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Hem/Onc > leukocyte disorders pt 1 > Flashcards

leukocyte disorders pt 1 Flashcards

1
Q

A procedure in which a hole is drilled into the bone to allow for aspiration of the cellular contents of the bone marrow

A

Bone Marrow Aspiration and Biopsy

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2
Q

indications for bone marrow aspiration and biopsy

A
  1. diagnosis, staging, and therapeutic monitoring of bone marrow disorders
  2. unexplained elevation or decrease in any hematologic cell line
    - i.e anemia, leukocytosis
  3. lymphoma, solid tumor
  4. evaluation of iron metabolism & stores when routine testing is inadequate
  5. fever of unknown origin
  6. unexplained splenomegaly
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3
Q

CI of bone
marrow aspiration/biopsy

A
  1. severe bleeding disorders
    - hemophilia, Disseminated Intravascular Coagulation (DIC)
  2. thrombocytopenia is not a CI
    consider platelet transfusion if plt count <20,000 prior to procedure
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4
Q

testings for bone marrow aspiration/biopsy

A

histology
cytogenetic testing
flow-cytometry

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5
Q

for a bone marrow aspiration/biopsy, you must avoid:

A

any area with signs of infection, injury or excessive overlying adipose tissue

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6
Q

what is the preferred site for bone marrow aspiration and biopsy

A

posterior iliac crest
- No major blood vessels or organs
- Easily accessible and less risky site

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7
Q

what site do you use for aspiration only

A
  1. tibia (under general anesthesia)
    - MC site used in infants < 12 m
  2. sternum (between 2nd and 3rd ICS)
    - reserved for only >12 yrs old and morbidly obese
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8
Q

a malignant disorder often as a result of chromosomal translocation

A

Acute Lymphoblastic Leukemia (ALL)

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9
Q

lymphoblast cell mutation results in: (4)

A
  1. rapid cell proliferation/self-renewal
  2. reduction in normal cell proliferation
  3. block in cell differentiation
  4. increase resistance in cell apoptosis
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10
Q

Accumulation of abnormal lymphoblasts in the BM causes what?

A
  1. suppress normal hematopoiesis
    - anemia
    - thrombocytopenia
    - neutropenia
  2. accumulate in other organs
    - meninges, gonads, thymus, liver, spleen, and lymph nodes
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11
Q

What environmental agents are linked to increased risk
of ALL

A
  1. In utero radiation exposure
  2. Chemicals - pre/postnatal exposure (questionable)
    - pesticides, tobacco, alcohol, nitrites, chemotherapy
  3. High birth weight - increased insulin-like growth factor (IGF-1)
  4. Lack of exposure to infections in the first few weeks/months of life
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12
Q

ALL is MC in what demographic?

A

children
males
White

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13
Q

Most deaths from ALL occur in ?

A

adults

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14
Q

MC presenting symptom of ALL

A

fever

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15
Q

s/s of ALL related to bone marrow infiltration

A
  1. neutropenia
    - secondary infections most often seen with ANC < 500/µL; severe infection with < 100/µL
  2. anemia
    - fatigue, dizziness, palpitations, exertional dyspnea, pallor
  3. thrombocytopenia
    - petechiae, ecchymosis, occult and gross blood loss
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16
Q

s/s of ALL Related to organ infiltration

A
  1. lymphadenopathy
  2. bone pain
  3. early satiety (splenomegaly)
  4. mediastinal mass
    - chest pain, dysphagia, or dyspnea
    - swelling of the neck, face, and upper limbs
  5. painless testicular swelling/mass
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17
Q

s/s of leukostasis from ALL leads to what?

