| |
|
|
| Year : 2011 | Volume
: 22
| Issue : 3 | Page : 454-461 |
|
| Orofacial manifestations of hematological disorders: Anemia and hemostatic disorders |
|
|
Titilope A Adeyemo, Wasiu L Adeyemo, Adewumi Adediran, Abd Jaleel A Akinbami, Alani S Akanmu
Departments of Haematology and Blood Transfusion and Oral and Maxillofacial Surgery, College of Medicine, University of Lagos, Nigeria
Click here for correspondence address and email
| Date of Submission | 06-Aug-2009 |
| Date of Decision | 09-Nov-2009 |
| Date of Acceptance | 06-Feb-2010 |
| Date of Web Publication | 3-Nov-2011 |
|
|
 |
|
 Abstract | | |
The aim of this paper is to review the literature and identify orofacial manifestations of hematological diseases, with particular reference to anemias and disorders of hemostasis. A computerized literature search using MEDLINE was conducted for published articles on orofacial manifestations of hematological diseases, with emphasis on anemia. Mesh phrases used in the search were: oral diseases AND anaemia; orofacial diseases AND anaemia; orofacial lesions AND anaemia; orofacial manifestations AND disorders of haemostasis. The Boolean operator "AND" was used to combine and narrow the searches. Anemic disorders associated with orofacial signs and symptoms include iron deficiency anemia, Plummer-Vinson syndrome, megaloblastic anemia, sickle cell anemia, thalassaemia and aplastic anemia. The manifestations include conjunctiva and facial pallor, atrophic glossitis, angular stomatitis, dysphagia, magenta tongue, midfacial overgrowth, osteoclerosis, osteomyelitis and paraesthesia/anesthesia of the mental nerve. Orofacial petechiae, conjunctivae hemorrhage, nose-bleeding, spontaneous and post-traumatic gingival hemorrhage and prolonged post-extraction bleeding are common orofacial manifestations of inherited hemostatic disorders such as von Willebrand's disease and hemophilia. A wide array of anemic and hemostatic disorders encountered in internal medicine has manifestations in the oral cavity and the facial region. Most of these manifestations are non-specific, but should alert the hematologist and the dental surgeon to the possibilities of a concurrent disease of hemopoiesis or hemostasis or a latent one that may subsequently manifest itself. Keywords: Anemia, hemostatic disorders, manifestation, orofacial
How to cite this article:
Adeyemo TA, Adeyemo WL, Adediran A, Akinbami AA, Akanmu AS. Orofacial manifestations of hematological disorders: Anemia and hemostatic disorders. Indian J Dent Res 2011;22:454-61 |
How to cite this URL:
Adeyemo TA, Adeyemo WL, Adediran A, Akinbami AA, Akanmu AS. Orofacial manifestations of hematological disorders: Anemia and hemostatic disorders. Indian J Dent Res [serial online] 2011 [cited 2022 Aug 8];22:454-61. Available from: https://www.ijdr.in/text.asp?2011/22/3/454/87070 |
A wide array of hematological disorders encountered in internal medicine has manifestations in the oral cavity and the facial region. [1] Most of these manifestations are non-specific, but should alert the hematologist and the dental surgeon to the possibilities of a concurrent hematological disorder or a latent one that may subsequently manifest itself. [1] These manifestations must be properly recognized if the patient is to receive appropriate diagnosis and referral for treatment. The importance of understanding the orofacial manifestations of these disorders also lies in the fact that orofacial signs and symptoms may be the first clinical presentation that alerts the dentist/hematologist to an underlying hematological disorder.
Disorders of the erythroid (anemia and polycythaemia), lymphoid and megakaryocytic-platelet compartment of the bone marrow, immune system deficiencies, coagulation as well as human immunodeficiency virus infection have been widely reported to manifest in the orofacial region. [1],[2],[3],[4],[5],[6]
In this review of orofacial manifestations of hematological disorders, orofacial manifestations of anemia and disorders of hemostasis are discussed.
A computerized literature search using MEDLINE was conducted for published articles on orofacial manifestations of hematological diseases, with emphasis on anemia. Mesh phrases used in the search were: oral diseases AND anaemia; orofacial diseases AND anaemia; orofacial lesions AND anaemia; orofacial diseases AND anaemic disorders. The Boolean operator "AND" was used to combine and narrow the searches. The full texts of these articles were thoroughly examined. References in these articles were also manually searched for non-Medline articles. Only relevant articles were selected for the review.
| Orofacial Manifestations of Anemia | |  |
Iron deficiency anemia
Iron deficiency anemia is the most common hematological disorder. [2] It may manifest in the orofacial region as atrophic glossitis, mucosal pallor and angular cheilitis. Atrophic glossitis "flattening of the tongue papillae" resulting in a smooth and erythematous tongue may mimic migratory glossitis [Table 1]. Migratory glossitis, also known as geographic tongue, is a condition of unknown etiology that affects 12% of the population. [1] It results in lesions on the tongue that are erythematous, non-indurate, atrophic and bordered by a slightly elevated, distinct rim that varies in color from gray to white. In atrophic glossitis, these areas do not have a white keratotic border and they increase in size rather than changing in position. In more severe cases, the tongue may be tender. Angular stomatitis (painful fissures at the corners of the mouth) and cheilosis (dry scaling of the lips and corners of the mouth) are also common findings associated with iron deficiency anemia. [1] Angular cheilitis, however, is often associated with fungal infections (Candida albicans), lip-sucking and dehydration. [7] Treatment must focus on correcting the deficiency state and providing adequate energy, protein, fluids and nutrients to promote healing. When angular cheilitis is due to opportunistic infections brought on by decreased resistance secondary to nutrient deficiencies, treatment should focus on antifungal therapy, correction of the nutrient deficiency and diet modification to make eating a more comfortable experience. [7]
The Plummer-Vinson syndrome More Details
This is otherwise called the Patterson-Brown-Kelly syndrome or sideropenic dysphagia. It is a symptom complex caused by iron deficiency. [8] This syndrome manifests as atrophic glossitis, or angular cheilitis, and, occasionally, hyperkeratotic lesions are seen in the oral mucosa. It is also associated with koilonychias (or spoon nails), pagophagia and dysphagia due to pharyngoesophageal ulcerations and esophageal webs. [8]
Megaloblastic anemia
This may be caused by a vitamin B12 deficiency (commonly from pernicious anemia, surgical resection of the ileum or small intestinal diverticula) or by a folic acid deficiency (most commonly from malnutrition). [9],[10] Vitamin B12 deficiency manifests in the oral cavity as part of megaloblastic changes in the entire gastrointestinal tract, which are so well demonstrated morphologically in the bone marrow. [9],[10] The oral manifestations of painful atrophy of the entire oral mucous membranes and tongue (glossitis), stomatitis as well as mucosal ulceration (recurrent aphthous ulcers) in vitamin B12 and folate deficiency have long been recognized [Table 1]. [9],[10] These oral changes may occur in the absence of symptomatic anemia or of macrocytosis. "Magenta tongue," which is said to be rather characteristic, may herald a B12 deficiency. [10]
Sickle cell anemia
Sickle cell disease is generically used to describe a group of disorders characterized by the production of abnormal hemoglobin S (HBS). [11] The entities include sickle cell anemia (HbSS), sickle cell Hb C disease (HbSC) and sickle cell-β thalassaemia.
