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CASE REPORT
Year : 2023 | Volume
: 14
| Issue : 2 | Page : 116-118
Gingival hypertrophy in acute promyelocytic leukemia – A rare clinical encounter
Arpit Shastri1, Anurag Rohatgi2, Shailaja Shukla3, Roopali Dahiya1, Aman Panchal2, Divita Rohatgi4
1 Department of Medicine, Atal Bihari Vajpayee Institute of Medical Sciences, Dr. Ram Manohar Lohia Hospital, New Delhi, India
2 Department of Medicine, Lady Hardinge Medical College and Associated Dr. Ram Manohar Lohia Hospital, New Delhi, India
3 Department of Pathology, Lady Hardinge Medical College, New Delhi, India
4 Medical Student, Department of Medicine, Hamdard Institute of Medical Sciences and Research, New Delhi, India
Correspondence Address:
Dr. Anurag Rohatgi
Department of Medicine, Lady Hardinge Medical College, New Delhi - 110 001
India
Source of Support: None, Conflict of Interest: None
DOI: 10.4103/injms.injms_26_23
Acute promyelocytic leukemia (APL) is a subtype of acute myeloid leukemia (AML), a cancer of the white blood cells. Its common manifestations are fatigue, fever, coagulopathy, anorexia, and pancytopenia. Oral manifestations are identified in huge majority of patients with AML of which gingival hypertrophy is more specific to acute monocytic (M5) and acute myelomonocytic (M4) subtypes of AML. Very rarely, gingival hypertrophy may be encountered in patients with APL. Herein, we present a case of a young male who presented with gingival hypertrophy and was subsequently diagnosed with APL (M3).
Keywords: Acute promyelocytic leukemia, gingival hypertrophy, oral manifestations
Acute promyelocytic leukemia (APL) is a unique subtype, accounting for 8% of all acute myeloid leukemia (AML) cases.[1],[2] It is characterized by bone marrow infiltration by abnormal dysplastic promyelocytes.[3] It commonly affects adult individuals in the 30–40 years age group.[4]
The common presenting features are fatigue, fever, coagulopathy, anorexia, and pancytopenia.[5] Coagulopathy at diagnosis is the most distinctive clinical feature in APL. This can manifest as gum bleeds, epistaxis, easy bruisability, purpuras, and petechiae. Intracranial hemorrhage is the most unfortunate and dreadful manifestation of coagulopathy in APL, which if untreated and unattended, can cause early hemorrhagic death.[6] Coagulopathy in APL is attributed to many causes. There is secondary thrombocytopenia due to reduced bone marrow production of platelets along with superimposed qualitative defects in platelet activity. Furthermore, there are increased levels of procoagulant factors leading to increased thrombin generation, which further promotes the coagulation cascade.[7] While symptoms of coagulopathy are the most striking, there may be other uncommon clinical manifestations in APL as well. Primary oral manifestations are one such subgroup. These manifestations also include findings secondary to coagulopathy, as discussed above. Moreover, mucosal pallor, ulceration, gingival enlargement, and localized infections are also encountered.[3],[8] However, gingival hypertrophy occurs rarely in APL. It is more commonly seen in acute monocytic leukemia (AML-M5), followed by acute myelomonocytic leukemia (AML-M4) and acute myelocytic leukemia (AML-M1, M2).[9],[10]
Owing to rapidly developing and worsening coagulopathic complications, APL is considered a medical emergency.[11] Immediate administration of all-trans retinoic acid (ATRA) is the rule of thumb, based on the preliminary hematological suggestion and even before the cytogenetic confirmation of characteristic, Promyelocytic leukemia/retinoic acid receptor alpha (PML-RARA) fusion transcript is available.[1] In such scenarios, uncommon presenting symptoms may cause a potential delay in accurate diagnosis. Hence, it is critical to be aware of such rare manifestations to avoid unnecessary delay in diagnosis. We hereby present a unique case of gingival hypertrophy as a rare, primary manifestation of APL.
