Term
| Clinical features of hematologic disease |
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Definition
| Infection (bacterial, fungal, viral), gingivitis, ulceration, recurrent aphthae, hemorrhage, pain & nerve damage, angular cheilitis, cervical lymphadenopathy & tonsil enlargment, Glossitis, petechiae, blood blister |
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Term
| Suitable screening hematologic failure: |
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Definition
1. BT (bleeding time)
2. CT (clotting time)
3. Platelet count
4. PT-protime
5. PTT - partial thromboplastin time or activated PTT |
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Term
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Definition
Decrease in # of circulating erythrocytes
Low hemoglobin, low RBC count, low HCT |
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Term
| Systemic manifestations of anemia |
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Definition
koilonychia, brittal nails
Weakness/tired, shortness of breath, pallor, tachycardia
paresthesias of extremities
Gait disturbances
motor impairment
Loss of position or vibration sense
Disturbances of taste and smell
Disturbances of memory & affect, forgetfullness, mental slow, depression, irritability or paranoid psychosis
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Term
| Oral manifestations of anemia |
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Definition
| Stomatitis, angular cheilitis (58%), atrophic glossitis, glossodynia, recurrent oral ulcerations, oral candidiasis, mucosal pallor, taste disturbances. |
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Term
| Etiology of Vitamin B12 deficiency (pernicious anemia) |
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Definition
Megaloblastic anemia due to impaired DNA synthesis
Autoimmune, gastrectomy, H. pylori infection |
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Term
| Clinical manifestations of pernicious anemia (Vit B12 deficiency) |
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Definition
| Generalized weakness; glossodynia, atrophic glossitis (Hunter's glossitis, Moeller's glossitis); numbness or tingling |
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Term
| Oral manifestations of pernicious anemia |
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Definition
Normal tongue, glossodynia, atrophic glossitis, recurrent aphthous ulcers, mucosal pallor
Fissured and mild geographic tongue |
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Term
| Lab findings/diagnosis of pernicious anemia |
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Definition
macrocytic erythrocytes, multisegmented neutrophils; decreased Vit B12; decreased MMA, Schiling test; atrophic glossitis;antibodies to intrinsic factor are present in serum and saliva
Oral biopsy: mucosal atrophy, increased N/C ratio, chronic inflammation |
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Term
| Treatment of pernicious anemia |
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Definition
Colabamin injection, high-dose oral or nasal spray
Life-long
Prevents further degernation of nerves |
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Term
| Prognosis of pernicious anemia |
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Definition
Good w/ treatment
Gastric carcinoma 1-2% |
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Term
| Folate deficiency etiology |
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Definition
- Megaloblastic anemia due to impaired DNA synthesis
- Associated with: alcoholism, hemodialysis, phenytoin, oral contraceptives, methotrexate
- Assoc. w/ cleft lip, cleft palate, neural tube defects, increased risk of colon cancer & heart disease & stroke
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Term
| Oral manifestations of folate deficiency |
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Definition
- Sore, atrophic glossitis
- Angular cheilitis
- Candidiasis
- Recurrent aphthous ulcers |
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Term
| Treatment & prognosis of folate deficiency |
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Definition
Replacement therapy
Excellent w/ treatment |
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Term
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Definition
| reduced production in # of cells |
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Term
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Definition
| decreased intake of iron, excessive blood loss women>men, increased demand, decreased absorption of iron (gastrectomy, celiac sprue) |
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Term
| Clinical features of iron deficiency |
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Definition
| Fatigue, palpitations, lightheadedness, lack of energy |
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Term
| Oral manifestations of iron deficiency |
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Definition
| Angular cheilitis, atrophic glossitis, mucosal atrophy, candidiasis, increased risk of aphthous ulcers |
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Term
| Lab diagnosis of iron deficiency |
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Definition
- CBC and differential reveals microcytic hypochromic anemia
- Total ferritin decreased, total iron binding capacity |
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Term
| Treatment and prognosis of iron deficiency anemia |
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Definition
- Replacement therapy