Sickle cell anemia (HbSS) is the most common type and represents the homozygous form where the individuals inherit a double dose of the abnormal gene that codes for hemoglobin S. The sickle hemoglobin abnormality is caused by substitution of valine for glutamic acid in the sixth position from the NH 2 terminal end of the β-globin chain. With decreased oxygen tension, the abnormal hemoglobin polymerizes, forming fluid polymers (tactoids) that cause the red cells to deform into a characteristic sickle shape that may plug different areas of the microcirculation or large vessels. [11]
The hallmark features of sickle cell disease are chronic hemolytic anemia and vaso-occlusion resulting in ischemic tissue injury. [11] While a wide spectrum of complications result from these, the major manifestation of concern is "sickle crisis" of aplastic, hemolytic or painful (vaso-occlusive) types leading to devastating multisystem complications, including stroke, pulmonary disease, delayed growth, osteomyelitis, organ damage and psychosocial dysfunction. All tissues and organs within the body are at risk of damage due to sickling.
Although relatively uncommon, a number of orofacial changes have also been observed in sickle cell disease. [12] When it occurs, the basic pathogenicity is similar to that in other organs. These orofacial changes in HbSS as reported in the literature include midfacial overgrowth attributable to marrow hyperplasia, [13],[14] other skull and jaw changes such as increased thickening of the skull and osteoporotic changes, [15],[16] mandibular infarction that may be followed by osteosclerosis, [17],[18] osteomyelitis of the mandible, [19],[20],[21],[22] anesthesia or paraesthesia of the mental nerve, [23],[24] asymptomatic pulpal necrosis, [25] orofacial pain, enamel hypomineralization and diastema [Table 1]. [26],[27],[28] These dentofacial deformities are radiographically characterized by a step-ladder appearance of the alveolar bone and areas of decreased densities and coarse trabecular pattern most easily seen between the root apices of the teeth and the inferior border of the mandible. [13],[14]
Mandibular osteomyelitis is an oral complication commonly observed in patients with sickle cell anemia, which is rarely manifested with other complications, making both its diagnosis and treatment easy. [19] The mandible is the most affected part of the face because the blood supply is relatively insufficient when compared with the maxilla. [20] Intravascular impairment can result in both ischemic infarct and osteonecrosis thus allowing bacterial proliferation by Streptococcus or Salmonella More Details. [19],[20],[21],[22] Aches in the mandible can be preceded by widespread painful crises and be accompanied by neuropathy involving the inferior alveolar nerve and paraesthesia of the lower lip. [29],[30]
The possibility of blood extravasations and hematoma secondary to sickle cell anemia-induced hemorrhage should be considered as a working diagnosis of a facial swelling in sickle cell disease. Scipio et al. [31] reported a case of a 14-year-old boy with sickle cell-related hemorrhage who developed an acute facial swelling, mimicking facial cellulitis of dental origin. Surgical exploration, however, revealed a large hematoma between the periosteum and the lateral aspect of the ramus of the mandible. Pools of blood were also found within the buccinators muscle. The boy also exhibited gingival enlargement, which was considered to be an outcome of repeated hemorrhagic episodes and fibrous repair. Gingival biopsies reported the presence of erythrocyte-filled intraepithelial blood vessels in the gingival epithelium.
Saint Clair de Velasquez and Rivera reported, in a study, that the most common soft tissue oral manifestation of sickle cell anemia in a Venezuelan population was buccal mucosa pallor, while the most common hard tissue finding was enlarged medullary spaces. [32]
The increased number of malocclusions in patients with sickle cell disease can be related to muscular imbalance, absence of labial sealing or changes in the osseous base thus leading to increased orthodontic intervention. [26]
Thalassaemia
These are a group of inherited hemolytic anemia involving defects in the synthesis of either the αor the β polypeptide chains of hemoglobin (α-thalassaemia, β-thalassaemia). Based on genetic and clinical entities, thalassaemia are classified as homozygous, heterozygous or compound heterozygous. The heterozygous form of the disease (thalassaemia minor) is mild and usually asymptomatic, the only manifestation being hypochromic microcytic anemia.
Homozygous β-thalassaemia, also known as Cooley's anemia or Mediterranean anemia, is chiefly seen in Mediterranean populations with prevalence as high as 15-20% in Greece, Turkey, Cyprus and southern Italy. [33]
The homozygous form of β-thalassaemia (thalassaemia major) exhibits the most severe clinical symptoms with marked orofacial deformities. [33] The onset of symptoms occurs early in infancy and the patients are severely anemic and have a short life expectancy. Patients with the most severe form of the disease rarely survive into adulthood because of cardiac failure, chronic anemia and hypoxia. [33],[34] However, with modern management, the prognosis has greatly improved.
