2.2 Hematologic oncology Flashcards
1
Q
Cancer is the:
1) Cause of 1 in _____ deaths in the U.S.
2) _____ leading cause of death behind heart disease
A
1) 1 in 4
2) 2nd
2
Q
Overall lifetime risk of developing cancer:
1) In men
2) In women
A
1) Men: 40.5%
2) Women: 38.9%
3
Q
1) Which is more common in men, lymphoma or leukemia?
2) Which is more common in women, lymphoma or leukemia?
A
1) Lymphoma
2) Lymphoma (by a lot)
4
Q
1) Which is more common in men, deaths from lymphoma or leukemia?
2) Which is more common in women, deaths from lymphoma or leukemia?
A
1) Leukemia
2) Leukemia (only slightly higher)
5
Q
Myeloproliferative disorders:
1) What causes them?
2) Qualitative & quantitative changes can be seen which cell lines?
3) Are they well defined?
4) Are they grouped together? Why or why not?
5) All myeloproliferative disorders may progress to what?
A
1) Acquired clonal abnormalities of the hematopoietic stem cell
2) Qualitative & quantitative changes
3) Classically produce characteristic syndromes with well-defined clinical & laboratory features
4) Grouped together as they may evolve from one into another (hybrid disorders are common)
5) All myeloproliferative disorders may progress to acute myeloid leukemia (AML)
6
Q
List 4 myeloproliferative disorders
A
1) CML
2) Polycythemia vera
3) Myelodysplastic syndromes
4) AML & related neoplasms
7
Q
1) Define myelodysplastic
2) Define myeloproliferative
A
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)
8
Q
True or false: MDS encompasses several heterogenous syndromes
A
True
9
Q
Myelodysplastic syndromes (MDS):
1) Define these
2) Describe them
A
1) A group of acquired clonal disorders of the hematopoietic stem cell
2) Cytopenias; A usually hypercellular bone marrow; Morphologic dysplasia; Genetic abnormalities
10
Q
What are the 2 main types of MDS? Which is more common?
A
1) Usually idiopathic (primary)
2) Possibly cytotoxic chemotherapy, radiation, or both (secondary)
11
Q
Describe who MDS is most common in
A
1) Usual age >60 years (median age 70)
2) M > F
3) Rare in children
12
Q
S/Sx of MDS
1) ~___% asymptomatic (incidental finding)
2) Usually present with what?
3) May present as what?
4) Possibly associated with what?
5) What is possibly found on physical exam?
A
1) ~50%
2) Fatigue (anemia), infection (neutropenia), or bleeding (thrombocytopenia)
3) Wasting illness with fever, weight loss, and/or general debility
4) Paraneoplastic syndromes prior to or following diagnosis
5) Possible splenomegaly on exam
13
Q
MDS: ________________ syndromesrefer to the disorders that accompany benign or malignant tumors (as a result of substances produced by neoplastic cells altering physiology of various body systems) but are not directly related to mass effects or invasion.
A
Paraneoplastic
14
Q
MDS labs:
1) When can anemia be significant? What may be seen?
2) What will the WBCs be like?
A
1) With normal or increased MCV (transfusion may be required)
-Macro-ovalocytes may be seen
2) Usually normal or reduced & neutropenia is common
-Possible abnormal morphology of neutrophils (decreased granules/nuclear segmentation, include bilobed nucleus [Pelger-Huët abnormality]) & left shift of myeloid series (promyelocytes or blasts)
15
Q
MDS labs:
1) What is the platelet count? What is a weird characteristic the platelets may have?
2) Describe the bone marrow
A
1) Count normal or reduced; may be hypo-granular
2) Bone marrow is typically hypercellular
Erythroid hyperplasia is common, with abnormal erythropoiesis
16
Q
MDS: _______________ evaluation can help distinguish from other cytopenias (number of blasts)
A
Bone marrow
17
Q
List 5 DDxs for MDS
A
1) Megaloblastic anemia
2) Aplastic anemia
3) Myelofibrosis
4) HIV-associated cytopenias
5) Acute or chronic drug effect
18
Q
True or false: MDS Tx depends on multiple factors
A
True
19
Q
List the Txs when each of the following is present with MDS:
1) Asymptomatic low-risk MDS
2) Anemia
3) Primarily severe neutropenia
4) Thrombocytopenia
A
1) Expectant monitoring (e.g., treat infections, transfusions for critical cytopenias, health maintenance, etc.)
