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What is lymphadenopathy?
abnormal enlargement of the lymph nodes, typically >1cm
what are patients s/p splenectomy at an increased risk of?
sepsis caused by encapsulated bacteria such as s. pneumoniae, h. influenzae, and n. meningitides
What do children s/p splenectomy require until adulthood?
prophylactic antibiotics: PCN VK
What are the abnormal cells that multiply in leukemia called?
blasts
What is the hallmark of leukemia?
pancytopenia or leukocytosis w/ circulating blasts
(abnormal counts w/ circulating blasts)
What abnormal labs would require an emergent hematology/oncology consult?
presence of blasts in a peripheral blood differential or smear
What is required for definitive diagnosis of leukemia?
bone marrow aspiration and biopsy
What is flow cytometry?
cells are suspended in a fluid and flow one at a time through a specialized light → cells are tagged w/ markers (CD) for identification
can be performed on peripheral blood or BM aspirate to identify/categorize blasts/abnormal cells (its quick but you have to know what you’re looking for)
What is the most common acute leukemia in adults?
AML (more common >60 y/o)
How is AML classified?
risk stratification based on specific mutations - high, intermediate, low risk
(no stages for leukemia)
what is therapy related AML?
develops bc of previous chemotherapy or radiation
worse prognosis
clinical presentation of AML (will prob be on a vignette Q)
fatigue, SOB due to anemia, inc infection, inc brusing/bleeding (epistaxis, gingival bleeding, menorrhagia), fever (tumor fever vs infection), night sweats, bone and joint pain
what would you find on a peripheral smear in AML?
auer rods- rod shaped structures in cytoplasm
What would you see on a CBC in an AML patient?
anemia, neutropenia, thrombocytopenia, blasts on differential
In a bone marrow biopsy, what percentage of myeloblasts in considered AML?
over 20%
What is the tx for AML that is considered good risk?
chemotherapy alone
What is the tx for AML that is considered poor risk?
chemotherapy followed by allogenic stem cell transplant
what is induction chemotherapy?
initial tx for AML that should be initiated immediately after dx. goes is to de-bunk disease and induce remission
consolidation chemotherapy AML
given after remission is achieved, goal is to consolidate any microscopic remaining disease
HiDAC- high-dose cytarabine x 4 cycles 1 x month
allogeneic stem cell transplant
tx given to intermediate / high risk AML that replaces damaged/diseased bone marrow with healthy donor’s
(risk of prolonged immunosuprresion & GVHD)
what is seen w/ tumor lysis syndrome?
hyperuricemia, hyperkalemia, hyperphosphatemia, elevated Cr, or hypocalcemia
What medical emergency is seen w/ AML?
APL- acute promyelocytic leukemia
maturation process stopped at promyelocytes, high rate of mortality due to hemorrhage (DIC)
How do you tx APL?
ATRA- all-trans retinoid acid (vitamin a); start therapy ASAP
once confirmed give them arsenic
What do you need to watch out for when treating APL w/ ATRA?
differentiation syndrome and hyperleukocytosis
Etiology of CML
specific genetic abnormality- Philadelphia chromosome (Ph)- balance gene translocation b/ long arms of chromosome 9 and 22 [t(9;22)]
What is the clinical presentation of CML?
often asx- incidentally discovered on routine CBCs
fevers, fatigue, splenomegaly
What is the blast crisis phase of CML?
aggressive disease, BM blasts >20%, essentially conversion to AML
Is CML curable?
no (except for allogeneic transplant) but it is highly controllable
What tyrosine kinse inhibitors (TKIs) are used for CML?
Imatinib (Gleevec)- first class
Dasatinib (Sprycel)- 2nd generation
What is a leukemoid reaction?
elevated WBC (like leukemia) but due to a prolonged, severe infection
WBC >50,000 w/ predominante of neutrophils and bands, but NO blasts or Philadelphia chromosome are present
What is the most common childhood malignancy?
acute lymphoblastic leukemia (ALL)
What are the acute onset sx of ALL?
fever, fatigue, inc infections, increased. bleeding/bruising, focal neurologic deficits/seizures due to CNS involvement
in a bone marrow biopsy on an ALL patient, what would you see a >20% increase in?
lymphoblasts
What do both adult and pediatric treatment regimens include in ALL?
CNS prophylaxis due to high prevalence of CNS disease
What is the most common leukemia overall?
CLL
What is the difference between CLL and SLL?
same disease but:
CLL- disease cells in peripheral circulation
SLL- disease cells form solid masses in lymph nodes
What is the clinical presentation of CLL?
fatigue, LAD, hepatomegaly, splenomegaly, or asx (discovered on incidental CBC)
what is seen on a peripheral smear for CLL?
smudge cells- fragile B-lymphocytes break as a result of smear creation
What is the tx regimen for CLL?
