2. WBC Neoplasms Flashcards
What are the 3 Etiologies of Leukemia?
- Genetic Factors
- Some Syndromes
- Gene translocation (philadelphia translocation)
- Genetic alterations of stem cells
- Myelodysplasia
- Environmental
- Pesticides, Benzene
- Ionizing Radiation (WWII)
- Viruses
- HTLV-1
What is the Pathophysiology of Leukemia?
- Replacement of normal hematopoietic cells in bone marrow
- Derived from a single transformed cell exhibiting clonal growth
What are the General Symptoms of Leukemia? (3)
-
Fatigue, SOB, Pallor
- Decreased RBCs
-
Easy Bruising
- Decreased Platelets
-
Infection
- Decreased WBCs
What does Leukemia cause? (3)
-
Neutropenia
- __Bacterial, viral and fungal infections
- Oral ulcers, herpes, candida
-
Thrombocytopenia
- Gingival bleeding
- Palatal petechiae
-
Myelophthisic Anemia
- Lack RBCs - hypoxia
- Causes Extramedullary Hematopoiesis
What are the Oral Presentations and in what type of Leukemia are they normally seen?
Myelomonocytic forms of leukemia
-
Focal Soft Tissue Tumor Mass
- Myeloid Sarcoma or Granulocytic Sarcoma
- Firm, redish/purple mass
- ~ Kaposi sarcoma
-
Diffuse Gingival Enlargement
- Not just gm, but down in the vestibule
-
Mimic Periapical Ds
- RL in bone
How is Leukemia usually diagnosed?
- Increased #s of atypical WBCs in blood and bone marrow
- Type is determined by:
- Immunohistochemical and Cytogenetic studies
What are the Clinical Features of Hodgkin Lymphoma? (4)
- Teenagers/young adults OR >50yrs
- EBV linked to a significant number of lesions
- 1 or more rubbery-firm, enlarging, non-tender lymph nodes
-
Rare intraorally
- NHL is in the oral cavity
What are the Constitutional Symptoms of Hodgkin Lymphoma? (4)
- Progressive Fever OR Pel-Ebstein Fever
- Night sweats
- Weight loss
-
Generalized Pruritis (itchiness all over body)
- UNIQUE SYMPTOM
What does the Diagnosis of Hodgkin Lymphoma depend on?
-
Reed-Sternberg cells
- Large, either multinucleated, or have a bilobed nucleus “owl eye”
What is the Treatment of Hodgkin Lymphoma?
- ABVD
- Adriamycin
- Bleomycin
- Vinblastine
- Dacarbazine
What is the prognosis of Hodgkin Lymphoma
Stage - most important prognostic indicator
- 10 yr survival
- Stage I/II = 80-90%
- Stage III/IV = 55-75%
What are the Clinical Features of Non-Hodgkin Lymphoma (NHL)? (4)
- Most are B-cell in origin (85%)
-
Middle-aged to Older Adults
- Not in kids, like HL can be
- May develop in nodes (~75%) or extranodal sites:
- spleen, mucosal lymphoid tissue (MALT), skin, CNS, bone, viscera
- Some may involve bone marrow and spill over into blood becoming a leukemia
What is the nodal disease progression of NHL?
- Nontender mass or lymph node enlarging over months, may be freely moveable –>
- Expands to involve a set of local lymph nodes (cervical, axillary, inguinal) –>
- Nodes become fixed/matted –>
- Invasion into normal tissue
What are the Clinical Features of NHL in the Oral Cavity?
- Usually extranodal ds
-
In Soft Tissue
- __non-tender, diffuse swelling
- Buccal vestibule, posterior hard palate, or gingiva
- Normal to red/purple
- Possibly ulcerated, often with a boogy consistency
-
In the Jaws
- Vague pain, paresthesia, “numb chin” sign
- Ill-defined/ragged RL
- With time, expansion and perforation into soft tissue
What is the Histopathology of NHL?
- Variably differentiated lymphocytic cells
- Grow as infiltrative, diffuse sheets of relatively uniform cells
-
Diffuse Large B Cell Lymphoma
- Wall to wall cells, with lots of apoptic cells
-
Follicular B Cell Lymphoma
- Expansion of follicle germinal center
-
Diffuse Large B Cell Lymphoma
- IHC always needed to ID which type of NHL
What is the Treatment for Low Grade (indolent type) NHL?
- “Incurable”
- Chemotherapy: Anti-CD20 monocolonal antibodies +/- CHOP (Cytoxan, Hydroxyrubicin (adriamycin), Oncovin (vincristine), Prednisone
What is the Treatment for Intermediate/High Grade (aggressive types) Localized Disease?
- radiation therapy + chemotherapy
What is the Treatment for Intermediate/High Grade (aggressive types) Generalized Disease?
Chemotherapy
What constituets 98% of NHL Oral Lesions?
-
B Cell Lymphomas
- Diffuse Large B cell lymphomas (~60%)
- Follicular Lymphoma (~15%)
- Marginal Zone Lymphoma (~12%)
What are the Clinical Features of Diffuse Large B Cell Lymphoma?
- Older adults
- Localized
-
Rapidly growing mass in:
- lymph nodes, waldeyer’s ring or extranodal sites (oral cavity)
- Also can occur:
- Transformation from a previous low grade lymphoma
- Associated with HIV and other immunodeficiencies
What is the pathogenesis of Burkitt Lymphoma?
8:14 translocation causes rapid growth
What is the histologic pattern of Burkitt Lymphoma?
-
“starry sky”
- macs gobbling up all of the dead cells leaving holes in the tissues
What are the 2 types of Burkitt Lymphoma?
