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

Leukocyte disorders: part 1- Exam 2 Flashcards

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
Q

What is the post-remission therapy for older patients or unable to tolerable the effects of chemo?

A

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

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24
Q

What is the management of leukostasis in ALL?

A

prophylaxis for tumor lysis syndrome via IV hydration and hypouricemic agents

emergent chemotherapy

leukapheresis

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25
Q

For ALL, what is the cure rate in children? Adults?

A

90% in children

50% in adults

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26
Q

What is considered a relapse for ALL? What is the common timeframe?

A

a reappearance of leukemic cells at any site

most often occurs within first 2 years

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27
Q

What are good prognostic factors for ALL?

A

No chromosomal abnormalities¹
Age younger than 39 years
White blood cell (WBC) count of < than 30,000/μL
Complete remission within 4 weeks

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28
Q

What are poor prognostic criteria for ALL?

A

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

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29
Q

_______ a malignant lymphoid neoplasm that is characterized by the accumulation of long-lived, functionally incompetent, small mature B cells

A

Chronic Lymphocytic Leukemia (CLL)

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30
Q

______ results from dysfunction in the maturation of the B-cell. Results in B-cells that are unable to respond to immunologic stimulation

A

Chronic Lymphocytic Leukemia (CLL)

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31
Q

_____ is the MC form of leukemia in the US

A

Chronic Lymphocytic Leukemia (CLL)

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32
Q

_____ mainly occurs after the age of 50, median age of onset is 72

A

Chronic Lymphocytic Leukemia (CLL)

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33
Q

What is the clinical presentation of CLL?

A

slow onset
lymphadenopathy
recurrent infections (usually viral)
HSM
anemia/thrombocytopenia

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34
Q

What tests would you order if you suspect CLL? **What will the tests show?

A

**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

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35
Q

**What is a hallmark finding on a CBC with a CLL pt?

A

isolated absolute lymphocytosis (hallmark)
persists for > 3 months

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36
Q

What do you expect to find on a peripheral smear for a CLL pt?

A

predominantly small mature lymphocytes, **(+) smudge cells¹, may see prolymphocytes

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37
Q

What are the 2 direct predecessor of a B lymphocyte?

A

small lymphocyte then prolymphocyte

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38
Q

How is a CLL dx confirmed? What would you expect to see?

A

Peripheral blood via flow cytometry - confirms diagnosis

confirms the presence of various abnormal B-lymphocyte surface antigens

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39
Q

What will the bone marrow bx show of a pt with CLL? Is it required?

A

infiltrated with small lymphocytes

NOT always required to make diagnosis

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40
Q

**What is the staging system for CLL? Describe how to stage a pt.

A

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

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41
Q

What is the management for CLL based on?

A

staging

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42
Q

If a pt is low risk pt for CLL, what is the management? What would change that?

A

observation

symptoms would increase risk: worsening fatigue, symptomatic lymphadenopathy/HSM, anemia, or thrombocytopenia

43
Q

What are treatment options for CLL? What is the curative therapy?

A

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
Q

What are the complications of CLL?

A

Obstructive lymphadenopathy - compressing on internal organs

Transformation into aggressive large cell lymphoma (Richter Syndrome)

Autoimmune hemolytic anemia or thrombocytopenia¹

45
Q

a pt with CLL who is a low risk stage, what is the average survival?

Intermediate/high risk?

A

low: average survival 10-15 years

Intermediate/high risk: 2 year survival is > 90%, 5-year survival is > 70%

46
Q

_____ A malignant disease of the bone marrow resulting from an arrest in the early development of myeloid precursors

A

Acute Myelogenous Leukemia (AML)

47
Q

What is the pathophysiology of AML?

A

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
Q

What is the pathogenesis of AML? Who is the MC pt population?

A

chromosomal translocations and other genetic mutations

median age is 70, white, men, developed countries

49
Q

What is the MC risk factor for AML? Describe what is happening?

