Hairy cell leukaemia (HCL) is a rare haematological malignancy characterised by abnormal B cell lymphocytes causing pancytopenia and splenomegaly. It is uncommon in the general population, accounting for 1-2% of all leukaemias. It is particularly rare in young females, therefore there are limited cases reported in the literature of HCL affecting pregnancy. This review will discuss two cases seen at a tertiary maternity hospital over the past four years. We will detail their presentation eventuating to diagnosis, the investigations carried out and the management during the antenatal, intrapartum and postpartum periods. Although rare, it is important to review these cases and the management initiated in order to optimise outcomes for future pregnancies affected by this disease.
First and second line treatment for HCL are medications that would usually be avoided in pregnancy: purine analogues and biological therapies. These are classified by the Australian Therapeutic Goods Administration as category D and C drugs respectively. Given these classifications, it is optimal to delay their use in pregnancy until after delivery has been achieved. A review of the literature revealed twelve articles relating to HCL in pregnancy. All detailed treatment with purine analogues, interferons and/or splenectomy. This case review is important as it avoided the use of potential teratogens during the antenatal period. Successful supportive therapy (e.g. blood transfusions and prophylactic antibiotics) was instead initiated, and achieved term delivery in both cases.
Patient A had a diagnosis of relapsing HCL at the time of pregnancy, and Patient B was first diagnosed during the second trimester. Although both patients had low total white cell counts (normal neutrophil counts) antenatally, only Patient B was initiated on oral prophylactic antibiotics. Both patients delivered at term: Patient A had an elective caesarean section and Patient B had a vaginal birth. The patients received differing antibiotic regimes at delivery: patient A received cefazolin and metronidazole compared to Patient B who received piperacillin/ tazobactam. The rationale for this management is unclear; both patients remained afebrile, no risk factors for peripartum infection were identified and their white cell counts remained stable. There does not appear to be a standardised protocol to guide antibiotic therapy in patients who are at risk of developing neutropenia in the antenatal, intrapartum or postpartum period. This is an area that requires further research in order to standardise antibiotic therapy. This would allow optimisation of patient care and ultimately improve maternal and fetal outcomes.