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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 9  |  Issue : 2  |  Page : 183-186

Pyogenic Granuloma in a Thalassemia Minor Patient: Consequence or Coincidence?


1 Department of Periodontology, Faculty of Dentistry, Jamia Millia Islamia, New Delhi, India
2 Department of Pediatric and Preventive Dentistry, Faculty of Dentistry, Jamia Millia Islamia, New Delhi, India
3 Department of Oral Pathology and Microbiology, Faculty of Dentistry, Jamia Millia Islamia, New Delhi, India

Date of Submission17-Dec-2021
Date of Decision01-Mar-2022
Date of Acceptance23-Mar-2022
Date of Web Publication22-Aug-2022

Correspondence Address:
Deepika Bablani Popli
Oral Pathology, Faculty of Dentistry, Jamia Millia Islamia, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jdrr.jdrr_190_21

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  Abstract 


Thalassemia is a blood disorder inherited as an autosomal single gene characterized by a reduced or absent amount of hemoglobin chains. It is considered to be the most common genetic disorder with defect in either alpha or beta chains which produce abnormal blood cells. The present report describes an inflammatory enlargement over gingiva in the region of an erupting maxillary right premolar in a 10-year-old male who had minor thalassemia. The case was surgically handled using LASER, being a conservative management option, especially in such patients. The histopathological examination confirmed it to be a pyogenic granuloma. The paper describes the management of such lesions using LASERS as minimally invasive surgical techniques. It also tries to establish a hypothesis of a possible pathogenic association between intraoral pyogenic granuloma and thalassemia.

Keywords: Gingival hyperplasia, lasers, pyogenic granuloma, thalassemia


How to cite this article:
Sultan N, Sultan A, Popli DB, Juneja A. Pyogenic Granuloma in a Thalassemia Minor Patient: Consequence or Coincidence?. J Dent Res Rev 2022;9:183-6

How to cite this URL:
Sultan N, Sultan A, Popli DB, Juneja A. Pyogenic Granuloma in a Thalassemia Minor Patient: Consequence or Coincidence?. J Dent Res Rev [serial online] 2022 [cited 2022 Oct 2];9:183-6. Available from: https://www.jdrr.org/text.asp?2022/9/2/183/354200




  Introduction Top


Soft-tissue enlargements in the oral cavity often pose a diagnostic challenge as there is a diverse group of pathological and sometimes physiological conditions which can cause such enlargements. Pyogenic granuloma (PG) also referred to as lobular capillary hemangioma is one such clinical and histopathologic entity which can occur in the oral cavity and pose diagnostic challenge, especially in a pediatric patient. Oral PG is the most common gingival tumor occurring in 75% of cases, followed by lips, tongue, and buccal mucosa.[1] According to Vilmann et al., the majority of PG occur along the marginal gingiva with only 15% of cases on the alveolar crest.[2] In our case, the growth was present around the erupting maxillary premolar over the alveolar crest which is not the common site of occurrence for PG.

The present case report highlights a case of PG at an uncommon location in a 10-year-old patient who also had thalassemia. The case report puts forward a unique association between the intraoral PG and thalassemia and also tries to establish a pathogenic connection among them.


  Case Report Top


A 10-year-old male patient visited the outpatient department with the chief complaint of difficulty in chewing from the upper right back teeth region for 10 days (as reported by his mother). On examination, there was an erupting maxillary first premolar with inflamed pericoronal gingival mass and partially visible buccal and lingual cusps. The tissue was erythematous, hyperplastic, about 2 cm × 0.5 cm in dimension all around, semi-firm in consistency, minutely lobulated, and mildly tender on palpation [Figure 1]. There were some plaque deposits, there was bleeding on slight provocation and no other significant intraoral findings were found. Radiographic findings of that region were insignificant and only showed an erupting maxillary premolar.
Figure 1: Preoperative intraoral view of the growth

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Medical history

The family's medical profile was thoroughly studied. The patient had minor thalassemia. Both of his parents had minor thalassemia. He had two siblings, the younger brother (6 years of age) had β-thalassemia major (homozygous) diagnosed at the age of 6 months, on weekly blood transfusion and iron chelation therapy since then. The youngest brother was medically healthy.

