Introduction: Myelodysplastic syndrome (MDS) is a hematopoietic stem cell disorder characterized by pancytopenia in the peripheral blood due to ineffective hematopoiesis in the bone marrow. MDS is a disease of the elderly, with a median age at diagnosis of 70 years. Less than 10% of patients are younger than 50 years of age. MDS in pregnant patients is even rarer with only a few case reports published on the management of MDS in this patient population. We present a case of MDS which was successfully managed during pregnancy and delivery.
Case Report: A 27-year-old primigravid Chinese lady had her booking visit with the Obstetric team at UMMC at 27 weeks of gestation. She was diagnosed with Langerhans cell histiocytosis at the age of 5 and subsequently underwent chemotherapy. Patient was previously transfusion dependent from the age of 5 until 21. In 2011, bone marrow aspirate and trephine showed marked erythroid hyperplasia with relative suppression of granulopoiesis and megakaryocytosis. Flow cytometry immunophenotyping was suggestive of myelodysplasia. Patient was asymptomatic of anemia with no bleeding tendencies. At booking, hemoglobin was 8.4 g/dL and platelet 47. Patient was keen for spontaneous vaginal delivery following full discussion. In view of normal pregnancy progression and satisfactory fetal growth, transfusion was not indicated although regular blood investigations showed gradual exacerbation of her anemia and worsening thrombocytopenia. At 35 weeks hemoglobin and platelet were at levels of clinical concern (hemoglobin 7.9 g/dL, platelet 35) which prompted decision for induction of labour (IOL) to enable time controlled delivery. A multidisciplinary team meeting was held where the obstetricians, hematologists and anaesthetists decided on the management of labour; including a transfusion protocol, analgesia during active phase of labour, steps to prevent excessive blood loss at delivery and thromboprophylaxis. IOL was performed at 37 weeks. Once in established labour, hemoglobin and platelet levels were optimized through transfusion of blood products. A healthy baby girl weighing 2.7 kg was delivered via vacuum assisted vaginal delivery. Post delivery, patient received two units of platelets. Patient and her baby were discharged well day three post delivery with hemoglobin 8.1 g/dL and platelet 71.
Conclusion: The controversial effect of MDS on the outcome of pregnancy is not well described in the literature. This is further compounded by the lack of evidence based management guideline. Nonetheless, rigorous, supportive care should be provided by a multidisciplinary team. This includes vigilant monitoring of symptoms and blood investigations, transfusion of packed cell or platelet as clinically indicated and decision making on the timing and mode of delivery.