2.2 highlights Flashcards
Myeloproliferative disorders:
1) Caused by acquired clonal abnormalities of the ____________ stem cell
2) Qualitative & quantitative changes can be seen in _____ cell lines
3) All myeloproliferative disorders may progress to what?
1) hematopoietic
2) all
3) Acute myeloid leukemia (AML)
1) Define Myelodysplastic
2) Define Myeloproliferative
1) Bone marrow underproduces one or more types of healthy mature blood cells (RBCs, WBCs, & platelets)
2) Bone marrow overproduces one or more types of blood cells (RBCs, WBCs, & platelets)
Myelodysplastic syndromes (MDS): A group of acquired clonal disorders of the hematopoietic _____ cell
stem
MDS (Myelodysplastic syndromes): AML has ≥____% blasts
20%
MDS (Myelodysplastic syndromes)
Key distinction is an increase in bone marrow blasts (>5% of marrow elements but <20%), representing a more aggressive form & often leading to AML
MDS (Myelodysplastic syndromes): Syndromes _____________ excess blasts are characterized by the degree of dysplasia
without
MDS (Myelodysplastic syndromes):
1) Usually presents with at least one of what 3 things?
2) Possible ____________ on exam
1) Fatigue (anemia), infection (neutropenia), or bleeding (thrombocytopenia)
2) splenomegaly
MDS: Atypical clinical manifestations in a patient with cancer should prompt consideration of a ________________ syndrome
paraneoplastic
MDS:
1) Anemia can be significant with ___________ or increased MCV (transfusion may be required).
2) WBC usually normal or ___________, & neutro_______ is common.
3) What WBC abnormality is possible?
1) normal
2) reduced; neutropenia
3) Pelger-Huët abnormality
What is the only curative therapy for MDS?
Allogeneic stem cell transplantation
List 4 factors that can help determine MDS Tx
1) Asymptomatic low-risk MDS
2) Anemia
3) Primarily severe neutropenia
4) Thrombocytopenia
You should refer all patients with suspected (or diagnosed) myelodysplasia to ______________
hematologist
MDS key points:
1) Cytopenias with ___________ bone marrow
2) Morphologic abnormalities in one or more ____________ cell lines
3) <____% blasts in blood and bone marrow
4) What age demographic?
5) What are the 2 main presentations?
1) hypercellular
2) hematopoietic
3) <20%
4) Older patients (70)
5) Asymptomatic OR fatigue, infection, and/or bleeding
List 3 types of acute leukemia
1) Acute myeloid leukemia (AML)
2) Acute promyelocytic leukemia (APL)
3) Acute lymphoblastic leukemia (ALL)
Classification of _____________ is according to cell type and lineage
leukemias
What is the key difference between AML and ALL?
1) AML: most common in older pts
2) ALL: most common in children
Acute leukemia: ~50% patients have symptoms ____ months before diagnosis
< 3 months
List some key Sx of acute leukemia
Weight loss, sweats, fever or infection, bone pain
Acute leukemia: Most dramatic presentation is headache, confusion, & dyspnea caused by __________________
hyperleukocytosis
Acute leukemia with hyperleukocytosis: requires emergent _______________ with adjunctive ______________
chemotherapy; leukapheresis
Acute leukemia: Possible gingival hypertrophy/stomatitis & rectal fissures in acute ____________ leukemia
monocytic (AML)
Acute leukemia:
1) Hallmark is combination of _____________ (low RBCs, high or low WBCs, & low PLTs) with circulating ___________ (peripheral blood)
2) Usually >_____% blasts in bone marrow
3) __________ (eosinophilic needle-like inclusion in cytoplasm) is strongly associated with AML
1) pancytopenia; blasts
2) >20%
3) Auer rod
Acute leukemia:
1) Refer all patients to a _____________
2) Admit _______ ____________for treatment
1) hematologist
2) all patients
How should you Tx most pts with AML or ALL?
Combo chemotherapy
Key points of acute leukemia:
1) Which is more common in older patients? What abt younger?
1) How long do the Sx last? What do these include?
1) AML: older patients (70)
ALL: young children (3-7)
2) Short duration of symptoms (fatigue, fever, infection, bleeding)
Key points of acute leukemia:
1) What will you see on a CBC?
2) What will you see in bone marrow?
3) What is found on a periph. smear with AML?
1) Pancytopenia with circulating blasts
2) >20% blasts
3) Auer rods
Chronic myeloid leukemia hallmark is ____________________ present (bcr/abl gene)
Philadelphia chromosome
List the 3 phases of CML
1) Chronic/Early CML
2) Accelerated
3) Acute blast
What is a complication of CML?
Leukocytosis [with blurred vision, respiratory distress, altered mental status, or priapism (hyperleukocytosis + decreased tissue perfusion)]
CML labs:
1) What is a main finding in the chronic phase?
2) What is the hallmark that allows for definitive Dx?
