Lymphoreticular Disorders Flashcards
What oral path lesion is considered to be a group of related disorders caused by proliferation of Langerhans cells?
langerhans cell histiocytosis
What are the 3 clinical presentations of langerhans cell histiocytosis?
- acute disseminated histiocytosis
- chronic disseminated histiocytosis
- eosinophilic granuloma
True or false: Langerhans cells are related to monocytes and serve as antigen-presenting cells.
true
What is another name for acute disseminated histiocytosis? What age group is most affected? What is the clinical appearance?
- Letterer-Siwe disease
- infants
- skin rash, splenic, and hepatic involvement; usually very aggressive malignant course
What is another name for chronic disseminated histiocytosis? What age group is most affected? What is the clinical appearance?
- Hand-Schuller-Christian disease
- older children
- classic triad of: exophthalmous, diabetes insipidus, and bone lesions (well-defined radiolucencies); triad is actually rather uncommon
What age group is most affected by eosinophilic granuloma (langerhans cell histiocytosis)? What are the 2 presentations? What is its clinical appearance?
- teenagers and adults
- polyototic (teenagers) or monostotic (adults)
- well-defined but not corticated radiolucency
What is the most common clinical presentation of langerhans cell histiocytosis?
eosinophilic granuloma
Describe the histological appearance of langerhans cell histiocytosis.
- 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
What is the treatment and prognosis of each clinical presentation of langerhans cell histiocytosis?
- ACUTE: chemotherapy; poor prognosis
- CHRONIC: radiation and/or chemotherapy; guarded prognosis
- EOSINOPHILIC GRANULOMA: curettage or radiation; good prognosis
What are the 4 labels for leukemia?
- lymphocytic and myelomonocytic
- acute and chronic
Describe the clinical appearance of leukemia.
- 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
Oral involvement is most frequently seen in what form of leukemia?
myelomonocytic form
How is leukemia usually diagnosed?
- 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
What is the recommended treatment for leukemia?
- chemotherapy
- bone marrow or stem cell transplantation
- targeted gene therapy (experimental but promising)
What are the 2 types of lymphoma? What is the difference between them?
- hodgkin lymphoma (develops in the lymph nodes)
- non-hodgkin lymphoma (develops in nodes, soft tissue, or bone)
Which type of lymphoma is the “good lymphoma”?
hodgkin lymphoma
What age group is most affected by hodgkin lymphoma? What is the site of initial involvement?
- bimodal age distribution (2nd and 3rd decades and also >50 years old)
- head and neck
Describe the clinical appearance of Hodgkin lymphoma. The histological appearance?
- 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”)
What is the treatment and prognosis for hodgkin lymphoma?
- 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
What age group is usually affected by non-hodgkin lymphoma? What sites are most involved?
- generally older age group of patients than with Hodgkin lymphoma
- most present in lymph nodes but 30-40% are extranodal
Which is more common: hodgkin or non-hodgkin lymphoma?
non-hodgkin lymphoma
Describe the clinical appearance of non-hodgkin lymphoma.
- diffuse mass of the soft palate or the buccal vestibule
- “numb chin” sign
- “moth-eaten” or ill-defined radiolucency
What is the recommended treatment for non-hodgkin lymphoma?
- LOCALIZED: radiation therapy +/- chemotherapy
- GENERALIZED: multi-agent chemotherapy
What is the difference in the prognosis of hodgkin and non-hodgkin lymphoma?
- HODGKIN: good; 95% 10-year survival
- NON-HODGKIN: more variable; depnds on type, grade, and stage
What is the difference between plasmacytoma and multiple myeloma?
- plasmacytoma: localized
- multiple myeloma: generalized
What are the 3 different types of plasmacytoma/multiple myeloma?
- solitary plasmacytoma
- extramedullary plasmacytoma
- multiple myeloma
Plasmacytoma/multiple myeloma comprises nearly __% of malignancies involving bone, excluding metastases.
50%
What is the age range most affected by plasmacytoma? What gender? What ethnicity?
- mean age is 65 years; rare to be
What are the signs and symptoms of plasmacytoma?
- 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”)
When looking at a blood work-up of a patient with plasmacytoma, what do you expect to see?
high kappa or lambda value
Describe the histology of plasmacytoma.
- 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
What is the treatment of plasmacytoma? Prognosis?
- chemotherapy (prednisone + alkylating agent), thalidomide, bone marrow transplant
- poor to guarded, 5-year survival is 46%
