د.زينة كاملORAL MEDICINE
Dental management and Oral manifestation of blood diseases
1-Disorders of RBCs
ANEMIA
Anemia, which is defined as a reduction in the oxygen-carrying capacity of the blood, usually is associated with a decreased number of circulating RBCs or an abnormality in the Hb contained within the RBCs.Anemia is not a disease but rather a symptom complex that may result from one of three underlying causes:(1) decreased production of RBCs (iron deficiency, pernicious anemia, and folate deficiency). (2) Blood loss (3) Increased rate of destruction of circulating RBCs (hypersplenism, autoimmune destruction).
Iron Deficiency Anemia
Iron deficiency anemia is a microcytic anemiathat can be caused by excessive blood loss, poor iron intake, poor iron absorption, or increased demand for iron.Blood loss may occur with menstruation or by bleeding from the gastrointestinal tract.Poor intake is more common in children who live in developing countries. Malabsorption of iron can result from gastrectomy or intestinal disease that reduces absorption of iron from the duodenum and the jejunum. Increased demand is associated with chronic inflammation (autoimmune disease).In women, menstruation and pregnancy contribute to the development of iron deficiency anemia.
Oral manifestations
• Paresthesia.
• Loss of papillae on dorsum of tongue.
• In rare cases, infection and bleeding complications.
• In patients with dysphagia, increased incidence of carcinoma of oral and pharyngeal areas (Plummer-Vinson syndrome).
Dental management
• Detection and referral for diagnosis and treatment.
In general no modification in treatment planning is required.
Folate Deficiency Anemia and Pernicious Anemia
Vitamin B12 (cobalamin) and folic acid are needed for RBC formation and growth within bone marrow.Vitamin B12 is a cofactor in methionine-associated enzymatic reactions required of protein synthesis and thus in the maturation of RBCsFolate is needed for enzymatic reactions required for the synthesis of purines and pyrimidines of deoxyribonucleic acid (DNA) and ribonucleicacid (RNA) and thus for the synthesis of proteins.
A deficiency in daily intake (as in chronic alcoholism) or absorption (due to celiac disease or tropical sprue) of these vitamins can result in anemia.
Pernicious anemia is caused by a deficiency of intrinsic factor, a substance secreted by the stomach parietal cells that is necessary for absorption of vitamin B12 (cobalamin).Because vitamin B12 may be stored for several years, this form of nutritional deficiency is rare and usually does not develop until late adulthood.
Oral manifestations
• Paresthesia of oral tissues (burning, tingling, numbness)
• Delayed healing (severe cases), infection, bald red tongue, and angular chelitis.
• Petechial hemorrhages.
Dental management
Detection and medical treatment (early detection and treatment can prevent permanent neurologic damage). No modification required in treatment planning if the patient under medical care.
Hemolytic Anemia: Glucose-6-Phosphate Dehydrogenase Deficiency
Glucose-6-phosphate dehydrogenase (G-6-PD) is an enzyme that helps the RBC to turn carbohydrates into energy.The G-6-PD gene is located on the X chromosome; thus, disease inheritance is gender linked.Drugs are the most common trigger for hemolysis in G-6-PD MED deficiency. Of more than 40 drugs that can induce hemolysis, those of significance in dental practice include acetylsalicylic acid, acetophenetidin (phenacetin), dapsone, ascorbic acid, and vitamin K. Fava bean ingestion is the most common dietary cause of hemolytic anemia in persons with G-6-PD deficiency.
Oral manifestation….. None
Dental management
• Control infection.
• Avoid drugs such as certain antibiotics, or that contain aspirin, or acetaminophen, which may increase risk for hemolytic anemia.
• Be aware that these patients also often have increased sensitivity to sulfa drugs and chloramphenicol.
• Usually no modification in treatment planning is required unless anemia is severe; then, performs only procedures to meet urgent dental needs.
Sickle Cell Anemia
Hemoglobinopathies such as sickle cell anemia are inherited as autosomal recessive traits.The RBC in sickle cell anemia becomes sickle-shaped when blood experiences lowered oxygen tension or decreased pH, or when the patient becomes dehydrated.Sickle cell disorders are distinguished by the number of globin genes affected.
