leukemia, lymphoma, plasma cell dyscarsias

etiology of lymphoma/leukemia

usually unknown

viral occasionally - HTLV1 and HTLV2 viruses

EBV implicated in some leukemia and lymphoma

environmental factors include chemicals, radiation

in leukemia which gender is more affected

males

in leukemia what blood cell line is involved

any white blood cell line

leukemia diagnosis made by

peripheral smear/bone marrow exam

leukemia begins in

bone marrow, malignant cells circulate in blood

acute leukemia of myeloid origin

AML

acute leukemia of lymphoid origin

ALL

chronic leukemia of myeloid origin

CML

chronic leukemia of lymphoid origin

CLL

What age group is most prominently affected by ALL?

Children and young adults

What types of cells are primarily involved in ALL?

T cell and B cell type (75%)

What is the most common cancer in children?

Acute Lymphoblastic Leukemia (ALL)

Which genetic condition is associated with an increased risk of ALL?

Trisomy 21 (Down syndrome)

What is the typical onset time for symptoms of ALL?

Abrupt onset within days to weeks

What is the prognosis for ALL after treatment?

Good

What percentage of patients are disease-free at 5 years after a bone marrow transplant for ALL?

70-90%

What does AML stand for?

Acute Myeloid Leukemia

What type of leukemia is AML?

Leukemia of adults

What type of cells are involved in AML?

Immature myeloid cells

What are the signs and symptoms of AML similar to?

Acute Lymphoblastic Leukemia (ALL)

What is the prognosis for AML?

Poor prognosis

What percentage of AML patients survive 3-5 years after a bone marrow transplant?

50%

What is CLL?

Chronic Lymphocytic Leukemia

What age group is most commonly affected by CLL?

Older patients over 50 years

What is the most common leukemia in adults?

Chronic Lymphocytic Leukemia (CLL)

What type of cells accumulate in CLL?

Mature lymphocytes

What is the prognosis for CLL?

Excellent

Is CLL often symptomatic at diagnosis?

Often asymptomatic

Are the symptoms of CLL specific or nonspecific?

Nonspecific

What is the median age of diagnosis for Chronic Myeloid Leukemia (CML)?

60

What is the male to female ratio in Chronic Myeloid Leukemia (CML)?

3:1

What genetic abnormality is associated with Chronic Myeloid Leukemia (CML)?

Philadelphia chromosome

What percentage of cells in blood/marrow are immature during acute transformation due to blast crisis in CML?

30%

What are some treatment options for Chronic Myeloid Leukemia (CML)?

Chemotherapy, radiation, interferon, marrow transplant

What are common oral manifestations of leukemia related to fever?

Fever, enlarged tonsils, and salivary glands with dry mouth.

What is gingival hyperplasia?

A condition characterized by the overgrowth of gum tissue, often seen in leukemia patients.

What are the oral symptoms associated with gum bleeds in leukemia?

Gum bleeds and tumor-like growths such as chroma or granulocytic sarcoma.

What types of infections can occur in the oral cavity due to leukemia?

Infections and oral ulcers.

What are white cells responsible for?

White cells are responsible for much of the body's response to injury and infection.

What causes increased white cell counts?

Increased white cell counts occur in response to bacterial infection or necrosis, allergic conditions, and viral conditions.

What can reduced white cell counts lead to?

Reduced white cell counts may lead to infections, especially around the oral cavity.

What condition is associated with reduced neutrophils?

Reduced neutrophils may be associated with leukemias.

What is leukemia?

Leukemia is a neoplastic condition of marrow cells which may differentiate into several types of cells.

How many types of leukemia are there?

There are 4 types of leukemia depending on the type of cells involved and presentation.

What characterizes acute leukemias?

Acute leukemias are aggressive and affect a younger age group.

What is the treatment for acute leukemias?

Treatment for acute leukemias is by chemotherapy and radiotherapy.

Who is more likely to have chronic leukemias?

Chronic leukemias are more usual in older patients.

What is chronic myeloid leukemia (CML) associated with?

Chronic myeloid leukemia (CML) is associated with the Philadelphia chromosome.

What is lymphoid hyperplasia?

Lymphoid hyperplasia is the enlargement of lymphoid tissue due to the proliferation of lymphoid cells in response to antigenic challenge.

What is the protective function of lymphoid tissue?

Lymphoid tissue has a protective function by responding to antigenic challenges.

What are the characteristics of acute lymphoid hyperplasia?

Acute lymphoid hyperplasia presents with enlarged, tender, soft, and freely movable nodes.

What are the characteristics of chronic lymphoid hyperplasia?

Chronic lymphoid hyperplasia presents with enlarged, rubbery, firm, non-tender, and freely movable nodes.

Where does intraoral lymphoid hyperplasia affect?

