A 24-year-old pregnant woman presented with paralysis and prickling pain in her head and neck, headache and aching pain throughout her body. The patient's previous history included an appendectomy, a successful pregnancy 2 years previously and an induced abortion 8 months previously. At the time of presentation, she was 36-weeks pregnant. She was pale, with sternal tenderness and lymphadenopathy of her neck and axillae; her WBC and platelets were 100 000 and 44 000/μL, respectively, and her BM had 67% blasts. A diagnosis of B-lineage ALL was made. She received immediate dosage of dexamethasone (7 mg) to relieve the tumor burden. A healthy preterm male weighting 2000 g was delivered successfully the night she was admitted. After delivery, she developed fever and had paralysis of the intestine. She was diagnosed with a pulmonary infection (complicated by intestinal obstruction), which was confirmed by computerized tomographic scanning. Following 7 days after delivery, a successful partial enterectomy was carried out after leukapheresis, temporary CY (CTX) intervention and continued dexamethasone therapy. The patient recovered rapidly following antibiotics and nutritional support. Two weeks after delivery, she received additional combination chemotherapy consisting of vindesine and 80 mg of DNR. She achieved hematological CR 25 days after delivery, after which prophylactic intrathecal chemotherapy was given. Two weeks later, she was readmitted for recurrence of bone ache and prickling pain. Leukemia relapse was confirmed by the discovery of 86.5% blast cells in a BM aspirate. Cytogenetic analysis by G-banding showed 46, XX, der(19) t(1;19)(q23;p13) in six metaphases. TCF3-PBX1 transcripts were detected by reverse transcription PCR. The patient received an allogeneic BMT (allo-BMT) following successful salvage re-induction chemotherapy consisting of mitoxantrone, vindesine and dexamethasone (from a fully matched sibling donor). The conditioning regimen was composed of BU and CTX but not TBI. GVHD prophylaxis was given consisting of CsA and short-term MTX. One month after BMT, the engraftment was checked by BM aspiration and biopsy analysis, but leukemic cells were not detectable. The post-SCT course was complicated by moderate cutaneous and gastrointestinal GVHD, which was treated with methylprednisolone and low-dose CTX. About 280 days after BMT, an isolated hard painless mass with a 4-cm diameter in her left breast was found. Ultrasound of the breast showed two low echogenic areas with abundant blood flow, located around the left nipple. A radical left mastocarcinoma was performed 293 days after BMT. Two dark red masses were removed and submitted for pathology. Immunohistochemistry of a paraffin-embedded specimen showed positive staining of CD10, CD43, CD79a, scattered detection of CD3, and no myeloperoxidase. The TCF3–PBX1 fusion gene was detected by FISH in interphase tumor cells. Extramedullary relapse (EMR) was diagnosed. Her BM remained in remission during the course of treatment. The patient was given palliative local radiotherapy and was then lost to follow-up.
ALL rarely occurs during pregnancy, and the successful delivery of a healthy neonate is extremely challenging. To the best of our knowledge, this is the first reported case of extramedullary tumors (EMT) after allo-BMT in a postpartum woman with ALL. Treatment of leukemia in pregnancy is challenging. Obstetric complications such as iatrogenic and spontaneous preterm delivery can be further exacerbated by treatment-related fetal myelosuppression, especially if close to the time of delivery.1 We commenced the dexamethasone therapy without additional cytotoxic drugs. Once delivery ended, integrated individualized therapy including cytotoxic drugs, nutritional and anti-infective drugs should be given promptly. Many authors reported satisfactory remission rates when cytotoxic chemotherapy was given after delivery.2 Allogeneic hematopoietic SCT (allo-HSCT) is susceptible to a growing relapse risk of delayed chemotherapy due to pregnancy. Chelghoum et al.3 surveyed a series of 37 patients with a diagnosis of acute leukemia during pregnancy, with 3 ALL cases diagnosed in the third trimester. There were 13 normal deliveries, and 15 of 23 healthy babies had been exposed to chemotherapeutic agents. CR was achieved in 34 patients. Five patients underwent post remission allo-HSCT. Patients who enrolled in allo-HSCT were still in remission at a median follow-up of 3.4 years. Isolated EMR of ALL post-allo-HSCT is a rare occurrence. Weak GVL effect in a localized area might facilitate EMR. Diagnosis of focal leukemic lesion should be confirmed by pathological examination. For the diagnosis of isolated EMR, FISH was very helpful for those patients with recurrent cytogenetic abnormalities when first diagnosed.4 To date, an optimal treatment has not yet been established. Isolated relapse may be well controlled with radiation therapy5 and the prognosis of it may be better than that of BM relapse.
This is a preview of subscription content, access via your institution
