Ann Lab Med.  2012 Mar;32(2):162-166. 10.3343/alm.2012.32.2.162.

A Case of B-cell Lymphoma, Unclassifiable, with Features Intermediate between Diffuse Large B-cell Lymphoma and Burkitt Lymphoma in a Korean Child

• Ahn JY
• Seo YH
• Park PW
• Kim KH
• Park MJ
• Jeong JH
• Park SH
• Song YH

• KMID: 1245258
• DOI: http://doi.org/10.3343/alm.2012.32.2.162

Abstract

B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma (BL) (intermediate DLBCL/BL), is a heterogeneous group with some features resembling DLBCL and others resembling BL. Here, we report a case of intermediate DLBCL/BL in a Korean child. A 2-yr-old male was admitted for evaluation and management of left hip pain. Immunohistochemistry of a biopsy of the femur neck revealed tumor cells positive for CD20, CD10, BCL2, BCL6, and Ki67. A bone marrow (BM) aspirate smear revealed that 49.3% of all nucleated cells were abnormal lymphoid cells, composed of large- and medium-sized cells. Immunophenotyping of the neoplastic cells revealed positivity for CD19, CD10, CD20, and sIg lambda and negativity for CD34, Tdt, and myeloperoxidase (MPO). Cytogenetic and FISH analyses showed a complex karyotype, including t(8;14)(q24.1;q32) and IGH-MYC fusion. Intensive chemotherapy was initiated, including prednisone, vincristine, L-asparaginase, daunorubicin, and central nervous system prophylaxis with intrathecal methotrexate (MTX) and cytarabine. One month after the initial diagnosis, BM examination revealed the persistent of abnormal lymphoid cells; cerebrospinal fluid cytology, including cytospin, showed atypical lymphoid cells. The patient was treated again with cyclophosphamide, vincristine, prednisone, adriamycin, MTX, and intrathecal MTX and cytarabine. The patient died of sepsis 5 months after the second round of chemotherapy.

Keyword

Diffuse large B-cell lymphoma; Burkitt lymphoma; Gray zone lymphoma

MeSH Terms

Antineoplastic Agents/therapeutic use
Bone Marrow Cells/pathology
Child, Preschool
Chromosomes, Human, Pair 14
Chromosomes, Human, Pair 8
Cyclophosphamide/therapeutic use
Doxorubicin/therapeutic use
Drug Therapy, Combination
Femur Neck/pathology
Humans
Immunohistochemistry
Immunophenotyping
In Situ Hybridization, Fluorescence
Karyotyping
Lymphoma, B-Cell/*diagnosis/drug therapy
Male
Methotrexate/therapeutic use
Oncogene Proteins, Fusion/genetics
Prednisolone/therapeutic use
Republic of Korea
Translocation, Genetic
Treatment Outcome
Vincristine/therapeutic use

Figure

• 

  Fig. 1 Immunohistochemistry shows positivity for BCL2 (A), BCL6 (B), CD10 (C), and Ki67 (D) of tumor cells (Femur neck, immunohistochemical stains, 400× magnification).

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  Fig. 2 Bone marrow smear (A, Wright-Giemsa stain, 1,000× magnification) and biopsy (B, H&E stain, 1,000× magnification) reveal abnormal lymphoid cells composed of large and medium sized cells. Large neoplastic cells showed irregular nuclei with 1-2 distinct nucleoli and abundant deeply basophilic cytoplasm. Medium-sized cells showed round nuclei with 1-4 prominent nucleoli and had scantly to moderately basophilic cytoplasm with some vacuoles.

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  Fig. 3 Giemsa-trypsin banding showed the following karyotypes: 46,XY,t(8;14)(q24.1;q32),del(11)(q13),dup(11)(q22q13),der(17)del(17)(p12)t(1;17)(q21;q25)[29]/46,idem,t(12;19)(q13;p13.2)[4]/46,idem,add(19))(p11)[4]/46,idem,add(13)(q34)[3].

• 

  Fig. 4 FISH analysis using Vysis LSI IGH/MYC, CEP 8 tri-color, dual fusion translocation probe reveals IGH-MYC rearrangement with 2 fusions (IGH-MYC fusions on der(8)t(8;14) and der(14)t(8;14)), 1 orange (native MYC), 1 green (native IGH), and 2 aqua (D8Z2) signals.

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