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Medical Recall - Oncology > Lymphoproliferative disorder > Flashcards

Lymphoproliferative disorder Flashcards

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USMLE® Step 1 flashcards
1
Q

What is a lymphoproliferative disorder?

A

A clonal neoplastic proliferation of lymphocytes

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2
Q

What are the lymphoprolif- erative disorders?

A
  • CLL
  • hairy cell leukemia
  • LGL leukemia
  • lymphoma (e.g., Hodgkin disease and NHL)
  • plasma cell dyscrasias (e.g., MM and WM)
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3
Q

What is the most common form of leukemia in Western countries?

A

CLL comprises 30% of all cases of adult leukemia, with an annual incidence of 4 in 100,000 persons.

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4
Q

What is CLL?

A
  • CLL = Chronic lymphocytic leukemia
  • A neoplastic proliferation with accumulation of immune-incompetent lymphocytes within the bone marrow, peripheral blood, and lymphoid organs
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5
Q

What are the morphologic characteristics of CLL?

A

Small, mature lymphocytes with clumped chromatin and scant cytoplasm

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6
Q

What is the median age of onset of CLL?

A

65 years

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7
Q

What are the symptoms of CLL?

A

Up to 70% of persons with CLL are asymptomatic at diagnosis.

  • Generalized lymphadenopathy, fever, night sweats, weight loss, easy fatigability, weakness, and increased bleeding are common complaints.
  • Frequent infections and exaggerated responses to insect bites are occasionally noted.
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8
Q

How is the diagnosis of CLL made?

A

An increase in the absolute number (>5,000) of lymphocytes in the peripheral blood, which, on the peripheral smear, appear as small, mature lymphocytes.

Flow cytometry shows a monoclonal population that coexpresses CD19 and CD5.

Frequently, there is lymphadenopathy, splenomegaly, and bone marrow infiltration, making it difficult to distinguish CLL from its lymphomatous counterpart, SLL.

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9
Q

What is the staging system for CLL?

A

The Rai system is the one most commonly used:

  • Stage 0—lymphocytosis alone
  • Stage I—lymphocytosis with lymphadenopathy
  • Stage II—lymphocytosis with splenomegaly or hepatomegaly
  • Stage III—lymphocytosis with anemia
  • Stage IV—lymphocytosis with anemia and thrombocytopenia
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10
Q

What are the median survival times for Stage 0?

A

15 years

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11
Q

What are the median survival times for Stage I?

A

9 years

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12
Q

What are the median survival times for Stage II?

A

5 years

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13
Q

What are the median survival times for Stage III?

A

2 years

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14
Q

What are the median survival times for Stage IV?

A

2 years

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15
Q

What specific hematologic complication can be associated with CLL?

A

AIHA (autoimmune hemolytic anemia)

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16
Q

What is the treatment for CLL?

A
  • Observation if the patient is asymptomatic.

- Oral alkylating agents or fludarabine are commonly used in symptomatic patients.

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17
Q

What are the indications for the treatment for CLL?

A

Some indications include the presence of autoimmune hemolytic anemia, autoimmune thrombocytopenia, bulky lymphadenopathy, progressive hyperlym- phocytosis, and frequent bacterial infec- tions, but the exact time to initiate treatment is an area of much debate.

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18
Q

Chronic myleogenous leukemia

A

(Please see Chapter 6, “Myeloproliferative Diseases” section.)

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19
Q

What is hairy cell leukemia?

A

A fairly uncommon chronic lymphoprolif- erative disorder with a clonal neoplastic proliferation of a lymphocyte that is related to memory B cells, activated B cells, and preplasma cells

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20
Q

What is the morphologic appearance of hairy cell leukemia?

A
  • A large lymphocyte with an eccentric nucleus (looks like a fried egg), with delicate, lacy chromatin and small nucleoli as well as abundant grayish-blue cytoplasm with fine irregular filamentous projections.
  • These projections can be very hard to see on a peripheral smear.
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21
Q

What is the median age of onset of hairy cell leukemia?

A

50–55 years of age.

Not reported in children or teens

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22
Q

For hairy cell, what is the male to female ratio?

A

There is a male to female ratio of 4:1.

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23
Q

What ethnic group has a higher preponderance of hairy cell?

A

More common in Ashkenazi Jewish males

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24
Q

What are the presenting symptoms of hairy cell leukemia?

A

Weakness, weight loss, recent pyogenic infection, or symptoms attributable to splenomegaly

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25
Q

What are the physical findings of hairy cell leukemia?

A

Splenomegaly occurs in 80% of cases.