A thorough hematology work-up of the patient was done. The patient had slight anemia (Hb: 10.1 gm%) and reduced red blood cell (RBC) count (3.3 million/cu mm) and packed cell volume (30%). His peripheral blood smear showed microcytic hypochromic RBCs exhibiting anisocytosis (unequal size), poikilocytosis (abnormally shaped), target cells (appearance of a bullseye), and dacryocytes (teardrop cells) [Figure 2]. Oral prophylaxis was done, and under local anesthesia, surgical exposure of the erupting maxillary premolar was planned. Soft-tissue diode laser was used for better patient compliance, minimal use of local anesthesia, and better hemostasis. Noneugenol periodontal dressing was placed at the surgical site. Postoperative instructions were given to the patient. Postprocedural antibiotics and anti-inflammatory medicines were prescribed. The patient was advised chlorhexidine mouth rinses twice daily for a week. The tissue specimen was submitted for histopathological examination. The patient was reviewed during the postoperative phase and showed an uneventful healing at the biopsy site [Figure 3].
Figure 2: Peripheral blood smear with microcytic hypochromic red blood cells exhibiting anisocytosis, poikilocytosis, target cells (black arrows), and dacryocytes (red arrows), Leishman's stain, ×400

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Figure 3: Postoperative intraoral photograph (10-month follow-up)

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Histopathologic examination: Hematoxylin- and eosin-stained section showed parakeratinized stratified squamous epithelium which was hyperplastic at places. The underlying fibrous connective tissue showed the presence of numerous small capillary vessels which were dilated with prominent endothelial cell proliferation. Foci of hematopoietic tissue were seen in a few areas. The presence of dense chronic inflammatory infiltrate was also noted [Figure 4]. The lesion was suggestive of PG.
Figure 4: Histomicrograph with numerous proliferating blood vessels, hematoxylin and eosin stain, ×100

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  Discussion Top


Thalassemia is an inherited, autosomal recessive, single, or multiple gene blood disorder characterized by reduced or absent hemoglobin.[3] It is commonly seen in people living in the Mediterranean region, lower socioeconomic strata, and in families with consanguineous marriages. Hemoglobin is a heterotetramer protein composed of two α-chains and two β-chains. In thalassemia, defects occur either in α-chains or β-chains forming defective RBCs with hemoglobin either reduced or completely absent.

In α-thalassemia, two genes HB α1 and HB α2 at chromosome 16 are involved and inherited, resulting in excess of β-globin chains in adults and γ-globin chains in newborn babies. The excess β-chains form unstable tetramer which causes deranged oxygen transport. In β-thalassemia, there is mutation in the HB β-gene at the chromosome # 11. According to the severity, it is subclassified as (1) thalassemia major (both the parents have defective genes), (2) thalassemia minor (only one of the parents has the defective genes), and (3) thalassemia intermedia. The disease causes multisystem involvement secondary to chronic anemia, iron overload, and complication of repeated blood transfusions. There is sufficient literature available citing the facial features and oral manifestations of thalassemia.[4]

Occurrence of pyogenic granuloma in pediatric patients

PG is a common benign tumor-like growth of the oral cavity. The term “PG” is a misnomer as the lesion neither contains pus nor is granulomatous. Although it can occur at any age, peak age of occurrence is the third decade of life with greater predilection in females due to the influence of hormones. There are few reported cases in the literature citing the occurrence of PG during the first decade of life and early adolescence.[5],[6],[7] Here, we report a case of PG in a 10-year-old male which is otherwise not a common occurrence.

Radiographically, there is usually no bone involvement but can cause slight superficial bone destruction. Jain et al. reported a case of PG in an 8-year-old male in alveolar crest causing superficial bone resorption and displacement of the underlying tooth bud.[8] Very few cases (3%) are reported of PG showing extensive bone loss and mobility of teeth mimicking a malignant tumor.[9] That is why excision should be always followed by histopathological examination.