The most common orofacial manifestations are due to intense compensatory hyperplasia of the marrow and expansion of the marrow cavity [Table 1] and a facial appearance known as "chipmunk" face: enlargement of the maxilla, bossing of the skull and prominent molar eminences. [35],[36] Overdevelopment of the maxilla frequently results in an increased over jet and spacing of maxillary teeth and other degrees of malocclusion. [37],[38],[39],[40]
Aplastic anemia
Aplastic anemia commonly presents with oral manifestation and can be the first clinical manifestation of the disease [Table 1]. The most common orofacial manifestation of the disease is multiple hemorrhages, which most often develop in patients with platelet counts <25 × 10 9 /liter. [Figure 1]a and b shows multiple orofacial hemorrhages in a 12-year-old patient with aplastic anemia. The other common manifestations are oral ulceration, candidiasis and viral infection. [41]  | Figure 1: (a) A 12-year-old boy with aplastic anemia. Note the subconjunctival ecchymosis. (b) The same patient as in Figure 1a. Note ecchymosis of the lower lip and cheek as well as sublingual hematoma
Click here to view |
Orofacial manifestations of disorders of hemostasis
Interaction of several basic mechanisms produces normal hemostasis, which can be divided into four general phases: the vascular phase; the platelet phase; the coagulation cascade phase, consisting of intrinsic, extrinsic and common pathways; and the fibrinolytic phase. [3]
An abnormal tendency to hemorrhage or thromboembolism occurs in the mixed group of disorders known as hemostatic disorders. All hemorrhagic hemostatic disorders, both inherited and acquired, may produce a variety of orofacial manifestations, including petechiae, nose bleeding, spontaneous and post-traumatic gingival hemorrhages and prolonged post-extraction bleeding [Table 2]. [3] Minimal trauma, such as occurs in eating or in tooth brushing, may be sufficient to provoke gingival hemorrhage, which, when it does, is characterized by its persistence rather than its profusion, and the total volume of blood loss may be important. [42]
Vascular disorders of hemostasis
The vascular disorders are a heterogenous group of conditions characterized by easy bruising and spontaneous bleeding from the small vessels. [43] Frequently, the bleeding is mainly in the skin, causing petechiae, ecchymoses or both and in some cases there is bleeding also from the mucous membrane. [43] Vascular defect of hemostasis may be acquired or inherited. [43] Most cases of bleeding from the vascular defect alone are not severe or life threatening. Hereditary hemorrhagic telangiectasia [44] and Ehlers-Danlos syndrome More Details, [45] in which some patients express the characteristic facial appearance of large eyes, small chin, thin nose and lips, lobeless ears are examples of hereditary vascular disorders that may present with petechiae or ecchymotic lesions on the lips, tongue and oral mucosa and epistaxis.
Platelet disorders in hemostasis
Abnormal bleeding associated with thrombocytopenia (low platelet count) or abnormal platelet function is characterized by spontaneous skin purpura, mucosal hemorrhages and prolonged bleeding after trauma. [42]
Facial petechiae, conjunctivae hemorrhage and hemorrhagic bullae in the oral mucous membrane occur in primary deficiency of platelets. These features are also seen in secondary thrombocytopenia due to the myelophthisic syndrome, autoimmune disorder, aplastic anemia [Figure 1]a and b, infections, collagen vascular disease, disseminated intravascular coagulopathy and drugs. [42] These features may be seen in von Willebrand's disease (VWD) and have also been observed in severe hemophilia. [42]
Thrombocytopenia secondary to an autoimmunologic disorder is called idiopathic thrombocytopenic purpura (ITP). It is an autoimmune disease characterized by the production of antibodies against ones own platelets. These antibodies adhere to the platelets and are recognized and destroyed by the reticuloendothelial system. Consequently, the platelet count gradually diminishes and is insufficient for the maintenance of primary hemostasis. Several reports have documented cases of ITP presenting as post-extraction hemorrhage. [46],[47]
Disorders of platelet function are suspected in patients who show skin and mucosal hemorrhages and in whom the bleeding time is prolonged despite a normal platelet count. [48] Glanzmann thrombasthenia (GT) is an exceedingly rare but well-defined inherited disorder of platelet function caused by a defect in the glycoprotein IIb/IIIa complex. [3] The association of GT with consanguinity has been noted, especially in geographic regions in which such intermarriage is common. In most patients, GT is diagnosed during early infancy or before the age of 5 years. Common manifestations of this hemorrhagic disorder are gingival hemorrhage, purpura, epistaxis, petechiae and menorrhagia. [6] Bernard-Soulier syndrome and grey platelet syndrome [49] are the other well-defined, inherited disorders of platelet function while the use of antiplatelet drugs like aspirin and clopidogrel, uremia and hyperglobulinemia are some acquired disorders of platelet function with similar orofacial manifestations. [50]
Coagulation disorders of hemostasis
In the 2007 World federation of hemophilia global survey where 89% of the world population was covered, 52,545 persons were identified with VWD, 105,018 with hemophilia A, 21,384 with hemophilia B, while 18,762 persons were identified with other bleeding disorders. In this survey, the total number of people identified with bleeding disorders was 213,904. [51]
von Willebrand's disease
VWD is one of the most common hereditary coagulation abnormalities in humans and shows a worldwide distribution. [52] It is classified into type 1, type 2A, 2B, 2M, 2N disease. Type 1 disease is the most common form of VWD, accounting for approximately 80% of all cases. It is transmitted as an autosomal-dominant trait and thus there may be evidence of a family history of excessive bleeding. Most forms of the disease show incomplete penetrance of the phenotype and variable expressivity of bleeding symptoms within families. [53] In contrast, the severe type 3 form of the disease shows a recessive pattern of inheritance with parents that do not usually manifest clinical symptoms. Type 2N VWD, in which isolated low FVIII levels occur, also shows a recessive pattern of inheritance and, thus, here again, a family history may be absent. [53]