Rarely, patients have lymphadenopathy or hepatomegaly.

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26
Q

Do patients with hairy cell leukemia have an elevated WBC count?

A

Not usually; 80% of patients have leukopenia.

Pancytopenia is a common presentation.

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27
Q

What is unusual about the bone marrow aspirate?

Hairy cell leukemia

A

The bone marrow is often difficult to aspirate, resulting in a “dry tap.”

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28
Q

How is the diagnosis of hairy cell leukemia made?

A
  • By the appropriate clinical scenario and by “hairy cells” seen on peripheral smear or bone marrow examination.
  • A special stain called “TRAP” (tartrate-resistant acid phosphatase) is confirmatory, as is flow cytometric data.
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29
Q

What is the treatment for hairy cell leukemia?

A
  • The nucleoside analogs cladribine and pentostatin are used with good response rates, which are durable for most patients.
  • Because the hairy cells express CD20, the anti-CD20 antibody, rituximab, is also gaining some popularity.
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30
Q

What is an LGL?

A

On peripheral smear, an LGL appears as a large lymphocyte with abundant pale cytoplasm with prominent azurophilic granules.

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31
Q

What are the 2 types of LGL syndromes?

A

T cell and NK cell

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32
Q

How is the diagnosis of LGL syndrome made?

A

Increase in LGLs on peripheral smear, which, by flow cytometry, shows clonality with the appropriate phenotype (CD3, CD56)

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33
Q

What is the presentation of T-cell LGL?

A

Chronic, sometimes severe, neutropenia with frequent bacterial infections. Infil- tration of the spleen, bone marrow, and liver is not uncommon. Interestingly, 25% of cases are associated with rheumatoid arthritis, making it difficult to distinguish from Felty syndrome.

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34
Q

What is the presentation of NK-cell LGL?

A
  • Usually, an acute clinical course involving fever and B symptoms (see the following text).
  • Anemia and thrombocytopenia are more common than with T-cell LGL.
  • Massive hepatosplenomegaly, lymph node involvement, and GI symptoms are common.
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35
Q

What is lymphoma?

A

A heterogeneous group of malignancies of lymphocytes that usually arise in lymph nodes but may originate in any organ

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36
Q

What are the 2 broad categories of lymphomas?

A
  • Hodgkin disease

- NHL

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37
Q

How do NHL and Hodgkin disease differ in their natural history?

lymphoma

A
  • NHL commonly presents with diffuse disease.

- Hodgkin disease presents more commonly with localized disease.

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38
Q

What are the typical presenting symptoms of lymphoma?

A
  • Persistent painless adenopathy and B symptoms.
  • Generalized pruritus with unexplained lymphadenopathy and pain in lymph nodes after alcohol ingestion are highly suggestive of Hodgkin disease.
  • Lymphomas can present with symptoms attributable to enlarged lymph nodes anywhere in the body.
39
Q

What are B symptoms?

lymphoma

A

Fever (>38°C x 3 consecutive days), drenching night sweats, and weight loss (>10% of body weight over 6 months)

40
Q

What are the typical physical findings in lymphoma?

A

Lymphadenopathy and hepatosplenomegaly are the most predominant findings.

41
Q

What are the typical presenting symptoms for CNS lymphoma?

A
  • Headache
  • altered mental status
  • focal neurologic findings
42
Q

What is Waldeyer’s ring?

lymphoma

A

The ring of lymphoid tonsillar tissue in the oropharynx.

43
Q

What are the typical presenting symptoms for lymphoma involving Waldeyer’s ring?

A
  • Sinusitis

- earaches

44
Q

What are the typical presenting symptoms of mediastinal lymphomas?

A
  • Cough
  • shortness of breath
  • chest pain
  • hemoptysis
45
Q

What are the typical presenting symptoms of abdominal lymphomas?

A
  • Abdominal pain
  • nausea
  • vomiting
  • back pain
46
Q

What are the WHO histologic subtypes of Hodgkin disease?

A
  • Nodular lymphocyte predominant

- Classic Hodgkin lymphoma

47
Q

What is classic Hodgkin lymphoma divided into?

A
  • nodular sclerosing
  • mixed cellularity
  • lymphocyte rich
  • lymphocyte depleted
48
Q

What is the name of the pathologic cell in Hodgkin disease?

A

Reed-Sternberg cell

49
Q

What is the age distribution of Hodgkin disease?

A

There is a bimodal age distribution:

  • with a young adult form peaking from age 16 to 34 years
  • and an older adult form peaking from age 55 to 74 years.
50
Q

What is the staging system for Hodgkin disease?

A

The Modified Ann Arbor staging system is used.