Occurrence of pyogenic granuloma with thalassemia

After a thorough search of the literature, we could find only one article citing the occurrence of multiple PGs in a 10-year-old female, β-thalassemia major patient along with mobile lower anterior teeth.[10]

The association of PG in thalassemia patients could not be proved from the available literature as none of the studies or the reviews on thalassemia cites the occurrence of PG as one of the findings. After an in-depth analysis of the pathogenesis of thalassemia, it was established that PG could be a part of the spectrum of extramedullary hematopoiesis (EMH) seen in chronic anemia states such as thalassemia.[11] EMH refers to hematopoiesis outside of the medulla of the bone and has been widely reported in thalassemia patients. Ectopic EMH is most frequently seen in the liver and spleen but can occur in any tissue of the body such as lymph nodes, glands, lymph nodes, skin, breast, thymus, kidneys, gastrointestinal tract, nerves, and paraspinal regions.[12],[13] Ineffective erythropoiesis in thalassemia patients triggers EMH outside the liver and spleen, and these masses are referred to as extramedullary hematopoietic pseudotumors [Figure 5][14] Extramedullary hematopoietic pseudotumors are seen more in nontransfusion thalassemia patients (~20%) as compared to β-thalassemia major patients who are regularly transfused (<1%). Extramedullary hematopoiesis has been reported mainly in β-thalassemia intermedia and hemoglobin E/β-thalassemia patients, although it has also been reported in thalassemia minor.[15],[16] Although there are no direct links established between PG and thalassemia, there are few reports of extramedullary hematopoiesis in PG as an isolated finding in otherwise healthy subjects.[17],[18] Waraasawapati et al. conducted a study on 157 cases of PG and found that almost 10% of these lesions had foci of EMH.[18] The present case was also a nontransfusion thalassemia patient with PG showing abundant cell proliferation.
Figure 5: Flowchart describing the pathogenesis of extramedullary hematopoietic pseudotumors

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To our knowledge, this is the first case report of an intraoral PG in a thalassemia minor patient where the lesion has been hypothesized to be a type of extramedullary hematopoietic tumors. There is a need to envisage research in this direction and explore further the association of the occurrence of PG in various hemoglobinopathies to test this hypothesis.

Management includes excision of the lesion up to the periosteum along with removal of the local predisposing factors to reduce the chances of recurrence. The recurrence after surgical excision has been reported to be about 16% and most commonly attributed to incomplete removal.[19] The present case was followed up for a period of 3 years and there was no recurrence.

The treatment of PG includes excision or complete removal of the lesion. The present case was managed by excisional biopsy of the growth using diode lasers. Of the various advantages of lasers, such as reduced tissue trauma, faster and better healing, comfortable postoperative phase, especially in pediatric patients; the most relevant reason was the hemostasis and minimal or no bleeding during the procedure, especially in a patient with any bleeding disorder as in our case the patient was thalassemic. The use of lasers in the excision of exophytic growths in such patients becomes all the more vital as lasers arrest the bleeding in the field by contracting the vascular wall collagen as they act.[20]

Our case is probably first of its kind (as per the authors), as it states the occurrence of PG in a 10-year-old boy; the site, the age, the appearance of the growth which innocuously looked like the tissue around the erupting tooth and its rare association with β-thalassemia in the patient. It also elaborates on the pathogenesis behind the coexistence of these two conditions and illustrates the management of the case by performing excisional biopsy, done with the intention of surgical exposure of the erupting permanent maxillary premolar, using lasers.

In conclusion, soft-tissue enlargements of the oral cavity pose a diagnostic challenge as a diverse group of pathological processes can produce the same. The presence of any such enlargement in a thalassemic patient becomes a matter of utmost care as it can have implications as to how these cases should be managed both during the intraoperative phase and the postsurgical phase with the choice of medications, anesthesia, measures to achieve hemostasis, and satisfactory healing. A dentist should have profound knowledge about the management of such cases and hematological consultations should be taken wherever required. The present case reports the management of one such case successfully with diode lasers. The authors hypothesize that the thalassemia patients may have an inclination to develop PG as they often show extramedullary hematopoiesis. Thus, we advocate further research and investigation in this field to explore the association of extramedullary hematopoietic tumors in the oral cavity of patients with various hemoglobinopathies.

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.

