Isolated Intra-Oral Relapsed B-Acute Lymphoblastic Leukemia (B-ALL): A Case Report

Case Report

Ann Hematol Onco. 2023; 10(1): 1414.

Isolated Intra-Oral Relapsed B-Acute Lymphoblastic Leukemia (B-ALL): A Case Report

Al-Mohaya MA1, Al-Ali MA2, Binmayouf MS3, Sewaralthahab SS4* and Elyamany G5

1Consultant of Oral Medicine, Oral Medicine & Special Care Dentistry, Prince Sultan Military Medical City, KSA

2Resident of Saudi Board of Oral Medicine and Patholog, Prince Sultan Military Medical City, Riyadh, KSA

3Medical Intern, King Saud University, KSA

4Assistant Professor of Internal Medicine & Consultant Hematologist, King Saud University College of Medicine, King Saud University Medical City, Saudi Arabia.

5Consultant of Hematopathology, Department of Central Military Laboratory and Blood Bank, Saudi Arabia

*Corresponding author: Sarah Salah Sewaralthahab Assistant Professor of Internal Medicine & Consultant Hematologist, King Saud University College of Medicine, King Saud University Medical City, Riyadh, Saudi Arabia.

Received: December 15, 2022; Accepted: January 13, 2022; Published: January 20, 2023


B-Lymphoblastic Leukemia (B-ALL) is the second most common acute leukemia in adults. Its clinical manifestations include leukocytosis or leukopenia, anemia, and thrombocytopenia with an overall poor prognosis in elderly patients. If relapse occurs, it commonly involves the bone marrow but can also involve Extramedullary (EM) sites such as the brain, testis, liver, spleen and lymph nodes. Isolated extramedullary involvement of the oral cavity in relapse B-ALL is extremely rare with only a few reposted cases in the literature in pediatrics and Adolescent-and-Young-Adult (AYA) population. Here we describe the case of an elderly gentleman who presented with isolated EM B-ALL relapse involving the oral cavity and gnathic bones which was initially misdiagnosed as an odontogenic infection.

Keywords: Relapse; Acute lymphoblastic leukemia; Lymphoblastic lymphoma; Oral cavity; Gnathic bone

Abbreviations: B-ALL: B-Lymphoblastic Leukemia; EM: Extramedullary; B-LBL: B-Lymphoblastic Lymphoma; AYA: Adolescent And Young Adults; FISH: Fluorescence In Situ Hybridization, CVD: Cyclophosphamide, Vincristine, Dexamethasone; POMP: Mercaptopurine, Vincristine, Methotrexate, Prednisone; HSCT: Hematopoietic Stem Cell Transplantation


B-Lymphoblastic Leukemia (B-ALL) is a neoplasm of precursor lymphoid cells committed to the B cell lineage, involving the bone marrow, blood, and at times Extramedullary sites (EM) with a blast percentage of more than 20% [1]. It represents 80–85% of Acute Lymphoblastic Leukemia (ALL), in contrast to B-Lymphoblastic Lymphoma (B-LBL) which accounts only for 10% [2]. By age, B-ALL is the most common malignant neoplasm found in pediatrics/ Adolescent and Young Adults (AYA) population with about 60% of cases diagnosed in those younger than 20 years of age and the second most common acute leukemia in adults [3].

Patients commonly present with fatigue, bone pain, bleeding, infections, anorexia, or malaise [4]. The CNS, testes, liver, spleen, and lymph nodes are common sites for extramedullary disease; other less common reported sites include the ovaries, breast, and kidneys. However, involvement of the head and neck area is quite uncommon [5,6] and B-ALL relapse primarily involving the oral cavity and gnathic bone are extremely rare with only a few reported cases in the literaturein pediatrics/ AYA [7,8].

Herein, we describe a case of a 78-year-old Saudi gentleman with a history of B-ALL who presented with an intraoral swelling as an isolated manifestation of relapsed B-ALL.

