|Year : 2021 | Volume
| Issue : 3 | Page : 328-332
Acute myeloid leukemia presenting in oral cavity – A report of two cases
A Winnifred Christy, T Jones Raja Devathambi, B Deepika Lakshmi, Gheerthana Venkatesh
Department of Oral Medicine and Radiology, CSI College of Dental Sciences and Research, Madurai, Tamil Nadu, India
|Date of Submission||29-Apr-2021|
|Date of Decision||17-Jun-2021|
|Date of Acceptance||25-Jul-2021|
|Date of Web Publication||28-Sep-2021|
Dr. A Winnifred Christy
Professor and Head, Department of Oral Medicine and Radiology, CSI College of Dental Sciences and Research, Madurai - 625 001, Tamil Nadu
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Acute myeloid leukemia is a malignant disease of the bone marrow. Because of its high morbidity, early diagnosis and prompt treatment are essential to save lives. This article describes the case reports of a 33-year-old female and a 60-year-old male who came to our dental hospital with gingival overgrowth, gingival bleeding, epistaxis, and facial swelling for about 1 week. After doing a thorough hematological examination, acute myeloid leukemia was diagnosed The oral health care professionals need to be able to recognize oral physiological characteristics, and, when identifying a change of normalcy, investigate it requesting additional tests or referring the patient to a specialized professional.
Keywords: Epistaxis, gingival enlargement, hemogram, leukemia, oral cavity, swelling of the lip
|How to cite this article:|
Christy A W, Raja Devathambi T J, Lakshmi B D, Venkatesh G. Acute myeloid leukemia presenting in oral cavity – A report of two cases. J Indian Acad Oral Med Radiol 2021;33:328-32
|How to cite this URL:|
Christy A W, Raja Devathambi T J, Lakshmi B D, Venkatesh G. Acute myeloid leukemia presenting in oral cavity – A report of two cases. J Indian Acad Oral Med Radiol [serial online] 2021 [cited 2022 Aug 8];33:328-32. Available from: https://www.jiaomr.in/text.asp?2021/33/3/328/326891
| Introduction|| |
Leukemia is a neoplastic hematologic disorder resulting from the proliferation of a clone of abnormal hematopoietic cells with impaired differentiation, regulation, and programmed cell death. Acute myeloid leukemia (AML) is predominantly a disease of adulthood, with an annual incidence of around 3.6 per 100,000 people in India. General manifestations of leukemia may include fatigue, pallor secondary to anemia, lymphadenopathy, recurrent infection due to neutropenia, bleeding and purpura as a result of thrombocytopenia, and bone and abdominal pain due to infiltration of blast cells.
Acute leukemia is often associated with a high incidence of oral complications such as mucosal pallor, petechiae, ecchymosis, bleeding, ulceration, and gingival enlargement that are secondary to pancytopenia, and rarely with trismus, mental nerve neuropathy, facial palsy, and infections.
Two patients who presented with gingival enlargement, bleeding, and epistaxis as an early sign of acute leukemia are described here, who were diagnosed by oral physicians, which stresses the importance of being aware of the signs and symptoms of leukemia.
| Case Reports|| |
A 33-year-old female came to our dental clinic with painful and bleeding gums for the past 1 week along with fever, malaise, and sore throat. Her gingival bleeding was spontaneous and increased with mastication and oral hygiene practices. The patient had unusual menstrual bleeding for 2 weeks and visited a gynecologist who prescribed tranexamic acid for symptomatic relief but failed to investigate further.
History of epilepsy, hypertension, or any long-term medication is known to cause gingival enlargement was negative.
Physical examination revealed a fatigued appearance and skin pallor with limited mouth opening. Lymph node examination revealed mild, tender, bilateral submandibular, and cervical lymphadenopathy. She was afebrile with an increased respiratory rate of 22 breaths per minute
She had mild hepatosplenomegaly, and her abdomen was soft. Intraoral examination revealed poor oral hygiene with fetor oris, generalized staining, and the presence of calculus throughout the dentition. The marginal gingival appeared soft, friable, and edematous with a generalized loss of stippling. There was generalized bluish-red cyanotic-looking gingiva that bled spontaneously. Gingival enlargements were seen in buccal, lingual, and palatal aspects in the anterior region of both maxilla and mandible. The gingiva in the mandibular anterior region, extending from 33 to 43, had a 3 mm gingival recession with ulceration on the buccal gingiva, and also necrosis with pseudomembrane formation was evident in some regions. There was severe gingival hemorrhage in the same region, which was controlled with pressure. Lower anterior teeth had Grade I mobility with tenderness on percussion. A provisional diagnosis of idiopathic thrombocytopenic purpura was made with leukemia considered as a differential diagnosis [Figure 1]a.
