|Year : 2021 | Volume
| Issue : 1 | Page : 37-40
Primary osseous burkitt lymphoma mimicking multiple myeloma – A case report and review of literature
Somnath Roy1, Tanmoy Kumar Mandal1, Lingaraj Nayak2, Avinash Gupta3, Dhanlaxmi Lalit Shetty4
1 Department of Hematology and Medical Oncology, Homi Bhabha Cancer Hospital, Tata Memorial Centre, Varanasi, Uttar Pradesh, India
2 Department of Hematology and Medical Oncology, Tata Memorial Centre, Mumbai, Maharashtra, India
3 Department of Haematopathology, Homi Bhabha Cancer Hospital, Tata Memorial Centre, Varanasi, Uttar Pradesh, India
4 Department of Cancer Cytogenetics, ACTREC, Tata Memorial Centre, Navi Mumbai, Maharashtra, India
|Date of Submission||30-Jun-2020|
|Date of Decision||18-Aug-2020|
|Date of Acceptance||12-Sep-2020|
|Date of Web Publication||15-Mar-2021|
Dr. Somnath Roy
Department of Hematology and Medical Oncology, Homi Bhabha Cancer Hospital, Tata Memorial Centre, Varanasi, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
Burkitt lymphoma (BL) is one of the aggressive subtypes of non-Hodgkin lymphoma commonly seen among pediatric and young population. Primary BL of pelvic bone presenting with back pain, paraparesis, and multifocal osteolytic lesion in the elderly is a rare entity. We report a case of primary BL of pelvic bone in a 65-year-old female presenting with back pain and paraparesis, facing diagnostic dilemma as the lesions were radiologically mimicking multiple myeloma. Subsequently, bone marrow flowcytometry and cytogenetic evaluation by fluorescence insitu hybridization confirmed the diagnosis. We highlight the diagnostic challenge, clinical presentation, and treatment strategy in such a rare case along with a review of literature.
Keywords: Bone primary, burkitt lymphoma, rare presentation
|How to cite this article:|
Roy S, Mandal TK, Nayak L, Gupta A, Shetty DL. Primary osseous burkitt lymphoma mimicking multiple myeloma – A case report and review of literature. J Appl Hematol 2021;12:37-40
|How to cite this URL:|
Roy S, Mandal TK, Nayak L, Gupta A, Shetty DL. Primary osseous burkitt lymphoma mimicking multiple myeloma – A case report and review of literature. J Appl Hematol [serial online] 2021 [cited 2021 Apr 20];12:37-40. Available from: https://www.jahjournal.org/text.asp?2021/12/1/37/311324
| Introduction|| |
Primary bone lymphoma (PBL) is a rare form of extranodal lymphoma, which is limited to the bone or bone marrow without any systemic involvement; it represents <1% of malignant lymphomas. The most common histology of PBL is diffuse large B cell lymphoma; other histologies such as small lymphocytic, anaplastic large cell, and follicular lymphoma have also been reported. Burkitt lymphoma (BL) is a monoclonal proliferation of B-lymphocytes accounting for about 40% of childhood non-Hodgkin lymphoma (NHL). We report a case of primary BL of pelvic bone in a 65-year-old female presenting with back pain and paraparesis, facing diagnostic dilemma as the lesions were radiologically mimicking multiple myeloma. We highlight the diagnostic challenge, clinical presentation, and treatment strategy in such a rare case along with a review of literature.
| Case Report|| |
A 65-year-old female presented to our hematology oncology clinic in January 2020 with 2 months' history of back pain with progressively increasing weakness of both lower limbs. On examination, she had a performance status of ECOG-3 with moderate pallor and tenderness over the lumbo-sacral spine without any lymphadenopathy or organomegaly. Neurological examination revealed a power of 3/5 of both lower limbs with bilateral flexor type of planter reflex with features suggestive of bladder involvement without any sensory deficit. Baseline routine blood parameters showed anemia with hemoglobin of 6.5 g/dL, creatinine of 1.6 mg/dL, glomerular filtration rate of 27 mL/min, hypercalcemia with corrected calcium of 11 mg/dL, serum total protein of 7.1 g/dL, albumin of 2.6 g/dL, and globulin of 4.5 g/dL.
Contrast enhanced computed tomography scan of the thorax, abdomen, and pelvis showed multiple foci of osteolytic lesion in the S1 vertebrae and in the bilateral iliac bone along the sacroiliac joint [Figure 1]. With the above clinical and laboratory findings, the possibility of multiple myeloma was thought and planned for serum protein electrophoresis with immuno-fixation, skeletal survey and bone marrow aspiration, with trephine biopsy. Initial morphology of the marrow aspirate sample was suggestive of high-grade lymphoma.
|Figure 1: Contrast enhanced computed tomography scan of the thorax, abdomen, and pelvis showing multiple foci of osteolytic lesion in the S1 vertebrae and in the bilateral iliac bone along the sacroiliac joint|
Click here to view
Subsequently, bone marrow multi-parametric flow-cytometry revealed large (high forward scatter), monoclonal (kappa restricted) B cell population (red color). This population was CD45 bright and moderately positive for B cell markers (CD19, CD10, and CD20) and negative for CD5, CD23, CD11c, CD103, and CD25, which were consistent with a high-grade B cell lymphoma [Figure 2]. Bone marrow biopsy was suggestive of involvement by malignant lymphoma.