2) RBC transfusions OR Erythropoiesis-stimulating agents
3) Myeloid growth factors (e.g., filgrastim)
4) Oral thrombopoietin analogues (e.g., romiplostim, eltrombopag) OR Platelet transfusions
20
Q
List the Txs when each of the following is present with MDS:
1) Occasional patients
2) Non-responders & high-risk MDS
A
1) Immunosuppressive therapy (Anti-Thymocyte Globulin [ATG])
2) Various chemotherapy & immunomodulating agents
21
Q
True or false: MDS patients may benefit from clinical trials
A
True
22
Q
Course and progress of MDS:
1) What is the ultimate outcome?
2) What are the 2 most common causes of death?
3) Describe the survival rates of different forms
A
1) An ultimately fatal disease
2) Infections or bleeding
3) Some forms have favorable 5-year survival rates >90%
Other forms with < 2-year survival without allogeneic stem cell transplantation
23
Q
When should you admit a pt with MDS?
A
Only for specific complications (ie, severe infection)
24
Q
What is the main difference between acute & chronic leukemias?
A
1) Acute leukemias have a quick onset and quickly deteriorate without treatment.
2) Chronic leukemias have a slow, insidious onset and worsen over time.
25
1) Define acute leukemia
2) What occurs during this disease?
1) Malignancy of hematopoietic progenitor cell (myeloblasts & lymphoblasts)
2) Abnormal cells proliferate & replace bone marrow
26
What are the classifications of acute leukemia? Describe each
1) Acute myeloid leukemia (AML): myeloblastic subtype (6 WHO classifications/groups with 22 different sub-types)
2) Acute promyelocytic leukemia (APL)
3) Acute lymphoblastic leukemia (ALL): lymphoblastic subtype
4) Mixed phenotype acute leukemias
27
Acute leukemia Etiology:
1) What is the most common etiology?
2) What may be implicated?
3) What etiology is there no evidence for?
1) Mostly idiopathic (established etiology is rare)
2) Genetic predisposition, radiation, chemical/other occupational exposures, & drugs (ie, chemo)
3) Viral etiology
28
1) Who is AML usually found in?
2) Who is ALL usually found in?
1) Most common acute leukemia in older patients (median age of 67 at diagnosis)
-Causes 62% leukemic deaths
2) Most frequent neoplastic disease in children; peak incidence at ages 3-7
-Comprises 80% of acute leukemias in childhood
-Comprises ~20% of adult acute leukemias
29
Describe the Sx of acute leukemia
1) ~50% patients have symptoms <3 months before diagnosis
2) Gradual or abrupt onset (most ill for few days or weeks)
3) Fatigue frequently 1st symptom (anemia)
4) Anorexia, weight loss, bleeding, & easy bruising common
5) Fever + infection initial symptom in ~10% patients (cellulitis, pneumonia, peri-rectal, fungal)
6) Bone pain, lymphadenopathy, nonspecific cough, headache, or diaphoresis may occur
30
1) What is the most dramatic presentation of acute leukemia?
2) What is this due to?
3) What does it require?
1) HA, confusion, & dyspnea caused by hyperleukocytosis
2) Markedly elevated circulating blast count (total WBC > 100,000/mcL)
3) Emergent chemotherapy with adjunctive leukapheresis*
31
List the signs of acute leukemia
1) Fever, infection, & bleeding often found at time of diagnosis
2) Pale appearance, purpura, petechiae
3) Possible gingival hypertrophy/stomatitis & rectal fissures (in acute monocytic leukemia – a subtype of AML seen more commonly in children)
32
What are 3 less common signs of acute leukemia?
Splenomegaly, hepatomegaly, & lymphadenopathy
33
Acute leukemia:
1) What is the hallmark lab finding?
2) What % blasts in bone marrow?
