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Hem/Onc > Leukocyte disorders: part 1- Exam 2 > Flashcards

Leukocyte disorders: part 1- Exam 2 Flashcards

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1
Q

What are some indications to order a bone marrow biopsy?

A

dx/staging bone marrow disorders

increase or decrease in hematologic cell lines

lymphoma

evaluation of iron metabolism

fever of unknown origin

unexplained splenomegaly

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2
Q

What is the CI to bone marrow biopsy? What is NOT a CI?

A

severe bleeding disorders

thrombocytopenia is NOT a CI, but you would give a plt transfusion first

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3
Q

What is the preferred site for a bone marrow biopsy? What is the second area?

A

posterior iliac crest

anterior iliac crest

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4
Q

What sites are commonly used for bone marrow aspiration? Which one is commonly used in infants? morbidly obese?

A

tibia: MC site used in infants

sternum between 2nd and 3rd ICS: reserved for only >12 yrs old and morbidly obese

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5
Q

_____ a malignant disorder that originates in a single lymphocyte progenitor (lymphoblast) and results from an abnormal expression of genes, often as a result of chromosomal translocation

A

Acute Lymphoblastic Leukemia (ALL)

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6
Q

**What is the pathophysiology behind ALL?

A

abnormal lymphoblasts that are resist to death, keeping making more and more, no longer needs the stem cell to replicate, abnormal lymphoblasts in the BM suppress normal hematopoiesis, abnormal lymphoblasts accumulate in other organs and the organs are enlarged upon PE

rapid cell proliferation/self-renewal

reduction in normal cell proliferation

block in cell differentiation

increase resistance in cell apoptosis

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7
Q

What are the risk factors for ALL?

A

In utero radiation exposure

Chemicals (questionable): pesticides, tobacco, alcohol, nitrites, chemotherapy

High birth weight - increased insulin-like growth factor (IGF - 1)

Lack of exposure to infections in the first few weeks/months of life¹

Only 5% are linked to genetics

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8
Q

______ is MC in children less than 5 years old and older than 60. Most deaths are in what pt population? What ethnicity and gender?

A

ALL

more deaths in adults

Slightly more common in males

Caucasian > African Americans

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9
Q

What is the clinical presentation of ALL?

A

fever of unknown origin

related to bone marrow infiltration

neutropenia, anemia, thrombocytopenia

lymphadenopathy

bone pain

LUQ abdominal fullness, early satiety (splenomegaly)

mediastinal mass: chest pain, dysphagia, or dyspnea

swelling of the neck, face, and upper limbs

painless testicular swelling/mass

Leukostasis: WBC> 100K that leads to inadequate circulation

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10
Q

What are some s/s of leukostasis?

A

HA, altered mental status, blurred vision, dyspnea, priapism

increased risk of intracranial hemorrhage

risk persists for at least 1 week after reduction of WBC

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11
Q

What is the mortality of leukostasis is not treated properly in 2 days?

A

40% within 2 days

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11
Q

What tests should you order on a pt if you suspect ALL?

A

CBC
CMP
blood cultures
CXR
CT/MRI of the brain w/o contrast

additional:
peripheral smear
LDH
CT chest with contrast
CSF analysis
Flow cytometry
bone marrow aspiration and biopsy

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12
Q

What will the CBC look like on a pt with ALL?

A

decreased RBC, platelet and neutrophils
WBC may be normal, high or low

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13
Q

Why do you order a CXR if you suspect ALL?

A

r/o pneumonia as a source of infection, assess for signs of mediastinal mass

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14
Q

What would you expect to find on a peripheral smear of a pt wit ALL?

A

pancytopenia with circulating lymphoblasts

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15
Q

Why would you order a chest ct with contrast on a pt with ALL?

A

assess lymphadenopathy, further assess mediastinal mass

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16
Q

Pt has ALL with neuro s/s and you do a CSF analysis, what would you expect to find? What will flow cytometry show?

A

(+) lymphoblast cells in CSF - with spinal infiltration of dz.

atypical antigens confirmation

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17
Q

How do you definitively dx ALL?

A

Bone marrow biopsy with greater than 20% lymphoblasts

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18
Q

What is the management for ALL?

A

Refer to hematology/oncology

Screen and treat for active infections in febrile patients

ALL treatment begins with “induction chemotherapy” and CNS prophylaxis, followed by post-remission therapy with or without stem cell transplantation

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19
Q

What is the goal of induction chemo? What are the guidelines?

A

Multi-drug chemotherapy over the course of 4-6 weeks

Initiated in the hospital¹

Goal: remission induction

Complete remission achieved at 65-85%

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20
Q

When treating ALL, _____ is vital during all stages to prevent CNS recurrence

A

Intrathecal therapy

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21
Q
A
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22
Q

After the induction phase of chemo, what are the next treatment steps based off of?

A

Based upon patient age and toleration of
induction chemo

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22
Q

What is the post-remission therapy for young patients with tolerable effects of chemo?

A

readministration of induction regimen or other high dose chemotherapeutic agents after normal hematopoiesis is restored for 4-8 months

continue less intensive weekly/daily maintenance therapy for 2-3 years

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23
What is the post-remission therapy for older patients or unable to tolerable the effects of chemo?
Allogeneic Stem Cell Transplantation Patient receives intensive chemotherapy prior to transplant to ensure destruction of as many cancer cells as possible. IV transplantation of stem cells over an hour Stem cells travel to the bone marrow and begin to produce new blood cells
24
What is the management of leukostasis in ALL?
prophylaxis for tumor lysis syndrome via IV hydration and hypouricemic agents emergent chemotherapy leukapheresis
25
For ALL, what is the cure rate in children? Adults?
90% in children 50% in adults
26
What is considered a relapse for ALL? What is the common timeframe?
a reappearance of leukemic cells at any site most often occurs within first 2 years
27
What are good prognostic factors for ALL?
No chromosomal abnormalities¹ Age younger than 39 years White blood cell (WBC) count of < than 30,000/μL Complete remission within 4 weeks
28
What are poor prognostic criteria for ALL?
Chromosomal abnormalities Age older than 60 years Precursor B-cell WBCs with WBC count > than 100,000/μL Failure to achieve complete remission within 4 weeks
29
_______ a malignant lymphoid neoplasm that is characterized by the accumulation of long-lived, functionally incompetent, small mature B cells
Chronic Lymphocytic Leukemia (CLL)
30
______ results from dysfunction in the maturation of the B-cell. Results in B-cells that are unable to respond to immunologic stimulation
Chronic Lymphocytic Leukemia (CLL)
31
_____ is the MC form of leukemia in the US
Chronic Lymphocytic Leukemia (CLL)
32
_____ mainly occurs after the age of 50, median age of onset is 72
Chronic Lymphocytic Leukemia (CLL)
33
What is the clinical presentation of CLL?
slow onset lymphadenopathy recurrent infections (usually viral) HSM anemia/thrombocytopenia
34
What tests would you order if you suspect CLL? **What will the tests show?
**CBC: WBC >20,000 cell/µL isolated absolute lymphocytosis (hallmark) persists for > 3 months +/- decreased RBC, platelets peripheral smear: predominantly small mature lymphocytes, (+) smudge cells¹, may see prolymphocytes CMP: to asses liver/renal function flow cytometry bone marrow aspiration/biospy
35
**What is a hallmark finding on a CBC with a CLL pt?
isolated absolute lymphocytosis (hallmark) persists for > 3 months
36
What do you expect to find on a peripheral smear for a CLL pt?
predominantly small mature lymphocytes, **(+) smudge cells¹, may see prolymphocytes
37
What are the 2 direct predecessor of a B lymphocyte?
small lymphocyte then prolymphocyte
38
How is a CLL dx confirmed? What would you expect to see?
Peripheral blood via flow cytometry - confirms diagnosis confirms the presence of various abnormal B-lymphocyte surface antigens
39
What will the bone marrow bx show of a pt with CLL? Is it required?
infiltrated with small lymphocytes NOT always required to make diagnosis
40
**What is the staging system for CLL? Describe how to stage a pt.
Revised Rai staging system Low risk: Stage 0: Lymphocytosis alone lymphocyte > 15,000/μL, and > 40% lymphocytes in the bone marrow Intermediate risk: Stage I: Lymphocytosis with enlarged node(s) in any site, Stage II: splenomegaly or hepatomegaly or both High risk: Stage III: Anemia (hemoglobin level < 11.0 g/dL) Stage IV: Thrombocytopenia (platelets < 100,000/μL) aka look at blood levels and organs involved to determine stage LESHAT
41
What is the management for CLL based on?
staging
42
If a pt is low risk pt for CLL, what is the management? What would change that?
observation symptoms would increase risk: worsening fatigue, symptomatic lymphadenopathy/HSM, anemia, or thrombocytopenia
43
What are treatment options for CLL? What is the curative therapy?
multidrug chemo growth factors to decrease duration of neutropenia following chemo allogeneic stem cell transplant:**reserved for patients who are not controlled with standard therapies** Most elderly patients are NOT able to tolerate transplant after first round of cehmo splenectomy: **indicated for refractory splenomegaly and pancytopenia** stem cell transplant is the only known curative therapy
44
What are the complications of CLL?
Obstructive lymphadenopathy - compressing on internal organs Transformation into aggressive large cell lymphoma (Richter Syndrome) Autoimmune hemolytic anemia or thrombocytopenia¹
45
a pt with CLL who is a low risk stage, what is the average survival? Intermediate/high risk?
low: average survival 10-15 years Intermediate/high risk: 2 year survival is > 90%, 5-year survival is > 70%
46
_____ A malignant disease of the bone marrow resulting from an arrest in the early development of myeloid precursors
Acute Myelogenous Leukemia (AML)
47
What is the pathophysiology of AML?
rapid proliferation of myeloblast without differentiation into mature cells abnormal myeloblasts are resistant to apoptosis accumulation of myeloblasts in marrow, blood, spleen, liver reduction of normal hematopoiesis due to marrow accumulation aka loss of neutrophils, eosinophils, basophils and monocytes
48
What is the pathogenesis of AML? Who is the MC pt population?
chromosomal translocations and other genetic mutations median age is 70, white, men, developed countries
49
What is the MC risk factor for AML? Describe what is happening?
Myelodysplastic Syndrome (MDS) bone marrow disease of unknown etiology that occurs most often in older patients and manifests as progressive cytopenias that occur over months to years
50
Congenital/Genetic Disorders such as Trisomy 21, Bloom's syndrome, Fanconi anemia are associated with _____
AML
51
What would a CBC of a pt with AML show? **peripheral smear?
decreased RBC, platelet and neutrophils WBC may be normal, high or low **pancytopenia with predominantly circulating myeloblasts **Auer rod - eosinophilic needle-like inclusion in the cytoplasm of myeloblasts
51
**_____ confirms the presence of AML
auer rod on peripheral smear
52
What will bone marrow bx look like on a pt with AML?
hypercellular, predominant myeloblasts
53
How do you differentiate AML from ALL?
flow cytometry: AML will have myeloid antigens on the cell surface and ALL will not
54
AML work up includes ____ and ______. When are they indicated?
Brain MRI/CT - indicated for neurologic symptoms (leukostasis) Lumbar Puncture - symptomatic patients only, looking for CNS infiltration, CNS infiltration is rare for pts with AML
55
What is the broad management for AML?
refer to hem/onc multi-drug chemo intrathecal chemo if CNS is involved
56
AML pts, induction: multi-drug chemotherapy will induce remission in _____ of patients who are < 60 y/o and ____ in patients > 60 y/o
80-90% 50-60%
57
AML pts: Post-remission therapy includes _____ and/or _____
standard chemotherapy stem-cell replacement (can be autologous or allogeneic)
58
What factors contribute towards a good prognosis for AML pts? Poor prognosis factors?
Good: less than 60 yrs old remission after first chemo Poor: older than 60 yrs old poor response to first chemo treatment abnormal genetics/chromosomes detected in AML cells
59
______ A disorder characterized by dysregulated production and uncontrolled proliferation of mature and maturing granulocytes with normal differentiation
Chronic Myeloid Leukemia (CML)
60
What is the Philadephia chromosome? What disorder is it associated with?
results from a single specific genetic mutation translocation t (9:22) Chronic Myeloid Leukemia (CML)
61
What is the genetic bcr/abl known as? What does it possess?
translocation t(9:22) - Philadelphia chromosome bcr/abl gene produces a protein that possess overactive tyrosine kinase (TK) activity
62
What is TK responsible for? What disorder is it associated with?
controlling cell growth, differentiation, metabolism and apoptosis Chronic Myeloid Leukemia (CML)
63
Chronic Myeloid Leukemia (CML) average age of onset is ______. What is associated with?
55 y/o - “middle-aged” increased risk with exposure to ionizing radiation
64
What are the phases of CML? Chronic myeloid leukemia
First (chronic) Second (accelerated) Third (terminal blast crisis)
65
CML that is found in the chronic phase is found _____
incidentally
66
CML in the third "blast" phase, what is happening?
immature myeloid cells rapidly proliferate (fatal)
67
pruritus, diarrhea, flushing, gastrointestinal ulcers seen with elevated basophils due to overproduction of _____. What disorder are they associated with?
histamine CML: Chronic myeloid leukemia
68
What is the clinical presentation of CML?
Fever of unknown origin, bone pain, **splenomegaly**, signs of leukemia: anemia, neutropenia, thrombocytopenia
69
What is the MC PE finding for CML?
splenomegaly
70
What does the CBC of CML patient in the chronic phase look like? accelerated phase?
chronic phase: average white count 100,000-150,000 cells/μL granulocytosis with marked increase in mature neutrophils, mild increase in basophils and eosinophils accelerated phase: reduced platelets, RBC, increase in myeloblasts
71
What does the peripheral smear of CML patient in the chronic phase look like? accelerated phase?
chronic phase: confirmation of CBC findings accelerated phase : peripheral myeloblast cells, promyelocytes visualized
72
What two "things" are associated with leukostasis?
increased WBC AND signs of end organ damage
73
What will the Leukocyte Alkaline Phosphatase (LAP) stain tell you? What does it look like in CML?
a type of alkaline phosphatase found inside WBC’s LAP will be high in an infection will be DECREASED in CML
74
**What will a bone marrow biopsy look like in a CML pt?
hypercellular with increased granulocyte cells and their progenitors Blast phase: >20% blasts compromise BM cells
75
**What will a PCR test look like with CML?
can be performed on blood or marrow aspirate identifies bcr/abl DNA segment (aka Philadelphia Chromosome)
76
What is the definitive way to diagnosis CML?
bone marrow bx and PCR to check for the presence of the Philadelphia Chromosome
77
What is the management of chronic phase CML?
tyrosine kinase inhibitor - single drug chemotherapy
78
_____ results in CML cell death leaving healthy cells less affected compared to standard chemotherapy
tyrosine kinase inhibitor
79
_____ describes the dependency of certain tumor cells on a single activated oncogenic protein or pathway to maintain their malignant properties.
oncogene addiction
80
What are the parameters in order to be considered in hematologic remission of CML? How long does it normally take?
often seen within 3 months normal CBC and physical exam
81
What are the parameters in order to be considered in cytogenetic remission of CML? How long does it normally take?
seen within 3-6 months normal chromosome returns with < 10 % positive (Ph+) cells
82
What are the parameters in order to be considered in molecular remission of CML? How long does it normally take?
within 12 months negative PCR result for the mutational bcr/abl mRNA (absence of Ph+ cells)
83
How long do you need to continue therapy after molecular remission is reached in CML?
Continue therapy for 2 years after molecular remission is reached
84
What is the treatment for accelerated or blast phase CML?
TKI + multidrug chemotherapy consider allogeneic stem cell transplantation transplant is also indicated if lack of response to TKI
85
What is the treatment for symptomatic leukostasis?
emergent chemotherapy, leukapheresis, prophylaxis for tumor lysis syndrome
86
What are some signs of transition from chronic CML to accelerated CML?
splenomegaly inadequate decrease in granulocytes with standard therapy blast cells and promyelocytes in peripheral smear anemia, basophilia and thrombocytopenia new cytogenetic abnormalities or myelofibrosis seen on bone marrow biopsy
87
What is the prognosis for CML?
depends on the response to TKI 100% survival at 9 years with good response to TKI
88
_________ a neoplastic proliferation of plasma cells producing an overproduction of nonfunctional monoclonal immunoglobulins
multiple myeloma
89
Multiple Myeloma is preceded by a premalignant plasma cell proliferative disorder known as ???? What does it result from?
Monoclonal Gammopathy of Undetermined Significance (MGUS) abnormal plasma cell response to antigenic stimulation
90
What are the risk factors for multiple myeloma? Who is at the highest risk?
older age, immunosuppression, and environmental exposures radiation, benzene, organic solvents, herbicides, and insecticides median age: 65 years old M>F African American > Caucasian/Hispanics > Asian/Pacific Islanders
91
in MM, Proliferation of neoplastic plasma cells in the bone marrow results in _____
diminished hematopoiesis
92
What do neoplastic plasma cells of MM look like? What happens as a result?
monoclonal resulting in a lack of adequate immunoglobulin response to infection
93
Neoplastic plasma cells increase _____, ______ and _____ formation
osteoclastic activity, hypercalcemia, bone tumor
94
What do neoplastic plasma cells secrete? What does it harm?
an antibody called myeloma proteins which are harmful to the kidneys, nerves and other organs
95
What are plasmacytomas? What are they made of specifically?
a discrete solitary mass of neoplastic monoclonal plasma cells found in bone (MC vertebrae) or extramedullary spaces (upper airway/sinus being MC - other soft tissue locations possible)
96
What is the clinical presentation of Multiple myeloma?
Skeletal system: MC axial bone pain: MC back, hips and ribs spinal cord compression: back pain, weakness, numbness, or dysesthesias in the extremities pathologic fractures hypercalcemia due to skeletal destruction signs of anemia, neutropenia, thrombocytopenia renal: impaired function/failure, proteinuria, oliguria neuro: radiculopathy or neuro deficits from spinal nerve compression peripheral nerve compression - MC median nerve (Carpal Tunnel Syndrome) Plasmacytomas: bleeding, obstruction aerodigestive tract (MC), orbital, ear canal, cutaneous, gastric, rectal, prostatic, and retroperitoneal
97
What will the CBC of MM look like? peripheral blood smear? bone marrow bx?
pancytopenia RBC rouleaux formation infiltration by monoclonal plasma cells which are often morphologically abnormal
98
What are some specific tests that you want to order for multiple myeloma? What will be present to help dx MM?
Serum protein electrophoresis¹ (SPEP): (+) paraprotein (M-protein) found 24 hour urine collection with urine protein electrophoresis (UPEP): (+) Bence Jones protein² Quantitative immunoglobulin levels: suppression of the non-myelomatous immunoglobulins (IgG, IgA, IgM)
99
prognostic testing for MM is _____. What does it tell you?
Beta-2 microglobulin elevated - level is directly related to tumor burden
100
Why would you order x-rays for MM? Where are the MC places?
assess for lytic lesions and pathologic fractures MC seen in skull, spine, long bones
101
What is the management for MM?
chemo chemo then autologous stem cell transplant (usually in younger patients) localized radiation: to target bone pain related to tumor formation stabilize the bone due to pathological fractures IV steroids/neurosurgery consult to help reduce spinal cord compression
102
How do you manage bone disease/hypercalcemia in MM?
use bisphosphonates¹ (IV Reclast) in symptomatic patients with intact renal function
103
How do you manage anemia in MM? renal impairment?
initiate EPO therapy if persistent anemia when other causes have been ruled out plasmapheresis to remove M-proteins from circulation
104
What is the prognosis for MM?
Median survival is 3 years better survival rates in younger patients
105

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