-
African Type (endemic)
- __EBV almost always present
- Usually a jaw lesion (50-70%) in young pt
-
American Type (sporadic)
- Usually abdominal mass
- May spread to jaw
- Usually abdominal mass
What is the Treatment and Prognosis for Burkitt Lymphoma?
- Death within 6 months without tx
- Tx: intensive chemotherapy
What are the Clinical Features of Mycosis Fungoides? (4)
- Cutaneous T cell Lymphoma
- Stages:
- Erythematous patch (eczematous) –> plaque –> tumor
- Rare intraorally - usually appear late in ds course, after skin lesions
-
Sezary Syndrome
- Aggressive systemic involvement leading to death in 2-3 yrs
What are the Clinical Features of Angiocentric T Cell Lymphoma? (4)
- Destructive tumor of midline of palate and nasal cavity may cause palatal perforation
- Nasal stuffiness, epitaxis and pain may proceed
- Significant number linked to EBV
- Treatment
- Radiation –> localized lesions
- Chemotx –> disseminated ds
What are the Clinical Features, Treatment, and Prognosis of Plasmacytoma?
- Lymphoma of plasma cells, usually within bone but can be in soft tissue (extramedullary)
- Solitary, well-defined or ragged unilocular radiolucency
- ~25% of soft tissue lesions affect the H/N
- Least aggressive end of the spectrum of Multiple Myeloma
- Treated with Radiation
- ~50% develop multiple myeloma within 2-3 yrs
What are the Clinical Features of Multiple Myeloma? (5)
- Lymphoma of plasma cells forming tumor masses within bone, sometimes extramedullary sites
- Mostly in male, older adults
- 1/2 of all bone malignancies
- Begins in marrow with slow, asymptomatic growth over yrs –> elevation in its Ig (M protein), light chains (Bence-Jones protein) or heavy chains
- Diagnosed when bone lesions finally cause pain, fractures
What are the Secondary Effects of Multiple Myeloma? (4)
- Hypercalcemia from bone destruction
- Myelophthisic anemia, thrombocytopenia, neutropenia with attendant infections and coagulopathy
- Light chain proteins that filter into renal tubules are toxic
- Amyloid from light chains collects in organs (heart, tongue, GI tract, skin, and kidney)
What is the pathology of Multiple Myeloma?
Affected bones (vertebrae, ribs, skull, mandible) develop multiple punched-out radiolucencies containing sheets of plasma cells
How do you Diagnose Multiple Myeloma?
- Biopsy of radiographic lesions
- Bence-Jence Protein in urine
- Detection of M protein in blood (monoclonal gammopathy)
What is the Treatment and Prognosis for Multiple Myeloma?
-
Steroid + alkylating agents
- 60% initial response
- Frequent recurrence
-
Bisphosphonates
-
Inhibit bone resorption
- Reducing fractures, pain, and hypercalcemia
- Medication-related osteonecrosis of the jaws
-
Inhibit bone resorption
- 3-4 yr survival
What are the Clinical Features of Langerhans Cell Histiocytosis? (6)
- A group of related disorders caused by proliferation of Langerhans cells
- Langerhans cels are related to monocytes and present antigen to T cells
- Monoclonal proliferation (true neoplasm)
- BRAF mutation noted in 40-60% of lesions
- Lesions form where Langerhans and dendritic cells live
- skin, lymph nodes, marrow, mucosa
- Favored bones include:
- skull, ribs, vertebrae, mandible
- ~ locations to Multiple Myeloma
- skull, ribs, vertebrae, mandible
- Wide age range (50% over 15yrs)
- Infants with bone marrow, skin and visceral involvement
- Usually follows a very aggressive course
Acute Disseminated Histiocytosis
(Letterer-Siwe Ds)
- Bone, skin and viscera in older children
Chronic Disseminated Histiocytosis
(Hand-Schuler Christian Ds)
- Localized form in bone
- Adults (monostotic) and teenagers (polyostotic)
- Well-defined, but non-corticated radiolucency “scooped out”
Eosinophilic Granuloma of Bone
What is the most common Clinical Presentation of Langerhans Cell Histiocytosis? (5)
-
Bone lesions are the most common clinical presentation
- Dull pain and tenderness often present
- Punched-out or ill-defined RL
- Posterior mandible involvement “scooped out”
- May mimic severe periodontitis with teeth “floating in air”
- May mimic periapical disease
- Soft tissue mass may occur in isolation or from a lesion breaking out of bone
What are the Histopathologic Features of Langerhans Cell Histiocytosis?
- Sheets of large, histiocytic-appearing cells (neoplastic Langerhans cells)
- Variable numbers of eosinophils (eosinophilic granuloma), lymphocytes, plasma cells, multinucleated giant cells
What is the Treatment for Langerhans Cell Histiocytosis accessible bone lesions (Md/Mx)?
Curettage or intralesional steroid injection
What is the Treatment for Langerhans Cell Histiocytosis disseminated disease?
Single or multiagent chemotx depending on how widespread the disease is
When does a poorer prognosis occur in Langerhans Cell Histiocytosis?
- The earlier the onset of symptoms, the more widespread (more organs involved) the presentation
- and lack of response to induction chemotx
What is the biological behavior of lymph node group in Hodgkin Lymphom?
-
Cerivcal or Supraclavicular (70-75%)
- mediastinal node –> spleen, liver, bm, lung
- If enlarged nodes are absent it could present with a, mediastinal mass = massive nelargement on midline of lungs
- CLASSIC for HL
