Int J Dent Case Reports 2011; 1(3): 29-33 IJDCR 2011. All rights reserved www.ijdcr.

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CAS E REPORT LEUKEMIAS UNMAS KING THE DIS EAS E WITH THE AID OF ORAL MANIFES TATIONS T. Rajesh Singh 1 , S. Sujatha2 , T. Ramesh 3 , L.A. Swapna 4 , Raghavendra Mahadev Naik5 , D.Ajit 6
1

Department of Oral Medicine and Rad iology, Vishnu Dental College, Vishnupur, Bhimavaram, west Godavari district, Andhra Pradesh
2

Associate professor, Depart ment of Oral Medicine and Radio logy, Oxford dental college Bangalore

Associate Professor, Department of Oral Medicine and Radiology, Vishnu Dental College, Vishnupur, Bhimavaram, west Godavari d istrict, Andhra Pradesh
4

P.G. Student, Depart ment of Oral Medicine and Radio logy, Vishnu Dental College, Vishnupur, Bh imavaram, west Godavari district, Andhra Pradesh
5

Senior Lecturer, Depart ment of Oral Med icine and Radiology, Vishnu Dental College, Vishnupur, Bhimavaram, west Godavari district, Andhra Pradesh
6

Assistant professor, Depart ment of Oral Medicine & Rad iology, Oxfo rd dental college, Hosur Road, Bangalore

ADDRESS FOR CORRESPONDENC E Dr.L.A.Swapna Post graduate student, Depart ment of Oral Medicine and Radio logy, Vishnu Dental College, Bhimavaram, west Godavari d istrict, Pin code: 534202. Andhra Pradesh. E mail: laswapna123@g mail.co m ABSTRACT Oral signs and symptoms may often signify unseen underlying systemic disease. Periodontal lesions are co mmon in patients with acute leukemias throughout the course of the disease. Although many cases of gingival enlargement in patients with acute myeloid leukemias have been reported in literature, cases diagnosed by the oral man ifestations in India are very few. The two remarkab le cases, one with generalized gingival hyperplasia and the other with spontaneous hemorrhage, herald ing the presence of acute myelocytic leu kemias are reported here. Dentists should always be on guard to observe any unusual clin ical signs that may lead to the early diagnosis of systemic d isease processes. In day to day life oral physician plays a key ro le in d iagnosing various oral diseases and perhaps saving the life of few individuals by indentifying the underlying systemic disease condition by observing their oral man ifestations. Key words: packed cell volume (PCV); Erythrocytic Sedimentation Rate (ESR); Red Blood cell count (RBC)

Singh, Su jatha, Ramesh, Swapna, Naik, Ajit

Leukemias

INTRODUCTION The leukemias are a heterogeneous group of diseases characterized by infiltration of the blood, bone marrow, and other tissues by neoplastic cells of the hematopoietic system (1, 2). Defined as a malignant neoplasm characterized by a proliferation of abnormal white b lood cells within the bone marrow. There is a failu re of maturation of precursor cells (blasts) with the result that the blasts accumulate in the marrow and suppress normal haematopoietic stem cells by physical replacement and also other unknown mechanisms. As the number of malignant white blood cells developing in the marrow increases, they rapidly enter the circulation. Despite an increase in the white blood cell count, the leukaemic leukocytes are non-functional. The resulting paucity of functional white cells, red cells and platelets is responsible for immunodeficiency, anaemia and thrombocytopaenia respectively (3). The causes of leukaemia are poorly defined. Ho wever, radiat ion exposure, chromosomal abnormalities, chemical injuries and viral infect ions have been implicated. Leukemias accounts for about 4% of all deaths from malignant disease. The rate of progression varies considerably in different types of leukemias but death is the usual outcome in untreated disease as a result of co mpro mised production of mature blood cells (4, 5). Gingival bleeding is perhaps the most common manifestation of the in flammatory process that presents daily at the dental clin ic. Chronic marginal gingiv itis accounts for most of this and is usually managed with simple periodontal therapy followed by oral hygiene instructions. Significant gingival bleeding that is of sudden onset and difficult to control, warrants further investigation. . Case 1: A 35 year o ld male patient reported to the Depart ment of Oral Medicine and Rad iology, with a chief co mplaint of pain in the lower right back tooth region since one week and swelling since one day. Pain was sudden in onset, severe, intermittent and throbbing in nature, aggravated on chewing and subsided on taking medication. Swelling was sudden in onset and gradually increasing in size .He visited a dentist for the same and was advised extraction of the tooth. The dentist noticed profuse bleeding wh ile

removing the tooth and administered ethamsylate which resulted in cessation of bleeding and he was referred to Dental College. No significant past med ical and dental history was elicited .General examination revealed that the patient was moderately built and nourished with gross asymmetry of the right side of the face.