3) What inclusion is strongly associated with AML?
1) Hallmark is combination of pancytopenia (low RBCs, high or low WBCs, & low PLTs) with circulating blasts (peripheral blood)
2) Usually >20% blasts in bone marrow
3) Auer rod (eosinophilic needle-like inclusion in cytoplasm) strongly associated with AML
34
List 4 DDxs for acute leukemia
1) Other myeloproliferative disorders
2) CML
3) Myelodysplastic syndromes
4) Left-shifted bone marrow recovering from previous toxic insult
35
Acute leukemia:
1) Most pts up to age ____ treated with intent to cure.
2) What is the first step of Tx? Explain what this means
1) 60
2) Obtain complete remission: Normal peripheral blood (resolution of cytopenias), normal bone marrow (no excess blasts), & normal clinical status
36
Acute leukemia:
1) Type of initial chemo depends on what?
2) What should you do for all patients?
3) What patients should you admit?
1) Type of initial chemo depends on subtype
2) Refer all patients to a hematologist
3) Admit all patients for treatment
37
Differentiate the Txs of AML and ALL (incl. post-remission)
1) AML: Most pts: combo chemotherapy
-Post-remission therapy with curative intent (continued chemo & stem cell transplant)
2) ALL: Adults & children: combo chemotherapy
-Post-remission: additional cycles of chemo or high-dose chemo & stem cell transplant
38
Differentiate the prognoses of AML and ALL
1) **AML:** ~70-80% adults < age 60 have complete remission & 50% cure rate with post-remission therapy
Up to 50% remission for adults > age 60 & only ~10-20% cure rate with post-remission therapy
2) **ALL: Younger pts:** 98% remission for children/adolescents, 60-80% cure rate for pts < age 39
**Age >39:** Remission in 90% but only 20-40% cure rate
39
Chronic myeloid leukemia:
1) Define it
2) What is the hallmark?
1) Overproduction of myeloid cells
2) Philadelphia chromosome (bcr/abl gene)
40
Chronic myeloid leukemia: What are the 3 phases? Describe each
1) Chronic/Early CML (does not behave like a malignant disease)
2) Accelerated (occurs if early CML untreated)
3) Acute blast (morphologically indistinguishable from acute leukemia)
41
1) Give the etiology for CML
2) What age is it most common in?
1) Mostly idiopathic; radiation, toxins, & chemo agents
2) Disease of middle age (median age 55 at presentation)
42
Give the S/Sx of CML
1) Some are asymptomatic (incidental finding)
2) Splenomegaly (often marked), sternal tenderness
>May have abdominal fullness related to splenomegaly
3) Rare syndrome of leukostasis with blurred vision, respiratory distress, altered mental status, or priapism (hyperleukocytosis + decreased tissue perfusion)
4) Accelerated phase often associated with fever (in absence of infection), bone pain, & splenomegaly
5) Usual presentation: fatigue, night sweats, & low-grade fevers
43
What is the usual presentation of CML? (3 Sx)
fatigue, night sweats, & low-grade fevers
44
CML: List the lab findings in the chronic phase
1) Elevated WBC count1 (median 150,000/mcL)
2) Left shifted myeloid series
3) Marrow and peripheral blasts usually ≤5% in chronic phase3
4) Usually not anemic
5) Platelets normal or elevated (thrombocytosis)
45
CML labs:
1) What 3 things are found in the accelerated & acute blast phases?
2) What abt just the acute blast phase?
3) What should be biopsied? Why?
1) Progressive anemia, thrombocytopenia, & increased blasts
2) >20% blasts in bone marrow
3) BM biopsy; for karyotype & confirm phase of disease
46
What is the hallmark lab finding of CML?
Hallmark is bcr/abl gene in peripheral blood & bone marrow with PCR (definitive diagnosis)
47
Amount of _________ increases as CML progresses from chronic (≤5%), accelerated (≥15%), to acute blast phase (>20%)
blasts
48
List DDxs for CML
1) Reactive leukocytosis associated with infection (WBC <50k) (differentiate from “early CML”)
2) Other myeloproliferative disease
49
1) What is the Philadelphia chromosome?