Complications of sickle cell anemia can occur at any age, but patients in the following age groups are more likely to manifest certain complications:
1. Birth to 20 years of age: painful events, stroke, acute chest syndrome (fever, chest pain, wheezing, cough, and hypoxia), acute anemia and infection
2. from 20 to 40 years of age: osteonecrosis of hip and shoulder joints, leg ulcers, priapism, liver disease, and gallstones
3. Older than 40 years of age: pulmonary hypertension, nephropathy, proliferative retinopathy, and cardiac enlargement, heart murmurs, and sudden death due to arrhythmias.
Oral manifestations
• Atypical trabecular pattern.
• Delayed eruption of teeth, growth abnormalities.
• Hypoplasia of teeth.
• Pallor of oral mucosa.
• Bone pain.
• Osteoporosis.
Dental management
• Consult with patient’s physician to ensure that condition is stable.
• Avoid any procedure that may produce acidosis or hypoxia (avoid long, complicated procedures).
• Drug modifications:
• Avoid excessive use of barbiturates and narcotics, because suppression of the respiratory center may occur, Use benzodiazepine instead.
• Avoid excessive use of salicylates; codeine and acetaminophen in moderate dosage can be used for pain control.
• Avoid the use of general anesthesia, because hypoxia can lead to precipitation of acute crisis.
• Nitrous oxide may be used, provided that 50% oxygen is supplied at all times; it is critical to avoid diffusion hypoxia at the termination of nitrous oxide administration. For nonsurgical procedures, use local without vasoconstrictor; for surgical procedures, use 1:100,000epinephrine in anesthetic solution.
1. Aspirate before injecting.
2. Inject slowly.
3. Use no more than two cartridges.
4. It is necessary to prevent infection. Use prophylactic antibiotics for major surgical procedures.
5. If infection occurs, manage aggressively, with the use of:
. Incision and drainage
. Antibiotics
. Corrective treatment (e.g., extraction, pulpectomy)
. Avoid dehydration in patients with infection and in patients who are receiving surgical treatment.
Aplastic Anemia
Aplastic anemia occurs when the bone marrow is unable to produce adequate numbers of RBCs, white blood cells, and platelets.The hematopoietic stem cells are unable to proliferate, differentiate, or give rise to mature blood cells.Some cases of aplastic anemia are caused by drugs, viruses, organic compounds, and radiation.Anticonvulsants, antibacterial, antidiabetic drugs, diuretics, sulfonamides, and synthetic antithyroid drugs are the drugs most commonly associated with aplastic anemia. The most common viral infection associated with aplastic anemia is viral hepatitis.Aplastic anemia is described as moderate, severe, or very severe based on the degree of pancytopenia.
Oral manifestation
• Gingival bleeding
• Petechiae
• Ecchymosis
• Oral infection
• Pallor of mucosa
Dental management
• Referral for medical diagnosis and treatment.
•Avoidsome drugs (anticonvulsants, antithyroid drugs, select antidiabetic agents, diuretics and sulfonamides) are associated with higher incidence of aplastic anemia
• During periods of low blood count (platelets, neutrophils, red blood cells), provide emergency care only.
• Antimicrobial agents and supportive therapy are needed for oral infection.
Thalassemia
It is a kind of severe anemia; Thalassemia can be classified according to the chain involved. In beta thalassemia there is deficientsynthesis of beta globin, whereas in alpha thalassemia there is deficient synthesis of alpha globin.
Oro-facial manifestations of thalassemia
- Enlargement of the upper jaw.
- Migration and spacing of upper anterior teeth.
- Increase in dental decay.
- Delayed dental development.
- Change in dental morphology.
- Alveolar bone may have a ‘chickenwire-like’ radiological appearance
- Painful swelling of parotids and xerostomia (due to iron deposits)
- Mucosal Pallor and dental discolouration
- Sore or burning tongue due to folate deficiency
- Oral ulceration (very rare)
- Necrotizing gingivostomatitis (very rare)
Dental management
Before beginning the treatment the following should be determined:
Type of Thalassemia
Present hemoglobin level of the patient
Degree of iron overload in the body and Relative organ involvement with iron
Presence or absence of splenomegaly and History of splenectomy
Overall patient prognosis and life expectancy
The teeth can be significantly affected in patients with thalassemia, but proper transfusion therapy can prevent many of the changes. However, close dental and orthodontic monitoring is crucial. In addition to regular annual dental care, thalassemia patients should be evaluated by a dentist to determine if bony changes requiring orthodontic treatments have developed.
