The Zebra Files: Adult T-Cell Leukemia-Lymphoma During Pregnancy

Each week we present to you a rare medical case that defies common diagnosis and procedure.

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Adult T-Cell Leukemia-Lymphoma During Pregnancy

1. Introduction

Adult T-cell leukemia-lymphoma (ATL) is an uncommon lymphoid malignancy associated with human T-cell lymphotropic virus type 1 (HTLV-1) infection. HTLV-1 infection is endemic in Japan and Caribbean basin and occurs sporadically in Africa, Central and South America, Middle East, and southeastern United States. The global seroprevalence of HTLV-1 is 4.4% [1], with higher prevalence in endemic areas. ATL develops in 2%–4% of HTLV-1 infected people [2]. There were only 3 previously reported cases of ATL during pregnancy based on the reporters’ comprehensive review of the literature [3–5]. We report a case of a Jamaican female diagnosed with ATL during pregnancy.

2. Case Report

A 27-year-old female Jamaican immigrant, at 28 weeks of age of gestation, presented with 1-week history of progressive confusion, poor appetite, vomiting, and joint pains. During admission, she was noted to have small nodular lesions around the mouth and neck, multiple bilateral tender cervical lymphadenopathy, multiple areas of tenderness on her right shoulder and ribs, and a gravid abdomen. Pertinent laboratory findings include leukocytosis (13,900/mL3), hypercalcemia (18.4 mg/dL), elevated ionized calcium (11.04 mg/dL), normal serum phosphorus (2.6 mg/dL), and elevated PTH- related protein (46 pg/mL) (see Table 1 for full discussion). HIV antibody test was negative. Chest radiograph revealed diffuse lytic lesions and fractures in the clavicles and ribs (Figure 1).
Liberal hydration with intravenous normal saline was started and patient was given intravenous furosemide for hypercalcemia. Cautious emergent hemodialysis was instituted in the ICU to further control hypercalcemia. However, serum calcium remained elevated. Thus, intravenous calcitonin was started. Core biopsy of the largest cervical lymph node revealed intermediate to large peripheral T cells with immunophenotype classic for HTLV-1 lymphoproliferative disorder (Figure 2). Peripheral blood showed occasional atypical polylobulated T-lymphocytes. Qualitative HTLV-1 DNA test was positive. Screening test for HTLV-1 antibodies was also positive. This was subsequently confirmed by Qualitative HTLV-1 Line Immunoassay. Intravenous allopurinol was started because of subsequent hyperuricemia. In order to initiate chemotherapy promptly, it was decided through a multispecialty meeting to deliver the baby via cesarean section. Betamethasone was not given before delivery due to a high risk of tumor lysis. She gave birth to a baby girl, weighing 1280 grams, with an Apgar score of 1/2/3, who was admitted to the neonatal ICU. Following delivery, the patient received intravenous zoledronic acid and rasburicase for hypercalcemia and hyperuricemia, respectively. Fluoroscopy-guided lumbar puncture yielded cerebrospinal fluid that was positive for lymphoma cells. MRI of the brain revealed scattered subcortical T2-hyperintense foci and enhancing nodules in the calvarium, consistent with metastatic disease (Figure 3).

Bone marrow biopsy and aspirate did not show evidence of involvement. The patient was started on E-CHOP (etoposide, cyclophosphamide, hydroxydaunorubicin, oncovin, and prednisone) chemotherapy. She also underwent Ommaya reservoir placement for intrathecal methotrexate. Her prolonged hospital course was complicated by deep venous thrombosis, central line-associated Enterococcus faecalis bacteremia, catheter-related fungal uri- nary tract infection, neutropenic fever, and central diabetes insipidus. These complications were treated accordingly and patient was discharged after 4 weeks of hospital stay. She will complete a total of six cycles of E-CHOP, as well as weekly intrathecal methotrexate. She will be referred for hematopoietic stem cell transplantation upon completion of chemotherapy.