The diagnosis of VWD is established by finding a prolonged bleeding time, a low level of factor VIII procoagulant activity and abnormally low levels of factor VIII - von Willebrand protein by immunologic assay and diminished platelet aggregation in response to ristocetin. [6],[54] The clinical bleeding symptoms of this disorder are notoriously heterogeneous and may range from virtually no symptom to mild symptoms to a disease resembling factor VIII deficiency and include oral mucosal bleeding, soft tissue hemorrhage, menorrhagia in women and rare hemarthrosis. [54] Continuous oral bleeding over long periods of time fosters deposits of hemosiderin and other blood degradation products on the tooth surfaces, turning them brown. If the history suggests VWD and oral surgery is contemplated in these patients, hematology consultation should be obtained and the patient's blood should be typed. [54]
Hemophilia
Hemophilia is an X-linked hereditary disorder. Hemophilia A is a deficiency of factor VIII while hemophilia B (Christmas disease) is a deficiency of factor IX. Factors VIII and IX are important in the intrinsic phase of blood coagulation and their deficiency is considered severe when plasma activity of the deficient factor is <1 IU/dl (normal range, 50-100), moderate if it ranges between 2 and 5 IU/dl and mild if it is between 6 and 40 IU/dl. [55]
The deficiency of factors VIII and XI is characterized by bleeding from multiple sites, frequently manifested in the mouth as gingival and post-extraction hemorrhages. [56] Hemophiliacs may experience many episodes of oral bleeding over their lifetime. Sonis and Musselman [57] reported an average 29.1 bleeding events per year, serious enough to require factor replacement in F VIII-deficient patients, of which 9% involved oral structures. Location of oral bleeding according to Sonis and Musselman are labial frenum, 60%; tongue, 23%; buccal mucosa, 17%; gingiva and palate, 0.5%. [57] Bleeding occurrences were most frequent in patients with severe hemophilia, followed by moderate and then mild hemophilia, and most often resulted from traumatic injury. [57] Bleeding events may also be induced by poor oral hygiene practices and iatrogenic factors. [57] Kaneda and colleagues [58] reported frequency of oral hemorrhage by location in individuals' deficient of F VIII and F IX as follows: gingiva, 64%; dental pulp, 13%; tongue, 7.5%; lip, 7%; palate, 2%; buccal mucosa, 1%. Many minor oral bleeds, such as those from the gingiva or dental pulp, can be controlled by local measures while more major forms will require factor replacement. [58]
Hemarthrosis is a common complication in a hemophiliacs' weight-bearing joints, yet it rarely occurs in the temporomandibular joint (TMJ). There are very few reported case of TMJ hemarthrosis. [59],[60] Chronic hemophilic TMJ arthropathy may also occur, which requires arthrotomy, arthroscopic adhesion lysis, factor replacement, splint therapy and physical therapy. [60] The treatment of patients with either hemophilia A or hemophilia B involves the replacement of the deficient clotting factors by intravenous infusion to either control or prevent bleeding. [55] Patients with mild factor VIII deficiency may be treated with DDAVP to raise the factor VIII level.
There is a group of rare inherited coagulation disorders that may present significant difficulties in diagnosis and management, [61] and with similar orofacial manifestations of hemophilia and VWD. These rare disorders include defects of fibrinogen, prothrombin, factor V, combined deficiency of factors V and VIII, factor VII, factor X, deficiency of vitamin K-dependent factors (II, VII, IX, X), factor XI, factor XII and factor XIII deficiency. [61] The overall frequency of these disorders in the general population is low (with the exception of factor XI deficiency). Homozygous deficiency varies from 1 in 500,000 for factor VII deficiency to 1 in 2 million for prothrombin. [62] All the disorders are autosomally inherited and, with the exception of factor XI deficiency, generally have no significant clinical manifestations in heterozygotes. [63] Severe deficiencies are more likely to be found in populations where marriage between blood relatives is common and, in rare cases, individuals may inherit more than one disorder. [63]
Factor XI deficiency, also known as hemophilia C, is the most common of the rare disorders and has a more variable bleeding tendency than hemophilia A or B. [64],[65] The deficiency is particularly common in Ashkenazi Jews, where the carrier rate is 8-9%. Severely deficient individuals (FXI<10 IU/dl) have a mild bleeding tendency after surgery, especially in areas with high fibrinolytic potential such as the mouth, the nose and the genitourinary tract. Spontaneous bleeding is rare, and hemarthroses are not a feature. [66],[67]
Preventive and restorative dental care, particularly for the patient with hereditary hemostatic disorder, is of paramount importance for the fact that advanced dental conditions and subsequent treatments have proven to be more complicated and risky. [57],[58],[59],[60] Quite often, dental health is neglected by hemophiliacs for fear of bleeding during procedures. Surprisingly, even dental specialists avoid these candidates and contribute to the conversion of a simple dental patient to an oral surgical patient. [59],[60] The complexities involved in diagnosing a bleeding disorder and the rarity of a standardized protocol to handle such patients contribute to this problem.
| Conclusion | | |
A wide array of disorders of red cells and hemostasis encountered in internal medicine has manifestations in the oral cavity and the facial region. Most of these manifestations are non-specific, but should alert the hematologist and the dental surgeon to the possibilities of a concurrent disease of hemopoiesis or hemostasis or a latent one that may subsequently manifest itself. These manifestations must be properly recognized if the patient must receive appropriate diagnosis and referral for treatment. Proper diagnosis is essential to initiate the correct treatment.
| References | | |
| 1. | Long RG, Hlousek L, Doyle JL. Oral manifestations of systemic diseases. Mt Sinai J Med 1998;65:309-15. 
|
| 2. | McFarlane DB, Pinkerton PH, Dagg JH, Goldberg A. Incidence of iron deficiency, with and without anaemia, in women in general practice. Br J Haematol 1967;13:790-6.
|
| 3. | Aster J, Kumar V. White cells and lymph nodes. In: Cotran RS, Kumar V, Collins T, editors. Robbins Pathologic Basis of Disease. 6 th ed, Kidlington, Oxford: WB Saunders; 1999. p. 657-8.
|
| 4. | Hou GL, Huang JS, Tsai CC. Analysis of oral manifestations of leukaemia: A retrospective study. Oral Dis 1997;3:31-8.
|
| 5. | Stafford R, Sonis S, Lockhart P, Sonis A. Oral pathoses as diagnostic indicators in leukemia. Oral Surg Oral Med Oral Pathol 1980;50:134-9.
