White Blood Cell DIsorders Flashcards
WBC
Granulocytes (neutrophils, eosinophils, basophils)
Lymphocytes (T/B lymphocytes, NK cells)
Monocytes (macrophages)
95% WBC present in the bone marrow (immature, maturing, functional)
5% in circulation
- marginal: adhere to vessel walls, readily available
- circulating
Lymphocyte location: lymph nodes, spleen, MALT (respiratory, GI tracts)
MALT mucosa-associated lymphoid tissues
Neutrophils: phagocytosis, enzymatic destruction
Eosinophils, basophils: inflammatory allergic reactions (granules). Eosinophils: combat parasitic infections
T cells: delayed, cellular immune rxn
B cells: immediate/humoral immune rxn (plasma cells, immunoglobulins)
Monocytes: phagocytosis, cytokines production
- antigen presenting cells: dendritic cells in lymph nodes, Langerhan cells i skin/mucosa
- tissue macrophages
WBC Count
normal: 4400 - 11,000 cells/uL
Leukocytosis > 11,000
Leukopenia: <4400
Leukocytosis causes (>11,000 cells/uL)
- physiologic (exercise, eotional stress, pregnancy)
- pathologic: infection, neoplasia, necrosis
Leukopenia causes (<4000 cells/uL)
- early stage of leukemia/lymphoma (excessive prolif of WBC causes crowding)
- agranulocytosis (reduction of granulocytes)
- pancytopenia (decreased RBC and WBC)
- chemotherapy
Leukemia
- Exponential prolif. of clonal lymphoid/myeloid cells in bone marrow and blood
- Acute: rapidly progressive. Immature functionless WBC
- Chronic: slower onset. More mature funcctional cells
- Prevalence: Adults > Children
Incidence (new cases in 2010)
CLL 15,000 (#1 adult)
AML 12,000
ALL 5330 (#1 children)
CML 4870
General Manifestation
Anemia: fatigue, malaise, flu-like symptoms, pallor, dyspnea
Thrombocytopenia: bleeding, petechiae, bruising
Granulocytopenia: infection, oral ulcers, fever
Leukemic infifltration of tonsils, gingiva
Enlargement of liver, spleen, lymph nodes
Infiltraison of CNS: increased intracranial pressure, meningeal signs
Acute Myelogenous Leukemia (AML) 12,000
Young adults, elderly
Cause of death: hemorrhage, infection, complications of chemotherapy
Poor prognosis: death in 1-3 months if untreated. Adult survival 30-50%
Acute Lymphoid Leukemia (ALL) 5,330
Typically in children.
#1 leukemia in children
Association: Down syndrome
Association: Philadelphia chromosome (shortened Cr22 from translocation 9/22) – poorer outcome
Prognosis:
Good in children: Cure rate >70%, Remission >90%
Adults survival 50-60% only
Chronic Myelogenous Leukemia (CML) 4870,
Older adults
Philadelphia chromosome (CMLbetter outcome and ALLworse outcome)
Initial chronic, then accelerated, followed by blast phase
- chronic phase (mature, low risk of infection)
- blast phase (immature: anemia, thrombocytopenia, infection)
Overall poor prognosis
Medical Management
- Chemotherapy: hydroxyurea, tyrosine kinase inhibitor
- Stem cell transplantation
Chornic Lymphocytic Leukemia (CLL) 15,000
Most common leukemia in adults. Older adults
Familial inheritance bigger role (Jewish from Russia/Eastern Europe)
Treatment has little effect on survival
Medical Management
Acute Leukemia
- Chemotherapy
- Remission: blood counts return to normal
- Cure: no leukemic cells remain
- Radiation therapy
- Marrow Transplant
- Stem cell Transplant
CML: Chemo, Stem cell transplant
CLL: usually no treatment
Oral Manifestations
Acute Leukemia
- Generalized boggy gingiva with easy bleeding
- leukemic infiltrate, thrombocytopenia
- Brusing of tongue
- Ulcer, Oral Infection
- Granulocytic sarcoma/chloroma (mass of leukemic cells in any part of body, maxilla, palate)
- Resolution: plaque control, chlorhexidine, cheomtherapy
Chronic Leukemia
- Generalized lymphadenopathy, mucosal pallor, soft tissue infection
Hodgkin Lymphoma (HL) - most common
