Advanced Practice Provider/ Nurse Practitioner The University of Texas MD Anderson Cancer Center Houston, Texas, United States
BACKGROUND Burkitt’s Lymphoma (BL) is an aggressive B-cell non-Hodgkin’s lymphoma (NHL) characterized by a high proliferation rate and the dysregulation of the c-myc gene. BL during pregnancy is extremely rare and just 40 cases have been reported since 1900. Given its aggressive nature, it can be fatal if left untreated to both the mother and fetus. We present a case of a pregnant woman with stage II extranodal BL diagnosed at 16 weeks gestational age (GA), who received a Methotrexate (MTX) free therapy as a bridge to postpartum HD-MTX therapy.
CASE REPORT A 31-year-old woman diagnosed with BL at 16 weeks GA, confirmed by core needle Immunohistochemistry demonstrated neoplastic cells positive for CD43, CD79a, C-MYC ( >90%), and BCL6 and negative for CD3, CD34, BCL2, MUM1, TDT, and CyclinD1. Ki-67 proliferation index was 100%. Bone marrow aspiration and biopsy were negative for lymphoma, but positive for Epstein Barr Virus (EBV) by PCR. Due to pregnancy, patient underwent MRI whole body without gadolinium contrast for staging purposes. Baseline echocardiogram was normal. She had stage II disease, elevated lactate dehydrogenase (LDH) and B symptoms including drenching night sweats at diagnosis. Due to the teratogenic nature of MTX, patient was initiated on cyclophosphamide, vincristine, doxorubicin, and dexamethasone (HyperCVAD) part A for four cycles prior to scheduled induction at 30 weeks plus intrathecal chemoprophylaxis using cytarabine. Following cesarean section delivery, restaging via PET/CT demonstrated a complete metabolic response. She continued with HD MTX, rituximab, and high dose cytarabine (part B). In total, the patient received a total of 6 cycles of R-HyperCVAD Part A and B, and achieved a complete response by PET/CT. Currently, she is over 24 months post chemotherapy with no evidence of disease by imaging. Her daughter is now 2 years old and is happy and healthy.
BRIEF DISCUSSION There are several variants of BL including endemic, sporadic, and Human Immunodeficiency Virus-associated BL. The sporadic variant is most commonly seen in the United States and Western Europe. BL comprises 30 percent of pediatric lymphomas and < 1 percent of adult NHL in the United States. Standard treatment for BL is multiagent chemoimmunotherapy including HD (MTX), which is highly teratogenic. In this patient’s case, proper care coordination and monitoring was critical. Patient was seen by a maternal fetal medicine (MFM) physician with ultrasound prior to each dose of chemotherapy and was monitored up to 2-3 times with weekly lab draws. Advanced Practioners (AP’s) played an important role in our patient’s care and coordination.
CONCLUSIONS Treating and managing BL in a pregnant patient is rare, but is extremely challenging. Patients require proper education and risk versus benefit discussions prior to treatment as well as close monitoring by a multidisciplinary team of both mother and unborn child during treatment with multi-agent chemoimmunotherapy. Studies have demonstrated improved outcomes in pregnant patients when they receive upfront aggressive chemotherapy. However, providers must also balance the potential life-threatening effects of this disease while minimizing treatment's toxic effects to the fetus. AP’s play a critical role in this care coordination and managing of patient’s potential toxicities while undergoing therapy and in the post-partum period.