2) How is the BCR:ABL fusion gene formed?
3) What does this lead to?
1) An abnormal chromosome that is made when pieces of chromosomes 9 and 22 break off and trade places.
2) The ABL gene from chromosome 9 joins to the BCR gene on chromosome 22 .
-The changed chromosome 22 with the fusion gene on it is called the Ph chromosome.
3) This fusion gene leads to production of an oncoprotein with constitutive tyrosine kinase activity > stimulates hematopoietic transformation and myeloproliferation
50
Is CML Tx usually emergent? Explain
Usually not emergent (even with WBC counts >200k)
-Majority of circulating cells are mature and smaller/more deformable than primitive leukemic blasts (sxs from hyperleukocytosis are rare and are treated as previously discussed)
51
CML Tx:
1) What is the goal of chronic phase Tx?
2) How can this be done? (2 ways) Which is the Tx of choice?
1) : normalize hematologic abnormalities & suppress malignant bcr/abl-expressing clone
2) Tyrosine kinase inhibitor (TKI): imatinib, nilotinib, dasatinib = treatment of choice (TOC): developed as a targeted therapy
-Stem cell transplant for inadequate response to TOC or disease progression despite therapy
52
CML:
1) What is the course?
2) What is the prognosis?
3) Which patients should you refer?
1) Most patients die from causes other than CML while still in remission
2) Excellent with good response to TKI
3) Refer all patients to a hematologist
53
When should you admit a pt with CML?
Rarely needed; reserved for symptoms of leukostasis at diagnosis or for transformation to acute leukemia
54
CLL:
1) Define it
2) How does it manifest clinically?
1) Clonal malignancy of morphologically mature but immune-incompetent B lymphocytes
(slowly progressive accumulation of long-lived small lymphocytes)
2) Immunosuppression, bone marrow failure, & organ infiltration with lymphocytes (advanced CLL)
55
CLL
1) What is the usual course?
2) What are some aggressive sub-types?
1) Indolent course
2) Prolymphocytic leukemia & large-cell lymphoma (Richter syndrome)
56
CLL:
1) Are there any exposures that put you at risk?
2) Is it genetic?
3) What age is it common in? What sex?
4) What race?
1) No definitive links to exposures; one of the only leukemias not linked to radiation exposure
a) Agent Orange exposure has been implicated (Vietnam conflict)
2) One of the most familial-associated malignancies (1st degree relative has 8.5-fold elevated risk vs. general population)
3) Disease of older patients (median age of 70 at presentation)
M > F (2:1)
4) Caucasians > Hispanic & African Americans > Asians (rare)
57
CLL
1) What is the most common S/Sx?
2) What do 80% of patients have?
3) What do 50% of patients have?
1) Asymptomatic: incidental discovery most common
Some present with fatigue & lymphadenopathy (usually not painful)
2) Diffuse lymphadenopathy
3) Enlarged liver or spleen
58
Lab findings in CLL:
1) What is the hallmark?
2) What is used to determine immunophenotype of circulating lymphocytes? What is this phenotype?
3) What is usually normal at presentation?
4) Desc. the bone marrow
5) What occurs in 50% of cases?
1) Isolated lymphocytosis (>5k; usually >20,000/mcL but may be several hundred thousand)
2) Flow cytometry (peripheral blood); coexpression of CD19, CD5
3) Hct & PLT counts
4) Bone marrow variably infiltrated with small lymphocytes
5) Hypogammaglobinemia (more common in advanced disease)
59
List at least 4 DDxs for CLL
1) Viral infections producing lymphocytosis
2) Pertussis
3) Other lymphoproliferative diseases (ie, Waldenström macroglobulinemia, hairy cell leukemia, or lymphoma in the leukemic phase)
4) Monoclonal B-cell lymphocytosis (precursor to CLL)
60
CLL Tx:
1) What do you do for early stage disease?
2) What are indications for Tx?
3) What is the usual initial therapy?