|
| 6. | Toygar HU, Guzeldemir E. Excessive gingival bleeding in two patients with Glanzman Thrombastenia. J Periodontol 2007;78:1154-8.
|
| 7. | Zegarelli DJ. Fungal infections of the oral cavity. Otolaryngol Clin North Am 1993;26:1069-89.
|
| 8. | Hoffman RM, Jaffe PE. Plummer-Vinson syndrome: A case report and literature review. Arch Intern Med 1995;155:2008-11.
|
| 9. | Faccini JM. Oral manifestations of vitamin B12 deficiency. Br J Oral Surg 1968;6:137-40.
|
| 10. | Field EA, Speechley JA, Rugman FR, Varga E, Tyldesley WR. Oral signs and symptoms in patients with undiagnosed vitamin B 12 deficiency. J Oral Pathol Med 1995;24:468-70.
|
| 11. | Embury SH. The not so simple process of sickle cell vasoocclusion. Microcirculation 2004;11:101-13 .
|
| 12. | Davies SC, Brozoviæ M. The presentation, management and prophylaxis of sickle cell disease. Blood Rev 1989;3:29-44.
|
| 13. | Konotey-Ahulu FI. The Sickle cell disease: Clinical manifestations including the 'sickle cell crisis'. Arch Intern Med 1974l;133:611-9.
|
| 14. | Brown DL, Sebes JI. Sickle cell gnathopathy: Radiologic assessment. Oral Surg Oral Med Oral Pathol 1986;61:653-6.
|
| 15. | Morris AL, Stahl SS. Intraoral roentgenographic changes in sickle cell anaemia, a case report. Oral Surg Oral Med Oral Pathol 1954;7:787-91.
|
| 16. | Prowler JR, Smith EW. Dental bone changes occurring in sickle cell diseases and abnormal haemoglobin traits. Radiology 1955;65:762-9.
|
| 17. | Mourshed F, Tuckson CR. A study of the radiographic features of the jaws in sickle cell anaemia. Oral Surg Oral Med Oral Pathol 1974;37:812-9.
|
| 18. | Walker RD, Schenck KL Jr. Infarct of the mandible in sickle cell anaemia: Report of a case. J Am Dent Assoc 1973;87:661-4.
|
| 19. | Hammersley N. Mandibular infarction occurring during a sickle cell crisis. Br J Oral Maxillofac Surg 1984;22:103-14.
|
| 20. | Patton LL, Brahim JS, Travis WD. Mandibular osteomyelitis in a patient with sickle cell anaemia: Report of a case. J Am Dent Assoc 1990;121:602-4.
|
| 21. | Shroyer JV 3rd, Lew D, Abreo F, Unhold GP. Osteomyelitis of the mandible as a result of sickle cell disease: Report and literature review. Oral Surg Oral Med Oral Pathol 1991;72:25-8.
|
| 22. | Olaitan AA, Amuda JT, Adekeye EO. Osteomyelitis of the mandible in sickle cell disease. Br J Oral Maxillofac Surg 1997;35:190-2.
|
| 23. | Friedlander AH, Genser L, Swerdloff M. Mental nerve neuropathy: A complication of sickle cell crisis. Oral Surg Oral Med Oral Pathol 1980;49:15-7.
|
| 24. | Gregory G, Olujohungbe A. Mandibular nerve neuropathy in sickle cell disease: Local factors. Oral Surg Oral Med Oral Pathol 1994;77:66-9.
|
| 25. | Andrews CH, England MC Jr, Kemp WB. Sickle cell anaemia: An aetiological factor in pulpal necrosis. J Endod 1983;9:249-52.
|
| 26. | Kelleher M, Bishop K, Briggs P. Oral complications associated with sickle cell anaemia: A review and case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1996;82:225-8.
|
| 27. | Okafor LA, Nonnoo DC, Ojehanon PI, Aikhionbare O. Oral and dental complications of sickle cell disease in Nigerians. Angiology 1986;37:672-5.
|
| 28. | Taylor LB, Nowak AJ, Giller RH, Casamassimo PS. Sickle cell anaemia: A review of the dental concerns and a retrospective study of dental and bony changes. Spec Care Dentist 1995;15:38-42.
|
| 29. | O'Rourke CA, Hawley GM. Sickle cell disorder and orofacial pain in Jamaican patients. Br Dent J 1998;185:90-2.
|
| 30. | Stinson J, Naser B. Pain management in children with sickle cell disease. Paediatr Drugs 2003;5:229-41.
|
| 31. | Scipio JE, Al-Bayaty HF, Murti PR, Matthews R. Facial swelling and gingival enlargement in a patient with sickle cell disease. Oral Dis 2008;7:306-9.
|
| 32. | Saint Clair de Velasquez Y, Rivera H. Sickle cell anaemia oral manifestations in a Venezuelan population. Acta Odontol Latinoam 1997;10:101-10.
|
| 33. | Weatherall JD, Clegg JB. The thalassaemia syndromes. 3 rd ed, Oxford: Blackwell Scientific: 1981. p. 132-74.
|
| 34. | Modell B. Management of thalassaemia major. Br Med Bull 1976;32:270-6.
|
| 35. | Weel F, Jackson IT, Crookendale WA, McMichan J. A case of thalassaemia major with gross dental and jaw deformities. Br J Oral Maxillofac Surg 1987;25:348-52.
|
| 36. | Kaplan RI, Werther R, Castano FA. Dental and oral findings in Cooley's anemia: A study of fifty cases. Ann N Y Acad Sci 1964;119:664-6.
|
| 37. | Cannell H. The development of oral and facial signs in thalassaemia major. Br Dent J 1988;164:50-1.
|
| 38. | Van Dis ML, Langlais RP. The thalassaemias: Oral manifestations and complications. Oral Surg Oral Med Oral Pathol 1986;62:229-33.
|
| 39. | Hes J, van der Waal I, de Man K. Bimaxillary hyperplasia: The facial expression of homozygous thalassaemia. Oral Surg Oral Med Oral Pathol 1990;69:185-90.