Intraoral lymphoid hyperplasia affects Waldeyer's ring, which includes aggregates of lymphoid tissue in the oropharynx, soft palate, lateral tongue, and floor of the mouth.

What is the color of lymphoid tissue affected by hyperplasia?

Lymphoid tissue affected by hyperplasia is normally deep pink in color, but may appear creamy yellow if closer to the surface.

What is the recommended management once lymphoid hyperplasia is diagnosed?

Once diagnosed, no treatment is required, but periodic observation is recommended.

lymphoma

proliferation of immature lymphocytes that accumulate. in soft tissues, lymph node, bone marrow

what is usually the first node to be involved in lymphoma

cervical node

classification of lymphoma

type, grade, stage

type

pathology, cell type

grade

malignant potential

stage

spread of disease

clinical staging of disease helps determine

prognosis

What is non-Hodgkin lymphoma (NHL)?

A diverse, complex group of lymphoreticular malignancies.

In which population does non-Hodgkin lymphoma primarily occur?

Primarily in adults.

Where do most non-Hodgkin lymphomas arise?

In lymph nodes as solid, non-tender masses.

Which lymph nodes are usually involved in non-Hodgkin lymphoma?

Cervical, axillary, or inguinal nodes.

What is the initial presentation of lymph nodes in non-Hodgkin lymphoma?

1-2 freely movable nodes initially.

How do lymph nodes change as non-Hodgkin lymphoma progresses?

They become more numerous, fixed, or matted together.

Who has an increased risk of developing high-grade non-Hodgkin lymphomas?

Immunosuppressed patients, such as transplant patients.

lymphoma in the oral cavity

soft tissue- diffuse swelling, often posterior hard palate, red/purple

bone- vague pain , ragged radiolucency

What is the gender incidence ratio for Hodgkin lymphoma?

Males > females

At what ages does the incidence of Hodgkin lymphoma peak?

20s and 50s

Where do Hodgkin lymphoma tumors commonly arise?

In lymph nodes, more common in the neck

What are Reed-Sternberg cells characterized by?

Binucleate (owl eye nuclei) or prominent nucleoli

What are the classical forms of Hodgkin lymphoma?

1. Nodular sclerosing, 2. Mixed cellularity, 3. Lymphocyte rich, 4. Lymphocyte depletion

How do Reed-Sternberg cells differ in lymphocyte predominance compared to classical types?

They have a distinctive B cell immunophenotype

What is the prognosis for lymphocyte predominance Hodgkin lymphoma?

Excellent

What is the most common form of Hodgkin lymphoma?

Nodular sclerosing

In which gender is nodular sclerosing Hodgkin lymphoma more common?

Females

What histological feature is associated with nodular sclerosing Hodgkin lymphoma?

Deposition of collagen in bands that divide involved lymph nodes into circumscribed nodules

What age group is nodular sclerosing Hodgkin lymphoma more common in?

Adolescents and young adults

What is the prognosis for nodular sclerosing Hodgkin lymphoma?

Excellent

What are B symptoms in the context of categorizing diseases?

Fever > 38C for > 15 days, night sweats, and worsening prognosis.

What category is assigned if B symptoms are present?

Category B

What category is assigned if there are no B symptoms?

Category A

What does Stage I of the Ann Arbor classification indicate?

Involvement of a single lymph node region (I) or a single extra lymphatic organ or site (IE)

What does Stage II of the Ann Arbor classification indicate?

Involvement of two or more lymph node regions on the same side of the diaphragm alone (II) or localized involvement of an extra lymphatic organ or site (IIE)

What does Stage III of the Ann Arbor classification indicate?

Involvement of lymph node regions on both sides of the diaphragm without (III) or with (IIIE) localized involvement of an extra lymphatic organ or site

What does Stage IV of the Ann Arbor classification indicate?

Diffuse involvement of one or more extra lymphatic organs or sites with or without lymphatic involvement

most important prognostic variable

tumor stage

best prognosis

lymphocyte predominant, lymphocyte rich and nodular sclerosis

Burkett lymphoma

starry sky histology: monotonous sea of dark tumor cells with occasional tangible body macrophages

adult t cell lymphoma

caused by RNA retrovirus HTLV1, common in oral cavity, long latency then rapidly fatal

increased risk of lymphoma

increased incidence in some autoimmune or immunodeficiency diseases - sjogren, oral transplant, AIDS, congenital immune deficiencies, celiac

lymph node enlargement may be

reactive or neoplastic

neoplastic lymphadenopathy may be

primary or secondary due to deposits of metastatic tumors

lymphoma is described as

malignant neoplasm of lymphoid tissue

two types of lymphoam

hodgkin and non hodgkin

hodgkin lymphoma is

neoplastic proliferation of atypical cell called R-S

lymphoma may be

nodal or extra nodal in origin, B or T cell type