- PX: iron deficiency w/ increased risk of cancer of upper aerodigestive tract; Plummer-Vinson Syndrome (patternson-Kelly syndrome, sideropenic dysphagia) - women in 4th-5th decades, mucosal atrophy, impaired cell-mediated immune response
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Term
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Definition
| Esophageal webs, difficulty swallowing (dysphagia), yellowish/dry skin, atrophic glossitis, increased risk of dev oral cancer and cancer of URT |
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Term
| Defintion of aplastic anemia "pancytopenia" |
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Definition
| Reduced # of cells due to failure in production |
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Term
| Etiology of aplastic anemia "pancytopenia" |
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Definition
| Immune mediated cytotoxic T lymphocytes, Idiopathic, radiation, chemo, drugs (chloramphenicol, gold, NSAIDs); toxins-benzene, viruses (papovavirus; HIV; non-A, non-B, non-C, non-G hepatitis); hereditary disease-Fanconi's anemia (autosomal recessive), dyskeratosis congenita |
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Term
| Clinical features of aplastic anemia |
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Definition
Lightheadedness, tachycardia, weakness
Thrombocytopenia-bruising & bleeding tendency
Bacterial and fungal infections |
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Term
| Oral manifestations of Aplastic anemia |
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Definition
| Oral petechiae (pinpoint), purpura, ecchymosis (large areas), hemorrhage, pale mucosa, minimal erythema around ulcers, gingival hyperplasia |
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Term
| Aplastic anemia: diagnosis and histology |
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Definition
Pancytopenia: decrease in all formed elements
DX: complete blood cell count & differential, bone marrow aspiration or biopsy
- Complete blood cell count exhibits at least 2 of the following: <500 granulocytes, <20,000 platelets, <10,000 reticulocytes
- Bone marrow aspiration and biopsy
- Histology: marrow hypoplasia (increased fat and decreased cellular elements)
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Term
| Treatment for aplastic anemia |
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Definition
Withdrawal of offending agent if known
Supportive therpay including transfusions
Androgenic steroids helpful in mild disease
If immuned mediated: corticosteroids, anti-lymphocyte globulin and cyclosporine
Bone marrow transplant
**very serious disease
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Term
| Asplastic anemia prognosis |
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Definition
Unpredictable-guarded
Anti-lymphocyte globulin and cyclosporine, help 90% 3 yr SR
Bone marrow transplant, risk of Graft vs. Host disease, may recur, increased risk of hematologic malignancy (acute leukemia) |
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Term
| Hemolytic anemia definition |
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Definition
| Anemia due to increased destruction or loss of cells; reduced RBC survival (destruction in spleen and liver) |
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Term
| Intrinsic defects for hemolytic anemia |
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Definition
- Hereditary spherocytosis: most common RBC membrane defect
Glucose-6-phosphate dehydrogenase enzyme defect
Pyruvate kinase deficiency = enzyme deficiency --> chronic hemolytic anemia
Thalassemia: alpha or beta chains; inherited autosomal recessive
sickle cell anemia
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Term
| Extrinsic defects of hemolytic anemia |
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Definition
Erythroblastosis fetalis
Transfusion rxns
Mechanical trauma-prosthetic heart valve
Disseminated intravascular coagulation (DIC)
Hyperplenism
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Term
| Characteristics of Thalassemia |
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Definition
Mediterranean, African, Indian, Southeast Asian
Excess alpha or beta globulin chains results in abnormal RBCs filtered out by spleen --> microcytic, hypochromic anemia
Common
Wide range of clinical severity (over 200 known mutations)
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Term
| Alpha genes of thalassemia |
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Definition
1 abnorm gene = no clinical disease
2 abnorm genes = alpha-thalassemia trait
3 abnorm genes = HbH disease
4 abnorm genes = hydrops fetalis |
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Term
| Beta genes of thalassemia |
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Definition
Minor: heterozygous
Major: homozygous, Cooley's anemia or Mediterranean fever |
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Term
| Diagnosis/Lab findings w/ Alpha Thalassemia |
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Definition
| Hypochromic, microcytic anemia (same with iron deficiency) |
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Term
| Diagnosis/Lab findings of beta thalassemia |
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Definition
| Hypochromia, dark cell is orthochromic normoblast, microcytic |
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Term
| Oral manifestations of thalassemia |
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Definition