|
| 40. | Abu Alhaija ES, Hattab FN, al-Omari MA. Cephalometric measurements and facial deformities in subjects with thalassaemia major. Eur J Orthod 2002;24:9-19.
|
| 41. | Sepúlveda E, Brethauer U, Rojas J, Le Fort P. Oral manifestation of Aplastic anaemia in children. J Am Dent Assoc 2006;137:474-8.
|
| 42. | Girolami A, Luzzatto G, Varvarikis C, Pellati D, Sartori R, Girolami B. Main clinical manifestations of a bleeding diathesis: An often disregarded aspect of medical and surgical history taking. Haemophilia 2005;11:193-202.
|
| 43. | Malfait F, De Paepe A. Bleeding in the heritable connective tissue disorders: Mechanisms, diagnosis and treatment. Blood Rev 2009;23:191-7.
|
| 44. | da Silva Santos PS, Fernandes KS, Magalhães MH. Osler-Weber-Rendu syndrome--dental implications. J Can Dent Assoc 2009;75:527-30.
|
| 45. | Létourneau Y, Pérusse R, Buithieu H. Oral manifestations of Ehlers-Danlos syndrome. J Can Dent Assoc 2001;67:330-4.
|
| 46. | Martini MZ, Lopez JS Jr, Gendler JL, da Fonseca EV, Soares HA, Franzi SA. Idiopathic thrombocytopenic purpura presenting as post-extraction haemorrhage. J Contemp Dent Pract 2007;8:43-9.
|
| 47. | Chandra J, Ravi R, Singh V, Narayan S, Sharma S, Dutta AK. Bleeding manifestations in severely thrombocytopenic children with immune thrombocytopenic purpura. Hematology 2006;11:131-3.
|
| 48. | Nurden AT. Inherited abnormalities of platelets. Thromb Haemost 1999;82:468-80.
|
| 49. | Young G, Luban N, White JG. Giant platelet disorders in African-American children misdiagnosed as idiopathic thrombocytopenic purpura. J Pediatr Hematol Oncol 1999;21:231-6.
|
| 50. | Hassan AA, Kroll MH . Acquired disorders of platelet function. Hematology Am Soc Hematol Educ Program 2005;1:403-8.
|
| 51. | Report on the Annual Global Survey 2007. World Federation of Hemophilia; 2008.
|
| 52. | Rodeghiero F, Castaman G, Dini E. Epidemiological investigation of the prevalence of von Willebrand's disease. Blood 1987;692:454-9.
|
| 53. | Miller CH, Graham JB, Goldin LR, Elston RC. Genetics of classic von Willebrand's disease: I. Phenotypic variation within families. Blood 1979;54:117-36.
|
| 54. | Goodeve A, Eikenboom J, Castaman G, Rodeghiero F, Federici AB, Batlle J, et al. Phenotype and genotype of a cohort of families historically diagnosed with type 1 von Willebrand disease in the European study, Molecular and Clinical Markers for the Diagnosis and Management of Type 1 von Willebrand Disease (MCMDM-1VWD). Blood 2007;109:112-21.
|
| 55. | Kasper CK. Hereditary plasma clotting factor disorders and their management. Haemophilia 2000;6:13-27.
|
| 56. | Rakocz M, Mazar A, Varon D, Spierer S, Blinder D, Martinowitz U. Dental extractions in patients with bleeding disorders. Oral Surg Oral Med Oral Pathol 1993;75:280-2.
|
| 57. | Sonis AL, Musselman RJ. Oral bleeding in classic haemophilia. Oral Surg Oral Med Oral Pathol 1982;53:363-6.
|
| 58. | Kaneda T, Shikimori M, Watanabe I, Minato F, Koide Y, Inoue N, et al. The importance of local hemostatic procedures in dental extractions and oral mucosal bleeding of haemophiliac patients. Int J Oral Surg 1981;10:266-71.
|
| 59. | Kaneda T, Nagayama M, Ohmori M, Minato F, Nakajima J, Shikimori M. Hemarthrosis of the temporomandibular joint in a patient with hemophilia B: Report of case. J Oral Surg 1979;37:513-4.
|
| 60. | Nishioka GJ, Van Sickels JE, Tilson HB. Hemophilic arthropathy of the temporomandibular joint: Review of the literature, a case report, and discussion. Oral Surg Oral Med Oral Pathol 1988;65:145-50.
|
| 61. | Bolton-Maggs PH, Perry DJ, Chalmers EA, Parapia LA, Wilde JT, Williams MD, et al. The rare coagulation disorders review with guidelines for management from the UKHCDO. Haemophilia 2004;10:593-628.
|
| 62. | Mannucci PM, Duga S, Peyvandi F. Recessively inherited coagulation disorders. Blood 2004;104:1243-52.
|
| 63. | Bolton-Maggs PH, Young Wan-Yin B, McCraw AH, Slack J, Kernoff PB. Inheritance and bleeding in factor XI deficiency. Br J Haematol 1988;69:521-8.
|
| 64. | Bolton-Maggs PH. Factor XI deficiency and its management. Treatment of Hemophilia monographs. 3 rd ed, vol 16. World federation of Hemophilia; 2008. p. 1-18.
|
| 65. | Brenner B, Lupo H, Laor A, Zivelin A, Lanir N, Seligsohn U. Predictors of bleeding in factor XI deficiency. Thromb Haemostas 1995;73:1441.
|
| 66. | Seligsohn U. Factor XI deficiency. Thromb Haemost 1993;70:68-71.
|
| 67. | O'connell NM. Factor XI Deficiency. Semin Hematol 2004;41:76-81.
