TY - JOUR
T1 - Subsequent development of diffuse large B-cell lymphomas and hodgkin lymphoma associated with primary immune disorder in a 6-year-old female
T2 - A case report and review of the literature
AU - Bautista-Quach, Marnelli A.
AU - Bedros, Antranik
AU - Wang, Jun
N1 - J Pediatr Hematol Oncol. 2011 Oct;33(7):e320-5. doi: 10.1097/MPH.0b013e318207a37a. Case Reports; Review
PY - 2011/10
Y1 - 2011/10
N2 - Neoplastic lymphoid proliferation may arise from immune deficiency or disordered regulation of the immune system. Often the neoplasms are associated with viral agents, such as Epstein-Barr virus, human immunodeficiency virus, or human herpes virus 8. Lymphoproliferative diseases have been documented in a variety of primary immune disorders. The most commonly encountered neoplastic lesion is diffuse large B-cell lymphoma (DLBCL), although Hodgkin lymphoma (HL), Burkitt lymphoma, and peripheral T-cell lymphomas and/or leukemias have also been documented in rare instances. We report a case of a 6-year-old girl with unclassifiable primary immunodeficiency diagnosed with 2 different clones of DLBCLs and subsequently developed lymphocyte-depleted, classical HL. Both neoplasms were associated with Epstein-Barr virus. To the best of our knowledge, this is the first reported occurrence of primary immune disorder-associated lymphoproliferative disease with sequential development of DLBCLs and HL in a pediatric patient. Thorough surveillance is paramount for accurate assessment of the associated lymphoproliferative disease and in ascertaining likely transformation to, or de novo evolution of a different lymphoid neoplasm. This is also important in evaluating treatment response with appropriate therapeutic adjustments if clinically indicated.
AB - Neoplastic lymphoid proliferation may arise from immune deficiency or disordered regulation of the immune system. Often the neoplasms are associated with viral agents, such as Epstein-Barr virus, human immunodeficiency virus, or human herpes virus 8. Lymphoproliferative diseases have been documented in a variety of primary immune disorders. The most commonly encountered neoplastic lesion is diffuse large B-cell lymphoma (DLBCL), although Hodgkin lymphoma (HL), Burkitt lymphoma, and peripheral T-cell lymphomas and/or leukemias have also been documented in rare instances. We report a case of a 6-year-old girl with unclassifiable primary immunodeficiency diagnosed with 2 different clones of DLBCLs and subsequently developed lymphocyte-depleted, classical HL. Both neoplasms were associated with Epstein-Barr virus. To the best of our knowledge, this is the first reported occurrence of primary immune disorder-associated lymphoproliferative disease with sequential development of DLBCLs and HL in a pediatric patient. Thorough surveillance is paramount for accurate assessment of the associated lymphoproliferative disease and in ascertaining likely transformation to, or de novo evolution of a different lymphoid neoplasm. This is also important in evaluating treatment response with appropriate therapeutic adjustments if clinically indicated.
KW - Epstein-Barr virus
KW - Hodgkin lymphoma
KW - diffuse large B-cell lymphoma
KW - lymphoproliferative diseases
KW - primary immune disorder
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U2 - 10.1097/MPH.0b013e318207a37a
DO - 10.1097/MPH.0b013e318207a37a
M3 - Review article
C2 - 21572347
SN - 1077-4114
VL - 33
SP - e320-e325
JO - Journal of Pediatric Hematology/Oncology
JF - Journal of Pediatric Hematology/Oncology
IS - 7
ER -