1) Early-stage disease (indolent): observation (most cases require no specific therapy)
2) Fatigue, symptomatic lymphadenopathy, anemia, or thrombocytopenia
3) Targeted biologic therapy
Alkylating agent & immunotherapy for older patients
Stem cell transplant for non-response to initial therapy
61
How do you determine the prognosis of CLL?
Rai classification system (stages 0-IV)
-Stage 0-1: median survival 10-15 years
-Stage III-IV: 5-year survival >70%
62
1) Define lymphoma
2) What are the 2 main types?
1) Cancer of the lymph nodes
2) Hodgkin's and non-hodgkins
63
Define Hodgkin lymphomas (HL) and what the cells look like
1) Malignancy of mature B lymphocytes
2) Large, dysplastic cells surrounded by variable mixtures of mature, non-neoplastic, inflammatory cells & fibrosis
64
Describe the etiology and epidemiology of HL (hodgkin lymphoma)
1) Suggested association with EBV; infection with HIV is a risk factor
2) Bimodal distribution (peak incidences in 20s & 80s)
Caucasians > African Americans
M > F
8,840 new cases in 2020 (U.S.)
65
HL (Hodgkin lymphoma):
1) What do most pts present with?
2) What do >50% of patients have?
3) What do 1/3 of pts present with?
1) Painless & non-tender lymphadenopathy (most common in neck, supraclavicular, & axilla)
2) Mediastinal adenopathy at diagnosis
3) Fevers, night sweats, and/or weight loss (aka, “B symptoms”)
66
HL:
1) Where does it typically start?
2) What occurs in 10-15% at presentation and can precede diagnosis by months to >1 year?
1) In one LN area then spread in an orderly fashion to contiguous LNs (late hematogenous spread)
2) Pruritus (usually generalized)
67
HL: What is an uncommon Sx (<10%) that's highly specific for classic Hodgkin?
Severe pain following alcohol ingestion (at sites of bony involvement or in involved lymph nodes)
68
List the lab findings with Hodgkin lymphoma (HL)
1) Tissue biopsy required for diagnosis & determination of histologic subtype
2) Reed-Sternberg cells on lymph node biopsy
3) Possible associated findings: hypercalcemia, anemia, eosinophilia, leukocytosis, thrombocytosis, lymphopenia, & hypoalbuminemia
69
List DDxs for Hodgkin's lymphoma
1) Other malignant lymphomas
2) Reactive lymph nodes:
-a) Infectious mononucleosis
-b) Cat-scratch disease
-c) Drug reactions (ie, phenytoin
70
1) What helps determine the extent of HL?
2) What type of staging is done?
3) What intent are all pts treated with?
1) Staging evaluation to determine extent: Serum chemistries, whole-body PET/CT, BM biopsy
2) Ann Arbor staging: I-IV plus “A” or “B”
3) All treated with curative intent
71
What is the mainstay of Hodgkin lymphoma Tx? Describe what's used for each stage
Mainstay: chemotherapy (ABVD standard 1st line regimen)
1) Low-risk (stage I or II): combo short-course chemo + INRT
2) High-risk (stage III or IV): full course chemo (ABVD) x 6 cycles
72
_____________ toxicity is a complication of chemo or radiation for Hodgkin lymphoma
Pulmonary
73
Hodgkin lymphomas:
1) Cure rates depend on # risk factors; what's the range?
2) What is the Advanced (stage III or IV) 10-year survival?
3) When should you refer or admit patients?
1) Cure rates depend on # risk factors: 55-90%
2) 10-year survival 50-60%
3) Refer all patients to a hematologist or oncologist
Admit patients for complications of the disease or its treatment
74
Non-Hodgkin lymphomas (NHL):
1) What are they?
2) What makes up ~90%? What abt the other ~10%?
3) How do you distinguish from HL?
1) Heterogeneous group of cancers of lymphocytes
2) ~90% are B-cell in origin
~10% are T-cell or NK-cell in origin
3) Absence of Reed-Sternberg cells
75
Non-Hodgkin lymphomas (NHL): What are the grades?