“watch and wait” active surveillance; initiate tx only when disease causes sx
What is the bimodal age distribution of Hodgkin lymphoma?
15-30 years and >50 years
What virus is associated w/ up to 40-50% of cases of Hodgkin lymphoma?
EBV (also known association w/ HIV)
What is the clinical presentation of Hodgkin lymphoma?
asymptomatic lump, B-symptoms, pruritus, rash, pain on drinking alcohol, LAD (usually cervical initially), leukocytosis/eosinophilia
What are the B-symptoms associated w/ Hodgkin lymphoma?
fever, night sweats, weight loss
generally correlates w/ slightly worse prognosis and decreased survival rate
Evaluation of Hodgkin lymphoma
excisional bx (core needle usually not and FNA definitely not sufficient for pathology)
CBC, ESR/LDH, PET or CT of neck, chest, abdomen, and pelvis, BM bx if advanced
pathology of Hodgkin lymphoma
reed-sternberg cells surrounded by healthy reactive cells; presentation in a single node initially, typically spreads in contiguous pattern
What are reed-sternberg cells?
large, bi nucleate pathologic cell w/ “owls eye” appearance; seen in Hodgkin lymphoma
what is the tx for hodgkin lymphoma?
chemo: ABVD X 6 cycles
± XRT (radiation therapy)
brentuximab vedotin monoclonal ab against CD30 (new drug)
autologous transplant for relapsed/refractory dz
(consider allogeneic or immunotherapy Nivolumab if transplant fails)
What is the risk with ABVD tx for hodgkin lymphoma? what is needed prior to starting tx?
cardio and pulmonary toxicity —> need ECHO and PFT’s prior to starting
What kind of biopsy is preferred for NHL?
excisional (but core is acceptable)
evaluation for NHL
lymph node bx (excisional preferred), PET or CT scan of neck, chest, abdomen, pelvis, BM bx, CBC, LDH, lumbar puncture (if sinus, testicular, orbital, epidural, or BM involvement)
what is follicular lymphoma (FL)?
indolent lymphoma, (20-30% of lymphoma cases), potential to transform into more aggressive, defined by translocation (14;18) genetics
What are indications for tx of follicular lymphoma?
symptomatic/bulky LAD, signification B symptoms, significant cytopenia, transformation to aggressive NHL
What is diffuse large B-cell lymphoma (DLBCL)?
intermediate aggressive lymphoma and the most common NHL (30%)
what is the goal of treatment for DLBCL?
CURE (50-60% cured after first line tx)
What is burkitt lymphoma?
very aggressive tumor of immature B cells; characteristic genetic abnormality t(8;14)
What is the pathognomic appearance of burkitt lymphoma?
starry sky- diffuse infiltration of small non-cleaved lymphocytes mixed w/ large cells
How does burkitt lymphoma present in Africa?
endemic form of children, presents as local tumor to jaw w/ metastases to kidney, ovaries, and CNS
strong EBV association
How does burkitt lymphoma present in western countries?
rapidly dividing tumor of abdominal LN;
no EBV, strong HIV association
what is the tx for burkitt lymphoma?
aggressive combo chemotherapy similar to leukemia (DA-R-EPOCH, CODOX-M-IVAC)
highly curable
what is lymphoplasmacytic lymphoma / waldenstrom’s macroglobulinemia ?
rare type of NHL that affects B cells when they are in the process of developing into plasma cells (lymp and plasma cells similar to MM)
has more of the large IgM in the blood
may have hyper viscosity of the blood
may have neuropathy or autoimmune hemolytic anemia
What is multiple myeloma?
malignant disorder of plasma cells
more common in older adults and African Americans
What is responsible for symptoms of multiple myeloma?
malignant plasma cels that over produce antibodies (M protein)
what is the clinical presentation of multiple myeloma?
sx of anemia are the most common presentaiton
bone pain, pathologic fractures, pallor, fatigue, ARF, infections (bc of inability to make normal abs), and hyperviscosity syndrome
What is hyperviscosity syndrome?
seen in multiple myeloma due extreme levels of antibodies creating “smudging” of the blood
sx include mucosa bleeding, vertigo, nausea, visual disturbances, AMS
evaluation of multiple myeloma
CBC- anemia, thrombocytopenia
CMP- hypercalcemia, elevated sCr
proteinuria
SPEP- elevated M-spike
UPEP- detects bence Jone proteins
skeletal survey- lytic lesions + “moth-eaten appearance” (PET preferred now)
BM bx- plasma cell infiltration of normal marrow
What would you see on a UPEP (urine protein electrophoresis) in multiple myeloma?
bence-jones proteins
What is crab criteria?
diagnostic for multiple myeloma:
Calcium elevation: (>11mg/dL)
Renal insufficiency: Cr >2mg/dL or CrCl <40 mL/min
Anemia: Hgb <10 g/dL
Bone damage: lytic lesions
What is rouleux formation?
