2. WBC Neoplasms Flashcards
(42 cards)
1
Q
What are the 3 Etiologies of Leukemia?
A
- Genetic Factors
- Some Syndromes
- Gene translocation (philadelphia translocation)
- Genetic alterations of stem cells
- Myelodysplasia
- Environmental
- Pesticides, Benzene
- Ionizing Radiation (WWII)
- Viruses
- HTLV-1
2
Q
What is the Pathophysiology of Leukemia?
A
- Replacement of normal hematopoietic cells in bone marrow
- Derived from a single transformed cell exhibiting clonal growth
3
Q
What are the General Symptoms of Leukemia? (3)
A
-
Fatigue, SOB, Pallor
- Decreased RBCs
-
Easy Bruising
- Decreased Platelets
-
Infection
- Decreased WBCs
4
Q
What does Leukemia cause? (3)
A
-
Neutropenia
- __Bacterial, viral and fungal infections
- Oral ulcers, herpes, candida
-
Thrombocytopenia
- Gingival bleeding
- Palatal petechiae
-
Myelophthisic Anemia
- Lack RBCs - hypoxia
- Causes Extramedullary Hematopoiesis
5
Q
What are the Oral Presentations and in what type of Leukemia are they normally seen?
A
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
6
Q
How is Leukemia usually diagnosed?
A
- Increased #s of atypical WBCs in blood and bone marrow
- Type is determined by:
- Immunohistochemical and Cytogenetic studies
7
Q
What are the Clinical Features of Hodgkin Lymphoma? (4)
A
- 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
8
Q
What are the Constitutional Symptoms of Hodgkin Lymphoma? (4)
A
- Progressive Fever OR Pel-Ebstein Fever
- Night sweats
- Weight loss
-
Generalized Pruritis (itchiness all over body)
- UNIQUE SYMPTOM
9
Q
What does the Diagnosis of Hodgkin Lymphoma depend on?
A
-
Reed-Sternberg cells
- Large, either multinucleated, or have a bilobed nucleus “owl eye”
10
Q
What is the Treatment of Hodgkin Lymphoma?
A
- ABVD
- Adriamycin
- Bleomycin
- Vinblastine
- Dacarbazine
11
Q
What is the prognosis of Hodgkin Lymphoma
A
Stage - most important prognostic indicator
- 10 yr survival
- Stage I/II = 80-90%
- Stage III/IV = 55-75%
12
Q
What are the Clinical Features of Non-Hodgkin Lymphoma (NHL)? (4)
A
- 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
13
Q
What is the nodal disease progression of NHL?
A
- 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
14
Q
What are the Clinical Features of NHL in the Oral Cavity?
A
- 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
15
Q
What is the Histopathology of NHL?
A
- 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
16
Q
What is the Treatment for Low Grade (indolent type) NHL?
A
- “Incurable”
- Chemotherapy: Anti-CD20 monocolonal antibodies +/- CHOP (Cytoxan, Hydroxyrubicin (adriamycin), Oncovin (vincristine), Prednisone
17
Q
What is the Treatment for Intermediate/High Grade (aggressive types) Localized Disease?
A
- radiation therapy + chemotherapy
18
Q
What is the Treatment for Intermediate/High Grade (aggressive types) Generalized Disease?
A
Chemotherapy
19
Q
What constituets 98% of NHL Oral Lesions?
A
-
B Cell Lymphomas
- Diffuse Large B cell lymphomas (~60%)
- Follicular Lymphoma (~15%)
- Marginal Zone Lymphoma (~12%)
20
Q
What are the Clinical Features of Diffuse Large B Cell Lymphoma?
A
- 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
21
Q
What is the pathogenesis of Burkitt Lymphoma?
A
8:14 translocation causes rapid growth
22
Q
What is the histologic pattern of Burkitt Lymphoma?
A
-
“starry sky”
- macs gobbling up all of the dead cells leaving holes in the tissues
23
Q
What are the 2 types of Burkitt Lymphoma?
A
-
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
24
Q
What is the Treatment and Prognosis for Burkitt Lymphoma?
A
- Death within 6 months without tx
- Tx: intensive chemotherapy
25
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
26
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
27
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
28
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**
29
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)
30
What is the pathology of **Multiple Myeloma**?
Affected bones (vertebrae, ribs, skull, mandible) develop **multiple punched-out radiolucencies** containing sheets of plasma cells
31
How do you Diagnose **Multiple Myeloma**?
* **Biopsy** of radiographic lesions
* **Bence-Jence Protein in urine**
* Detection of **M protein in blood** (monoclonal gammopathy)
32
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**
* 3-4 yr survival
33
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
* **Wide age range** (50% over 15yrs)
34
* **Infants** with bone marrow, skin and visceral involvement
* Usually follows a **very aggressive** course
Acute Disseminated Histiocytosis
| (Letterer-Siwe Ds)
35
* Bone, skin and viscera in **older children**
Chronic Disseminated Histiocytosis
| (Hand-Schuler Christian Ds)
36
* **Localized form** in **bone**
* **Adults** (monostotic) and **teenagers** (polyostotic)
* Well-defined, but non-corticated **radiolucency "scooped out"**
Eosinophilic Granuloma of Bone
37
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
38
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
39
What is the Treatment for **Langerhans Cell Histiocytosis accessible bone lesions (Md/Mx)**?
Curettage or intralesional steroid injection
40
What is the Treatment for **Langerhans Cell Histiocytosis disseminated disease**?
**Single or multiagent chemotx** depending on how widespread the disease is
41
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
42
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