1) Elevated WBC count (like 150K)
2) Bcr/abl gene in peripheral blood & bone marrow with PCR (definitive diagnosis)
1) Define Left shifted myeloid series
2) What condition may this occur in?
1) More immature circulating myeloid cells: bands, metamyelocytes, myelocytes, promyelocytes, and blasts
2) CML
CML: The ___________________ chromosome is an abnormal chromosome that is made when pieces of chromosomes ___ and ______ break off and trade places.
Philadelphia (Ph); 9 and 22
CML Philadelphia (Ph) chromosome:
1) The ABL gene from chromosome 9 joins to the BCR gene on chromosome 22 to form the _____________ gene.
2) This fusion gene leads to production of an _________ with constitutive _____________ activity that stimulates hematopoietic transformation and myeloproliferation.
1) BCR::ABL fusion
2) oncoprotein; tyrosine kinase activity
CML chronic phase:
1) Name a Tyrosine kinase inhibitor (TKI) used
2) Is this the TOC (treatment of choice)?
3) What should you use for inadequate response to TOC or disease progression despite therapy?
1) Imatinib, nilotinib, dasatinib
2) Yes; developed as a targeted therapy
3) Stem cell transplant
CML: Refer ________ patients to a hematologist
all
CML key points:
1) What is the common age?
2) What is elevated?
3) What is a unique characteristic?
4) What are the phases?
1) Middle age (~ 55)
2) Elevated WBC count
3) Left-shifted myeloid series (but still ≤5% blasts in chronic phase
4) Chronic, accelerated, & acute blast phases
CML key points:
1) What is the hallmark?
2) What are 2 treatments?
3) How can you remember what demographic it’s common in?
1) Bcr/abl gene (Philadelphia chromosome) present (definitive diagnosis)
2) TKI, stem cell transplant
3) Chronic “Middle-aged” Leukemia
CLL is a clonal malignancy of morphologically mature but immune-incompetent __________________
B lymphocytes
What is one of the only leukemias not linked to radiation exposure?
CLL
CLL: Some present with fatigue & lymphadenopathy; is this lymphadenopathy painful?
Lymphadenopathy
1) What is the hallmark lab finding for CLL?
2) What is used to determine immunophenotype of circulating lymphocytes? What is the immunophenotype?
1) Isolated lymphocytosis
2) Flow cytometry (peripheral blood); coexpression of CD19, CD5
CLL:
1) What is the Tx for early stage disease?
2) Give examples of indications for Tx
1) Observation
2) Fatigue, symptomatic lymphadenopathy, anemia, or thrombocytopenia
True or false: Refer all CLL patients to a hematologist, but rarely need to admit them
True
CLL key points:
1) What is the main finding?
2) What shows shows coexpression of CD19, CD5 on lymphocytes?
3) Is it caused by exposures?
1) Isolated lymphocytosis (mature B lymphocytes)
2) Flow cytometry
3) Not caused by exposures (except for maybe Agent Orange)
CLL key points:
1) Is it genetic?
2) What are the 2 main ways it can present?
3) What is the CLL mnemonic to remember common facts?
1) Strong familial association
2) Asymptomatic OR fatigue, painless lymphadenopathy, or hepatosplenomegaly
3) “Chronic Long-Life, Lots of Long-Lived Lymphocytes Leukemia”
HL (Hodgkin’s lymphoma):
1) Infection with _________ is a risk factor
2) What age group is it common in?
1) HIV
2) Bimodal distribution (peak incidences in 20s & 80s)
HL (Hodgkin’s lymphoma): Most present with _________ & _____________ lymphadenopathy (most common in neck, supraclavicular, & axilla)
painless & non-tender
HL (Hodgkin’s lymphoma):
1) What is a Sx seen in 10-15% that’s usually generalized?
2) What is a weird. unique, less common Sx?
1) Pruritis
2) Pain following alcohol ingestion
Reed-Sternberg cells on lymph node biopsy are indicative of what type of lymphoma?
Hodgkin lymphoma
What is the first line drug regimen for Hodgkin lymphoma?
Chemotherapy (ABVD/ doxorubicin [Adriamycin], bleomycin, vinblastine, dacarbazine)
(know these drugs)
Which type of cancer has the following defining events?:
C- calcium
R – renal
A – anemia
B – bone
(CRAB)
Myeloma
Hodgkin Lymphoma Key points:
1) What is the age group?
2) What is a risk factor?
3) What are the common Sx?
1) Bimodal (20s & 80s)
2) HIV is risk factor
3) Often painless, non-tender, & palpable lymphadenopathy
± “B symptoms”
Hodgkin lymphomas Key points:
1) Where does it typically start?
2) What skin Sx may they have?
3) What is an uncommon but specific Sx?
4) How is it diganosed?
1) Typically starts in one LN area then spreads
2) May have pruritus
3) Uncommon but specific symptom: pain in affected bone or LN after alcohol ingestion
4) Pathologic diagnosis with lymph node biopsy (Reed-Sternberg cells)
Non-Hodgkin lymphomas (NHL):
1) ~____% are B-cell in origin
2) Distinguished from HL by the absence of _________________ cells
1) 90%
2) Reed-Sternberg
Organ transplant recipients are at risk for what type of cancer?