Ethical considerations

Patient consent for publishing this work has been obtained. Photographs used for publication do not reveal the identity of the patient. Furthermore, the personal details have been delinked in this report.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

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Jafarzadeh H, Sanatkhani M, Mohtasham N. Oral pyogenic granuloma: A review. J Oral Sci 2006;48:167-75.  Back to cited text no. 1
    
2.
Vilmann A, Vilmann P, Vilmann H. Pyogenic granuloma: Evaluation of oral conditions. Br J Oral Maxillofac Surg 1986;24:376-82.  Back to cited text no. 2
    
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4.
Helmi N, Bashir M, Shireen A, Ahmed IM. Thalassemia review: Features, dental considerations and management. Electron Physician 2017;9:4003-8.  Back to cited text no. 4
    
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Willies-Jacobo LJ, Isaacs H Jr., Stein MT. Pyogenic granuloma presenting as a congenital epulis. Arch Pediatr Adolesc Med 2000;154:603-5.  Back to cited text no. 6
    
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Aguilo L. Pyogenic granuloma subsequent to injury of a primary tooth. A case report. Int J Paediatr Dent 2002;12:438-41.  Back to cited text no. 7
    
8.
Jain M, Singhal S, Goyal M, Sharma B. Pyogenic granuloma in eight-year-old child associated with bone loss and displacement of tooth bud: A unique case. Int J Exp Dent Sci 2015;4:134-6.  Back to cited text no. 8
    
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Martins-Filho PR, Piva MR, Da Silva LC, Reinheimer DM, Santos TS. Aggressive pregnancy tumor (pyogenic granuloma) with extensive alveolar bone loss mimicking a malignant tumor: Case report and review of literature. Int J Morphol 2011;29:164-67.  Back to cited text no. 9
    
10.
Naz M, Ahmed S, Haider SM, Naureen A. β-Thalassaemia: A case report of multiple pyogenic granulomas in thalassemic major patients. Pak J Surg 2018;34:169-71.  Back to cited text no. 10
    
11.
Bukhari SS, Junaid M, Rashid MU. Thalassemia, extramedullary hematopoiesis, and spinal cord compression: A case report. Surg Neurol Int 2016;7:S148-52.  Back to cited text no. 11
    
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Aessopos A, Tassiopoulos S, Farmakis D, Moyssakis I, Kati M, Polonifi K, et al. Extramedullary hematopoiesis-related pleural effusion: The case of beta-thalassemia. Ann Thorac Surg 2006;81:2037-43.  Back to cited text no. 12
    
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Al-Habib H, Hadzikaric N. Spinal cord compression due to intraspinal extramedullary hematopoiesis in thalassemia intermedia. Neurosciences (Riyadh) 2007;12:261-4.  Back to cited text no. 13
    
14.
Taher A, Vichinsky E, Musallam K, Cappellini MD, Viprakasit V. Guidelines for the management of non transfusion dependent thalassaemia (NTDT). In: Weatherall D, editor. Guidelines for the Management of Non Transfusion Dependent Thalassaemia (NTDT). Nicosia (Cyprus): Thalassaemia International Federation; 2013.  Back to cited text no. 14
    
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Musallam KM, Rivella S, Vichinsky E, Rachmilewitz EA. Non-transfusion-dependent thalassemias. Haematologica 2013;98:833-44.  Back to cited text no. 15
    
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Lalueza A, Silva CP, Alcalá-Galiano A, Arrieta E, Cánovas JM, Calero MR, et al. Presacral and intrathoracic extramedullary hematopoiesis in a patient with β thalassemia minor. Clin Respir J 2018;12:322-6.  Back to cited text no. 16
    
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Waraasawapati S, Koonmee S, Kusama H, Kudo M. Extramedullary hematopoiesis in pyogenic granuloma. Pathol Int 2013;63:492-5.  Back to cited text no. 17
    
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Vega Harring SM, Niyaz M, Okada S, Kudo M. Extramedullary hematopoiesis in a pyogenic granuloma: A case report and review. J Cutan Pathol 2004;31:555-7.  Back to cited text no. 18
    
19.
Hasanoglu Erbasar GN, Senguven B, Gultekin SE, Cetiner S. Management of a recurrent pyogenic granuloma of the hard palate with diode laser: A case report. J Lasers Med Sci 2016;7:56-61.  Back to cited text no. 19
    
20.
Mathur E, Sareen M, Dhaka P, Baghla P. Diode laser excision of oral benign lesions. J Lasers Med Sci 2015;6:129-32.  Back to cited text no. 20
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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