Onc: BOLDED ONLY (ty brenna)

What is lymphadenopathy?

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