Non-Hodgkin lymphomas (NHL)
True or false: NHL is far more common than HL
True
NHL S/Sx:
1) Usually present with _______ lymphadenopathy (peripheral or central)
2) _________ lymphomas usually ____________ at diagnosis with frequent bone marrow involvement (not considered curable)
1) painless
2) Indolent; disseminated
NHL: __________________ is required for diagnosis & classification (lymph node or involved extra-nodal tissue)
Tissue biopsy
NHL: Most patients with what kind are non-curable?
Indolent
NHL key points:
1) _____% are B-cell in origin.
2) What age demographic?
3) What cases are not considered curable? Explain
4) What does it often present with?
1) 90%
2) All age groups
3) Most indolent are disseminated at diagnosis (not considered curable)
4) Painless lymphadenopathy
NHL key points:
1) Where may it possibly occur?
2) How do you make a Dx?
3) How do you differentiate it from HL?
1) Possible extra-nodal sites
2) Diagnosis made by tissue biopsy
3) No Reed-Sternberg cells
Monoclonal immunoglobulin (paraprotein) formation is a key characteristic of what?
Plasma cell myeloma (PCM)
Plasma cell myeloma (PCM) used to be called what?
Multiple myeloma
PCM labs:
1) __________ is nearly universal (usually normocytic)
2) Normal RBC morphology, but _________ formation is common (may be marked)
1) Anemia
2) Rouleax
PCM labs:
1) What may be found on serum and/or urine protein electrophoresis (SPEP/UPEP) (and/or IFE)?
2) Free light chain assay may show what?
3) What may be in BM or tissue biopsy (or both) (not usually visible in peripheral blood)?
1) Hallmark monoclonal immunoglobulin (paraprotein)
2) Excess monoclonal light chains
3) Clonal plasma cells
PCM
List 4 myeloma defining events (hint: there’s a mnemonic)
CRAB
HyperCalcemia (Total calcium >11.0 mg/dL)
Renal injury (creatinine >2 mg/dL)
Anemia (Hgb <10 g/dL)
Bone disease (lytic lesions on x-ray, MRI, PET/CT)
What are most common in skull, spine, proximal long bones, & ribs with PCM?
Lytic lesions
Which type of myeloma may have ≥10% BM clonal plasma cells but no “myeloma-defining events”?
Smoldering MM
Which type of myeloma may have <10% BM clonal plasma cells?
MGUS
What findings would help you Dx PCM/MM (as opposed to smoldering MM or MGUS)?
(plasma cell myeloma)
≥10% clonal plasma cells in the bone marroworbiopsy-proven bony or soft tissue plasmacytoma
PLUS one of the following:
1) Any one of the “myeloma-defining events” (CRAB)
2) A biomarker associated with near inevitable progression to end-organ damage
PCM (plasma cell myeloma) Tx:
1) What Sx may hydration, glucocorticoids, bisphosphonates, and/or hemodialysis/calcitonin indicated for symptomatic patients help tx?
2) How do you Tx renal impairment?
3) What Sx may RBC transfusion, erythropoiesis-stimulating agents Tx?
1) Hypercalcemia
2) Directed at underlying cause
3) Anemia
PCM (plasma cell myeloma) Tx: Localized radiation therapy may palliate ___________ or eradicate tumor at site of pathologic fracture
bone pain
PCM (plasma cell myeloma) Key points:
1) What is the age demographic?
2) What is a typical Sx?
3) What is a more unique Sx?
4) What is the hallmark Sx seen on on Serum or urine protein electrophoresis (or IFE)?
1) Disease of older patients (69)
2) Bone pain (often in spine, hips, ribs, proximal long bones)
3) Rouleax formations
4) Hallmark monoclonal immunoglobulin (paraprotein)
PCM key points:
1) What may free light chain assay (serum or urine) show?
2) What may be seen in the bone marrow or in a tissue biopsy (or both)?
3) Organ damage due to plasma cells may occur; give examples.
4) What are most common in skull, spine, proximal long bones, & ribs?
1) Excess monoclonal light chains
2) Clonal plasma cells
3) HyperCalcemia, Renal, Anemia, Bones
4) Lytic lesions
Which hematologic cancer is common in?:
1) Any age
2) Childhood-predominant
3) Bimodal
4) Middle aged
5) Predominantly older adults (hint: 3 CAs)
1) NHL
2) ALL (ages 3-7)
3) Hodgkin lymphoma (20s & 80s)
4) CML (median age 55)
5)
AML: median age 67
CLL: median age 70
PCM: median age 69
1) Which hematologic cancer is most common in Hispanic male patients?
2) Which one is more common in Black male patients?
1) CML
2) PCM (plasma cell myeloma)
What is one of the most familial-associated malignancies?
CLL
What age demographic is CLL common in?
Disease of older patients