Case ReportA 26-year-old male, resident of Delhi, presented with complaints of easy fatigability and fever for 15 days, along with nasal and gum bleed for 4 days. There were no associated gastrointestinal or urinary complaints. The patient denied any significant drug history. Physical examination revealed pallor, gingival hypertrophy, and multiple bleeding spots in the oral mucosa [Figure 1]a and [Figure 1]b. Complete systematic examination was normal. Within laboratory investigations, complete hemogram revealed pancytopenia with hemoglobin of 10.5 g/dl, total leukocyte count of 830/μL, and platelet count of 20,000/μL. Peripheral smear (PS) showed marked leucopenia with the occasional presence of hypergranular blasts along with anemia and thrombocytopenia [Figure 2]. Additional tests such as dengue serology and malarial antigen test came out as negative. Tests for liver function, kidney function, and serum electrolytes were also within the normal limits. In view of pancytopenia and occasional blasts in PS, bone marrow aspiration was performed, which revealed hypercellular smears with numerous abnormal promyelocytes, many of them showing characteristic bundles of Auer rods More Details [Figure 3] and [Figure 4]. Immunocytochemistry highlighted these abnormal promyelocytes through strong granular, cytoplasmic positivity for myeloperoxidase [Figure 5]. Based on the characteristic bone marrow findings, a diagnosis of APL was rendered. The patient was then referred to a specialized oncology center where he was started on ATRA and arsenic trioxide. The patient is on regular follow-up with no complaint till his last visit.
Figure 1: (a) Clinical photograph depicting gingival hypertrophy adjacent to upper teeth along with bleeding spots. (b) Clinical photograph depicting gingival hypertrophy adjacent to lower teeth
Figure 2: PS showing hypergranular blast along with marked leucopenia and thrombocytopenia (Giemsa, ×100). PS: Peripheral smear
Figure 3: Bone marrow aspirate smear showing numerous abnormal promyelocytes (Giemsa, ×100). The arrow depicting one such promyelocyte
Figure 4: Bone marrow imprint smears showing abundant hypergranular, abnormal promyelocytes with many Auer rods (Giemsa, ×100)
Figure 5: Abnormal promyelocytes showing strong MPO positivity (MPO, ×100). MPO: Myeloperoxidase Discussion APL is a subtype of AML with typical clinical, hematological, and morphological features.[9] It constitutes 8% of all AML cases.[1],[2] The clinical history is usually short and coagulopathy is the prominent manifestation. Most patients have pancytopenia and abnormal promyelocytes.[9]
Gingiva is a unique site of involvement in many diseases and thus, forms a significant part of oral and systemic examination. Gingival hypertrophy occurs due to excessive overgrowth of the gingival tissue due to poor oral hygiene and drugs. It can occur as part of the clinical spectrum of many genetic disorders and systemic illnesses, including hematological diseases.[12] A subset of cases may even be idiopathic.[13] Within hematological malignancies, oral manifestations have been variably documented in AML, chronic myeloid leukemia, acute lymphocytic leukemia, and chronic lymphocytic leukemia. These manifestations encompass mucosal pallor, gum bleeding, mucosal ulcers, gingival enlargement, trismus, neuropathy, facial palsy, and oral infections.[3] Gingival involvement in form of hypertrophy has rarely been described in acute leukemia. It is more common in acute than chronic leukemia and in adults with aleukemic or subleukemic leukemia with no specific gender predisposition.[10] Approximately 5% of patients with AML exhibit gingival infiltration as the initial presenting complaint.[14] It is a more consistent finding in AML-M5 and AML-M4.[9],[10] Very rarely, APL can demonstrate similar oral manifestations as found in other types of acute leukemia. Gingival hypertrophy occurs as a result of infiltration of gingival tissue by leukemic cells.[3] Sometimes, APL patients without gingival hypertrophy can develop this abnormality after ATRA therapy.[10] However, in the index case, gingival hypertrophy was seen before APL was even diagnosed. This is a rare clinical scenario variably reported in literature. There is no concrete data regarding an exact incidence of gingival hypertrophy in APL; however, sporadic case reports have documented an incidence of 5.7% and 20%.[15] Hence, there is overall paucity of literature regarding the gingival hypertrophy in APL. The index case highlights a rare clinical presentation of APL, which should not be overlooked.
The subconscious hunt for more classic manifestations of coagulopathy in APL can mask the more subtle but important clinical clues to the underlying diagnosis. Thus, it must be borne in mind that gingival hypertrophy, although uncommon, can be a valuable hint to APL. Thus, APL should be considered a possible differential diagnosis in its evaluation. An early recognition and appropriate investigations can uncover the disease, allowing its timely diagnosis and prompt management.
ConclusionAPL can present with multiple clinical features, out of which gingival hypertrophy is extremely rare. Due to the presence of such atypical manifestations, the diagnosis is often missed out, which is extremely worrisome since APL is associated with increased morbidity and mortality. Awareness of such uncommon features can facilitate its early detection, timely management, and prevention of mortality.
Acknowledgment
The authors acknowledge the contribution of the Pathology Department of Lady Hardinge Medical College, New Delhi, India, for their support and help in the workup of the patients.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
None.
Conflicts of interest
There are no conflicts of interest.
References
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