A
  1. inadequate circulation
    - 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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18
Q

what is the initial work up for ALL

A
  1. CBC
    - decreased RBC, platelet and neutrophils
    - WBC may be normal, high or low
  2. Complete Metabolic Panel (CMP) - kidney/liver function
  3. Blood Cultures - if signs of infection
  4. Initial Imaging
    - CXR - r/o pneumonia as a source of infection, assess for signs of mediastinal mass
    - CT/MRI Brain (without contrast) - if neurologic s/s are present or leukostasis is suspected
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19
Q

what additional work ups could you do for ALL

A
  1. Peripheral smear - pancytopenia with circulating lymphoblasts
  2. LDH - ↑ due to tissue destruction
  3. CT chest w contrast - assess lymphadenopathy, further assess mediastinal mass
  4. CSF Analysis
    - (+) lymphoblast cells - with spinal infiltration of dz.
  5. Flow-cytometry
    - ALL cells will express CD19 antigens and (+/-) CD10 antigens
    - A lack of mature T-cell markers most of the time
  6. Bone Marrow Aspiration & Biopsy
    - definitive diagnosis - > 20% lymphoblasts (WHO classification)
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20
Q

management for ALL in EVERY pt

A
  1. Refer to hematology/oncology
  2. Screen and treat for active infections in febrile patients
  3. ALL treatment begins with
    - induction chemotherapy
    - CNS prophylaxis followed by
    - post-remission therapy with or without stem cell transplantation
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21
Q

pancytopenia with circulating lymphoblasts on peripheral blood smear
is the hallmark of

A

ALL

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22
Q

what is Induction Chemotherapy and its goal?

A
  • Multi-drug chemotherapy over the course of 4-6 weeks
  • Initiated in the hospital
  • Complete remission achieved at 65-85%
  • Goal: remission induction
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23
Q

why is CNS prophylaxis vital for ALL

A

Intrathecal therapy is vital during all stages to prevent CNS recurrence

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24
Q

what is the ALL management of post-remission therapy for young pts with tolerable effects of chemo

A
  1. Consolidation/Intensification therapy
    - readministration of induction regimen or other high dose chemotherapeutic agents after normal hematopoiesis is restored
    - Duration: usually for 4-8 months
    - Goal: increases time of remission
    — Small numbers of leukemic lymphoblasts will remain in the bone marrow after induction therapy
    — Recurrence and therapeutic resistance can occur if therapy isn’t continued
  2. Maintenance/Continuation Therapy
    - A less intensive regimen weekly and/or daily for 2-3 years
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25
Q

what is the ALL management of post-remission therapy for older patient or unable to tolerate SE of chemo

A
  1. Allogeneic Stem Cell Transplantation
    - Stem cells are collected from a matching donor
    - Patient receives intensive chemotherapy prior to transplant to ensure destruction of as many cancer cells as possible.
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26
Q

what is the tx for leukostasis from ALL

A
  1. prophylaxis for tumor lysis syndrome
    - IV hydration
    - hypouricemic agents
  2. emergent chemotherapy
  3. leukapheresis
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27
Q

the cure rate of ALL is higher in what demographic of pts

A

children - 90%
adults - 50%

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28
Q

relapse of ALL is MC when

A

within first 2 years

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29
Q

if a pt presented with:
1. No chromosomal abnormalities
2. Age younger than 39 years
3. White blood cell (WBC) count of < than 30,000/μL
4. Complete remission within 4 weeks
what is their prognosis

A

Good prognostic criteria

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30
Q

if a pt that has ALL presented with:
1. Chromosomal abnormalities
2. Age older than 60 years
3. Precursor B-cell WBCs with WBC count > than 100,000/μL
4. Failure to achieve complete remission within 4 weeks
what is their prognosis

A

bad prognostic criteria

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31
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)
- dysfunction in the maturation of the B-cell
- results in B-cells that are unable to respond to immunologic stimulation

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32
Q

What is the MC form of leukemia in the US

A

Chronic Lymphocytic Leukemia (CLL)

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33
Q

CLL is MC in what demographic

A

elderly
90% occur after age 50; median age of onset is 72 y/o
white
male

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34
Q

clinical presentation of CLL

A
  1. slow onset - may be found incidentally
  2. lymphadenopathy - MC
  3. recurrent infections (pneumonia, HSV, HZV)
  4. hepatosplenomegaly (HSM) - upper abdominal discomfort/fullness and early satiety
  5. s/s of anemia/thrombocytopenia - found later in course of disease
35
Q

isolated absolute lymphocytosis is the hallmark for what malignancy?

A

CLL
persists for > 3 months

36
Q

if a peripheral smear showed (+) smudge cells and prolymphocytes, what type of malignancy do they have?

