5 Hematologic Diseases (Malignancies) Flashcards
malignant lymphoproliferative disorder
hodgkin’s lymphoma
Reed-Sternberg cell, likely of B-cell origin, only makes up 1-3% of cells in the enlarged nodes
hodgkin’s lymphoma
approx 9,000 cases annually
hodgkin’s lymphoma
EBV linkage suggested
hodgkin’s lymphoma
male predilection
hodgkin’s lymphoma
Two age peaks of hodgkins lymphoma
15-35 and > 50
Clinical findings: persistently enlarging lymph nodes start off freely moveable but become fixed and matted to adjacent tissue
hodgkin’s lymphoma
30-40% of patients have other symptoms –> night sweats, weight loss, fever, generalized itching
hodgkin’s lymphoma
survival rate of hodgkins lymphoma stage I/II vs III/IV
Stage I or II = 80-90% relapse free 10-yr survival rate
Stage III/IV = 55-75% 10-yr survival
Clinical findings: an enlarging nontender discrete mass in one lymph node region
Hodgkin’s lymphoma
In 70-75% of cases of Hodgkin’s lymphoma, this is the initial site of presentation. What are other sites of involvement?
- cervical and supraclavicular lymphadenopathy
- axillary lymphadenopathy
- mediastinal lymphadenopathy
lymphoreticular malignancy, most often arise within lymph nodes and grow into solid masses
non-Hodgkin’s lymphoma
malignant cells do NOT usually circulate in the peripheral blood
non-Hodgkin’s lymphoma
70,000 cases/year in the US, 1/3 die of it each year
non-Hodgkin’s lymphoma
What does non-Hodgkin’s lymphoma originate from?
B-lymphocytes (85% of US cases)
T-lymphocytes (less common)
classification of non-Hodgkin’s lymphoma
- low, intermediate, or high grade (indicate increasing aggressiveness)
- REAL classification
extranodal site of development, make up 20-40% of lymphomas in US
non-Hodgkin’s lymphoma
can develop in the soft tissues or centrally within the jaws, present as nontender diffuse swellings
non-Hodgkin’s lymphoma
Oral manifestations:
- nontender diffuse swellings
- hard palate, buccal vestibule, gingiva
- erythematous
- purplish-blue
- may be ulcerated
- surface telangiectasia
non-Hodgkin’s lymphoma
tx of non-Hodgkin’s lymphoma
radiation, chemo, or a combo
high grade B cell lymphoma, linked to EBV infection as etiologic agent (90% of tumor cells express the EBV nuclear antigen)
Burkitt lymphoma
more common in males, children
Burkitt lymphoma
areas of the world with an increased prevalence of Burkitt lymphoma
sub-Saharan Africa, northeastern Brazil, immunodeficiency type
ill-defined, ragged radiolucency on radiographs
Burkitt lymphoma
malignancy of hematopoietic stem cell origin (one stem cell undergoes malignant transformation and proliferates within the marrow, overflows into the peripheral blood stream)
leukemia
2.9% of all cancers, 4.1% of all cancer deaths
leukemia
more frequently with certain genetic syndromes and alterations, also caused by environmental agents (benzene, pesticides, ionizing radiation, viruses)
leukemia
13 cases/100,000 people annually
leukemia
classification system of leukemia
Clinical course:
A or C: acute vs chronic
- acute = aggressive course, more lead to death, more common in children
- chronic = slow course but still fatal, more common in adults
Cell or origin:
M or L: myeloid or lymphocytic/lymphoblastic
- myeloid = broader age range, kids to age 40
- lymphocytic = most common type, older adults
- lymphoblastic = children
first genetic abnormality of leukemia reported
CML- 9:22 translocation (“Philadelphia chromsome”)
symptoms result from crowding out of normal red and white blood cells from the marrow, malignant cells replace normal cells and results in myelophthisic anemia
leukemia
specific anemia related to cancer (leukemia, multiple myeloma)
myelophthisic anemia
symptoms include fatigue, dyspnea with mild exertion, fever, frequent infections (intraoral herpes, candidiasis)
leukemia
frequent oral infections that occur in leukemia patients
intraoral herpes
candidiasis
easy bruising and bleeding from thrombocytipenia, spontaneous gingival bleeding can be seen
leukemia
cancerous cells can infiltrate the oral mucosa and cause diffuse, boggy swelling of the soft tissues (may simulate periapical disease clinically and radiographically)
leukemia
When malignant leukemia cells infiltrate organs:
splenomegaly
hepatomegaly
lymphadenopathy
dx of leukemia
presence of poorly differentiated leukemic cells in the peripheral blood and bone marrow
tx of leukemia
- chemo (induction or maintenance)
- radiation
- bone marrow transplant
induction vs maintenance chemo for tx of leukemia
Induction chemo- rapidly destroying the atypical cells
- hige dose toxic chemotherapeutics
- hopefully destroys all of the cancerous cells and puts the patient into remission
- transfusions with platelets
- encourage excellent OH to help prevent oral infections
Maintenance chemo- lower dose
- maintains remission following induction therapy
Why must radiation AND chemo be used to treat leukemia?
for the CNS –> many chemotherapeutics do not cross the BBB
malignancy of plasma cell origin, originates in bone (single malignant precursor cell gives rise to the entire proliferation, cells spread throughout the body and produce proteins, abnormal immunoglobulins, that deposit in soft tissue as amyloid)
multiple myeloma
~22,000 cases diagnosed annually
multiple myeloma
age = 60-70
multiple myeloma
2x more common in blacks than whites
multiple myeloma
most common hematologic malignancy in black patients
multiple myeloma
Clinical symptoms:
- bone pain
- pathologic fracture from tumor destruction of bone
- fatigue (myelophthisic anemia)
- petechial hemorrhages in soft tissues (10-15% of patients) due to accumulation of the light chain proteins
- kidney failure due to overload of light chain proteins
- increased Bence Jones proteins in the urine (30-50%)
multiple myeloma
rad: multiple, well-defined, punched-out radiolucencies, 30% of patients have jaw lesions
multiple myeloma
30% of patients have jaw lesions
multiple myeloma
tx of multiple myeloma
- chemotherapy + corticosteroids (60% of patients respond however most experience relapse)
- bisphosphonate medications given to reduce the possibility of myeloma related fractures but do not increase survival
- otherwise healthy patients under age 55-65 can try aggressive chemo and bone marrow transplantation (5 yr survival = 50%)
solitary/unifocal monoclonal, neoplastic proliferation of plasma cells usually arising within bone
plasmacytoma
most common site of plasmacytoma
spine
Name the hematologic malignancy: 55 yo average age, M: F = 2:1
plasmacytoma
symptoms include swelling or bone pain, can develop outside of bone as a soft tissue mass (extramedullary = 25% occur in the head and neck)
plasmacytoma
rad: well-defined, unilocular radiolucency, punched-out RL, may have ragged irregular borders
plasmacytoma
tx of plasmacytoma
radiation therapy (50% of patients show disseminated disease within 2-3 years)
increased Bence Jones proteins in the urine (30-50%)
multiple myeloma
Multiple myeloma cells produce proteins (abnormal immunoglobulins) that deposit in soft tissue as amyloid. What % of myeloma patients have deposition of amyloid on soft tissues due to accumulation of the light chain proteins? What is a symptom that arises from this deposition?
10-15%
kidney failure due to overload of light chain proteins (increased Bence Jones proteins in urine 30-50%)