|
Correspondence Address:
Titilope A Adeyemo
Departments of Haematology and Blood Transfusion and Oral and Maxillofacial Surgery, College of Medicine, University of Lagos
Nigeria
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0970-9290.87070
[Figure 1]
[Table 1], [Table 2] |
|
| This article has been cited by | | 1 |
Hemofili hastalarinda klinik yaklasim ile agiz ve dis sagliginin önemi |
|
| Aysem YURTSEVEN GÜNAY, Gülsüm AK | | ADO Klinik Bilimler Dergisi. 2022; 11(1): 1 | | [Pubmed] | [DOI] | | | 2 |
Oral Microbiota and Salivary Levels of Oral Pathogens in Gastro-Intestinal Diseases: Current Knowledge and Exploratory Study |
|
| Maria Contaldo, Alessandra Fusco, Paola Stiuso, Stefania Lama, Antonietta Gerarda Gravina, Annalisa Itro, Alessandro Federico, Angelo Itro, Gianna Dipalma, Francesco Inchingolo, Rosario Serpico, Giovanna Donnarumma | | Microorganisms. 2021; 9(5): 1064 | | [Pubmed] | [DOI] | | | 3 |
OROFACIAL MANIFESTATIONS OF HEMATOLOGICAL DISORDERS |
|
| Pallab Mandal | | INTERNATIONAL JOURNAL OF SCIENTIFIC RESEARCH. 2021; : 80 | | [Pubmed] | [DOI] | | | 4 |
The potential impact of vegetarian diet on the oral mucosa: A preliminary cytopathological study |
|
| Gabriela Wehbe Kesserwani, Núbia Carina de Oliveira, Thayse Caroline de Oliveira, Thiago Beltrami Dias Batista, Cassiano Lima Chaiben, Maria Ângela Naval Machado, Antonio Adilson Soares de Lima | | Journal of the Formosan Medical Association. 2021; | | [Pubmed] | [DOI] | | | 5 |
The Role of Red Blood Cells in Hemostasis |
|
| Amin A. Alamin | | Seminars in Thrombosis and Hemostasis. 2021; 47(01): 026 | | [Pubmed] | [DOI] | | | 6 |
Biological factors associating pulp necrosis and sickle cell anemia |
|
| Cyrene Piazera Silva Costa, Érika Bárbara Abreu Fonseca Thomaz, Cecília Cláudia Costa Ribeiro, Soraia de Fátima Carvalho Souza | | Oral Diseases. 2020; 26(7): 1558 | | [Pubmed] | [DOI] | | | 7 |
Oral Manifestations of Nutritional Deficiencies: Single Centre Analysis |
|
| Vladimíra Radochová, Radovan Slezák, Jakub Radocha | | Acta Medica (Hradec Kralove, Czech Republic). 2020; 63(3): 95 | | [Pubmed] | [DOI] | | | 8 |
Evidence-based dental management in the new era of sickle cell disease |
|
| Lewis L. Hsu, Judy Fan-Hsu | | The Journal of the American Dental Association. 2020; 151(9): 668 | | [Pubmed] | [DOI] | | | 9 |
Oral Health Profiles and Related Quality of Life in Thalassemia Children in Relation to Iron Overload: A Cross-Sectional Study |
|
| Hani T. Fadel, Mohammed A. Zolaly, Manal O. Alharbi, Lujain A. Qarah, Maher S. Alrehili, Abdulaziz D. Alamri, Ahmed M. Tarawah | | International Journal of Environmental Research and Public Health. 2020; 17(24): 9444 | | [Pubmed] | [DOI] | | | 10 |
Oral and Psychological Alterations in Haemophiliac Patients |
|
| Luca Fiorillo,Rosa De Stefano,Gabriele Cervino,Salvatore Crimi,Alberto Bianchi,Paola Campagna,Alan Scott Herford,Luigi Laino,Marco Cicciů | | Biomedicines. 2019; 7(2): 33 | | [Pubmed] | [DOI] | | | 11 |
Oral and Psychological Alterations in Haemophiliac Patients |
|
| Luca Fiorillo,Rosa De Stefano,Gabriele Cervino,Salvatore Crimi,Alberto Bianchi,Paola Campagna,Alan Scott Herford,Luigi Laino,Marco Cicciů | | Biomedicines. 2019; 7(2): 33 | | [Pubmed] | [DOI] | | | 12 |
Sensibilisation aux manifestations orales de pathologies générales |
|
| Thibaut Bila, Gratien Bonfort, Laurent Journo, Yvan Fronty, Hugues Lefort | | La Revue de l'Infirmičre. 2019; 68(253): 53 | | [Pubmed] | [DOI] | | | 13 |
Oral Changes in Patients with Sickle Cell Anemia of Dentistry Interest |
|
| Marcella Gomes Procopio da Silva, Cristhiane Almeida Leite, Álvaro Henrique Borges, Andreza Maria Fábio Aranha, Patrícia Leăo Castillo Eubank, Flávia Regina de Oliveira, Luiz Evaristo Ricci Volpato | | Journal of Health Sciences. 2018; 20(2): 94 | | [Pubmed] | [DOI] | | | 14 |
Sociodemographic profile and oral health status of thalassemic patients attending the National Thalassaemia Centre, Kurunegala, Sri Lanka |
|
| Wickramasooriyage Nandana Kularatne,Rasika M. Jayasinghe,Mahinda Chandika Diyunugala,Dayananda Bandara,Sachith Abeysundara,Irosha Perera | | Journal of Investigative and Clinical Dentistry. 2018; 9(2): e12293 | | [Pubmed] | [DOI] | | | 15 |
Sociodemographic profile and oral health status of thalassemic patients attending the National Thalassaemia Centre, Kurunegala, Sri Lanka |
|
| Wickramasooriyage Nandana Kularatne,Rasika M. Jayasinghe,Mahinda Chandika Diyunugala,Dayananda Bandara,Sachith Abeysundara,Irosha Perera | | Journal of Investigative and Clinical Dentistry. 2018; 9(2): e12293 | | [Pubmed] | [DOI] | | | 16 |
Haematinic deficiencies and patient clinical profiles in Polish patients with recurrent aphthous stomatitis (RAS) |
|
| Zuzanna Slebioda,Ewa Krawiecka,Elzbieta Szponar,Barbara Dorocka-Bobkowska | | Journal of Oral Pathology & Medicine. 2018; 47(5): 531 | | [Pubmed] | [DOI] | | | 17 |