Furthermore, splenectomy can complicate dental care due to increased risk of infection. Prior to dental work, which is likely to cause bleeding of the gums, Periodontitis and gingivitis, patients should receive dental prophylaxis antibiotics.Proper coagulation and Hb leveltests should be performed before undergoing any surgical procedure.Hepatotoxic drugs as Tetracycline, metronidazole and erythromycin should be avoided entirely. Paracetamol is a safealternative to NSAIDS and aspirin.
Dental management of a thalassemia patient requires special concern as this patient could be a heart patient, a diabetic, a splenectomy or a patient with compromised immunity and liver function. A dentist should have profound knowledge to manage this medically compromised state of a thalassemia patient. The best way is to have a multidisciplinary approach involving a dental surgeon, a hematologist and an orthodontist to safely provide dental treatment to these patients with seemingly blood dyscrasia.
2-Disorders of WBCs
The following box shows the classification and features of WBC dyscrasias:
LEUKOCYTOSIS AND LEUKOPENIA
The number of circulating WBCs normally ranges from 4400 to 11,000/μL in adults. The differential WBC count is an estimation of the percentage of each cell type per microliter of blood.The term leukocytosis is defined as an increase in the number of circulating WBCs (lymphocytes or granulocytes) to more than 11,000/μL, and leukopenia as a reduction in the number of circulating WBCs.
Cyclic Neutropenia
An important form of leukopenia involving the cyclic depression of circulating neutrophils is a disorder called cyclic neutropenia.In this condition, patients have a periodic decrease (at least a 40% drop) in the number of neutrophils (about every 21 to 28 days). During the period in which few circulating neutrophils are present.
Oral manifestations of cyclic neutropenia
• Periodontal disease
• Oral infection
• Oral ulceration similar to that of aphthous stomatitis.
Dental management of cyclic neutropenia
• Antibiotics should be given to prevent infection.
• Serial white blood cell (WBC) counts should be performed to identify the safest period for dental treatment (i.e., when the WBC count is closest to normal level).
• Modifications not required when the WBC count (neutrophils) is normal.
• If the WBC count (neutrophils) is depressed severely, antibiotics should be provided to prevent postoperative infection.
Leukemia
Leukemia is cancer of the WBCs that affects the bone marrow and circulating blood. It involves exponential proliferation of a clonal myeloid or lymphoid cell and occurs in both acute and chronic forms.Acute leukemia is a rapidly progressive disease that results from accumulation of immature, functionless WBCs in the marrow and blood. Chronic leukemia have a slower onset, which allows production of larger numbers of more mature (terminally differentiated), functional cells.The cause of leukemia remains unknown. Increased risk is associated with large doses of ionizing radiation, certain chemicals (benzene), and infection with specific viruses (e.g., Epstein-Barr virus [EBV], human lymphotropic virus [HTLV]-1). Cigarette smoking and exposure to electromagnetic fields also have been proposed to be causative.
ACUTE MYELOGENOUS LEUKEMIA
AML is a neoplasm of myeloid (immature) WBCs, which demonstrate uncontrolled proliferation in the bone marrow space and subsequently appear in the peripheral blood.AML arises de novo in younger adults or secondarily in elderly persons as a consequence of myelodysplasia.
ACUTE LYMPHOID LEUKEMIA
ALL is the result of uncontrolled monoclonal proliferation of immature lymphoid cells in the bone marrow and peripheral blood. These neoplastic cells may also expand in the lymph nodes, liver, spleen, or CNS.Although environmental, infectious, and genetic factors are considered likely causes of the disease, causal links for ALL have not been established.
CHRONIC MYELOGENOUS LEUKEMIA
(CML) is a neoplasm of mature myeloid WBCs.
CHRONIC LYMPHOCYTIC LEUKEMIA
(CLL) is a neoplasm of mature clonal CD5+ B lymphocytes.
Oral manifestations of leukemia
• Gingival swelling/ enlargement
• Mucosal or gingival bleeding
• Oral infection and mucositis.
• delayed healing.
Dental management of leukemia
• Referral for medical diagnosis, treatment, and consultation
• Complete blood count to determine risk for anemia, bleeding, and infection
• Antibiotics, antivirals, and antifungals provided during chemotherapy to prevent opportunistic oral infection
• Chlorhexidine rinse to manage mucositis.
