|
|
CASE REPORT |
|
|
|
| Year : 1995 | Volume
: 49
| Issue : 1 | Page : 9-10 |
| |
Sickle - Cell disease simulating Burkitt's lymphoma: Case report*
EML Endeley, EO Enwerem, C Holcombe, RV Patel
Division of Pediatric surgery, Department of Surgery, College of Medical sciences & Unimaid Teaching Hospital, Maiduguri - Nigeria - West Africa,
| Date of Submission | 13-Jan-1992 |
| |
Correspondence Address:
R V Patel
210. AG Office Staff Quarters, Near Saurashtra University, Rajkot 360005, Gujarat, India
PMID: 7590994
How to cite this article:
Endeley E, Enwerem E O, Holcombe C, Patel R V. Sickle - Cell disease simulating Burkitt's lymphoma: Case report*. Indian J Med Sci 1995;49:9-10 |
The clinical and radiological manifestations of sickle-cell disease are protean and no organ is spared by the disease. A tumor like presentation of the disease is very rare and infrequently reported. [1] We wish to communicate a case of sickle cell disease having jaw swelling which simulated Burkitt's tumor clinically and radiologically.
| ¤ Case Report | |  |
A 3-year old girl had a rapidly growing right lower jaw swelling of 4 weeks duration. She was first born, had failure to thrive, fever and weight loss. She was taken to a district hospital where an X-ray of jaw swelling showed dental anarchy and estolytic lesions in the underlying mandible and considerable soft tissue mass. A. provisional diagnosis of Burkitt's tumor was made. There were no facilities for cytological or histological confirmation, patient was poor enough to go to teaching hospital and the jaw tumor was increasing in size. It was decided to give a therapeutic trial of one dose of cyclophosphamide. Following this injection, her general condition deteriorated including fever, icterus, pallor and the size of the swelling started increasing rapidly. It was thought to be drug reaction and patient was referred to us for further management. Upon admission, a relatively painless and mildly tender right lower jaw tumor of 16 cm diameter was noted without any separate palpable lymphnodes. She was febrile, icteric and anemic. The hemoglobin was 6.8 gm %, total bilirubin 95 /umol/ IL, conjugated bilirubin 25/umol/L and peripheral smear was suggestive of sickle cell disease. The sickling test was positive and hemoglobin electrophoresis confirmed Hb-SS pattern. Repeated biopsies of the mass through intra oral route showed nonspecific inflammation and features of reparative giant cell granulma. She was given antibiotics, analgesics, folic acid and vitamin B complex supplements, intravenous fluids and blood transfusion. Dental consultation and treatment was taken for local treatment of the granuloma. She responded to treatment and histological examination of the excised tissue showed features of osteomyelitis and reparative giant cell granuloma. There was no evidence of malignant lymphoma in several sections studied and clinically she is free of any recurrence at our years follow up.
| ¤ Discussion | | |
Burkitt's tumor, malignant lymphoma of Africa, is the commonest malignant disease of children between the ages of 3 and 12 years inclusive living in the equitorial regions of Africa and majority of them present with jaw swellings. [2] Although sickle cell disease is the commonest disease in tropical Africa South of the Sahara and north of the River Zambezi, it is very rare for such patient to have inferection and/or osteomyelities of jaw bone following poor Oral hygiene and dental caries in these susceptible patients. Bones of the face are exception as there is considerable soft tissue swelling without infection of underlying bone even during inferective crisis. [1] The radiological changes of sickle cell disease or Burkitt's lymphoma are nonspecific. However in the former, the alveolar margins of the jaws may show a coarse cortical bone (lamina dura) for the tooth sockets while it is first to disappear in the latter. In both, lesions first appear as multiple small osteolytic deposits which coalesec to form larger areas of bone destruction. [2],[3] Burkitt tumor is unusually sensitive to even a single dose of chemotherapy and it is not unusual for many district hospitals in tropical Africa to administer therapeutic trial. [4] However it is recommended that histological confirmation of Burkitt's tumor should be insisted upon and in the regions were sickle cell disease is very much prevalent hemoglobin, peripheral smear, sickling test and hemoglobin electrophoresis should be carried out lest chemotherapeutic agent may induce hemolytic or other crisis as was evident in our present case.
| ¤ References | | |
| 1. | Endeley EML, Enwerem E0. Holcombe C, et al. Sickle cell anemia presenting as periorbital tumour. Ind J Med SC 1990;44:120-122.  |
| 2. | Scott RAP and Mclvor J. The lymphas tics and lymph nodes. In : Bailey and Love's short practice of surgery eds. Rains AJH and Mann CV, 12 Ed, London, ELBS/HK Lewis, 1989;PP, 118-131. |
| 3. | Lagundoye SB. Radiology of sickle cell disease. In : Sickle cell disease. Ed Fleming AF, Edl, Edinburgh. Churchill Livingstone, 1982;PP 57-72. |
| 4. | Nwako FA. Text book of Paediatric surgery in the Tropics, 1 Ed, London. Macmillan Press, 1980. |
|