| Prominent premaxilla, malocclusion, mucosal pallor, development of dentition delayed by one year |
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Term
| Radiographic features of thalassemia |
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Definition
| Osteoporosis, loss of trabeculae, coarse trabeculae, "Hair on end" |
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Term
| Treatment for thalassemia |
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Definition
Thalassemia major-blood transfusion
Bone marrow transplant
Surgery to remodel facial defects |
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Term
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Definition
w/out treatment-death in 1st year of life w/ major
Hemochromatosis-toxic to heart, liver, endocrine
Prevention - screen to detect carries; prenatal diagnosis |
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Term
| Etiology of sickle cell anemia |
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Definition
point mutation HbS
Thymine substitution adenine in DNA, codon altered to valine rather than glutamic acid in the beta-globin chain of hemoglobin
RBCs - marked tendency for deformation |
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Term
| Inheritance pattern of sickle cell anemia |
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Definition
Autosomal co-dominant, one gene 40-50% Hgb S
Heretozygous-trait, homozygous-disease
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Term
| Clinical features of sickle cell anemia |
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Definition
| Low oxgen tension, infection, hypothermia, dehydration |
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Term
| Clinical features of sickle cell anemia |
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Definition
| Low oxygen tension, infection, hypothermia, dehydration |
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Term
| What is sickle cell crisis? |
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Definition
Sequestration of RBCs, infarcts in femoral head, osteomyelitis, spleen --> atrophy, skin of ankle ulcers, CHF, CVA
Aplastic splenic atrophy predisposes to infection |
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Term
| Treatment for sickle cell anemia |
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Definition
| Penicillin, hydoxyurea to increase fetal hemoglobin F, bone marrow transplant |
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Term
| Prognosis of sickle cell anemia |
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Definition
| Variable, genetic screening/prenatal diagnosis |
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Term
| Definition of erythoblastosis fetalis |
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Definition
| Hemolytic anemia of newborns |
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Term
| etiology of erythoblastosis fetalis |
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Definition
| blood component incompatibility b/t mother and fetus, usually Rh factor |
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Term
| Clinical features of erythoblastosis fetalis |
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Definition
Intrinsic stain (blood pigments deposited in teeth, green-blue-brown)
Enamel hypoplasia |
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Term
| Differential diagnosis of erythoblastosis fetalis |
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Definition
tetracycline yellow-brown
porphyria red-brown |
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Term
| Treatment for erythoblastosis fetalis |
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Definition
Dental: none necessary, usually affects only primary dentition
Systemic: whole body transfusion |
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Term
| Prognosis of erythoblastosis fetalis |
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Definition
| Use of anti-antigen gamma globulin after delivery prevents formations of antibodies that would endanger future pregnancies |
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Term
| Definition of polycythemia rubra vera |
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Definition
myeloproliferative disease: increased #s of RBCs and platelets, plus or minus increase in granulocytes
>40 years, median 60 M=F |
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Term
| Etiology of polycythemia rubra vera |
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Definition
| 95% tyrosine kinase gene mutation (Janus kinase 2) |
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Term
| Clinical features of polycythemia rubra vera |
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Definition
Headache, weakness, dizziness, drowsiness, visual disturbances, sweating, weight loss, dyspnea, epigastric pain, generalized pruritis (40%), erythromelalgia
Vascular thrombosis --> CVA, MI
Hemorrhage, epistaxis, ecchymoses, ginival |
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Term
| Treatment for polycythemia rubra vera |
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Definition
| Phlebotomy, aspirin, antihitamines, anagrelide hydrochloride (selectively inhibits megakaryocyte maturation and platelet production), myelosuppressive agents, hydroxyurea |
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Term
| Prognosis for polycythemia rubra vera |
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Definition
Fair, average survival 10-12 yrs after diagnosis
Myelofibrosis 15%
Leukemia 2-10% usually acute |
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