8, MGUS (monoclonal gammopathy of undetermined significance)
what is a paraprotein
immunoglobulin or immunoglobulin light chain produced in excess by clonal proliferation of plasma cells often associated w/ benign MGUS
what is the avg age at presentation of MM
65
who is affected more by MM
males more than females blacks twice as much as whites
what is the % of 5 yr survival rate of MM
25-30% median survival 30-36 mo.
what are the clinical features of MM
- bone marrow failure: anemia, thrombocytopenia, neutropenia - renal failure - bone dz w/ skeletal destructucion: lytic lesions, gen. decrease in bone density - hypercalcemia - hyperviscosity syndrome - recurrent infections - amyloidosis
what are the labs done for MM
RBC/morphology, neutrophil/plt counts, calcium/proteins, erythrocyte sedimentation rate, PBS, serum protein electrophoresis, immunoelectrophoresis, urine IEP, B microglobulin level, urinalysis, bone marrow bx, bone xray, radionucleotide bone scaan
what is the pattern of anemia in MM
almost universal
what is seen in the RBC morphology of MM
normal, but rouleau formation common and may be marked
what is seen in the neutrophil and plt counts in MM
normal at presentation
what is the pattern of calcium and protein in MM
hypercalcemia and bence jones proteins (monoclonal globulin protein)
what is seen in the erythrocyte sedimentation rate of MM
elevated
what is seen in the PBS of MM
plasma cells rarely visible (plasma cell leukemia)
what is seen in the serum protein electrophoresis of MM
it demonstrates paraprotein in the majority demonstrateable as a monoclonal spike in B or y globulin region
what is seen in the immunoelectrophoresis of MM
monoclonal protein- 60% IgG 25% IgA 15% light chains only
what is seen in the urine IEP of MM
either complete immunoglobulin or light chains in 15% who have no demonstratable paraprotein in serum
what is seen in the B-microglobuling level
>3mg/L associated w/ poor survival
what is seen in the urinalysis in MM
may reveal proteinuria but dipstick test unreliable for light chains
what is the dipstick test used for
detects primarily albumin
what is the pattern of proximal renal tubular acidosis in MM
is seen w/ phosphaturia, glucosuria, uricosuria, and aminoaciduria
what is seen in the bone marrow bx in MM
infiltration by variable #'s of plasma cells (5% to 100%); dismal outcome if bone marrow cytogentic analysis shows deletions of chromosome 13q
what are the imaging tests done for MM
bone x rays, radionucleotide bone scan
what is seen in the bone xray of MM
lyric lesions especially i axial skeleton (skull, spine, proximal long bones and ribs) or generalized osteoporosis
what is the acute Tx plan for MM
-comb chemo w/ vincristine, doxorubicin (adriamycin) and dexamethosone (VAD) - Thalidomide and derivatives, experimental agents CC5013/PS341 (protease inhibitor) - thalidomide, berexomib (PS341, velcade), experimental agent CC5013 (revimid) - mobilization, hydration, biophosphonates - biophosphonates
what are thalidomide/derivatives and experimental agents CC5013/PS341 (protease inhibitor) used for MM
refractory dz
what is thalidomide, berexomib and experimental agent CC5013 (revimid) used for in MM
refractory dz
what is mobilization hydration and biophosphates used for in MM
hypercalcemia
what are the biophosphates used in MM
pamidronate 90 mg, zoledronate 4 mg IV Q month)
what are the biophosphates used for in MM
to reduce pathological fractures in pt w/ significant bony dz aredia/zometa
what is the chronic Tx plan for MM
observe w/out therapy, autologous stem cell transplantation, allogenic transplantation, less toxic forms of allogenic trasplantation, follow height of paraprotein spike on SPEP
when would you observe w/out therapy in chronic MM
if minimal dz or unclear whether paraproteinemia benign (MGUS) or malignant, since no advantage to early Tx of asymptomatic MM
wheb would you use analogous stem cell transplantation in MM
in pt <70, in relapse if dz still chemo sensitive
why would you use allogenic transplantation in MM
it is potentially cureative, but role limited by unusually high mortality rate (40-50%) in myeloma pt.
what are the less toxic forms of allogenic transplantation in MM
use nonmyeloablative regimens have produced encouraging results
why would you follow height of paraprotein spike on SPEP in MM
useful marker for monitoring response to therapy
what are the prognoses of MM
poor: >65, high tumor mass, high beta microglobulin, renal failure, hypercalcemia