ISSN 2997-1036
International Journal of Hematology | Vol. 9, No. 1, January 2018 | pp. 1–8
DOI: 10.46882/2018/IJH/000097
Case Report
Title: Acquired pure white cell aplasia associated with chronic large granular lymphocytic leukemia: Successful resolution with low-dose methotrexate
Names of Authors: K. L. Musa¹, M. N. Lawal², O. P. Dikko³
Authors’ Affiliations: ¹Department of Haematology, Federal Medical Centre, Katsina, Nigeria; ²Department of Medicine, Bayero University, Kano, Nigeria; ³Department of Pathology, Ahmadu Bello University Teaching Hospital, Zaria, Nigeria
Abstract: Acquired pure white cell aplasia is an exceptionally rare bone marrow failure syndrome characterized by an absolute absence of granulocytic precursors with preserved erythropoiesis and megakaryopoiesis. We report a 58-year-old female presenting with recurrent, severe soft tissue infections, a total white blood cell count of 0.8 × 10⁹/L, and an absolute neutrophil count of 0.0 × 10⁹/L lasting 4 weeks. Bone marrow aspirate and trephine biopsy examinations confirmed a complete lack of myeloid maturation past the myeloblast stage. Flow cytometric analysis of peripheral blood lymphocytes identified a clonal expansion of T-cell large granular lymphocytes co-expressing CD3, CD8, and CD57, confirming an underlying large granular lymphocytic leukemia. Immunomodulatory therapy utilizing low-dose oral methotrexate (10 mg weekly) was initiated. Granulopoietic recovery commenced by week 4, achieving sustained normalization of the absolute neutrophil count (2.4 × 10⁹/L) by month 3 of therapy. The patient remained infection-free and in complete hematological remission at a 12-month follow-up. This case demonstrates that low-dose methotrexate effectively suppresses the auto-reactive cytotoxic clones driving myeloid arrest.
Keywords: Pure white cell aplasia, large granular lymphocytic leukemia, agranulocytosis, methotrexate, marrow failure
Manuscript Timeline: Received: October 18, 2017; Revised: November 25, 2017; Accepted: December 14, 2017; Published: January 18, 2018