A portion of expanded granular lymphocytes cause pure white cell aplasia?
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Pure white cell aplasia (PWCA) is a rare disorder characterized by selective hematopoietic failure of both granulocytic and monocytic lineages . Herein we report a case with T cell granular lymphocyte (GL)-proliferative disorders (T-GLPD)-associated PWCA, which acquired prolonged remission from PWCA in spite of that GL counts had no apparent change before and after receiving alemtuzumab therapy.
The patient initially received immunosuppressive therapy including cyclosporine A and rabbit anti-thymocyte globulin. Absolute neutrophil count (ANC) was elevated to 1.49 × 109/L 3 months after receiving the therapy. Shortly thereafter, the neutrophil and monocyte counts were gradually decreased below 0.5 × 109/L. CD5− and CD5low CTLs reappeared, suggesting the recurrence of PWCA (Fig. 1a). The patient next received oral cyclophosphamide, while the pathogenetic cell populations failed to be eradicated (Fig. 1a). She further received low-dose alemtuzumab therapy. After a 3 mg initial dose, alemtuzumab was administered intravenously at 10 mg once a week, resulting in a total dose of 163 mg. Again, rapid recovery of the ANC was observed concomitantly with the disappearance of CD5− and CD5low CTLs (Fig. 1a). Although Southern blot analysis of TCR Jγ gene showed no clonal bands, the Cβ gene rearranged bands continued to be detected even 1 year after receiving the alemtuzumab therapy (Fig. 1b). The patient remained well without the recurrence of PWCA in spite of that GL fractions accounted for around 30% of lymphocytes.
The prolonged resolution of PWCA with disappearance of CD5− and CD5low effector CTLs suggests the cytotoxicity-related etiology. Even though all clones failed to be eradicated, cytopenia was resolved when the pathogenetic CTLs disappeared after the alemtuzumab therapy. Heterogeneity in clonal expanded GLs suggests that the present case has a reactive condition rather than leukemia-like neoplasms showing homogeneous cell populations. It may be appropriate to consider T-GLPD and the true neoplastic leukemia separately in T-LGLL.
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Conflict of interest
The authors declare that they have no conflict of interest.
Informed consent was obtained from the patient.
- 2.Jerez A, Clemente MJ, Makishima H, Koskela H, LeBlanc F, Ng KP et al (2012) STAT3 mutations unify the pathogenesis of chronic lymphoproliferative disorders of NK cells and T-cell large granular lymphocyte leukemia. Blood 120:3048–3057. https://doi.org/10.1182/blood-2012-06-435297 CrossRefPubMedPubMedCentralGoogle Scholar
- 3.Chan WC, Foucar K, Morice WG, Catovsky D (2017) T-cell large granular lymphocytic leukemia. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H et al (eds). WHO classification of tumours of haematopoietic and lymphoid tissues. Revised 4th edn, Vol. 2. IARC Press, Lyon, FR, pp 348–350Google Scholar
- 5.Risitano AM, Selleri C, Serio B, Torelli GF, Kulagin A, Maury S, Halter J, Gupta V, Bacigalupo A, Sociè G, Tichelli A, Schrezenmeier H, Marsh J, Passweg J, Rotoli B, on behalf of the Working Party Severe Aplastic Anaemia (WPSAA) of the European Group for Blood and Marrow Transplantation (EBMT) (2010) Alemtuzumab is safe and effective as immunosuppressive treatment for aplastic anaemia and single-lineage marrow failure: a pilot study and a survey from the EBMT WPSAA. Br J Haematol 148:791–796. https://doi.org/10.1111/j.1365-2141.2009.08027.x CrossRefPubMedPubMedCentralGoogle Scholar
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