|Figure 1: Friable gingiva with profuse bleeding evident (a) and orthopantomogram showing generalized mild interdental bone loss (b)|
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Panoramic radiograph showed generalized bone loss consistent with deposition of local factors [Figure 1]b. Complete blood count showed alarming values with decreased red blood cells (RBC) and platelet count (<40,000 mm3) and a marked increase in white blood cell (WBC) count of more than 200,000 [Table 1].
Peripheral blood smear report showed the evidence of 90% blasts, 4% neutrophils, 5% lymphocytes, and 1% monocytes; diminished platelets; and increased WBC count with 90% blasts that were large with multiple nucleoli granular cytoplasm [Table 2] and [Figure 3]a.
|Figure 2: Diffuse swelling and ecchymosis evident in the upper lip region (a) and occlusal radiograph showing no periapical pathology in upper anterior teeth (b)|
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|Figure 3: Peripheral smear showing atypical blast cells seen in both cases (a and b)|
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Complete blood count and peripheral smear values gave the impression of acute myelomonocytic leukemia (m4).
The patient was prescribed 0.12% chlorhexidine mouthwash twice a day to control gingival inflammation and tranexamic 5% mouthwash to arrest bleeding, and then referred to hemato-oncologist on the same day for further management. The patient was counseled regarding the nature of the disease and advised not to delay visiting a hemato-oncologist.
In a tertiary cancer hospital, bone marrow aspiration was done, which showed myeloblasts around 80%, suppressed erythropoiesis with the absence of megakaryocytes suggestive of acute leukemia. Immunophenotyping was done, which suggested AML m4. The cytogenetic study showed normal karyotyping. She was started on a cytarabine/daunorubicin 7 × 3 regimen with blood product and antibiotic support. However, the patient did not survive after the first induction chemotherapy because the disease failed to respond to the treatment.
A 60-year-old gentleman came to our dental hospital with bleeding from the nose intermittently and painful swelling in the upper lip region for the past 1 week. The pain was mild, intermittent, and aggravating on a movement of jaws and relieved with analgesics. He denied a history of trauma, allergy, or any bleeding disorders. He had a fever, malaise, and weight loss of about 3 kg for the past 1 week. His past medical history was unremarkable.
The patient was moderately built, appeared fatigued, and looked pale. Ecchymosis was evident below the right ala of the nose. The review of systems was normal except for mild splenomegaly. There was diffuse swelling of the upper lip that extended superiorly from the lower border of the nose inferiorly up to the lower border of the upper lip [Figure 2]a. No discharge was evident. Intraoral examination revealed no discernible dental cause. A provisional diagnosis of the acute dentoalveolar abscess was made with bacterial infections, allergies, trauma, orofacial granulomatosis, and syndromic presentations listed in the differential diagnosis.
Electric pulp testing elicited a vital response in the upper anterior teeth. No periapical pathology was evident on the radiograph [Figure 2]b.
The patient has been advised of a complete blood count and peripheral blood smear. The total leucocyte count was very high [Table 3], and the peripheral smear too showed 20% blast cells [Table 4] and [Figure 3]b. The blood picture confirmed acute myelomonocytic leukemia.
Therapeutic intervention and follow-up
The patient was advised antibiotics and analgesics for pain and then referred to a hemato-oncologist for further management. Follow-up was not possible as the patient delayed visiting the oncologist and succumbed to the disease without any treatment in less than a week.
Time line showing the onset and progress of the disease and therapeutic intervention of both the patients are given in [Table 5].
| Discussion|| |
AML is an aggressive neoplasm that accounts for about 25% of all leukemias in adults. The clinical manifestations are due to the underlying thrombocytopenia, anemia, and leukocytosis with increased blast cell levels in circulating blood.
Oral mucosa may exhibit mucosal pallor related to anemia along with spontaneous bleeding and petechial hemorrhages of gingivae, palate, tongue, or lips resulting from thrombocytopenia. Nonspecific oral ulcers can occur as a sign of neutropenia, and gingival hyperplasia can result due to leukemic infiltration. The immunosuppressive state may lead to the occurrence of bacterial, viral, and candidal infections.
