Abstract
Double hit B-cell lymphomas are rare tumors that are defined by a chromosomal breakpoint affecting the MYC/8q24 locus in combination with another recurrent breakpoint, mainly a t(14;18)(q32;q21) involving BCL2. These tumors mostly occur in adults and carry a very poor prognosis. Double hit lymphomas can occur de novo, or arise from transformation of follicular lymphoma. We report a case of a 69-year-old female with abdominal distention and progressively worsening weakness over six months. Patient presented with severe hypercalcemia and multiple intra-abdominal/pelvic masses. Histopathology results of the abdominal mass were compatible with a double hit B-cell lymphoma. However, bone marrow biopsy results showed a low grade follicular lymphoma, thus suggesting peripheral transformation of follicular lymphoma to double hit B-cell lymphoma. Patient was transferred to a tertiary care center and was started on combination chemotherapy (EPOCH: doxorubicin, etoposide, vincristine, cyclophosphamide, and prednisone). Our paper highlights not only transformation of follicular lymphoma to double hit B-cell lymphoma and the challenges encountered in diagnosing and treating these aggressive tumors, but also the association of new onset/worsening hypercalcemia in such patients.
Highlights
Double hit B-cell lymphomas are rare tumors that are defined by a chromosomal breakpoint affecting the MYC/8q24 locus in combination with another recurrent breakpoint, mainly a t(14;18)(q32;q21) involving BCL2
Double hit B-cell lymphomas usually show a poor response to standard chemotherapy regimens, otherwise used for treating B-cell lymphomas
Parker et al reported two cases of Double hit lymphomas (DHLs) successfully treated with aggressive immunochemotherapy followed by autologous stem cell transplantation and radiation therapy [24]
Summary
Double hit B-cell lymphomas are rare tumors that are defined by a chromosomal breakpoint affecting the MYC/8q24 locus in combination with another recurrent breakpoint, mainly a t(14;18)(q32;q21) involving BCL2. The partner of BCL2/18q21 breakpoint mostly is the IGH locus at 14q32, and in some cases a t(8;14;18) may be present. Double hit B-cell lymphomas can arise either de novo or from transformation of follicular lymphoma. More than half of the patients present with widespread, often extranodal disease. Patients usually present with poor prognostic factors such as elevated LDH, bone marrow/CNS involvement, and a high international prognostic index score. Double hit B-cell lymphomas usually show a poor response to standard chemotherapy regimens, otherwise used for treating B-cell lymphomas
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