Haematinic deficiencies and patient clinical profiles in Polish patients with recurrent aphthous stomatitis (RAS) |
|
| Zuzanna Slebioda,Ewa Krawiecka,Elzbieta Szponar,Barbara Dorocka-Bobkowska | | Journal of Oral Pathology & Medicine. 2018; 47(5): 531 | | [Pubmed] | [DOI] | | | 18 |
Gingival enlargement in children with sickle cell disease |
|
| Mine Ö. Tonguç,Selma Ünal,Rabia B. Arpaci | | Journal of Oral Science. 2018; 60(1): 105 | | [Pubmed] | [DOI] | | | 19 |
Gingival enlargement in children with sickle cell disease |
|
| Mine Ö. Tonguç,Selma Ünal,Rabia B. Arpaci | | Journal of Oral Science. 2018; 60(1): 105 | | [Pubmed] | [DOI] | | | 20 |
Orofacial Manifestations associated with Anemia |
|
| Shreya Gupta, Neeti Swarup, Harita Sairam, Nidhi Sinha, Suresh S Nair | | World Journal of Anemia. 2017; 1(2): 44 | | [Pubmed] | [DOI] | | | 21 |
Oral pathology in inflammatory bowel disease |
|
| Miranda Muhvic-Urek,Marija Tomac-Stojmenovic,Brankica Mijandrušic-Sincic | | World Journal of Gastroenterology. 2016; 22(25): 5655 | | [Pubmed] | [DOI] | | | 22 |
Oral pathology in inflammatory bowel disease |
|
| Miranda Muhvic-Urek,Marija Tomac-Stojmenovic,Brankica Mijandrušic-Sincic | | World Journal of Gastroenterology. 2016; 22(25): 5655 | | [Pubmed] | [DOI] | | | 23 |
The association between dmft index and haemoglobin levels in 3–6 year-old Saudi children with anaemia: A cross sectional study |
|
| Medhat A. Abdallah,Hassan H. Abed,Gehan Hamza,Elham N. Alsahafi | | Journal of Taibah University Medical Sciences. 2016; 11(1): 72 | | [Pubmed] | [DOI] | | | 24 |
The association between dmft index and haemoglobin levels in 3–6 year-old Saudi children with anaemia: A cross sectional study |
|
| Medhat A. Abdallah,Hassan H. Abed,Gehan Hamza,Elham N. Alsahafi | | Journal of Taibah University Medical Sciences. 2016; 11(1): 72 | | [Pubmed] | [DOI] | | | 25 |
Evaluation of the effects of serum iron levels on lacrimal gland secretion |
|
| Firat Erdogan,Mustafa Eliaçik,Evrim Senkal,Sevil Karaman Erdur,Kudret Kulak,Ilke Ozahi Ipek | | The Kaohsiung Journal of Medical Sciences. 2015; 31(8): 426 | | [Pubmed] | [DOI] | | | 26 |
Evaluation of the effects of serum iron levels on lacrimal gland secretion |
|
| Firat Erdogan,Mustafa Eliaçik,Evrim Senkal,Sevil Karaman Erdur,Kudret Kulak,Ilke Ozahi Ipek | | The Kaohsiung Journal of Medical Sciences. 2015; 31(8): 426 | | [Pubmed] | [DOI] | | | 27 |
Oro-facial characteristics and the surgical correction of patients affected by beta-thalassaemia: a review of the literature and report of a case |
|
| Azita Tehranchi, Hossein Behnia, Mostafa Sadeghi Ghochani, Farnaz Younessian | | Australasian Orthodontic Journal. 2015; 31(1): 98 | | [Pubmed] | [DOI] | | | 28 |
Immune Thrombocytopenic Purpura Presenting as Unprovoked Gingival Hemorrhage: a Case Report |
|
| Mehmet V Bal,Cenker Z Koyuncuoglu,Isil Saygun | | The Open Dentistry Journal. 2014; 8(1): 164 | | [Pubmed] | [DOI] | | | 29 |
Immune Thrombocytopenic Purpura Presenting as Unprovoked Gingival Hemorrhage: a Case Report |
|
| Mehmet V Bal,Cenker Z Koyuncuoglu,Isil Saygun | | The Open Dentistry Journal. 2014; 8(1): 164 | | [Pubmed] | [DOI] | | | 30 |
Surgical Correction of Facial Skeletal Deformity of Patients with Thalessemia |
|
| Amir Ali Badri,Seied Omid Keyhan,Faranak Jalalian,Hamid Reza Fallahi,Mohammad Ali Asayesh,Kazem Khiabani | | The American Journal of Cosmetic Surgery. 2014; 31(2): 107 | | [Pubmed] | [DOI] | | | 31 |
Surgical Correction of Facial Skeletal Deformity of Patients with Thalessemia |
|
| Amir Ali Badri,Seied Omid Keyhan,Faranak Jalalian,Hamid Reza Fallahi,Mohammad Ali Asayesh,Kazem Khiabani | | The American Journal of Cosmetic Surgery. 2014; 31(2): 107 | | [Pubmed] | [DOI] | | | 32 |
dental infections increase the likelihood of hospital admissions among adult patients with sickle cell disease |
|
| laurence, b. and haywood jr., c. and lanzkron, s. | | community dental health. 2013; 30(3): 168-172 | | [Pubmed] | | | 33 |
oral manifestations of anaemia [projevy anémií v dutině ústní] |
|
| paulusova, v. and radocha, j. and slezák, r. | | prakticky lekar. 2013; 93(1): 4-9 | | [Pubmed] | | | 34 |
Odontologic urgency after tooth extraction in a patient with platelet hypoaggregation: case report |
|
| | | Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology. 2012; 114(4): e122 | | [Pubmed] | [DOI] | | | 35 |
Orofacial manifestation of hematological disorders: Hemato-oncologic and immuno-deficiency disorders |
|
| Adeyemo, T. and Adeyemo, W. and Adediran, A. and Akinbami, A.J. and Akanmu, A. | | Indian Journal of Dental Research. 2011; 22(5): 688-697 | | [Pubmed] | |
|
|
|
|
|
|
|
|
|
|
|
|
| Article Access Statistics | | | Viewed | 86437 | | | Printed | 1377 | | | Emailed | 41 | | | PDF Downloaded | 2574 | | | Comments | [Add] | | | Cited by others | 35 | | |
|
|
Users online:
