Mrs Tatiana Yanez1, Profesor Cristian Contreras1, Profesor Monica Theodor1, Profesor Patricio Rojas1
1Pontificia Universidad Católica De Chile, Santiago, Chile
Biography:
Mrs. Yañez, a postgraduate in Internal Medicine from Pontificia Universidad Católica of Chile, currently works as an Assistant Teacher in the department. She has 7 years of experience in hospitalist medicine and medical education. Recently, she joined the Obstetric team as a consultant
Abstract:
Hairy cell leukemia (HCL) is a rare B-cell disorder characterized by spleen enlargement, low blood counts, and leukemic cell infiltration in the bone marrow, liver, and spleen. Diagnosis relies on identifying "hairy" lymphocytes via immunohistochemistry and flow cytometry. Treatment typically involves purine analogs like cladribine.
During pregnancy, HCL is uncommon and presents significant management challenges. Immune-mediated thrombocytopenia (PTI) is one of the clinical manifestations associated with HCL. Treatment options for PTI during pregnancy include corticosteroids, rituximab, and immunoglobulins. Although splenectomy is considered relatively safe in the second trimester and has been performed in pregnant patients with HCL, some cases may require additional treatments after delivery.
A 36-year-old primiparous woman at 24 weeks of gestation presented with dizziness and epistaxis, revealing severe thrombocytopenia (platelet count: 29,000/μL) and hepatosplenomegaly via CT scan. After excluding infectious and rheumatologic causes, a hematologic evaluation confirmed hairy cell leukemia and secondary immune-mediated purpura (PTI). Initial treatments included corticosteroids, transfusion support, and immunoglobulin therapy, which was unsuccessful in raising platelet levels. Treatment was escalated to weekly rituximab for four weeks and a thrombopoietin receptor agonist for seven days up to delivery. Despite therapy, her condition worsened with persistent severe thrombocytopenia and increased splenomegaly, leading to a decision for surgical intervention. A splenectomy followed by a Cesarean section resulted in the delivery of a healthy newborn, improving the mother's platelet count to 78,000/μL. Post-delivery, the patient received cladribine chemotherapy for hairy cell leukemia but developed complications including cytomegalovirus reactivation and Pseudomonas aeruginosa bacteremia, which were managed effectively. She was discharged to continue outpatient chemotherapy treatment.
Managing hairy cell leukemia (HCL) during pregnancy requires a multidisciplinary approach to balance treatment efficacy and safety for both mother and fetus, emphasizing the need for further research.
Keywords
hairy cell, analogs purine, splenectomy, thrombocytopenia.
References
1. Kreitman, Robert J."Hairy Cell Leukemia: Present and Future Directions".Leukemia & Lymphoma.2019;60(12):286979. https://doi.org/10.1080/10428194.2019.1608536.
2.Daver N, Nazha A, Kantarjian HM, Haltom R, Ravandi F. Treatment of hairy cell leukemia during pregnancy: Are purine analogues and rituximab viable therapeutic options. Clin Lymphoma Myeloma Leuk [Internet]. 2013;13(1):86–9. Disponible en: http://dx.doi.org/10.1016/j.clml.2012.06.009
3. Mendez-Hernandez A, Moturi K, Hanson V, Andritsos LA. Hairy cell leukemia: Where are we in 2023? Curr Oncol Rep [Internet]. 2023;25(8):833–40. Disponible en: http://dx.doi.org/10.1007/s11912-023-01419-z