The early manifestations of AML may include gingival bleeding, oral ulceration, and gingival hyperplasia.,, Shephard et al. observed that 13 symptoms were independently associated with acute leukemia, the three strongest being nosebleeds and/or bleeding gums, fever, and fatigue. In the first case, the gynecologist failed to find out the etiology of abnormal menstrual bleeding in a female in the reproductive age group. By the time we saw the patient, she had progressed into the later stage of the disease and hence succumbed during the treatment. Infection and bleeding remain a concern for dentists. Nonspecific, spontaneous oral bleeding should be viewed with caution and maybe a warning sign of an underlying serious abnormality as in our case.
In the second case, the sick appearance of the patient along with a history of epistaxis prompted us to think of a systemic cause for the swelling in the upper lip. The erythematous color and tensed skin over the swelling suggested a probable secondary infection that may be due to the underlying neutropenia. This case is reported for a slight variation in its presentation and unaware dentists may miss the diagnosis that may increase the chances of fatality.
In both cases, the patients presented late as they had a fever, malaise, and weight loss, which reflect the patients' hesitancy in seeking medical interventions early. Time elapsed before diagnosis proves expensive later in terms of morbidity.
Apart from diagnosing patients, dentists play a vital role in maintaining patients' oral health and giving symptomatic relief during the treatment phase. The main complications of antineoplastic therapy include oral mucositis, bleeding, salivary gland dysfunction leading to xerostomia, recurrent infections due to neutropenia, and dysgeusia. The stomatologist helps diagnose the side effects of chemotherapy and helps in treating the same thereby improving the quality of life in these patients.,
In both cases, the fatal hematologic malignancy was first diagnosed by the oral physicians. Leukemias presenting in the oral cavity are rare in India, and less than 10 case reports have been published in the English literature so far from this part of the globe.
| Conclusion|| |
Hematogenous malignancies in adults have high a mortality rate despite advances in health care. Occasionally, oral lesions may be the first and only manifestation of potentially fatal conditions such as leukemia. These cases remind that the dentist should have a keen eye in observing even minute changes intraorally and in the maxillofacial region and be aware of the importance of recognizing oral manifestations of systemic diseases quite early as oral cavity is considered a mirror of systemic health. Hemogram and peripheral smear are useful and widely available investigations that can alert the clinician of any hematologic abnormalities, and dentists can use this investigation when in doubt as it helps in early diagnosis and timely referral of patients averting fatality.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Cooper CL, Lowen R, Shore T. Gingival hyperplasia complicating acute myelomonocytic leukaemia. J Can Dent Assoc 2000;66:78-9.
Rajeev PC, Anirudhan D. Clinico epidemiological aspects of acute myeloid leukemia – An observational study. Ann Int Med Den Res 2018;4:MC01-7.
Guan, G. and Firth, N. Oral manifestations as an early clinical sign of acute myeloid leukaemia: A case report. Aust Dent J 2015;60:123-7.
Chang F, Shamsi TS, Waryah AM. Clinical and hematological profile of acute myeloid leukemia (AML) patients of Sindh. J Hematol Thrombo Dis 2016;4:239.
da Silva Santos PS, Fontes A, de Andrade F, de Sousa SC. Gingival leukemic infiltration as the first manifestation of acute myeloid leukemia. Otolaryngol Head Neck Surg 2010;143:465-6.
Shephard EA, Neal RD, Rose PW, Walter FM, Hamilton W. Symptoms of adult chronic and acute leukaemia before diagnosis: Large primary care case-control studies using electronic records. Br J Gen Pract 2016;66:e182-8.
Francisconi CF, Caldas RJ, Oliveira Martins LJ, Fischer Rubira CM, da Silva Santos PS. Leukemic oral manifestations and their management. Asian Pac J Cancer Prev 2016;17:911-5.
Zimmermann C, Meurer MI, Grando LJ, Gonzaga Del Moral JÂ, da Silva Rath IB, Schaefer Tavares S. Dental treatment in patients with leukemia. J Oncol 2015;2015:571739. doi: 10.1155/2015/571739.
[Figure 1], [Figure 2], [Figure 3]
[Table 1], [Table 2], [Table 3], [Table 4], [Table 5]