|Figure 2: Bone marrow multi-parametric flow-cytometry revealing large (high forward scatter), monoclonal (kappa restricted) B cell population (red color). This population is CD45 bright and moderately positive for B cell markers (CD19, CD10, and CD20) and negative for CD5, CD23, CD11c, CD103, and CD25; which are consistent with a high-grade B Cell lymphoma|
Click here to view
Bone marrow cytogenetic by fluorescence in situ hybridization (FISH) studies were positive for IgH/MYC fusion: t(8:14) (q24;q32) with standard positive signal pattern 2F1R1G in 30% of cells [Figure 3]a,[Figure 3]b,[Figure 3]c. Cerebrospinal fluid cytology was negative for lymphomatous involvement. She was treated with prephase chemotherapy (consisting of cyclophosphamide and dexamethasone) and then started on DA-REPOCH with a satisfactory clinical outcome.
|Figure 3: Bone marrow cytogenetics by fluorescence in situ hybridization (a) CMYC- interphase nuclei showing 1F1G1R signal pattern by using dual color break apart probe. (b) IgH – interphase nuclei showing 1F1G1R signal pattern by using dual color break apart probe. (c) Positive for IgH/MYC fusion: t(8;14)(q24;q32) with standard positive signal pattern 2F1R1G in 30% cells (Probe used, Vysis, Abott, USA, Metasystem, GmbH, Germany, Zytovision, GmbH, Germany)|
Click here to view
| Discussion and Conclusion|| |
Primary NHL of the bone generally occurs with an infiltrative and osteolytic pattern that affects mainly the femur, humerus, scapula, or tibia; axial skeleton is rarely involved. BL first described by Dennis Burkitt in 1958, is a highly aggressive NHL arising from the mature B cells. Endemic BL commonly involves the jaws and facial bones; sporadic BL presents with abdominal mass; and immunodeficiency-associated BL presents with nodal involvement.
Although there are few case series and case reports of primary BL of the appendicular skeleton, primary BL of the iliac bone and lumbosacral spine is rarely described.,, In a prospective study of 72 adults with BL, 55 patients had extranodal involvement, but only four had bone involvement. In a review of sixty cases of PBL, there was only one adult case with BL.
Although a definitive diagnosis was established by flowcytometry and confirmed through cytogenetic by FISH, this case highlights the diagnostic challenge when mimicked multiple myeloma along with a lytic lesion on imaging. Even with this aggressiveness, it is curable and highly chemosensitive. Therefore, early diagnosis and rapid initiation of treatment is essential. Our patient received prephase chemotherapy and then treated with DA-REPOCH, which includes rituximab, etoposide, vincristine, doxorubicin, cyclophosphamide, and prednisolone in addition to intrathecal methotrexate with growth factor support with satisfactory clinical outcome. This case is probably one of the rarely reported cases of primary BL involving the iliac bone and lumbo-sacral spine as the only site of disease.
To conclude, it is important that the treating oncologist be aware of this kind of atypical presentation of such an aggressive curable lymphoma so that without any diagnostic dilemma prompt treatment should be initiated. Definitive diagnosis requires flowcytometry and cytogenetics in suspected cases.
Written informed consent was obtained from the patient. The patient has given her consent for her images and other clinical information to be reported in the journal.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initial will not be published, and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
The authors would like to thank Mr. Anil Kumar Yadav, SO-C, Department of Cancer Cytogenetic, Homi Bhabha Cancer Hospital, Tata Memorial Centre, Varanasi, Uttar Pradesh.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Bhagavathi S, Fu K. Primary bone lymphoma. Arch Pathol Lab Med 2009;133:1868-71.
Jawad MU, Schneiderbauer MM, Min ES, Cheung MC, Koniaris LG, Scully SP. Primary lymphoma of bone in adult patients. Cancer 2010;116:871-9.
Bishop PC, Rao VK, Wilson WH. Burkitt's lymphoma: Molecular pathogenesis and treatment. Cancer Invest 2000;18:574-83.
Suresh TM, Babu S, Ganguly S, Babu KG, Lakshmaiah KC, Shankaranand B, et al
. Burkitt lymphoma of bone: A single institutional experience. Onc Gas Hep Rep 2014;3:23-5. [Full text]
Geetha N, Sreelesh KP, Nair RA, Jacob PM. Burkitt's lymphoma of the humerus. J Cancer Res Ther 2015;11:1025.
Cadavid L, Sastoque JM, Gutiérrez C, Yabur M, Molina G. Primary osseous Burkitt lymphoma with nodal and intracardiac metastases in a child. Radiol Case Rep 2017;12:185-90.
Diviné M, Casassus P, Koscielny S, Bosq J, Sebban C, Le Maignan C, et al
. Burkitt lymphoma in adults: A prospective study of 72 patients treated with an adapted pediatric LMB protocol. Ann Oncol 2005;16:1928-35.
Qureshi A, Ali A, Riaz N, Pervez S. Primary non-Hodgkin's lymphoma of bone: Experience of a decade. Indian J Pathol Microbiol 2010;53:267-70.
] [Full text]
[Figure 1], [Figure 2], [Figure 3]