Dentomaxillofacial Radiology (2009) 38, 289291 2009 The British Institute of Radiology http://dmfr.birjournals.

org

CASE REPORT

Acute lymphoblastic leukaemia: an unusual radiological presentation

R Ali*,1, A Brooke2 and J Luker2
Department of Oral and Dental Science, Division of Restorative Dentistry, Bristol Dental Hospital, UK; 2Department of Oral and Dental Science, Division of Oral Medicine, Pathology and Microbiology, Bristol Dental Hospital, UK
1

A 14-year-old female patient attended Bristol Dental Hospital for an oral screening prior to undergoing a bone marrow transplant as treatment for her acute lymphoblastic leukaemia. Maxillofacial radiographs revealed multiple, well-defined, non-corticated radiolucent lesions throughout the vault of her skull and mandible. These radiological features (coupled with the patients age) would have correlated with a diagnosis of Langerhans cell histiocytosis. However, a previous bone marrow biopsy confirmed that the patient did indeed have acute lymphoblastic leukaemia. The lytic lesions were present throughout her entire skeletal frame and had previously led to episodes of leg and abdominal pain. We feel that this radiological presentation of leukaemia needs to be reported as these features could easily have been confused with other haematological or even malignant conditions. Dentomaxillofacial Radiology (2009) 38, 289291. doi: 10.1259/dmfr/53260198 Keywords: leukaemia, well defined, non-corticated, radiolucent, lesions

Case report The Bone Marrow Transplant (BMT) Unit at Bristol Childrens Hospital has performed over 800 transplants on both paediatric and adult patients over the last 10 years. As part of their BMT work-up, all patients are dentally screened before undergoing chemotherapy to identify any potential areas of sepsis/oral pathology. A 14-year-old female patient attended the Primary Care Unit at Bristol Dental Hospital for an oral screen, prior to undergoing a BMT as treatment for her acute lymphoblastic leukaemia (ALL). The patient had previously received comprehensive chemotherapy following the diagnosis of ALL in 2006, was allergic to penicillin, and her current medications included fusidic acid, omeprazole and septrin. Intraoral examination revealed no obvious oral mucosal lesions and her oral hygiene was excellent. The patient was fully dentate (with the exception of all four third molars) and there was clinical evidence of caries in both upper first permanent molars. Bitewing radiographs confirmed the presence of early caries in the upper first permanent molars. A panoramic radiograph (PR) revealed full crown development
*Correspondence to: Mr Rahat Ali, Department of Oral and Dental Science, Bristol Dental Hospital, Lower Maudlin Street, Bristol, BS1 2LY, UK; E-mail: rahat224@hotmail.com Received 12 March 2008; revised 19 May 2008; accepted 19 May 2008

of all four third molars and significant radiolucencies in the body and ascending ramus bilaterally (Figure 1). The radiolucencies seen in Figure 1 were multiple, round, well-defined and non-corticated, and extended throughout the body and ascending ramus of the mandible bilaterally. The lesions themselves varied from 12 cm in diameter and had a monolocular punched-out appearance. In certain areas, they appeared to have eroded the bony cortex. Further radiological investigations were requested and a lateral skull radiograph was taken (Figure 2). This view confirmed the presence of multiple radiolucent areas throughout the cranial vault. These lesions had a similar punched-out, non-corticated appearance to those present on the PR. Classically, multiple punched-out non-corticated lesions are associated with malignant multiple myeloma1 and Langerhans cell histiocytosis.2 Discussion with the paediatric oncologist revealed that the patients original referral prior to diagnosis with ALL had been due to lower abdominal and leg pain. Radiographic investigation showed multiple radiolucencies in the scapula (Figure 3) and further investigation showed similar radiolucencies throughout the skeletal frame. A bone marrow biopsy and haematological investigation confirmed a diagnosis of ALL.

290

Acute lymphoblastic leukaemia R Ali et al

Figure 1 Dental panoramic radiograph of the patient. Arrows indicate some of the punched-out radiolucent lesions in the mandible