A

CLL

37
Q

what is the work up for CLL

A
  1. CBC
  2. peripheral smear - smudge cells + prolymphocytes
  3. CMP
  4. Peripheral blood via flow cytometry - confirms diagnosis
    - confirms the presence of various abnormal B-lymphocyte surface antigens
  5. Bone marrow aspiration/biopsy -
    not always required to make diagnosis
38
Q

what is the Staging CLL: Revised Rai staging system

A
  1. Low risk
    - Stage 0: Lymphocytosis aline
    — lymphocyte > 15000/μL, and > 40% lymphocytes in the bone marrow
  2. Intermediate risk
    - Stage I: Lymphocytosis - enlarged node(s)
    - Stage II: splenomegaly or hepatomegaly or both
  3. High risk
    - Stage III: Anemia (hemoglobin level < 11.0 g/dL)
  4. Stage IV: Thrombocytopenia (platelets < 100,000/μL)
39
Q

if a pt is considered at the “low risk” stage (Asx) of CLL, what is their management?

A

observation

40
Q

indication of tx for CLL is ?

A

symptomatic disease!!
1. Initial “high risk” stage
2. Progressive symptomatic “intermediate risk” staging
- worsening fatigue, symptomatic lymphadenopathy, anemia, or thrombocytopenia

otherwise, just observation!!

41
Q

tx/management for CLL (symptomatic)

A
  1. multidrug chemotherapy
    - Growth factors - utilized to decrease duration of neutropenia following chemotherapy
  2. Allogeneic stem cell transplant
    - reserved for patients who are not controlled with standard therapies
  3. Splenectomy - for refractory splenomegaly and pancytopenia
42
Q

complications with CLL

A
  1. Obstructive lymphadenopathy - compressing on internal organs
  2. Transformation into aggressive large cell lymphoma (Richter Syndrome)
    - weight loss, fevers, night sweats, muscle wasting, increasing hepatosplenomegaly and lymphadenopathy
  3. Autoimmune hemolytic anemia or thrombocytopenia
43
Q

what makes a good prognosis with CLL

A
  1. Low risk stage - average survival 10-15 years
  2. Intermediate/high risk
    - 2 year survival is > 90%,
    - 5-year survival is > 70%
44
Q

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

A

Acute Myelogenous Leukemia (AML)

45
Q

pathophys of Acute Myelogenous Leukemia (AML)

A
  1. rapid proliferation of myeloblast without differentiation
  2. resistant to apoptosis
  3. accumulation of myeloblasts in marrow, blood, spleen, liver
  4. reduction of normal hematopoiesis
46
Q

Myelodysplastic Syndrome (MDS) is the MC risk factor for what malignancy?

A

Acute Myelogenous Leukemia (AML)
it is a progressive cytopenias that occur over months to years

47
Q

causes of acute myelogenous leukemia (AML)

A
  1. myelodysplastic syndrome (MDS) (MC risk factor)
  2. congenital/genetic disorders
    - Trisomy 21
    - Bloom’s syndrome
    - Fanconi anemia
  3. environmental exposures
    - radiation, smoking, benzene⁴, soot, creosote, inks, dyes, tanning solution, coal dust
  4. Chemotherapeutic agents
48
Q

what is the epidemiology of acute myelogenous leukemia (AML)

A

~70y/o
white
males
developed countries

49
Q

if a pt is experiencing bone marrow failure, what might be the presentation

A

amenia, neutropenia, thrombocytopenia

50
Q

if a peripheral smear showed auer rod, what is that indicative of ?

A

acute myelogenous leukemia (AML)

51
Q

work up for AML

A
  1. CBC - decreased RBC, platelet and neutrophils
    - WBC may be normal, high or low
  2. Peripheral smear
    - predominantly blasts seen
    - Auer rod - eosinophilic needle-like inclusion in the cytoplasm of myeloblasts (Confirmation)
  3. CMP
  4. LDH
  5. BM - hypercellular, predominant blasts
  6. Flow Cytometry
  7. imaging
  8. LP - sx only
51
Q

work up for AML

A
  1. CBC
    - decreased RBC, platelet and neutrophils
    - WBC may be normal, high or low
  2. Peripheral smear
    - Auer rod
  3. CMP
  4. Blood Cultures - if signs of infection
  5. Lactic dehydrogenase level (LDH) -↑ with tissue destruction
  6. Bone Marrow - hypercellular, predominant blasts
  7. Flow Cytometry
  8. imaging studies
    - brain MRI/CT - indicate for neurologic symptoms (leukostasis)
  9. lumbar puncture - for sx pt ONLY
    - to look for leukemic (myeloblast) cells
    - only for sx pt only bc CNS infiltration is rare
52
Q

what differentiates AML from ALL by detecting myeloid antigens on cell surface

A

flow cytometry

53
Q

what confirms the presence of AML?

A

auer rods in peripheral smear

54
Q

management for acute myelogenous leukemia (AML)

A
  1. induction: multi-drug chemo
  2. post-remission therapy - standard chemo and/or stem-cell replacement
    - allogeneic preferred
  3. CNS involvement
    - intrathecal chemo w/wo local radiation
  4. leukostasis
    - emergent chemotherapy, leukapheresis and prevention of tumor lysis syndrome
55
Q
  • disorder characterized by dysregulated production and uncontrolled proliferation of mature and maturing granulocytes with normal differentiation
  • results from a single specific genetic mutation
A

Chronic Myeloid Leukemia (CML)

56
Q

the philadelphia chromosome is the hallmark for which disorder?

A

Chronic Myeloid Leukemia (CML)

57
Q

translocation t(9:22), also known as philadelphia chromosome, is a result in a genetic defect known as ?

A

bcr/abl
produces overactive tyrosine kinase (TK) activity
- TK is responsible for controlling cell growth, differentiation, metabolism and apoptosis

58
Q

epidemiology of CML

A

55y/o or middle aged

59
Q

etiology of CML

A

exposure of ionizing radiation
unknown otherwise

60
Q

what are the 3 phases of CML

A
  1. First phase: chronic - WBC’s differentiate
    - mostly found incidentally
    - chronic for 3-5 years if left untreated
  2. Second phase: accelerated - additional cytogenetic abnormalities occur
  3. Third “blast” phase: terminal blast crisis - immature myeloid cells rapidly proliferate (fatal)
61
Q

what is often the first symptom pts present with CML

A

fatigue and weight loss

62
Q

pruritus, diarrhea, flushing, gastrointestinal ulcers seen with elevated basophils due to overproduction of ?

A

histamine

63
Q

clinical presentation of CML

A
  1. fatigue and weight loss (MC)
  2. fever of unknown origin, bone pain, marked splenomegaly
  3. signs of acute leukemia
64
Q

what is the MC PE finding for CML

A

splenomegaly

65
Q

work up for CML

A
  1. CBC
    - chronic phase - granulocytosis with marked increase in mature neutrophils,
    - accelerated phase - reduced platelets, RBC
  2. Peripheral Smear
    - chronic phase - confirmation of CBC findings
    - accelerated phase - peripheral blast cells, promyelocytes visualized
  3. Leukocyte Alkaline Phosphatase (LAP)
    - low LAP = resistance to apoptosis
  4. CMP
  5. Bone Marrow Aspiration/Biopsy
    - hypercellular with increased granulocyte cells and their progenitors
    - Blast phase: >20% blasts
  6. Polymerase chain reaction (PCR)
    - can be performed on blood or marrow aspirate
    - identifies bcr/abl DNA segment (aka Philadelphia Chromosome)
66
Q

management of chronic phase with CML

A
  1. Standard therapy: tyrosine kinase inhibitor - single drug chemotherapy
    - targets abnormal bcr-abl protein
    - inhibits the cancer cells “addiction” to tyrosine kinase
    - results in CML cell death = healthy cells less affected compared to standard chemotherapy
67
Q

what are the goals and course expectations with management of standard therapy with CML

A
  1. Hematologic remission - ~3 months
    - normal CBC and physical exam
  2. Cytogenetic remission - 3-6 months
    - normal chromosome returns
  3. Molecular remission - ~12 months
    - negative PCR of bcr/abl
  4. Continue for 2 years
68
Q

tx for accelerated/blast phase of CML

A
  1. TKI + multidrug chemotherapy
  2. consider allogeneic stem cell transplantation
    - indicated if lack of response to TKI
69
Q

what are the signs of chronic turning into accelerated phase of CML?

