Gingival enlargement in myelodysplastic syndrome.

The myelodysplastic syndrome (MDS) is characterized by peripheral blood cytopenias and increased risk of transformation to acute myeloid leukemia. This syndrome affects blood cell production and behavior. MDS is difficult to diagnose because of the absence of symptoms in the early stage of the disease. Often it is accidentally discovered during a routine physical exam/blood test. Till date, only a few cases of gingival enlargement associated with MDS are reported in the literature. Here is a remarkable case of gingival enlargement heralding the presence of MDS.

INTRODUCTION

Gingival enlargement is the overgrowth of gingiva characterized by an expansion and accumulation of connective tissue with the occasional presence of increased number of cells. According to the etiologic factors and pathologic changes, there is a variety of gingival enlargements including inflammatory, drug-induced, neoplastic, false enlargement, and those associated with systemic diseases.[1] Systemic diseases that cause gingival enlargement are granulomatous diseases and leukemia. Leukemic enlargement is due to gingival infiltration of immature (blasts) and proliferating leukocytes and most commonly occur in acute monocytic leukemia and acute myelomonocytic leukemia.[2]

The myelodysplastic syndromes (MDS) are a heterogeneous group of blood diseases, usually presenting as refractory anemia or cytopenia with an approximately 25% risk of progression toward acute myeloid leukemia (AML). Most cases also show dysplastic changes in one or more hematopoietic cell lines. Cytogenetic and molecular data provide evidence for a clonal hematopoietic stem cell disorder in the majority of cases. Causation, unless associated with prior chemotherapy, radiation, or toxic exposure, eludes discovery.[3] In the general population, the incidence rate of MDS is about five cases per 100,000/year persons. It is commonly seen in elderly males with the mean age of 65 years.[45]

We present here a case of generalized gingival enlargement associated with MDS, which is a rare entity in the literature.

CASE REPORT

A 60-year-old male patient was referred to his oncologist for the management of his dental problems. He reported to the outpatient Department of Periodontology, K. M. C. T. Dental College, Kerala, with a chief complaint of swollen gums, since 2 weeks. He had difficulty in chewing because of enlarged gums and also presented with spontaneous bleeding from gums, for which he was prescribed tranexamic acid tablets by his doctor.

A significant finding in this patient was that his medical history revealed a diagnosis of MDS-refractory anemia with excess blasts (MDS-RAEB-II) and was under treatment for the same. The hematological investigation revealed pancytopenia (anemia, neutropenia, and thrombocytopenia) and also occasional dysplastic neutrophils were found in peripheral blood [Table 1]. Bone marrow picture shows blasts cells around 12% of marrow cells.

Table 1

PB examination

The patient is also a known diabetic since 4 years and under medication (tablet Metformin 500 mg). His history also revealed a deleterious habit of tobacco chewing, since 30 years.

On intraoral examination, there was generalized diffuse gingival enlargement, which was bulbous and lobulated near upper right and left posterior teeth [Figures 1 and 2], and lower left posterior teeth region [Figure 3]. Gingiva appeared pale pink, shiny, and moderately firm in consistency. Gingival enlargement with hematoma formation was seen in the area of first and second upper right molar [Figure 4]. A white lesion on the enlarged gingival surface near the left mandibular first molar was also observed [Figure 3]. There was generalized bleeding on probing. His oral hygiene was poor with generalized extrinsic stains and calculus.

Figure 1

Generalized gingival enlargement

Figure 2

Bulbous gingival enlargement in upper posterior teeth region

Figure 3

Gingival enlargement with white lesion

Figure 4

Gingival enlargement with hematoma formation

The patient appeared very fatigued and weak, and he was uncooperative and irritated. Since his platelet count was below 60,000, the decision to perform a gingival biopsy and oral prophylaxis was postponed. Oral hygiene instruction was given to the patient, and 0.2% chlorhexidine mouthwash prescribed. Patient referred to the oncology center for further management and asked to keep dental follow-up. However, he did not turn up for further dental treatment.

Based on medical diagnosis of MDS and gingival findings a provisional diagnosis of gingival enlargement caused by systemic disease (MDS) was given but a definitive diagnosis could not be made because of lack of gingival biopsy, which could not be performed.

DISCUSSION

MDS is a group of hematological disorders, where the bone marrow cells fail to make enough healthy blood cells. MDS can be classified by using two systems: (1) French-American-British (FAB) classification system (2) World Health Organization (WHO) classification system [Table 2].[6] The current WHO classification system of MDS recognizes several major subtypes of MDS. These subtypes are distinguished from each other by the degree to which normal blood cell production is affected, the number of blast cells present, and the likelihood of transformation to AML.[3] International Prognostic Scoring System is used to determine the progression of the disease. This system turns patient blood test and bone marrow biopsy data into a score. The score tells how quickly an MDS case is likely to progress. The patients can be classified into four risk groups (low, intermediate-1, intermediate-2, or high) according to the number of cytopenias, bone marrow blast percentage, and type of cytopenic abnormalities.[7]

Table 2

WHO classification

The symptoms of MDS are caused by low blood counts. Some of the symptoms include tiredness, pale skin, mouth sores, skin infections, petechia, and bleeding gums. Treatment options available include: (1) Supportive care, such as red blood cell and platelet transfusions, antibiotics, growth factors, and iron chelators; (2) medication approved by the Food and Drug Administration (FDA) to treat MDS such as azacitidine, decitabine, and lenalidomide; (3) Immunosuppressive therapy, (4) chemotherapy, (5) stem cell transplantation.[8]

A frequent site of MDS manifestations as a result of quantitative and qualitative blood abnormalities, as well as chemotherapy, used is the oral cavity.[9] The incidence of oral complication in MDS ranges between 15% and 85%.[10] Gingivitis and periodontitis, spontaneous hemorrhage from the periodontium, soft tissue ulcerations, exacerbation of chronic periodontal and/or dental pathoses, and bacterial, fungal; and viral infections of oral tissues are the common oral complications that may occur.[8]

There are only limited reports in the literature of leukemic gingival infiltration and enlargement in the MDS. This case documents the gingival changes seen in this patient with MDS. The diffuse gingival enlargement associated with hematoma formation, a feature in leukemia[1] near first and second upper right molars; in this case, may be an indication of transformation of MDS to leukemic condition. Approximately, 30% of patients with MDS transforms to AML and One way of separating MDS from AML is based on the percentage of blast cells in bone marrow (AML > 20%).[11] AML with myelodysplasia related features is a subtype of AML, according to WHO 1997 AML classification. This category includes patients with prior documented MDS that then has transformed in to AML, or who have cytogenetic abnormalities characteristic for this type of AML (with previous history of MDS or MDS that has gone unnoticed in the past, but the cytogenetics is still suggestive of MDS).

Dental treatment including oral prophylaxis remains controversial in MDS patients. On the other hand, oral evaluation and treatment of preexisting dental disease have been suggested in immunocompromised patients to reduce the risk of septicemia arising from the oral cavity.[11] The purpose of this case report was to emphasize that gingival enlargement can be an oral manifestation of MDS. The physician and dentist must be aware of the changes in the oral health status of patients suffering from these hematologic disorders. Thus, patients with a MDS should be given periodic oral examinations as a routine part of their management.[9]

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.