DISORDERS OF THE WHITE BLOOD CELLS (3)

• 4,400 – 11,000 / µL

Normal WBC Count

Leukocytosis

• Increase in the number of circulating WBCs (lymphocytes or granulocytes) to greater that 11,000/µL

• WBC > 11,000 / µL

Physiologic Leukocytosis

a. Exercise

b. Pregnancy

c. Emotional stress

Pathologic Leukocytes

a. Infection

b. Neoplasia

c. Necrosis

d. Pyogenic infections

Pyogenic infections

- Increased neutrophils

Tuberculosis, syphilis, and viral infections

- Increased in lymphocytes

Protozoal infections

- Increased in monocytes

Allergies and parasitic infections

- Increased in eosinophils

Cellular necrosis, carcinoma of glandular tissues

- Increased in circulating neutrophils

Leukemia

- Increased circulating immature leukocytes

Leukopenia

Reduction in the number of circulating WBCs (usually to <4400/µL)

Leukopenia

Causes:

• Early leukemia/lymphoma

• Bone marrow suppression

• Drugs (especially chemotherapy 💊)

• Agranulocytosis (↓ granulocytes)

• Pancytopenia (↓ all blood cells)

CYCLIC NEUTROPENIA

Patients have a periodic decrease (at least a 40% drop) in the number of neutrophils (about every 21–28 days)

CYCLIC NEUTROPENIA

During low phase:

• Severe infection risk

• Oral ulcers

• Possible death (10%) due to:

  • Pneumonia

  • Cellulitis

  • Peritonitis

LEUKEMIA

Cancer of WBC that affects the bone marrow and circulating blood

Acute Leukemia

• Rapidly progressive disease that results from accumulation of immature, nonfunctional WBCs in the marrow and blood

• Rapid, immature cells

Chronic Leukemia

• Have a slower onset, which allows production of larger numbers of more mature (terminally differentiated), functional cells

• Slow, more mature cells

ACUTE MYELOGENOUS LEUKEMIA

• Neoplasm of myeloid (immature) WBCs, which demonstrate uncontrolled proliferation in the bone marrow space and subsequently appear in the peripheral blood

• Cancer of immature myeloid cells

ACUTE MYELOGENOUS LEUKEMIA

Arises de novo in younger adults or secondarily in older adults as a consequence of myelodysplasia

Myelodysplastic syndromes

diverse group of clonal disorders of hematopoietic stem or progenitor cells resulting in abnormal cellular differentiation

ACUTE MYELOGENOUS LEUKEMIA

Has a sudden onset and leads to death in 1 to 3 months if left untreated

ACUTE MYELOGENOUS LEUKEMIA

Patients are susceptible to excessive bleeding, anemia, poor healing, and infection after surgical procedures Hemorrhage and infection, frequent complications of chemotherapy, are the chief causes of death

ACUTE MYELOGENOUS LEUKEMIA

Diagnosis: when myeloblasts are found in the bone marrow or peripheral blood at a rate of at least 20%

ACUTE MYELOGENOUS LEUKEMIA

Signs and Symptoms

- Fatigue, easy bruising, and bone pain

- Flu-like symptoms for 4 to 6 weeks before the diagnosis

- Anemia and thrombocytopenia: malaise, pallor, dyspnea on exertion, and bleeding and small hemorrhage (petechiae, ecchymoses) in skin and mucous membranes

- Granulocytopenia: recurrent infections (nonhealing wounds), oral ulcerations, fever

ACUTE LYMPHOID LEUKEMIA

• Result of uncontrolled monoclonal proliferation of immature lymphoid cells in the bone marrow and peripheral blood

• Cancer of immature lymphocytes

ACUTE LYMPHOID LEUKEMIA

Common in:

• Children

• Associated with Down syndrome

ACUTE LYMPHOID LEUKEMIA

Diagnosed when massive replacement of the bone marrow space with leukemic blast cells is observed

ACUTE LYMPHOID LEUKEMIA

Symptoms:

• Bone pain (affects walking)

• Fever

• Bleeding

• Enlarged lymph nodes, liver, spleen

1st Phase / Induction

Tx wherein Aggressively induce a state of remission by killing tumor cells with cytotoxic agents

2nd Phase / Consolidation or Intensification

Tx wherein Focuses on consolidating consolidating the kill of remaining leukemic cells

3rd Phase / Complete Remission

Tx wherein Maintenance therapy, to prevent expansion of any remaining leukemic cell mass

Sanctuaries

areas in the body where leukemic cells migrate and cannot reach by chemotherapeutic agents

Marrow transplant or peripheral blood stem cell transplant

preceded by high-dose chemotherapy (including busulfan) and radiation therapy

Bone marrow transplantation (BMT)

for patients younger than 45 years of age and for children and young adults who relapse when a suitable sibling match is available (allogeneic)

- Localized or generalized gingival enlargement: gingiva is boggy and bleeds easily

- Fetor oris

- Ulceration

- Infection

• Oral Manifestations of Acute Leukemia

CHRONIC MYELOGENOUS LEUKEMIA

Neoplasm of mature myeloid WBCs

CHRONIC MYELOGENOUS LEUKEMIA

• Etiology is unknown, but radiation exposure increases risk for the disease

• Progresses slowly through a chronic phase for 3 to 5 years and then moves on to an accelerated phase followed by a blast phase (or crisis)

blast phase

More than 85% of patients with CML die in the

CHRONIC MYELOGENOUS LEUKEMIA

Diagnosis

a. Complete blood count (CBC)

b. Presence of Philadelphia (Ph) Chromosome

CHRONIC MYELOGENOUS LEUKEMIA

Oral Manifestations

• Less likely to demonstrate oral manifestations

• Generalized lymphadenopathy, pallor of the oral mucosa, and soft tissue infection