1) Indolent (low-grade)
2) Aggressive (intermediate- or high-grade)
76
Non-Hodgkin lymphomas (NHL): What are the risk factors? (3 main categories)
1) Immunodeficiency: HIV, organ transplant recipients, inherited & acquired immune deficiencies, & autoimmune conditions
2) 1st degree relatives with NHL, HL, or CLL
3) Infectious associations: EBV, HTLV-1, HIV, H. pylori, HCV, HHV-8
77
Non-Hodgkin lymphomas (NHL): Describe the epidemiology.
4% of all new cancers (M & F)
7th most common cause of all cancer-related deaths
~10 x incidence of HL
Affects all age groups
Incidence increases with age, especially after 40
M > F
Caucasians > African Americans
78
Give the S/Sx for NHL
1) Usually present with painless lymphadenopathy (peripheral or central)
2) Indolent lymphomas usually disseminated at diagnosis with frequent bone marrow involvement (not considered curable)
3) Many patients have “B” symptoms
4) Isolated or diffuse lymphadenopathy
5) Possible extra-nodal sites (GI tract, skin, liver, bone marrow)
6) Abdominal pain or fullness in patients with Burkitt lymphoma (common NHL in children)
79
What are the lab findings with NHL?
1) Tissue biopsy required for diagnosis & classification (lymph node or involved extra-nodal tissue)
2) Peripheral blood usually normal (even with extensive bone marrow involvement by lymphoma)
80
What are the DDxs for NHL?
1) Other malignant lymphomas
2) Reactive lymph nodes:
-a) Infectious mononucleosis
-b) Cat-scratch disease
-c) Drug reactions (ie, phenytoin)
81
NHL:
1) How is it staged?
2) What does indolent Tx depend on? Describe the Tx
3) What does aggressive Tx depend on? Describe the Tx
1) Whole body PET/CT, bone marrow biopsy, ± lumbar puncture
2) Depends on stage & clinical status
-Localized irradiation with curative intent (small # patients)
-Immunotherapy ± chemo only for symptoms or high tumor bulk (as most patients are non-curable due to disseminated disease)
3) Depends on type
-Options include: immunotherapy, immunochemotherapy, INRT, combo chemo, stem cell transplant, intrathecal chemo
82
What is the prognosis of NHL?
(more of an FYI than something we need to memorize)
Median survival for indolent disease 10-15 years (ultimately become refractory to chemotherapy, uncurable)
Cure rates for aggressive disease range from >80% (low-risk) to <50% (high-risk)
83
1) When should you refer NHL pts?
2) When should you admit them?
1) Refer all patients to hematologist or oncologist
2) Specific complications of disease or its treatment
or
Treatment of all high-grade disease
84
Plasma cell myeloma (PCM) (previously called multiple myeloma):
1) Define it
2) What is it characterized by?
1) Malignancy of hematopoietic stem cells terminally differentiated as plasma cells
2) Bone marrow infiltration, bone destruction (common), monoclonal immunoglobulin (paraprotein) formation
85
Plasma cell myeloma (PCM)
1) What aspect may cause spinal cord compression & soft-tissue problems?
2) What are these pts prone to?
1) Plasmacytomas
2) Recurrent infections (especially encapsulated organisms: Strep. pneumoniae & H. influenzae)
86
Describe the etiology/ epidemiology of PCM
1) Unknown etiology but associated with Agent Orange exposure
2) Risk increases with BMI
3) ~1-2% of all cancers, ~17% of hematologic malignancies
Disease of older adults (median age of 69 at diagnosis)
Uncommon under age 40
M > F
African Americans > Caucasians
87
Sx of plasma cell myeloma (PCM):
1) Most common presenting symptoms related to what 4 things?
2) Where is bone pain most common?
3) How can it present?
4) What is another common Sx?
5) What syndrome may occur
1) Anemia, bone pain, kidney disease, & infection
2) Back, hips, ribs, proximal long bones
3) As pathologic fracture (common in femoral neck & vertebrae)
4) Spinal cord compression (plasmacytoma)
5) Hyperviscosity syndrome (mucosal bleeding, vertigo, nausea, visual disturbances, alterations in mental status, hypoxia)
88
True or false: Many PCM (plasma cell myeloma) patients have normal lab findings as first indication
False: many have abnormal findings incl: elevated total protein, hypercalcemia, proteinuria, elevated ESR, or abnormalities on serum protein electrophoresis
89
List possible exam findings for PCM (plasma cell myeloma)
1) Pallor, bone tenderness, and/or soft tissue masses
2) Neurological signs related to neuropathy or spinal cord compression
3) Fever mainly with infection
4) Acute kidney injury (AKI)
90
Lab findings with PCM (plasma cell myeloma):
1) What is nearly universal?