Figure 1 Extra Oral Examination (figure 1) revealed a solitary diffuse swelling on the right middle and lower third of the face extending superio-inferiorly fro m the ala tragal line to inferior border of the mandib le. Anteroposteriorly fro m the corner of the mouth to the angle of the mandible. On palpation the swelling was firm and tender. Bilaterally solitary submandibular ly mph nodes were palpable measuring approximately 1 cm in diameter, roughly circular in shape, firm in consistency, mobile and tender. Intra Oral Examination (figure 2&3) revealed acute pericoronitis in relation to 48. Gingiva distal to 48 was traumatized and erythematous. Grayish discoloration was seen on the right side of hard palate associated with multip le purpuric spots measuring approximately about 3-5mm which did not blanch on pressure. Based on the above findings, a Provisional Diagnosis of Acute periapical abscess with buccal space infection in relat ion to 48 and Bleeding disorder was

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given. Differential Diagnosis of Thro mbocytopenic purpura, Von Willebrands disease and Leukemias

Figure 2

complaint of generalized swelling in the gu ms both labially and lingually since two weeks which was sudden in onset and slowly increased in size covering all the teeth, bleeds on slight provocation while brushing and associated with difficulty in chewing and swallowing .The patient visited a dentist one week back for the same and was prescribed Amo xicillin 500mg t id and Metrogyl gum paint and as the swelling d id not subside the patient was referred to The Oxfo rd Dental College .No significant past dental history was elicited . Patient gave history of menorrhagia, stomach ache and weight loss since three months. General physical examination revealed that the patient was thinly built and poorly nourished and pallor was present bilaterally on lower palpebral conjunctiva.

Figure 3 was given. Investigations carried out include complete hemogram, peripheral s mear and bone marrow aspiration. Co mplete hemogram revealed Hemoglobin was 7.8g m%, Platelet count 46000cells/mm3, WBC count 3.2 lakh cells/ mm3, ESR 60mm/hr and PCV 26%.Peripheral s mear depicted numerous blast cells Bone marrow aspiration report revealed hypercellular marrow with sheets of neoplastic promyelocytes and myeloblasts and many faggot cells. Other elements were suppressed. Based on the above findings, a Final Diagnosis of Acute Myelocytic leukemias M3Hypergranular type was given. The case was referred to Kidwai Memorial Institute of Oncology where the patient passed away a few days later on account of internal bleed ing. Case 2: A 25 year o ld female patient reported to the Depart ment of Oral Medicine and Radiology, The Oxford Dental Co llege, Bangalore with a ch ief

Figure 4

Figure 5 On Extra Oral Examination bilateral solitary submandibular ly mph nodes were palpable measuring approximately 0.5cm in diameter, circu lar, firm, mobile and tender. Intra Oral Examination (figure

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4&5) revealed cro wding of upper and lower anterior teeth. There was diffuse generalized gingival enlargement involving buccal and lingual surfaces extending up to middle third of the crown. Generalized in flammation of g ingiva was present with loss of stippling, rolled out marg ins, smooth surface erythematous with bluish tinge. On palpation gingiva was firm & edematous, tender and bleding on slight provocation. A solitary shallow ulcer was seen on the tip of the tongue measuring approximately 0.2cm in diameter roughly oval in shape, floor covered with slough, surrounded by erythema and tender on palpation. Based on the above findings, a Provisional Diagnosis of Leukemic g ingival enlargement was given. A Differential Diagnosis of Hormonal gingival enlargement and Tuberculous gingival en largement was given. Investigations carried out included complete hemogram and peripheral smear. Co mp lete hemogram revealed Hemog lobin was 6.5 g m% , ESR 30mm/hr, PCV 20% , RBC count 2.2 4 million/ mm3, Platelet count 31000 cells/mm3, Total WBC count 1.82 lakh cells/ mm3, Ly mphocytes 14 % and Blasts 86%. Peripheral smear revealed microcytic hypochromic anemia, thro mbocytopenia, leucocytosis and presence of blast cells. Based on the above findings, a Final Diagnosis of Acute Myelocytic Leu kemias was given. The patient was referred to St.Johns Hospital where she passed away a couple of days later. DISCUSS ION Oral lesions may be the presenting feature of acute leukemias s and are therefore important d iagnostic indicators of the disease. Oral manifestations of the disease lead majority of the acute leukemic patients to consult the dentist and in most of the cases the underlying disease is diagnosed fro m clin ical findings during periodontal examination (11). Although the etiology of leukemias remains unknown, there is a genetic predisposition along with several well-known associated syndromes (ie, Down syndrome, Bloom syndrome, Neuro fibro matosis, Shwach man syndrome, ataxia -telangiectasia syndrome, Klinefelter, Fanconis anemia, and Wiskott- Aldrich syndrome) (12). Gingival hyperplasia is secondary to infiltration of the gingival tissue with leukemias cells and is well

