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DSE > Heme/onc part 2 > Flashcards

Heme/onc part 2 Flashcards

1
Q

What are more than 90% of the cases of CML due to?

A

Philadelphia chromosome
Translocation of the long arm of chromosome 22 and 9
Results in the shortening of chromosome 22

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2
Q

Presentation of CML

A

Usually discovered incidentally with leukocytosis on CBC
Many times pts will have splenomegaly on PE
CML
CML can transform into acute leukemia, will see an increase in blasts and new chromosomal abnormalities

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3
Q

S/sx of CML

A 
Low-grade fever
Splenomegaly
Decreased appetite
Chronic fatigue
Wt loss
Excessive sweating
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4
Q

CBC findings of CML

A

Elevated WBC (20,000-60,000)
Lymphocyte count will be nl
Will usually see an increase in other granulocytic cells (basophils, eosinophils, etc)

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5
Q

Other lab findings of CML

A

Could have low or absent ALP
Leukocyte alkaline phosphatase (LAP) stains very low to absent in most cells, resulting in a low score
BMBx: hypercellular (many myeloid cells seen, such as basophils, neutrophils, eosinophils)

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6
Q

Stage of CML

A

Dz is biphasic, sometimes triphasic
Chronic phase
Accelerated phase
Acute phase (blast phase)

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7
Q

Tx of CML

A

Gleevec is the 1st line chemotherapy. It targets the BCR/ABL gene by inducing apoptosis in cells pos for BCR/ABL
Usual dosage is 400 mg PO daily
Bone marrow transplant is currently the only known cure for the dz

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8
Q

When does ALL most commonly occur?

A

In childhood

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9
Q

S/sx of ALL

A 
Pallor
Fatigue
Joint pain
Fever
Wt loss
SOB
Persistent and frequent infections
Gingival bleeding
LAD and hepatosplenomegaly is common in ALL, as the lymphocytes are the involved cell lineage
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10
Q

PE of ALL

A 
Petechiae
Pallor
Gingival edema 
Chloroma (mass of leukemic cells found outside of the bone marrow)
LAD
Testicular edema
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11
Q

Lab evaluation of ALL

A

CBC will reveal elevated WBC with lymphocytosis
Bone marrow bx will reveal leukemic blasts
CXR may revieal a mediastinal mass
Cytogenetic studies
-Translocation 12:22; most common and favorable prognosis
-Philadelphia chromosome is unfavorable prognosis in ALL

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12
Q

Tx of ALL

A

Induction and consolidation chemotherapy
>50% of children will be cured with chemo
Prognosis depends upon WBC and age at the time of dx

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13
Q

Chronic lymphocytic leukemia

A

Most common adulthood leukemia

Affects the B-cell lineage

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14
Q

Characteristics of CLL

A

Most pts are asymptomatic when discovered
CBC reveals leukocytosis, with predominant lymphocytes
-Must persist for >3 mos for dx

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15
Q

Further evaluation of CLL

A

Peripheral blood flow cytometry: Usually express CD19
FISH for CLL and cytogenetic studies: evaluates chromosomes 6, 11, 12, 13, 14, 17, 18, or 19
-Used as a prognostic indicator
If significant LAD present, needle bx should be obtained

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16
Q

Peripheral blood smear of CLL

A

Smudge cells present

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17
Q

S/Sx of CLL

A 
Enlarged lymph nodes, liver, or spleen
Recurring infections
Loss of appetite or early satiety
Abnl bruising (late-stage sx)
Fatigue
Night sweats
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18
Q

Tx of CLL

A

Early stage CLL is monitored closely. Routine CBCs performed q3-6 mos. Flow cytometry is performed about q6 mos
-Absolute monoclonal B-lymphocyte count <5000
-Lymph nodes <1.5 cm
-No anemia or thrombocytopenia
As dz progresses, chemo is necessary
-FCR is generally 1st line tx in pts <70 yo
–Fludarabine, cyclophosphamide, Rituxan

19
Q

How are NHL tumors characterized?

A

By the level of differentiation, the size of the cell of origin, the origin cell’s rate of proliferation, and the histologic pattern of growth

20
Q

S/sx of NHL

A

B sx

  • Fever
  • Night sweats
  • Wt loss
21
Q

Low-grade NHL

A

Generally are felt to be slow-growing indolent diseases
Treatable with chemo but not curable
Pathologically described as grade 1 follicular or small lymphocytic lymphomas
Typically older pts- usually feel well, generally have no sx, and often have their dz found incidentally in the workup of another medical problem
Pts can often live up to 10+ years without requiring tx with chemo

22
Q

Intermediate grade NHL

A

More aggressive
Can be found in both young and older pts
They are usually grade 2 or 3 and consist of the more common subtypes
-Diffuse large B cell, mantle cell, and follicular lymphomas
Pts usually are experiencing some or all of the B sx
This is very curable, even in pts with stage 3 or 4 dz
Tx should be started ASAP

23
Q

High grade NHL

A

The most aggressive
Generally present in the younger, adolescent pt population
Pathologically, they include types such as Burkitt’s lymphoma, T-cell lymphomas, and anaplastic lymphomas
CNS involvement can commonly occur
Pts are often symptomatic at the time of presentation, and again, tx should start ASAP

24
Q

Tx of low-grade NHL

A

Chemo initiated with progressive dz and bulky LAD
Usually tx with CHOP: Cyclophosphamide, hydroxydaurnorubicin, Oncovin, prednisone
-Chemo treatments do not have to be as aggressive in this situation

25
Q

Adjuvant tx to chemo in NHL

A

Rituxan, which is targeted therapy to inactivate CD20+ lymphoma cells
Given with the second cycle
Often used once chemo is complete as maintenance therapy is given every 3-6 mos

26
Q

Tx of intermediate/high grade NHL

A

If early stage I or II dz, can sometimes treat with radiation only, depending on type of lymphomas and site of involvement
Otherwise, will need to treat with chemo +/- Rituxan

27
Q

Characteristics of Hodgkin’s lymphoma

A

Characterized by Reed-Sternberg cells
EBV is related in 40-50% of cases
Usually arises in one lymph node group and spreads to other nodes

28
Q

Subtypes of Hodgkin’s lymphoma

A

Nodular sclerosis
Mixed cellularity
Lymphocyte depleted
Lymphocyte rich

29
Q

Incidence of Hodgkin’s lymphoma

A

Bimodal incidance of Hodgkin’s with the first peak in pts in the twenties and the second peak after the age of 50
Most children with the disease are males

30
Q

Clinical features of Hodgkin’s lymphoma

A

`Painless cervical, supraclavicular, and mediastinal LAD
B sx
Nodular sclerosis subtype is usually seen in young women

31
Q

B sx

A 
Night sweats
Intense pruritis
Unexplained fever
Unintentional wt loss
Fatigue and generalized weakness
32
Q

Tx of Hodgkin’s lymphoma

A

Early stage disease I or II can often be treated with radiation alone or a combination of short course chemo and involved field radiation therapy
Stage III or IV dz is treated with chemo
If there is bulky mediastinal involvement, radiation to the chest may also be done

33
Q

What should be done for pts that relapse after first line Hodgkin’s lymphoma tx?

A

Additional chemo is recommended along with auto stem cell transplant which can also afford long term survival and possible cure

34
Q

What is the most common hematologic malignancy in the African American population?

A

Multiple myeloma

35
Q

What is oftentimes the first presentation of multiple myeloma?

A

Hypercalcemia

36
Q

Lab work for multiple myeloma

A

CBC
Chemistries- including total protein, albumin, and calcium levels
Immunofixation studies from serum and urine
Immunoglobulin levels- IgG
Skeletal survey
Bone marrow bx
Radiographic studies to evaluate specific areas of pain

37
Q

Lab findings of multiple myeloma

A

Routine CBC may often shown anemia and/or thrombocytopenia
High total protein level, high calcium and an elevated creatinine
Skeletal survey will show classic signs of lytic lesions in the bone
A bone marrow bx that shows more than 20% plasma cells is usually diagnostic for multiple myeloma

38
Q

Stage I multiple myeloma

A

Pts must have all of the following along with a bone marrow bx showing <20% plasma cells:

  • Hgb >10
  • Serum calcium nl or <12
  • Relatively small amount of monoclonal immunoglobulin in the serum or urine
  • Skeletal survey nl or with solitary plasmacytoma
39
Q

Stage II

A

Pts who qualify for neither stage I nor III

-A moderate number or myeloma cells are present

40
Q

Stage III multiple myeloma

A

Pts that have any one of the following along with bone marrow bx showing >20% plasma cells

  • Hgb <8.5
  • Serum calcium >12
  • High M component in serum
  • UPEP M component >12 gm/24 hr
  • Extensive lytic lesions
41
Q

Tx of multiple myeloma

A

High doses of decadron is still a mainstay of tx
-Combined either with oral biologic agents such as Thalidomide or Revlamid or intravenous chemo agents
Some pts may qualify for high dose chemo and autologous stem cell transplant, depending on their age and extent of dz

42
Q

Tx of hypercalcemia and lytic bone lesions in multiple myeloma

A

IV chemo agents and bisphosphonates

Usually Zometa 4 mg IV q4 wks

43
Q

Kyphoplasty in multiple myeloma

A

A procedure done by neurosurgeons or orthopedic surgeons to stabilize vertebral bodies that have been compromised

44
Q

F/u of multiple myeloma

A

Pts should be monitored every 2-3 mos with repeat lab work to determine response to dz

  • SPEP/IPEP, IgG
  • A repeat bone marrow bx would be indicated if new lab abnormalities were to develop

DSE (14 decks)

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