Neoplasm of B lymphocytes with presence of Reed-Sternberg cell (owl’s eye appearance) in lymph nodes
- Young adult + 5th decade
- Children in developing countries
- Etio: unknown. Assoc’ EBV (immortalize B cells)
Unifocal at diagnosis
Painless firm enlarged nodes in mediastinum, neck, underarm, groin
nodes can obstruct lung / vessels –> cough, SOB, dysphagia
Fever, weight loss, night sweats
Spread: to other nodes, spleen, bone marrow, liver, lung
Death: bone marrow failure, infection
Tx: #1 Radiation + chemo, stem cell transplant
Treatment can cause secondary neoplasm, heart disease
Cure rate 90%
Non-Hodgkin Lymphoma (NHL)
B, T, NK cell lymphoma (predom B); over 20 types
Etio unknown: genetic, environemntal. H. pylori - gastric lymphoma
Ongogenic viruses: EB, Kaposi sarcoma herpesvirus, Retroviruses
Risk factors: Sjogren syndrome, AIDS
Usually multifocal at diagnosis
Affect: Waldeyer’s rings, abdominal mesenteric nodes
Extranodal common
Signs/Symptoms: painless enlarged nodes, fever, weight loss, extranodal tumor
Treatment: Chemo + Radiation, stem cell transplant
Curability < 25%
Hodgkin, Non-Hodgkin Lymphoma - Oral Manifestations
Cervical lymphadenopathy
Extranodal, intraoral tumor (painless/painful)
- Waldeyer’s ring: soft palate, oropharynx
- Salivary glands, mandible
- Swellign of head/neck lymph nodes
- Tumor of palate, gingiva, buccal sulcus, FOM
- Note: immunosupressed pts and Sjogren greater risk for lymphoma
Crateriform oral ulcers
Fever
burning mouth symptoms
Radiation:
xerostomia (salivary substitute, pilocarpine),
damage taste buds,
trismus of masticatory muscles,
affect craniomandibular development
Osteoradionecrosis (prophylactic Abx, hyperbaric O2, Abx post op)
Burkitt Lymphoma
Aggressive B cell lymphoma (NHL) with mature B cells expressing surface IgM
Histology: starry sky pattern
Most common lymphoma of childhood
Types (all assoc’ EBV)
- Endemic BL - Central Africa age 7, mostly affect jaw
- Sporadic BL - Western, older children, 30’s
- HIV
Rapid growing: double in size every 3 days
- obstruct airway, alimentary canal vasculature
Can spread to CNS
Medical Tx: chemotherapy
Presentation: extranodal sites
- Endemic type:
- predilection for jaw (young children)
- abdominal organs (kidney, ovary, adrenal glands)
- Sporadic type: abdominal mass
Oral Manifestation
Jaw lesion: ill-defined RL, floating teeth, distal to last mand molar (max/mand involv)
Pain, paresthesia
Multiple Myeloma
Overproduction of cloned malignant plasma cells with Ig secretion. Can result in multiple masses throughout skeletal system
Lab: Bence-Jones proteins in urine (light chain)
Pathophysiology:
- plasma cell prolif, Ig production,
- bone resorp (serum hypercalcemia)
- marrow replacement (anemia, leukopenia, thrombocytopenia)
- Infection #1 cause of death
- Renal failure #2 (dmg from excretion of light chains of Ig)
Signs/Symptoms
- Radiographic punched out leasions multiple
- Persistent bone pain (spine, rib, sternum), pathologic fracture
- Anemia
- Hypercalcemia
Treatment: Chemo, radiation, Bisphosphonates
Oral manifestations:
- jaw lesion,
- swollen/painful amyloid deposits in soft tissue
- oropharyngeal tumors
Dental Treatment of White Blood Cell Disorders
Supportive care only for terminal patients
Stable patients may receive any indicated Tx
Antibiotic Prophylaxis if WBC < 2000, neutrophil < 500
Mucositis management
Bleeding/bruising: thrombocytopenia (med consult, platelet infusion)
Cardiovascular: dmg by radiation and chemo
Immunosupressants, steroids (transplant)
- gingival hyperplasia with Cyclosporin
Paresthesia assc’ chemo
Extraction 3 weeks before chemo/rad
Infection, ulcers due to neutropenia
Fungal infection
Effect on jaw/teeth development (chemo)
Bisphosphonates