CHRONIC LYMPHOCYTIC LEUKEMIA

Neoplasm of mature clonal CD5+ B lymphocytes

Etiology is unknown

Risk factors: more related to familial inheritance

CHRONIC LYMPHOCYTIC LEUKEMIA

Oral Manifestation

• Generalized lymphadenopathy

• Pallor of the oral mucosa

• Oral soft tissue infection may become evident as the patient develops hypoglobulinemia

Stage A

- 2 or fewer lymph node groups, no anemia or thrombocytopenia

- Survival time is longer than 10 years

Stage B

• 3 or more lymph node groups, no anemia or thrombocytopenia

• 5 years

Stage C

• Anemia and thrombocytopenia, any number of lymph node groups

• 2 years

LYMPHOMA

• Cancer of the lymphoid organs and tissues that presents as discrete tissue masses

• Initial signs often occur in the mouth (e.g., Waldeyer Ring) and in the head and neck region

HODGKIN LYMPHOMA

Contains a characteristic tumor cell called Reed-Sternberg cell that represents usually less than 1% of the cellular infiltrate in affected tissues

lung or vascular obstruction

Enlarging Tumorous Nodes

cough, shortness of breath, or dysphagia

Enlarging Mediastinal Nodes

HODGKIN LYMPHOMA

Signs and Symptoms

- Painless mass or a group of firm, nontender,

enlarged lymph nodes, often affecting the

mediastinal nodes or the neck nodes

- Enlarged lymph nodes in underarm or groin

- Fever, weight loss, and night sweats

- Pruritus and fatigue develop, may precede

appearance of enlarging lymph nodes

- Palpation of the lymph nodes typically

reveals a rubbery consistency

HODGKIN LYMPHOMA

Diagnosis: nodal biopsy or bone marrow

aspirate

NON-HODGKIN LYMPHOMA

- Compromises a large group of

lymphoproliferative disorders classified as

of B- or T-cell origin (more than 80% are of

B-cell origin)

• Mostly B-cell origin

• More widespread + unpredictable

NON-HODGKIN LYMPHOMA

- Often marked by enlarged lymph nodes,

fever, and weight loss

- Usually is multifocal when first detected

- Extranodal lymphomas

- Most prominent sign: painless lymph nodes

swelling of longer than 2 weeks duration

extranodal disease

Lymphoma in the oral cavity usually appears

as

Waldeyer Ring (soft palate and oropharynx)

Intraoral lymphoma most commonly

involves

NON-HODGKIN LYMPHOMA

Infrequent findings include deep

“crateriform” oral ulcers and fever

Osteoradionecrosis

long-term risk

associated with radiation

BURKITT LYMPHOMA

- Aggressive B-cell (Non-Hodgkin) Lymphoma

- Described by Denis Burkitt

- Tumors are composed of mature B cells that

express surface IgM

- Most common lymphoma of childhood

BURKITT LYMPHOMA

Associated with translocation of the c-myc

gene (a gene involved in cellular

proliferation) onto chromosome 8

Endemic Burkitt Lymphoma

- Found most often in Central Africa

- Affects children with a peak prevalence of

about 7 years of age

- More than 50% - 70% present in jaws

Sporadic (Non-endemic) Burkitt Lymphoma

- More common in Western societies

- Affects slightly older children and adults in

their 30s

3. Immunod

Immunodeficiency-associated Burkitt

Lymphoma

- Occurs in persons infected with HIV

- More common among men

Nonendemic Burkitt Lymphoma

- Abdominal mass that involves the lymph

nodes of the intestine and peritoneum, with

jaw lesions being less common

- Tumors that enlarge as abdominal masses

are accompanied by fluid accumulation,

pain, and possibly vomiting

BURKITT LYMPHOMA

- Very aggressive and grows very rapidly

- Tumors can double in size every 3 days ->

obstruction of the airway, alimentary canal,

and vasculature is possible

- Also has a propensity for spread to the CNS

BURKITT LYMPHOMA

- Arise at extranodal sites

• Jaw involvement is more common in

patients younger than 5 years

- Rapidly expanding tumorous mass in the

posterior region of the maxilla or mandible

- Rapid growth displaces adjacent teeth

- Pain and paresthesia

- Radiographically: osteolytic lesion with

poorly demarcated margins, erosion of the

cortical plate, and soft tissue involvement

BURKITT LYMPHOMA

Medical Management

- High-dose chemotherapy

- Tumors are particularly sensitive to

cyclophosphamide

- Combination chemotherapy with

vincristine, doxorubicin, methotrexate, or

cytarabine has achieved remission

MULTIPLE MYELOMA

Lymphoproliferative disorder that results

from overproduction of cloned malignant

plasma cells that results in multiple

tumorous masses scattered throughout the

skeletal system

MULTIPLE MYELOMA

Consist of plasma and myeloma cell

proliferation, immunoglobulin production,

bone resorption at tumor sites, and bone

marrow replacemen

MULTIPLE MYELOMA

Radiographically: multiple “punched-out”

lesions or mottled areas, which represent

areas of tumor that appear in the spine,

ribs, and cortical regions of the skull

- Osteolytic lesions of the jaw occur in up to

30% of patients

- Most prominent symptom is persistent

bone pain – along the spine, ribs, and

sternum