• Inspect head, neck, and radiographs for undiagnosed or latent disease (e.g., retained root tips, impacted teeth) and infections that require management before chemotherapy.
• Eliminate infections before chemotherapy.
• Extractions should be performed at least 10 days before initiation of chemotherapy.
• Plaque control measures and chlorhexidine during chemotherapy.
• Use prophylactic antibiotics if WBC count is less than 2000/μL, or neutrophil count is less than 500/μL (or 1000 at some institutions).
• Platelet replacement may be required (if platelet count is <50,000/μL) when invasive dental procedures are performed.
LYMPHOMAS
Lymphoma is cancer of the lymphoid organs and tissues that presents as discrete tissue masses. Lymphomas represent the seventh most common malignancy worldwide.These diseases are of importance in dental management because initial signs often occur in the mouth.
HODGKIN LYMPHOMA
Hodgkin lymphoma (HL) is a neoplasm (exhibiting uncontrolled growth) of B lymphocytes.It is the most common lymphoma in young adults. HL has two peaks of incidence, one in early adulthood and the other around the fifth decade of life. For a long time HL was referred to as Hodgkin’s disease and NHL as non-Hodgkin’s lymphoma.Men are at slightly higher risk for developing the disease (1.4: 1 male-female ratio). In developing countries, HL is found primarily in children, and the incidence decreases with age, in contrast with industrialized countries, where it is uncommon in children.
NON-HODGKIN LYMPHOMA
NHL comprises a large group of lymphoproliferative disorders classified as of B cell or T cell origin. More than 80% of these neoplasms are of B cell origin.
BURKITT LYMPHOMA
Burkitt lymphoma is an aggressive B cell (non-Hodgkin) .The tumors are composed of mature B cells that express surface IgM.
Oral manifestation of lymphomas
• Extranodal oral tumors in Waldeyer’s ring or osseous soft tissues.
• Xerostomia in patients treated by radiation; some of these patients prone to osteonecrosis.
• Burning mouth or tongue symptoms
• Petechiae or ecchymosis if thrombocytopenia present because of tumor invasion of bone marrow.
• Cervical lymphadenopathy.
• Mucositis in patients treated by radiation therapy or chemotherapy.
- Non-Hodgkin lymphoma may be found in patients with AIDS; hence, transmission of infectious agents may be a problem.
Dental management of lymphomas
• Patients with generalized lymphadenopathy, extranodal tumors, and osseous lesions must be identified and referred for medical evaluation and treatment.
• The dentist can do biopsy extranodal or osseous lesions to establish a diagnosis; patients with lesions involving the lymph nodes should be referred for excisional biopsy.
• Medical history and medical consultation will be needed to establish current status.
• Before invasive procedures, a complete blood count should be obtained to determine risks for bleeding and infection.
• Patients who have been treated by irradiation to the chest area may develop acute and chronic cardiovascular complications such as arrhythmias or valvular heart disease. Medical consultation is needed to confirm their current status.
• Patients in terminal phase should receive only supportive dental treatment.
• Patients under “control” may receive any indicated treatment; however, complex restorative treatment may not be indicated in cases with a poor prognosis.
• Platelet replacement may be needed for patients with thrombocytopenia.
• Consider prophylactic antibiotics if the WBC count is less than 2000/μL, or the neutrophil count is less than 500 (or 1000 at some institutions).
MULTIPLE MYELOMA
Multiple myeloma (MM) is a lymphoproliferative disorder that results from overproduction of cloned malignant plasma cells that results in multiple tumorous masses scattered throughout the skeletal system.
Oral manifestation
• Soft tissue tumors
• Osteolytic lesions
• Amyloid deposits in soft tissues
• Unexplained mobility of teeth
• Exposed bone
Dental management
• Patients with oral soft tissue lesions and/or osseous lesions should have them biopsied by the dentist or should be referred for diagnosis and treatment as indicated.
• Medical history and medical consultation is needed to establish current status.
• Be aware of and take precautions for bisphosphonate-induced osteonecrosis of the jaws.
• For patients in terminal stage, provide supportive dental care only.
• Long-term prognosis is poor, so complex dental procedures may not be indicated.
• If thrombocytopenia or leukopenia is present, special precautions (platelet replacement, antibiotic therapy) are needed to prevent bleeding and infection when invasive dental procedures are performed.