Studied by 1 personWhat does ALL stand for?
acute lymphocytic leukemia
What does AML stand for?
acute myelogenous leukemia
What does CLL stand for?
chronić lymphocytic leukemia
What does CML stand for?
chronic myelogenous leukemia
What is lymphadenopathy?
abnormal enlargement of the lymph nodes, typically >1cm
What are bacterial ecologies for LAD?
TB, cat scratch fever
What are viral etiologies for viral LAD?
HIV, infectious mononucleosis
What are protozoan etiologies for LAD?
toxoplasmosis
What labs do you order for LAD?
CBC w/ diff
VDRL or RPR (r/o syphillis)
HIV
LFTs
Hepatitis panel
EBV (r/o mononucleosis)
ANA (r/o rheumatic disease)
TSH
CT neck/chest/abdomen/pelvis- ID adenopathy
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 need to be given before a splenectomy?
pneumococcal (pneumovax 23, Prevnar - 13), Hib, and meningococcal vaccinations
What do children s/p splenectomy require until adulthood?
prophylactic antibiotics: PCN VK
What is leukemia?
progressive, malignant disease of bone marrow (blood forming organ) marked by distorted proliferation and development of leukocytes and/or their precursors in the bone marrow and peripheral blood
What are the abnormal cells that multiply in leukemia called?
blasts
What are etiologies of leukemia?
unknown, ionizing radiation, toxins (benzene) chemotherapeutic agents (alkylating), viruses (EBV, HTLV-1)
incidence increases w/ age and FHx
How are leukemias classified?
cell type- myeloid or lymphoid
time of onset- acute or chronic
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 M/E ratio?
myeloid / erythroid ratio- compares number of myeloid cells (WBC precursors) to erythroid cells (RBC precursors)
What does a bone marrow aspiration and biopsy show you?
M/E ratio, cell differential, presence of abnormal cells, cellularity, bone marrow structure
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 de novo AML?
no pre-existing hematologic condition
what is therapy related AML?
develops bc of previous chemotherapy or radiation
worse prognosis
What is secondary AML?
arises form previous hematologic condition (MDS, PNH, MF, ET)
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
physical exam findings for AML
allow, petechiae/purpura, stomatitis, gingival hyperplasia, myeloid sarcoma, leukemia cutis, ± LAD, ± hepatosplenomegaly
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 prognosis of AML based on?
the chromosomal abnormalities that are driving the leukemia
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
What drugs are used to induction chemotherapy for AML?
anthracycline (daunorubicin) + cytarabine (Ara-C)
requires 1 month hospital stay
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 are complications seen w/ AML?
anorexia, weight loss, N/V, infection, tumor lysis syndrome, DIC (more common in APL)
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)
What is considered high risk APL?
WBC >10,000
What is considered low risk APL?
WBC <10,000
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
epidemiology of CML?
results form overproduction of myeloid cells
insidious onset, but can become acute if not identified
onset typically >65 y/o
What is the 5 year survival rate of CML?
90%
Etiology of CML
specific genetic abnormality- Philadelphia chromosome (Ph)- balance gene translocation b/ long arms of chromosome 9 and 22 [t(9;22)]
results in creation of BCR-ABL oncogene which results in uncontrolled proliferation
What is the clinical presentation of CML?
often asx- incidentally discovered on routine CBCs
fevers, fatigue, splenomegaly
What are the 3 phases of CML?
chronic phase, accelerated phase, blast crisis
what is the chronic phase of CML?
indolent disease, raised WBC w/ left shift
what is the accelerated phase of CML?
worsening WBC count, unresponsive to therapy, 10-19% blasts in BM, >20% basophils in blood, additional chromosomal abnormalities (“clonal evolution”)
What is the blast crisis phase of CML?
aggressive disease, BM blasts >20%, essentially conversion to AML
What is the goal of therapy in CML?
control disease in the chronic phase and prevent progression to blast crisis (AML)
Is CML curable?
no (except for allogeneic transplant) but it is highly controllable
What revolutionized treatment for CML?
the discovery of Philadelphia chromosome and the creation of BCR-ABL tyrosine kinase inhibitors
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
difference between leukemia and lymphoma
leukemia- when present in large numbers in blood/marrow, can affect organs; originates from blasts
lymphoma- localized in lymphoid tissue; originates from mature lymphocytes
Lymphoid neoplasms form from a malignant transformation of what cells?
B cells, T cells, and NK cells
What is the most common childhood malignancy?
acute lymphoblastic leukemia (ALL)
Are outcomes of ALL better in children or adults?
children
What are the two peak of incidences of ALL?
children under 5 and adults over 50
What are the acute onset sx of ALL?
fever, fatigue, inc infections, increased. bleeding/bruising, focal neurologic deficits/seizures due to CNS involvement
What are physical exam findings of ALL?
petechiae, pallor, splenomegaly, hepatomegaly, LAD
(due to crowding in bone marrow)
What would you see on a CBC in an ALL patient/
marked anemia, neutropenia, thrombocytopenia, blasts on differential, kidney injury
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
pediatric tx for ALL
intensive combination chemotherapy given sequentially over 2-3 years (female- 2 years, male- 3 years)
stem cell transplant reserved for relapsed/refractory disease
adult tx for ALL (and why is it different than pediatrics)
combination therapy (can’t tolerate the heavy tx kids receive)
stem cell transplant reserved for those w/ good performance status to be able to tolerate the treatment
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 leukemia is a neoplastic transformation of the mature B-lymphocyte?
CLL
What is the median age at diagnosis of CLL?
71 (disease of the elderly)
What is the clinical presentation of CLL?
fatigue, LAD, hepatomegaly, splenomegaly, or asx (discovered on incidental CBC)
What do you see on a CBC for CLL?
inc absolute lymphocyte count (ALC)
lymphocytosis >5,000
± anemia, thrombocytopenia
what is seen on a peripheral smear for CLL?
smudge cells- fragile B-lymphocytes break as a result of smear creation
What do you see in a BM bx for CLL?
predominance of mature lymphocytes
How is CLL staged?
Rai system (more common in US) and Binet system (more common in Europe)
utilizes LAD, splenomegaly, and cytopenias to determine prognosis
The prognosis of CLL depends on chromosomal abnormalities. What would result in a very poor prognosis?
deletion of 11 or 17p oncogene
What is the tx regimen for CLL?
“watch and wait” active surveillance; initiate tx only when disease causes sx
When a CLL patient is having sx, what is the tx?
chemo-immunotherapy and allogeneic stem cell transplant (reserved for aggressive disease in younger patients)
What are the chemo-immunotherapy drugs used for CLL tx?
fludarabine/chlorambucil/rituxan (FCR)- young fit patients
Ibrutinib- oral, targeted agent, improved prognosis of 17p deletion
What is 30% of all malignant lymphomas?
hodgkin lymphoma
What are the 2 primary subtypes of Hodgkin lymphoma?
classical and lymphocyte predominant
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 staging system does Hodgkin lymphoma use?
modified Ann Arbor
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 is the origin of 95% of non-hodgkin lymphoma (NHL)?
B-cell
What ages are the peak incidence of NHL?
20-40
What are the classifications of non-hodgkin lymphoma NHL?
indolent: “watch and wait” if no sx (CLL/SLL, follicular lymphoma)
intermediate (DLBCL)
aggressive (burkitt lymphoma, ALL)
what is the clinical presentation of NHL?
painless enlargement of lymph nodes
B symptoms (fever, night sweats, wt loss)
advanced- SVC syndrome, airway obstruction, cord compression, gastric outlet obstruction
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