2) Describe the RBCs
3) What are usually normal at presentation?
1) Anemia nearly universal (usually normocytic)
2) Normal RBC morphology but Rouleax formation is common (may be marked)
3) Neutrophil & platelet counts usually normal at presentation
91
PCM (plasma cell myeloma) labs:
1) Where would you find Hallmark monoclonal immunoglobulin (paraprotein)?
2) What may show may show excess monoclonal light chains in serum and urine (sometimes the only way to identify the paraprotein)?
3) Clonal plasma cells seen in BM or tissue biopsy (or both) (not usually visible in peripheral blood). What indicates their abnormality?
1) On serum and/or urine protein electrophoresis **(SPEP/UPEP) (and/or IFE)**
2) Free light chain assay
3) Marked skewing of the normal kappa-to-lambda light chain ratio will indicate their clonality
92
PCM (plasma cell myeloma) imaging:
1) What is important to establish Dx?
2) Lytic lesions are most common where?
3) What is preferred for known/ suspected disease?
4) What is unhelpful?
1) Bone radiographs
2) In skull, spine, proximal long bones, & ribs
3) MRI & PET/CT preferred (more sensitive than x-rays)
4) Bone scan
93
List some DDxs for PCM (plasma cell myeloma)
1) Monoclonal gammopathy of undetermined significance (MGUS)
2) Smoldering multiple myeloma (SMM)
3) Waldenström macroglobulinemia (WM)
4) Solitary plasmacytoma
5) Primary amyloidosis (AL)
6) POEMS syndrome
7) Metastatic carcinoma
94
Initial Tx for PCM (plasma cell myeloma):
1) What is the purpose?
2) What is the goal?
3) Who is eligible for an autologous transplant?
4) Who is not eligible for an autologous transplant?
1) Purpose is to alleviate symptoms, reverse cytopenias, & decrease end-organ damage
2) Goal is to achieve sustained response, improve quality of life, & prolong overall survival
3) 3-6 months of induction chemotherapy + hematopoietic stem cell transplant (HCT)
4) 8-12 months of induction chemotherapy followed by maintenance
95
PCM (plasma cell myeloma) Tx:
1) Hypercalcemia
2) Renal impairment
3) Anemia
1) Hydration, glucocorticoids, bisphosphonates, and/or hemodialysis/calcitonin indicated for symptomatic patients
2) Directed at underlying cause
3) Red blood cell transfusion, erythropoiesis-stimulating agents
96
PCM Tx:
1) Localized radiation therapy may do what?
2) When should you do surgery?
3) What do bisphosphonates do?
1) Localized radiation therapy may palliate bone pain or eradicate tumor at site of pathologic fracture
2) For vertebral collapse
3) Can reduce pathologic fractures
97
PCM: Describe the prognosis and when to refer
1) Median survival more than 7 years (depends on stage)
2) Refer all patients to a hematologist or an oncologist
98
When should you admit a pt with PCM? (3 reasons)
1) Treatment of AKI, hypercalcemia, or suspicion of spinal cord compression
2) Certain chemotherapy regimens
3) Autologous hematopoietic stem cell transplantation
99
List the epidemiology of the hematologic cancers
AML: M>F, Caucasian>other
ALL: M>F, Caucasians>African Americans
CML: M>F, Hispanic>other
CLL/SLL: M>F, Caucasian>African Americans or Asian Pacific Islanders
HL: M>F, Caucasians>African Americans
NHL: M>F, Caucasians>African Americans
PCM: M>F, African Americans > Caucasians
100
The WHO classifies myelodysplastic syndromes (MDS) as ________________ disorders.
myeloproliferative
