Lymphoreticular disorders Flashcards
_____________ are considered to be a group of related disorders caused by proliferation of Langerhans cells
Langerhans Cell Histiocytosis
T/F: Langerhans cells are related to monocytes and serve as antigen-presenting cells
true
what are the 3 clinical presentations of Langerhans cell Histiocytosis?
1) Acute disseminated histiocytosis
2) Chronic disseminated histiocytosis
3) Eosinophilic granuloma, which may be monostotic or polyostotic
what form of Langerhans Cell Histiocytosis is seen only in infants?
what are its symptoms?
Acute disseminated histiocytosis
- Skin rash, splenic and hepatic involvement
- Usually a very aggressive, malignant course
what form of Langerhans cell histiocytosis is found in older children?
Chronic disseminated histiocytosis
what are the symptoms of Chronic disseminated histiocytosis?
Classic triad of:
1) exophthalmos
2) diabetes insipidus
3) bone lesions (well-defined radiolucencies) is actually rather uncommon
Eosinophilic granuloma of bone affects what age groups?
Teenagers and adults
what are the 2 types clinical forms of Eosinophilic granulomas?
what is the radiographic presentation/appearance?
- May be polyostotic (teenagers) or monostotic (older adults)
- Well-defined, but non-corticated radiolucency
what are the Histopathologic Features of Langerhans cell histiocytosis?
Sheets of large, histiocytic-appearing cells (neoplastic Langerhans cells)
Variable numbers of eosinophils (“eosinophilic granuloma”)
What are the treatment options for Langerhans cell histiocytosis?
Acute – chemotherapy; poor prognosis
Chronic – radiation and/or chemotherapy; guarded prognosis
Eosinophilic granuloma – curettage or radiation; good prognosis
___________ are a group of hematologic malignancies characterized by tumor cells circulating in the blood
Leukemia
how is Leukemia classified/categorized?
A) Broadly divided into lymphocytic and myelomonocytic types
B) Further divided into acute and chronic forms
what are the symptoms of Leukemia?
A) Patients often present with signs and symptoms related to myelophthisic anemia (normal bone marrow cells replaced by leukemic cells)
B) Fatigue, SOB, pallor (decreased RBC’s)
C) Easy bruising (decreased platelets)
D) Infection (decreased WBC’s)
Oral involvement of Leukemia is most frequently seen in the ________________ forms
myelomonocytic
what lesion forms in the oral cavity as a result of Leukemia?
“granulocytic sarcoma”
A) Focal mass of leukemic cells may develop at one soft tissue site – “granulocytic sarcoma”
B) Diffuse gingival enlargement
how is leukemia diagnosed?
1) This diagnosis is usually based on finding increased numbers of atypical WBC’s in the circulation
2) Type of leukemia is determined by immunohistochemical/cytogenetic studies
3) If gingival enlargement caused by leukemia is biopsied, atypical cells can be characterized
Treatment options for Leukemia:
Chemotherapy
Bone marrow or stem cell transplantation
Targeted gene therapy – experimental, but promising
_________ are malignancies of Lymphoid cells
Lymphoma
how are Lymphomas classified?
Usually divided into Hodgkin and non-Hodgkin forms
1) Hodgkin lymphoma develops in the lymph nodes
2) Non-Hodgkin lymphoma may develop in nodes, soft tissue or bone
what age group/groups are at risk for Hodgkins Lymphomas
Bimodal age distribution:
2nd-3rd decades and > 50 yrs old
~9,000 cases in US in 2015
what are the clinical features/characteristics of Hodgkins Lymphoma?
Head and neck is common site of initial involvement
One or more rubbery-firm, enlarging, painless lymphadenopathy (cervical, supraclavicular)
Hodgkins Lymphoma treatment and prognosis:
give for both low and high stages
Based primarily on stage
1) Low stage (localized disease)
Chemotherapy and radiotherapy, ~100% 5 yr survival
2) High Stage (widespread disease)
Chemotherapy; 50% 5 yr survival
T/F: Non-Hodgkins lymphoma Generally effects an older age group of patients than with Hodgkin lymphoma
True
Characteristics of Non-Hodgkins Lymphoma:
how many new cases per year, where they occur
71,000 cases in US in 2015
Most present in the lymph nodes, but 30-40% are extra nodal
what are the oral presentations of Non-Hodgkins Lymphoma? What are the radiographic findings?
1) Oral involvement of soft tissue is usually seen as a diffuse mass of the soft palate or the buccal vestibule
2) Mandibular involvement may be associated with “numb chin” sign
3) “Moth-eaten” or ill-defined radiolucency seen radiographically
what is the treatment for Lymphomas?
Localized disease – radiation therapy, with or without chemotherapy
More generalized disease – multi-agent chemotherapy
Prognosis For Hodgkins, and Non-Hodgkins lymphomas:
Hodgkin lymphoma – good, with many series reporting a 95% 10-year survival rate, especially with low-stage disease
Non-Hodgkin lymphoma- much more variable prognosis, depends on the type, grade and stage of disease
what is the definition of “Plasmacytoma/Multiple Myeloma”?
Neoplastic proliferation of plasma cells, frequently arising in bone marrow
what are the 3 forms of Plasmacytomas?
1) Solitary plasmacytoma
2) Extramedullary plasmacytoma
3) Multiple myeloma
Plasmacytomas comprises nearly ____% of malignancies involving bone, excluding metastases
how many cases of Plasmacytomas are diagnosed each year?
50%
Over 26,000 cases annually in the US
Mean age of 65 years; rare
T/F: a black male is 4 times as likely to develop Plasmacytomas than a caucasian female
TRUE
2:1 male predilection; black males affected 2X more than white males
what are the symptoms of Plasmacytomas?
A) Initially present with bone pain
B) Myelophthisic anemia may cause fatigue, susceptibility to infection, fever, petechial hemorrhage
C) Renal failure due to circulating monoclonal immunoglobulin light chains
what causes Bence Jones protein (during multiple myeloma)?
Circulating monoclonal immunoglobulin light chains spill over into the urine
what is an Amyloid?
Circulating monoclonal immunoglobulin light chains deposited in tissues as acellular eosinophilic material – “amyloid”
Give the normal levels in the blood for the following compounds:
1) IgG
2) Kappa
3) Lambda
4) K/L ratio
1) IgG = 700-1,500
2) Kappa = 3.3-19.4
3) lambda = 7.5-26.3
4) K/L ratio = .26-1.65
what are the histological findings for a person with Plasmacytoma/Multiple myeloma?
1) Monotonous sheets of atypical plasma cells
2) Varying stages of differentiation
3) IHC studies show a monoclonal light chain restriction (kappa or lambda) in lesional cells
4) Similar finding of monoclonal gammopathy on serum protein immunoelectrophoresis
Treatment, prognosis, and survival rates for Plasmacytoma/Multiple Myeloma:
A) Treatment: chemotherapy (prednisone plus an alkylating agent); thalidomide; bone marrow transplant
B) Prognosis: poor to guarded
C) Overall 5 yr survival: ~ 46%