A
  1. progressive splenomegaly
  2. inadequate decrease in granulocytes
  3. blast cells and promyelocytes in peripheral smear
  4. anemia, basophilia, thrombocytopenia
  5. new cytogenetic abnormalities or myelofibrosis (bone marrow bx)
70
Q

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

A

Multiple Myeloma (MM)

71
Q

pathogenesis of Multiple Myeloma (MM)
is preceded by what disorder?

A
  1. Monoclonal Gammopathy of Undetermined Significance (MGUS)
    - from abnormal plasma cell response to antigenic stimulation
72
Q

risk factors for multiple myeloma

A
  1. older age, immunosuppression, and environmental exposures
    - radiation, benzene, organic solvents, herbicides, and insecticides
73
Q

epidemiology of multiple myeloma

A
  1. ~65y/o
  2. Males
  3. african americans > caucasian/hispanics > asian/pacific islanders
74
Q

pathophys of multiple myeloma

A
  1. diminished hematopoiesis
  2. Neoplastic plasma cells are monoclonal = lack of adequate immunoglobulin response to infection
  3. increase osteoclastic activity, bone tumor formation and hypercalcemia
  4. M-proteins - harmful to the kidneys, nerves etc
  5. plasmacytomas
75
Q

if a pt presents with bone pain in the axial skeleton via back, what is the possible diagnosis?

A

multiple myeloma
Skeletal system - MC in the axial skeleton
- bone pain - MC presenting symptom in the back, hips, ribs

76
Q

clinical presentation of multiple myeloma

A
  1. Skeletal system - MC in the axial skeleton
    - bone pain - MC presenting symptom in the back, hips, ribs
    - spinal cord compression = back pain, weakness, numbness, or dysesthesias in the extremities
    - pathologic fracture
    - hypercalcemia (from skeletal destruction)
  2. Bone marrow
    - anemia, neutropenia, thrombocytopenia
  3. Renal
    - impaired function/failure, proteinuria, oliguria
  4. Neuro
    - radiculopathy or neuro deficits
    - peripheral nerve compression - MC median nerve (Carpal Tunnel Syndrome)
  5. Plasmacytomas
    - bleeding, obstruction
    - aerodigestive tract (MC), orbital, ear canal, cutaneous, gastric, rectal, prostatic, and retroperitoneal
77
Q

work up for multiple myeloma

A
  1. CBC - pancytopenia
  2. Peripheral blood smear
    - RBC rouleaux formation
  3. CMP
  4. Bone marrow aspiration/biopsy
    - monoclonal plasma cells
  5. Serum protein electrophoresis (SPEP)
    - (+) paraprotein (M-protein)
  6. 24 hour urine collection with urine protein electrophoresis (UPEP)
    - (+) Bence Jones protein
  7. Quantitative immunoglobulin levels
    - suppression immunoglobulins (IgG, IgA, IgM
  8. imaging
    - x-rays - complete skeletal survey - MC seen in skull, spine, long bones
    - CT scan - for monitoring
    - MRI spine - assess spinal nerve compression
78
Q

if a peripheral smear presents with RBC rouleux formation, what is it indicative of ?

A

multiple myeloma

79
Q

how can you determine the prognosis/”tumor burden” of multiple myeloma

A

beta-2 microglobulin
high = more burden

80
Q

which disease is incurable

A

multiple myeloma
Goal of treatment: prolong life, reduce complications and improve symptoms

81
Q

hem/onc will manage multiple myeloma how?

A
  1. Initial induction therapy - triple agent chemotherapy
  2. Chemotherapy is followed by autologous stem cell transplant (reserved for younger patients)
  3. Localized radiation - palliative for bone pain related to tumor formation
82
Q

complications with multiple myeloma? tx for each

A
  1. pathologic fractures
    - stabilize the bone and irradiate the lesion
  2. vertebral body collapse
    - vertebroplasty/kyphoplasty
  3. spinal cord compression
    - IV steroids
    - radiation
    - consult neuro for surgical intervention
  4. Bone disease/Hypercalcemia
    - bisphosphonates (for sx pts with good renal function)
  5. anemia
    - EPO for persistent anemia
  6. renal impairment
    - plasmapheresis to remove M-proteins
83
Q

what is the only known curative therapy for CLL

A

Allogeneic stem cell transplant

Hem/Onc (13 decks)

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