Acute promyelocytic leukemia (APL) is a clinically distinct and rare type of acute myeloid leukemia and represents an oncologic emergency. Even rarer is the incidence of APL in pregnancy with less than 60 cases described in the literature. A 33-year-old pregnant female at 34 week gestation presented to hospital with reports of abdominal pain. On admission she was found to have acute onset pancytopenia with a WBC count of 1.2, Hemoglobin of 9.7g/dl, and platelet count of 26000. Initial history, exam, and investigations including a peripheral smear, coagulation panel, liver function, vitamin b12 and folate levels did not reveal possible etiology of pancytopenia. Given worsening pancytopenia, bone marrow biopsy was done which showed 58% promyelocytes and 11% blasts with numerous Auer rods present. Cytogenetics showed abnormal female karyotype with t(15:17) and FISH analysis revealed PML/RARA fusion in 76.5% of analyzed cells. The above findings were diagnostic of APL. After multidisciplinary discussion with high risk obstetrics physician, it was decided to immediately induce labor for immediate initiation of treatment of APL. She had a prolonged labor requiring aggressive blood product support and initiation of All trans retinoic acid (ATRA) before delivery given concerns of coagulopathy. Induction treatment with Arsenic trioxide (ATO) was started the day after her delivery. Repeat bone marrow biopsy on day 24 showed complete morphologic remission. Shortly thereafter, she started cycle 1 of consolidation with ATRA and arsenic trioxide. APL is characterized by a translocation between chromosome 15 and 17. Coagulopathy is a pathognomonic feature of this leukemia and often the reason for high mortality in early course of disease. APL when treated with ATRA and ATO, has excellent remission rate and 99% overall survival at 2 years. APL in pregnancy is associated with increased risk of preterm delivery, perinatal mortality, and miscarriage. Following pregnancy, there is an increased risk of bleeding, infection, or placental abruption. ATRA, one of the pillars around which treatment of APL revolves, is highly teratogenic during the first trimester and has low risk later in pregnancy. Treatment is directed by the trimester of pregnancy. Termination of pregnancy or treatment with single agent conventional chemotherapy is preferred in the first trimester whereas treatment with ATRA prior to delivery and use of chemotherapy after delivery is the preferred approach in the 2nd and 3rd trimester. This case is an example of individualized approach with a multidisciplinary team need in the setting of scarce data.
| Identifer | oai:union.ndltd.org:ETSU/oai:dc.etsu.edu:asrf-1699 |
| Date | 28 April 2020 |
| Creators | Franklyn, Lindsey, Mhadgut, Hemendra, Sinha, Alok, Singal, Sakshi |
| Publisher | Digital Commons @ East Tennessee State University |
| Source Sets | East Tennessee State University |
| Detected Language | English |
| Type | text |
| Source | Appalachian Student Research Forum |
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