described in the literature (6, 7). In the most extensive review of the topic, gingival hyperplasia was observed in acute myelogenous leukemias (AM L) with a frequency of 3% to 5% among 1,076 patients receiving anti-leu kemias chemotherapy at a referral centre. Gingival hyperplasia is most commonly seen with the AML subtypes acute monocytic leukemias (M5) (66.7%), acute myelo monocytic leukemias (M4) (18.5%), and acute myelocytic leukemias (M 1, M2, M 3) (3.7%) (8-10).Gingival hyperplasia is characterized by progressive enlargement of the interdental papillae as well as the marginal and attach ed gingiva. In the conditions most pronounced form, the cro wns of the teeth may be covered. Gingiva appear swollen, devoid of stippling and pale red to deep purple in colour. Mucosal hemorrhages, ulcerative gingivit is, infectious gingivitis and odontalgia may be observed. Pallor, spontaneous hemorrhage, petechiae and ulceration have been described to occur more frequently in acute than chronic leukemias (10-14). More atypical oral findings that have been reported include cracked lips and the presence of haemorrhagic bullae on the anterior dorsum of the tongue, buccal and labial mucosa, toothache, tooth mobility and petechiae (17).Lynch and Ship (10) have also reported that the incidence of intra-oral b leeding in patients with acute leukaemia was prognostically noteworthy. They found that at the end of 12 months, 25 per cent of patients with acute leukaemia without intra-oral bleeding were alive co mpared with 0 per cent survival in patients with intra-oral bleed ing. Presenting features were different in both of the cases. In Case1, there was a swelling associated with profuse bleeding fro m the extraction socket. Whereas in Case 2 the gingiva was pink and firm with marked generalized enlargement. In both cases the patients were unaware of the underlying disease and the diagnosis was confirmed by the authors after clin ical examination and obtaining hematological investigation report. No surgical interventional procedures had been carried out because acute exacerbation with serious consequences has been reported after such procedures (16, 17, 18). Both the patients were referred to the oncology centre for the further management but within a span of four weeks the authors had been informed that both patients had died.

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CONCLUS ION As oral lesions are one of the earlier manifestations of acute leu kemias and may serve as diagnostic indicator of the disease, the dental surgeon may be the first to diagnose it. The dentist should always be aware of the presenting feature and various complications associated with leukemias to enable early diagnosis, timely and pro mpt referral for subsequent management.

1.

Fauci A.S, Braunwald E, Kasper D.L, Hauser S.L, Longo D.L, Jameson J.L, Lascalzo J. Harrison's Princip les of Internal Medicine. 7th edition McGraw-Hill Access Medicine,2008 p 271-283. 2. Bricker SL, Langlais RP, M iller SC. Oral diagnosis, Oral medicine and Treat ment planning. 2nd Ed BC Decker Inc 2002. pp 300. 3. Kinane DF, Marshall GJ. Periodontal man ifestations of systemic disease. Aust Dent J 2001;46:2-12. 4. Jemal, A, Siegel R, Ward E, Murray T, Xu J,Smigal C, et al. Cancer statistics, 2006. CA: A Cancer Journal for Clin icians. 2006; 56: 106 130. 5. Savona, M., and Talpaz, M. Chronic myeloid leukemias: Changing the treatment parad ig ms. Oncology.2006; 20: 707 711. 6. Barrett AP. Long term prospective clin ical study of neutropenic ulceration in leukemias . J Oral Med1987; 42:102-5. 7. Ishikawa G, Waldron CA. Diseases of the oral mucosa. In: Ishikawa CA, ed itor. Color at las of oral pathology. St. Louis (MO): Ishiyaku EuroA merica 1987; p. 71-102. 8. Bressman E, Decter JA, Chasens AI, Sackler RS. Acute myeloblastic leukemias with oral man ifestations. Report of a case. Oral Surg Oral Med Oral Pathol 1982; 54:401-3. 9. Dreizen S, McCredie KB, Keating MJ, Luna MA. Malignant gingival and skin infiltrates in adult leukemias . Oral Surg Oral Med Oral Pathol 1983; 55:572-9. 10. Lynch MA, Ship I. Oral man ifestations of leukemias ; a post diagnostic study. J Am Dent Assoc 1967; 75:1139-1144.

11. Stafford R, Sonis S, Lockhart P and Sonis A Oral pathoses as diagnostic indicators in leukemias . Oral Surg Oral Med Oral Pathol 1980;50(2):134-139. 12. Buffler PA, Kwan M L, Reynolds P, et al: Environmental and genetic risk factors for childhood leukemias : Appraising the evidence. Cancer Invest 23:60, 2005. 13. Aronovich S, and Connolly T. W. Pericoron itis as an Initial Manifestation of Acute Ly mphoblastic Leu kemias . J Oral Maxillofac Surg 2008; 66:804-808. 14. Cooper C. L, Loewen R, Shore T. Ging ival Hyperplasia Co mplicating Acute Myelomonocytic Leu kemias . J Can Dent Assoc 2000; 66:78-9. 15. Patil S , Kalla N, Ramesh D.N.S.V, Kalla A.R. Leukemic ging ival enlargement: a report of two cases Arch Orofac Sci 2010, 5 (2): 69-72. 16. Dean AK, Ferguson JW, Marvan ES. Acute leukaemia presenting as oral ulceration to a dental emergency service. Aust Dent J 2003;48:(3):195-197. 17. P Gleeson. Spontaneous gingival haemorrhage: Case report. Aust Dent J 2002;47:(2):174-175. 18. Anil S, Smaranayake LP, Nair RG and Beena VT. Ging ival en largement as a diagnostic indicator in leukaemia. Case report. Aust Dent J 1996; 41(4): 235-237.

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