A

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
Q

Congenital/Genetic Disorders such as Trisomy 21, Bloom’s syndrome, Fanconi anemia are associated with _____

A

AML

51
Q

What would a CBC of a pt with AML show?

**peripheral smear?

A

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
Q

**_____ confirms the presence of AML

A

auer rod on peripheral smear

52
Q

What will bone marrow bx look like on a pt with AML?

A

hypercellular, predominant myeloblasts

53
Q

How do you differentiate AML from ALL?

A

flow cytometry: AML will have myeloid antigens on the cell surface and ALL will not

54
Q

AML work up includes ____ and ______. When are they indicated?

A

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
Q

What is the broad management for AML?

A

refer to hem/onc

multi-drug chemo

intrathecal chemo if CNS is involved

56
Q

AML pts, induction: multi-drug chemotherapy will induce remission in _____ of patients who are < 60 y/o and ____ in patients > 60 y/o

A

80-90%

50-60%

57
Q

AML pts: Post-remission therapy includes _____ and/or _____

A

standard chemotherapy

stem-cell replacement (can be autologous or allogeneic)

58
Q

What factors contribute towards a good prognosis for AML pts? Poor prognosis factors?

A

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
Q

______ A disorder characterized by dysregulated production and uncontrolled proliferation of mature and maturing granulocytes with normal differentiation

A

Chronic Myeloid Leukemia (CML)

60
Q

What is the Philadephia chromosome? What disorder is it associated with?

A

results from a single specific genetic mutation
translocation t (9:22)

Chronic Myeloid Leukemia (CML)

61
Q

What is the genetic bcr/abl known as? What does it possess?

A

translocation t(9:22) - Philadelphia chromosome

bcr/abl gene produces a protein that possess overactive tyrosine kinase (TK) activity

62
Q

What is TK responsible for? What disorder is it associated with?

A

controlling cell growth, differentiation, metabolism and apoptosis

Chronic Myeloid Leukemia (CML)

63
Q

Chronic Myeloid Leukemia (CML) average age of onset is ______. What is associated with?

A

55 y/o - “middle-aged”

increased risk with exposure to ionizing radiation

64
Q

What are the phases of CML? Chronic myeloid leukemia

A

First (chronic)
Second (accelerated)
Third (terminal blast crisis)

65
Q

CML that is found in the chronic phase is found _____

A

incidentally

66
Q

CML in the third “blast” phase, what is happening?

A

immature myeloid cells rapidly proliferate (fatal)

67
Q

pruritus, diarrhea, flushing, gastrointestinal ulcers seen with elevated basophils due to overproduction of _____. What disorder are they associated with?

A

histamine

CML: Chronic myeloid leukemia

68
Q

What is the clinical presentation of CML?

A

Fever of unknown origin, bone pain, splenomegaly, signs of leukemia: anemia, neutropenia, thrombocytopenia

69
Q

What is the MC PE finding for CML?

A

splenomegaly

70
Q

What does the CBC of CML patient in the chronic phase look like? accelerated phase?

A

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
Q

What does the peripheral smear of CML patient in the chronic phase look like? accelerated phase?

A

chronic phase: confirmation of CBC findings

accelerated phase : peripheral myeloblast cells, promyelocytes visualized

72
Q

What two “things” are associated with leukostasis?

A

increased WBC AND signs of end organ damage

73
Q

What will the Leukocyte Alkaline Phosphatase (LAP) stain tell you? What does it look like in CML?

A

a type of alkaline phosphatase found inside WBC’s

LAP will be high in an infection

will be DECREASED in CML

74
Q

**What will a bone marrow biopsy look like in a CML pt?

A

hypercellular with increased granulocyte cells and their progenitors
Blast phase: >20% blasts compromise BM cells

75
Q

**What will a PCR test look like with CML?

A

can be performed on blood or marrow aspirate
identifies bcr/abl DNA segment (aka Philadelphia Chromosome)

76
Q

What is the definitive way to diagnosis CML?