Discussion There are four main types of leukaemia: acute lymphoblastic, acute myeloid, chronic lymphocytic and chronic myeloid. ALL is the most common leukaemia seen in children, accounting for 85% of paediatric cases.3 Clinical symptoms of leukaemia include anaemia, fever, an increased bleeding tendency, fatigue4,5 and even mental nerve paraesthesia.6 The radiological signs of ALL include disappearance of the inferior dental canal (IDC), an increased periodontal ligament space, loss of lamina dura and destruction of alveolar crestal bone.6 Disappearance of the IDC would certainly correlate with reports of sensory paraesthesia, which is classical of malignancy, inflammation and trauma. However, we had never encountered a patient with ALL who presented with multiple punched-out radiolucencies throughout their body. Osteopathy is certainly one of the most common initial symptoms of ALL in adolescents, but multiple sites of osteolysis throughout the body are rarely observed.7 This in itself seems paradoxical as serum parathyroid hormone related-peptide (PTHrp) levels are increased in patients with ALL and the lymphoblasts themselves can produce the peptide.7 Given that parathyroid hormone can stimulate the generation of ruffled borders on osteoclasts, and therefore bony resorption,8 the blast cells may be responsible for the multiple osteolytic lesions seen in ALL. Punched-out non-corticated mandibular lesions are often seen in multiple myeloma.9,10 However, given the patients relatively young age, this would have been an unlikely cause for the lesions shown in Figures 1 and 2. A more likely candidate would have been Langerhans cell histiocytosis. Eosinophillic granuloma (EG) is a form of Langerhans cell disease and accounts for over 50% of cases.11 The incidence of the disease tends to peak in the first three decades of life11and can affect multiple bones throughout the body (including the skull and mandible). Given that EG can also present radiographically as non-corticated punched-out lesions,12,13 we initially felt that it may have been responsible for the lesions shown in Figures 1 and 2. It came as a surprise when the patients oncologist
Dentomaxillofacial Radiology

Figure 2 Lateral skull radiograph of the patient. Arrows indicate some of the punched-out radiolucent lesions

informed us that ALL was responsible for the osteolytic lesions and that this had been confirmed by a bone marrow biopsy. We feel that this is both an interesting and unusual radiological presentation of ALL and warrants reporting in the dental literature as these radiological features

Figure 3 Radiograph of the patients scapula. Arrows indicate some of the punched-out lesions

Acute lymphoblastic leukaemia R Ali et al

291

could easily be confused with other conditions, and would clearly affect the treatment that the patient would receive. It also highlights the need to consider
References
1. Kesari S, Bundock EA. Punched-out skull. Arch Neurology 2004; 61: 958959. 2. Hoover KB, Rosenthal DI, Mankin, H. Langerhans cell histiocytosis. Skel Radiol 2007; 36: 95104. 3. Scully C, Cawson A. Medical problems in dentistry (5th edn). New York, NY: Elsevier Churchill Livingston, 2004, 156170. 4. Henderson ES. Acute leukemia: General considerations. In: Williams WJ, Beutler E, Erslev AJ, Lichtman MA (eds). Hematology (4th edn). New York, NY: McGraw-Hill Publishing Company, 1990, 236251. 5. Mahoney DH. Acute lymphoblastic leukemia in childhood. In: Oski FA, DeAngelis CD, Feigin RD, McMillan JA, Warshaw J (eds). Principle and practice of paediatrics (2nd edn). Philadelphia, PA: JB Lippincott Company, 1994, 17011710. 6. Hiraki A, Nakamura S, Kichachiro A, Takenoshita Y, Horinouchi Y, Shiohara, M, et al. Numb chin syndrome as an initial symptom of acute lymphocytic leukaemia: report of three cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1997; 83: 555561. 7. Shimonodan H, Nagayama J, Nagatoshi Y, Hatanaka M, Takada A, Iguchi JH, et al. Acute lymphocytic leukaemia in adolescence with multiple osteolytic lesions and hypercalcaemia mediated by lymphoblast-producing parathyroid hormone-

ALL in the differential diagnosis of any persistent bone or joint pain, especially when radiographs reveal multiple radiolucencies throughout the skeletal frame.

8. 9.

10. 11.

12.

13.

related peptide: a case report and review of the literature. Pediatr Blood Cancer 2005; 45: 333339. Miller SC. Rapid activation of the medullary bone osteoclast cell surface by parathyroid hormone. J Cell Biol 1978; 76: 615618. Witt C, Borges AC, Klein K, Neumann HJ. Radiographic manifestations of multiple myeloma in the mandible: a retrospective study of 77 patients. J Oral Maxillofac Surg 1997; 55: 450453. Scutellari PN, Orzincolo C. [Mandibular lesions in multiple myeloma]. Radiolog Med (Torino) 1992; 83: 219223 (in Italian). Yasko AW, Fanning CV, Ayala AG, Carrrasco CH, Murray JA. Percutaneous techniques for the diagnosis and treatment of localised Langerhans cell histiocytosis eosinophillic granuloma of bone. J Bone Joint Surg 1998; 82: 219228. Dagenais M, Pharoah MJ, Sikorski PA. The radiographic characteristics of histicytosis X. A study of 29 cases that involve the jaws. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1992; 74: 555561. Ardekian L, Peled M, Rosen D, Rachmiel A, Abu el-Naaj I, Laufer, D. Clinical and radiographic features of eosinophillic granuloma in the jaws: review of 41 lesions treated by surgery and low-dose radiotherapy. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1999; 87: 238242.

Dentomaxillofacial Radiology