51
Q

For Hodgkin disease, describe the criteria for Stage I?

A

Involvement of a single lymph node region or single extralymphatic site (IE)

52
Q

For Hodgkin disease, describe the criteria for Stage II?

A

Involvement of 2 or more lymph node regions on the same side of the diaphragm, or localized extranodal extension plus 1 or more nodal regions (IIE)

53
Q

For Hodgkin disease, describe the criteria for Stage III?

A

Involvement of lymph node regions on both sides of the diaphragm, may be accompanied by localized extralymphatic extension (IIIE) or splenic involvement (IIIS)

54
Q

For Hodgkin disease, describe the criteria for Stage IV?

A

Diffuse or disseminated involvement of 1 or more extralymphatic organs or tissues with or without associated lymph node involvement

55
Q

What are the common sites of extranodal Hodgkin disease?

A

Liver, lung, bone marrow, bone, and skin

56
Q

What are the standard tests used to work up Hodgkin disease?

A
  • H&P
  • CBC
  • chemistry panel; liver function tests
  • ESR
  • CT scan of the chest, abdomen, and pelvis and sometimes neck
  • and bilateral bone marrow biopsies (only for advanced disease, cytopenias or B symptoms).
  • PET scans are gaining wider use.
57
Q

What is the treatment for Hodgkin disease?

A

Combination chemotherapy with or without radiation therapy

58
Q

What are some of the long- term complications of Hodgkin disease?

A

See the following text.

59
Q

What risk factors are associated with the development of NHL?

A
  • Exposure to herbicides/pesticides by agricultural workers
  • autoimmune diseases
  • congenital immunodeficiency states
  • Helicobacter pylori
  • AIDS
  • EBV
  • HTLV-1
60
Q

What are the broad clinical subgroups of NHL?

A
  • The histologic classification of NHL is complex and controversial, a more useful subgrouping is by either indolent or aggressive clinical behavior.
  • The most common indolent lymphoma is follicular and the most common aggressive lymphoma is diffuse large B cell.
61
Q

What is the clinical behavior of indolent NHL?

A
  • Typically indolent.
  • Not curable.
  • Median survival is 7 years, with some patients having extremely prolonged survival with observation alone.
62
Q

What is the typical clinical behavior of aggressive NHL?

A
  • Rapid progression and death, if not treated.

- Cure is achieved in 40%–50% of patients treated with standard combination chemotherapy regimens.

63
Q

What is the staging system for NHL?

A

The Modified Ann Arbor staging system

-> see under section “Hodgkin Disease”

64
Q

What factors are crucial for choosing therapy for NHL?

A
  • Histologic subtype and stage.

- The International Prognostic Index (IPI) is also commonly used.

65
Q

What risk factors are assessed in the IPI?

NHL

A

Think “APLES”:

  • Age > 60 years
  • Performance status ECOG 2–4 (see the preceding text)
  • LDH > normal level
  • Extranodal sites of disease (>1)
  • Stage II–IV disease
66
Q

What is the treatment for indolent NHL?

A
  • Observation if the patient is asymptomatic.
  • Oral chemotherapy can be effective as initial treatment for symptomatic disease.
  • Localized symptomatic NHL can be effectively palliated with radiation therapy.
  • Combination chemotherapy and single-agent nucleoside analogs are effective but not proven to be better than oral regimens as initial treatment.
67
Q

What monoclonal antibody therapy is used in the treat- ment of NHL?

A
  • Rituximab.
  • Rituximab targets the CD20 receptor.
  • It is used with chemotherapy in the initial treatment and as single agent in the maintenance therapy.
68
Q

What is the treatment for aggressive NHL?

A

Combination chemotherapy with rituximab (if CD20 +) carries a 60%–70% complete response rate, but recurrence occurs in approximately half of patients within 1–2 years.

69
Q

What is a plasma cell dyscrasia?

A

An abnormal proliferation of plasma cells that usually secrete a monoclonal immunoglobulin

70
Q

What is the monoclonal protein associated with plasma cell dyscrasias?

A

An immunoglobulin.

  • The most common is IgG, with IgA being a close second. - All of the following may be present:
    • IgM, kappa light chains
    • lambda light chains
    • IgD
    • IgE monoclonal proteins.
71
Q

What are the major plasma cell dyscrasias?