A

bone marrow bx and PCR to check for the presence of the Philadelphia Chromosome

77
Q

What is the management of chronic phase CML?

A

tyrosine kinase inhibitor - single drug chemotherapy

78
Q

_____ results in CML cell death leaving healthy cells less affected compared to standard chemotherapy

A

tyrosine kinase inhibitor

79
Q

_____ describes the dependency of certain tumor cells on a single activated oncogenic protein or pathway to maintain their malignant properties.

A

oncogene addiction

80
Q

What are the parameters in order to be considered in hematologic remission of CML? How long does it normally take?

A

often seen within 3 months

normal CBC and physical exam

81
Q

What are the parameters in order to be considered in cytogenetic remission of CML? How long does it normally take?

A

seen within 3-6 months

normal chromosome returns with < 10 % positive (Ph+) cells

82
Q

What are the parameters in order to be considered in molecular remission of CML? How long does it normally take?

A

within 12 months

negative PCR result for the mutational bcr/abl mRNA (absence of Ph+ cells)

83
Q

How long do you need to continue therapy after molecular remission is reached in CML?

A

Continue therapy for 2 years after molecular remission is reached

84
Q

What is the treatment for accelerated or blast phase CML?

A

TKI + multidrug chemotherapy

consider allogeneic stem cell transplantation

transplant is also indicated if lack of response to TKI

85
Q

What is the treatment for symptomatic leukostasis?

A

emergent chemotherapy, leukapheresis, prophylaxis for tumor lysis syndrome

86
Q

What are some signs of transition from chronic CML to accelerated CML?

A

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
Q

What is the prognosis for CML?

A

depends on the response to TKI

100% survival at 9 years with good response to TKI

88
Q

_________ a neoplastic proliferation of plasma cells producing an overproduction of nonfunctional monoclonal immunoglobulins

A

multiple myeloma

89
Q

Multiple Myeloma is preceded by a premalignant plasma cell proliferative disorder known as ???? What does it result from?

A

Monoclonal Gammopathy of Undetermined Significance (MGUS)

abnormal plasma cell response to antigenic stimulation

90
Q

What are the risk factors for multiple myeloma? Who is at the highest risk?

A

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
Q

in MM, Proliferation of neoplastic plasma cells in the bone marrow results in _____

A

diminished hematopoiesis

92
Q

What do neoplastic plasma cells of MM look like? What happens as a result?

A

monoclonal

resulting in a lack of adequate immunoglobulin response to infection

93
Q

Neoplastic plasma cells increase _____, ______ and _____ formation

A

osteoclastic activity, hypercalcemia, bone tumor

94
Q

What do neoplastic plasma cells secrete? What does it harm?

A

an antibody called myeloma proteins which are harmful to the kidneys, nerves and other organs

95
Q

What are plasmacytomas? What are they made of specifically?

A

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
Q

What is the clinical presentation of Multiple myeloma?

A

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
Q

What will the CBC of MM look like? peripheral blood smear? bone marrow bx?

A

pancytopenia

RBC rouleaux formation

infiltration by monoclonal plasma cells which are often morphologically abnormal

98
Q

What are some specific tests that you want to order for multiple myeloma? What will be present to help dx MM?

A

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
Q

prognostic testing for MM is _____. What does it tell you?

A

Beta-2 microglobulin

elevated - level is directly related to tumor burden

100
Q

Why would you order x-rays for MM? Where are the MC places?

A

assess for lytic lesions and pathologic fractures

MC seen in skull, spine, long bones

101
Q

What is the management for MM?

A

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
Q

How do you manage bone disease/hypercalcemia in MM?

A

use bisphosphonates¹ (IV Reclast) in symptomatic patients with intact renal function

103
Q

How do you manage anemia in MM? renal impairment?

A

initiate EPO therapy if persistent anemia when other causes have been ruled out

plasmapheresis to remove M-proteins from circulation

104
Q

What is the prognosis for MM?

A

Median survival is 3 years

better survival rates in younger patients

105
Q
A

Hem/Onc (14 decks)

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