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AU15 - Oral Pathology Exam 2 > Lymphoreticular Disorders > Flashcards

Lymphoreticular Disorders Flashcards

1
Q

What oral path lesion is considered to be a group of related disorders caused by proliferation of Langerhans cells?

A

langerhans cell histiocytosis

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2
Q

What are the 3 clinical presentations of langerhans cell histiocytosis?

A
  • acute disseminated histiocytosis
  • chronic disseminated histiocytosis
  • eosinophilic granuloma
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3
Q

True or false: Langerhans cells are related to monocytes and serve as antigen-presenting cells.

A

true

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4
Q

What is another name for acute disseminated histiocytosis? What age group is most affected? What is the clinical appearance?

A
  • Letterer-Siwe disease
  • infants
  • skin rash, splenic, and hepatic involvement; usually very aggressive malignant course
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5
Q

What is another name for chronic disseminated histiocytosis? What age group is most affected? What is the clinical appearance?

A
  • Hand-Schuller-Christian disease
  • older children
  • classic triad of: exophthalmous, diabetes insipidus, and bone lesions (well-defined radiolucencies); triad is actually rather uncommon
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6
Q

What age group is most affected by eosinophilic granuloma (langerhans cell histiocytosis)? What are the 2 presentations? What is its clinical appearance?

A
  • teenagers and adults
  • polyototic (teenagers) or monostotic (adults)
  • well-defined but not corticated radiolucency
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7
Q

What is the most common clinical presentation of langerhans cell histiocytosis?

A

eosinophilic granuloma

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8
Q

Describe the histological appearance of langerhans cell histiocytosis.

A
  • sheets of large, histiocytic-appearing cells (neoplastic langerhans cells)
  • variable numbers of eosinophils
  • Note: cells have indented kidney-shaped nucleus with eosinophils mixed in; berbeck granules are rod-shaped and characteristic of langerhans
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9
Q

What is the treatment and prognosis of each clinical presentation of langerhans cell histiocytosis?

A
  • ACUTE: chemotherapy; poor prognosis
  • CHRONIC: radiation and/or chemotherapy; guarded prognosis
  • EOSINOPHILIC GRANULOMA: curettage or radiation; good prognosis
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10
Q

What are the 4 labels for leukemia?

A
  • lymphocytic and myelomonocytic

- acute and chronic

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11
Q

Describe the clinical appearance of leukemia.

A
  • patients often present with signs and symptoms related to myelophthisic anemia (normal bone marrow cells replaced by leukemic cells)
  • fatigue, shortness of breath, pallor (decreased RBC’s)
  • easy bruising (decreased platelets)
  • infection (decreased WBC’s)
  • focal mass of leukemic cells may develop at one soft tissue site (“granulocytic sarcoma”)
  • diffuse gingival enlargement
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12
Q

Oral involvement is most frequently seen in what form of leukemia?

A

myelomonocytic form

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13
Q

How is leukemia usually diagnosed?

A
  • diagnosis is usually based on finding increased numbers of atypical WBC’s in the circulation
  • type of leukemia is determined by immunohistocehmical and cytogenic studies
  • if gingival enlargmenet caused by leukemia is biopsied, atypical cells can be characterized
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14
Q

What is the recommended treatment for leukemia?

A
  • chemotherapy
  • bone marrow or stem cell transplantation
  • targeted gene therapy (experimental but promising)
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15
Q

What are the 2 types of lymphoma? What is the difference between them?

A
  • hodgkin lymphoma (develops in the lymph nodes)

- non-hodgkin lymphoma (develops in nodes, soft tissue, or bone)

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16
Q

Which type of lymphoma is the “good lymphoma”?

A

hodgkin lymphoma

17
Q

What age group is most affected by hodgkin lymphoma? What is the site of initial involvement?

A
  • bimodal age distribution (2nd and 3rd decades and also >50 years old)
  • head and neck
18
Q

Describe the clinical appearance of Hodgkin lymphoma. The histological appearance?

A
  • one or more rubbery-firm, enlarging, painless lymphadenopathy (cervical, supraclavicular)
  • Note: Reed-Sternberg cells are visible; large atypical cells with some bi-nucleated cells (“owl-eye appearance”)
19
Q

What is the treatment and prognosis for hodgkin lymphoma?

A
  • LOW STAGE (localized): chemotherapy and radiotherapy; approximately 100% 5-year survival
  • HIGH STAGE (widespread): chemotherapy; 50% 5-year survival
  • low risk for development of secondary treatment-related acute leukemia
20
Q

What age group is usually affected by non-hodgkin lymphoma? What sites are most involved?

A
  • generally older age group of patients than with Hodgkin lymphoma
  • most present in lymph nodes but 30-40% are extranodal
21
Q

Which is more common: hodgkin or non-hodgkin lymphoma?

A

non-hodgkin lymphoma

22
Q

Describe the clinical appearance of non-hodgkin lymphoma.

A
  • diffuse mass of the soft palate or the buccal vestibule
  • “numb chin” sign
  • “moth-eaten” or ill-defined radiolucency
23
Q

What is the recommended treatment for non-hodgkin lymphoma?

A
  • LOCALIZED: radiation therapy +/- chemotherapy

- GENERALIZED: multi-agent chemotherapy

24
Q

What is the difference in the prognosis of hodgkin and non-hodgkin lymphoma?

A
  • HODGKIN: good; 95% 10-year survival

- NON-HODGKIN: more variable; depnds on type, grade, and stage

25
Q

What is the difference between plasmacytoma and multiple myeloma?

A
  • plasmacytoma: localized

- multiple myeloma: generalized

26
Q

What are the 3 different types of plasmacytoma/multiple myeloma?

A
  • solitary plasmacytoma
  • extramedullary plasmacytoma
  • multiple myeloma
27
Q

Plasmacytoma/multiple myeloma comprises nearly __% of malignancies involving bone, excluding metastases.

A

50%

28
Q

What is the age range most affected by plasmacytoma? What gender? What ethnicity?

A
  • mean age is 65 years; rare to be
29
Q

What are the signs and symptoms of plasmacytoma?

A
  • bone pain initially
  • myelophthisic anemia (may cause fatigue, susceptibility to infeciton, fever, petechial hemorrhage)
  • renal failure due to circulating monoclonal immunoglobulin light chains
  • circulating monoclonal immunoglobulin light chains may spill over into urine (Bence Jones protein)
  • may be deposited in tissues as acellular eosinophilic material (“amyloid”)
30
Q

When looking at a blood work-up of a patient with plasmacytoma, what do you expect to see?

A

high kappa or lambda value

31
Q

Describe the histology of plasmacytoma.

A
  • monotonous sheets of atypical plasma cells
  • varying stages of differentiation
  • monoclonal light chain restriction (kappa or lambda) in lesional cells
  • monoclonal gammopathy on serum protein immunoelectrophoresis
  • Note: congo red stain will stain amyloid
32
Q

What is the treatment of plasmacytoma? Prognosis?

A
  • chemotherapy (prednisone + alkylating agent), thalidomide, bone marrow transplant
  • poor to guarded, 5-year survival is 46%

AU15 - Oral Pathology Exam 2 (4 decks)

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