Case Report

A 78 -year-old Saudi gentleman was diagnosed with B-ALL in June 2019 after presenting to the emergency department at Prince Sultan Medical Military City with fever, confusion, and severe generalized bony pain limiting his mobility. He had a medical history of chronic renal impairment, hypertension, dyslipidemia, and long-standing heavy smoking. At presentation, laboratory investigation showed elevations in creatinine (122 umol/L) and urea (7.7 mmol/L) leukopenia (0.7 x 10^9/L), anemia (hemoglobin 7.5 g/dL), thrombocytopenia (platelet count 47x 10^9/L) and low red blood cell count (2.7 x10^12/L). Further investigations revealed a normal free light chain ratio and normal immunoglobulin level. A plain radiograph was done showing multiple lytic lesions involving the pelvic bone. CT scan showed enlarged left para-aortic lymph node measuring 3.4x2.5 cm with no other lymphadenopathy and no hepatosplenomegaly.

Bone marrow aspiration and core biopsy showed a hypercellular marrow with normal trilineage hematopoiesis being replaced by infiltrating mononuclear malignant cells with round to oval, large, and slightly irregular nuclei having open chromatin, scant cytoplasm, and prominent nucleoli (blast cells, approximately 93% of BM cells). Flow cytometry analysis showed the blast cells express CD19, CD10, CD20 (dim),CD22, CD79a, HLADR and negative for MPO, CD3, CD34, TdT and other markers. On immunohistochemistry, the cells were positive for PAX 5, CD20 (weak), and BCL2 and negative for Kappa, lambda, MPO, CD3, TdT and CD34. Fluorescence in situ hybridization (FISH) analysis showed clonal mutations with MYC gene 8q24 rearrangement and duplications of the MLL 11q23 and ETV6-RUNX1 t (12:21) rearrangement.

Following diagnosis, the patient was placed on reducedintensity chemotherapy with mini-hyper-CVD (cyclophosphamide, vincristine, dexamethasone - part A included 50% reduction of cyclophosphamide and dexamethasone plus 2 mg of vincristine and no anthracycline, while part B included methotrexate 250mg/m2 and 4 doses of cytarabine 0.5 mg/m2). His treatment course was complicated by repeated attacks of severe neutropenic sepsis requiringmultiple hospitalizations. Maintenance chemotherapywith POMP (Mercaptopurine, Vincristine, Methotrexate, Prednisone) was also modified and reduced according to the patient’s tolerance as well. Follow-up bone marrow examination showed achievement of morphological remission with normal trilineage hematopoiesis. FISH confirmed resolution of chromosomal abnormalities previously detected at diagnosis.

In December 2020the patient presented to the oral medicine clinic with palatal swelling that fluctuated in size and responded minimally after empiric antibiotics and extraction of unsalvageable teeth. Extraoral examination was unremarkable with no cervical lymphadenopathy. However, the intraoral examination revealed a sessile dome-shaped exophytic palatal mass that extended to the alveolar ridge of extracted teeth #11 and #21. It had a smooth surface and was pinkish to purple in color with focal red discoloration where the lower teeth came in contact with the mass. The mass was fibrous in its consistency and measured about 3x5 cm in size (Figure 1). A CT scan revealed an asymmetrical destructive mass and MRI showed an enhanced soft tissue mass involving the anterior paramedian aspect of the maxilla extending to involve the alveolar process and floor of the nasal cavity.Laboratory investigation revealed elevations in creatinine level (125umol/L) and urea (6.4 mmol/L). White blood cell count was3.1x 10^9/L, platelet count was218 10^9/L and hemoglobin level was 10.2 g/dL. Peripheral blood smear showed no blast cells. Bone marrow aspiration and core biopsy showed hypocellular marrow with no increase in blast cells. An incisional biopsy from the palatal mass was performed under local anesthesia with histopathologic examination revealing diffuse infiltration of small to medium size oval to round cells with open chromatin and scanty cytoplasm. The tumor cells were positive for CD10, CD45, CD20 (week and subset), TdT (focal), and BCL-2 with high Ki-67 proliferation rate (70%-80%), but negative to CD3, CD30, and CD34 (Figure 2).Morphological and immunohistochemical profiles were consistent with extramedullary relapse of B-ALL.