RBC’s stack together in long chains and increases ESR
occurs when plasma proteins are high, particularly fibrinogen and globulins
(can be seen in MM)
What is the goal of tx for multiple myeloma?
prolong remission, increase quality of life
incurable
what are tx options for MM?
“triple-therapy” chemo-immunotherapy, autologous stem cell in first remission, XRT for pain control, BiTE therapy (bispecific abx) and supportive care
What is MGUS?
monoclonal gammopathy of undetermined significance
premalignant state of myeloma that is asymptomatic (detected by routine blood work), <10% BM plasma cells w/o end organ damage
common in adults over 70
what is tx for MGUS
active surveillance
What are leukemia and lymphoma survivors at an increased risk of?
secondary malignancies, osteonecrosis, Fe overload, chronic infection, chronic fatigue, depression, GVHD
When managing managing a patient who is a survivor of leukemia /lymphoma, what do you do when if any preemptive screenings are positive?
promo referral to appropriate specialist and notify the patient’s treating oncologist
Why do you get labs when a pt has LAD?
to r/o infxn
Which form of malignant splenomegaly is the largest?
myelofibrosis
Why do we give pts with AML prophylactic abx?
to prevent and manage infection
What is seen in tumor lysis syndrome?
uric acid elevation
what causes spinal cord compression?
cancers that metastasize to spinal column causing injury from edema, hemorrhage, and pressure induced ischemia
Where does spinal cord compression most commonly arise?
thoracic spine
what is often a late sign of spinal cord compression?
bowel and bladder dysfunction that can progress to incontinence
when dx spinal cord compression, what should you do if you are ever in doubt?
image the entire spine (MRI/Xray/bone scan) - don’t want to miss a tumor
what is the tx for spinal cord compression?
STAT corticosteroids to reduce edema in spinal cord (IV dexamethasone) and consult oncology / surgery
what is a life threatening sx of malignant pericardial effusions?
hypotension → may indicate cardiac tamponade
what is the most common paraneoplastic endocrine syndrome?
hypercalcemia
what causes the sx assoc. w/ hypercalcemia?
ionized/free calcium
Why would the total serum calcium concentrations in pt’s w/ low or high serum albumin not accurately reflect the calcium concentration?
Ca in serum is bound to proteins, usually albumin (if albumin is low, free calcium would be higher, etc)
need to calculate the corrected serum ca
what would a corrected serum ca >12 mg/dL indicate?
cardiac arrhythmias, which may lead to sudden death (possible short QT interval)
What cancers does tumor lysis syndrome (TLS) occur in?
most often during chemo in lymphoma and leukemias
can occur w/ other tumor types that have high proliferative rate (acute lmphyoblastic leukemia and burkitt lymphoma) or high sensitivity to chemo
what causes TLS?
massive relate of cellular material into systemic circulation (nucleic acids/uric acids, proteins, phosphorus, K+)
What abruptly develops with the impaired metabolism/excretion of TLS?
hyperuricemia, hyperkalemia, and hyperphosphatemia (w/ secondary hypocalcemia)
What TLS tx is reserved for high risk scenarios?
rasburicase
what is a medical emergency in patients w/ neutropenia?
infection / fever
A pt presents to the ED w/ SIRS and recently received systemic anticancer therapy w/in the past 6 weeks. What should you always assume for the diagnosis until proven otherwise?
neutropenic sepsis syndrome
What is systemic inflammatory response syndrome (SIRS)?
2 or more of the following:
fever >38C or < 36C
HR > 90 BPM
RR > 20 or PaCO2 < 32mmHg
abnormal WBC count (>12,00, <4,00, or >10% immature/band forms)
What should fever >100.4 in a patient w/ neutropenia be considered until proven otherwise?
infection- can be life threatening
Tx for febrile neutropenia
empiric broad spectrum abc begun asap after blood culture obtained - w/in 1 hr of presentation
what is the leading cause of cancer death in men and women?
lung cancer
what are the two main groups of lung/bronchogenic cancer?
non small cell lung cancer (NSCLC) and small cell lung cancer (SCLC)
which major group is associated w/ more rapid growth and increased metastases?
SCLC