A
  • MGUS
  • MM
  • WM
  • amyloidosis
72
Q

What are the diagnostic criteria for an MGUS?

plasma cell dyscrasias

A

An MGUS must have:

  • Serum M protein <3.5 g/dL (IgG) or <2 g/dL (IgA) <10% plasma cells in the bone marrow
  • Urine light chains <1 g/24 h
  • No lytic bone lesions
73
Q

What is the incidence of MGUS?

plasma cell dyscrasias

A

Increases with age with 1% at age 25 and 10% at age 80

74
Q

What is the rate of progression of MGUS to MM?

plasma cell dyscrasias

A

1%–3% per year

75
Q

What is MM?

A
  • Multiple Myeloma

- A malignant proliferation of terminally differentiated B lymphocytes (plasma cells) resulting in end-organ damage

76
Q

What is the incidence of MM?

A

1% of all cancers and 10% of all hematologic malignancies

77
Q

What is the median age for the development of MM?

A

The median age is 65 years.

78
Q

What are the risk factors for the development of MM?

A
  • The disease is much more common in African Americans.

- Exposures to radiation, alkylating agents, asbestos, pesticides, HHV-8, and SV40 have been implicated as risk factors.

79
Q

Do all patients with MM have an M protein in the serum?

A

No.
- Only 80% of patients have an M protein in the serum; 20% have only light chains, which can be measured in a 24-hour urine collection or with a serum light-chain assay.

  • These light chains will NOT be picked up on a regular urinalysis.
  • Approximately 1% of patients with MM are termed “nonsecretors” and have no identifiable M protein.
80
Q

In nonsecretors, where is the immunoglobulin?

A

On staining of the plasma cells, the protein is shown to be within the cytoplasm, but the plasma cells cannot excrete the immunoglobulin molecule.

81
Q

What are the clinical manifestations of MM?

A
  • Osteolytic bone lesions with an associated risk of pathologic fractures of the long bones, vertebrae, pelvis, and ribs
  • Anemia and pancytopenia
  • Hypercalcemia
  • Renal insufficiency
  • Recurrent bacterial and viral infections (due to hypogammaglobulinemia)
  • Hyperviscosity
  • Peripheral neuropathies
  • Spinal cord compression (not common)
  • Myelomatous meningitis (not common)
82
Q

What are the causes of renal insufficiency or failure in patients with MM?

A

Amyloidosis, light-chain deposition disease, hypercalcemia, hyperuricemia with uric acid crystallization within the collecting ducts and tubules, and plasma cell infiltration

83
Q

What are the major criteria for the diagnosis of MM?

A
  • M protein >3.5 g/dL (IgG) or >2 g/dL (IgA)
  • Marrow plasmacytosis >30%
  • Plasmacytoma
84
Q

What are the minor criteria for the diagnosis of MM?

A
  • Lytic bone lesions
  • Marrow plasmacytosis 10%–30%
  • M protein less than defined previously
  • Decreased levels of normal immunoglobulins
85
Q

How is the diagnosis of MM made?

A

Need 1 major criterion plus 1 minor criterion, or 3 minor criteria

86
Q

What tests should be done in the workup of a patient suspected to have MM?

A

CBC, chemistry panel to include uric acid, quantitative immunoglobulins, serum protein electrophoresis, 24-hour urine collection for protein electrophoresis, and Beta2-microglobulin, total body skeletal survey, and bone marrow aspirate and biopsy

87
Q

What is the treatment for MM?

A
  • Alkylating agent chemotherapy with corticosteroids was the traditional mainstay.
  • Now, thalidomide and its derivatives are used in the initial treatment of MM.
88
Q

How is solitary plasmacytoma treated?

MM

A

Can generally be treated with localized radiation therapy for cure; however, 80% of patients recur with MM.

89
Q

WM = ?

A

waldenström macroglobulinemia

90
Q

What is the characteristic cell type in WM?

A

A mature lymphocyte with plasma cell features that produces IgM. The disease is commonly referred to as a “lymphoplasmacytic disorder.”

91
Q

What are the common presenting symptoms of WM?

A

Weakness, fatigue, oral and nasal mucocutaneous bleeding, symptoms attributable to splenomegaly, and symptoms attributable to hyperviscosity

92
Q

What are the symptoms attributable to hyperviscosity?

A
  • Headache
  • blurred vision
  • paresthesias
  • focal or diffuse weakness
  • deafness
  • symptoms secondary to congestive heart failure
93
Q

What are the important physical findings of WM?

A

Hepatosplenomegaly and lymphadenopathy

94
Q

What is the treatment for WM?

A
  • Oral alkylating agents can be used.
  • Newer therapies include nucleoside analogs (fludarabine, cladribine), monoclonal antibodies (anti-CD20), and thalidomide and high-dose chemotherapy followed by stem cell transplantation for younger patients.

Medical Recall - Oncology (6 decks)

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