T-prolymphocytic Leukaemia With Spontaneous Remission
Shichishima, T., Kawaguchi, M., Ono, N., Oshimi, K., Nakamura, N., & Maruyama, Y. (2004). Gammadelta T-cell large granular lymphocyte (LGL) leukemia with spontaneous remission. American journal of hematology, 75(3), 168–172. https://doi.org/10.1002/ajh.10480
Abstract
T‐prolymphocytic leukaemia (T‐PLL) is a rare dis‐order with a poor prognosis. A 69‐year‐old man was diagnosed as having a small‐cell variant of T‐PLL according to the French–American–British classification by haematological, immunological and ultrastructural studies, although the cells had a CD7 − phenotype and no chromosomal abnormality. He had no symptoms or organomegaly. The number of his lymphocytes, 53.7 × 10 9 /l at the time of diagnosis, gradually decreased without therapy, and he was in complete remission 39 months later. A rearranged band in the T‐cell antigen receptor‐β gene, which was detected at the time of diagnosis, decreased or disappeared. This is the first report of a T‐PLL case with spontaneous complete remission.
Case Details
Disease Location
Bone marrowithblood
Personal Characteristics
69 -year-old male
Clinical Characteristics
Referred in oct 1993 for evaluation of leucocytosis following erupatiention wbc count was 63.9x10^9/l with 85% lymphocytes, peripheral blood lymphocytes were small and had round nuclei with indistinct or invisible nucleoli electron microscope exam showed most of lymphocytes had a distinct nucleolus, but appeared somewhat smaller than those in typical t-pll cells bone marrow aspirate demonstrated infiltration of 83% lymphocytes with the same morphology described above flow cytometry of the marrow mononuclear cells showed that 87.1% were positive for CD2, 3, 4, 7, 8, 19, and HLA-dr chromosomal analysis showed 46, xy (20/20) a rearranged band in the t-cell antigen receptor beta gene was detected patient was diagnosed with having a small-cell variant of t-pll
Remission Characteristics
Lymphocytosis persisted, but the number of lymphocytes gradually decreased over about 2 years without therapy and thereafter remained <5x10^9/l bm aspirate in feb 1997 showed a normal myelogram, with normal pb cell counts, flow cytometry of bm mononuclear cells showed improvement of the immunophenotype (values in article) immunological remission including normalization of percentage CD7+ cells and the CD4/8 ratio was confirmed in pb the rearrangement bands of the tcr-beta gene in the pb and/or marrow had decreased or disappeared between september 1996 and may 1998, during this period, the patient remained asymptomatic complete sr about 39 months after diagnosis, which continued
Treatment & Mechanisms
Proposed Remission Mechanisms
May be due to unusual features of cells i.e. No CD7+ phenotype or chromosome abnormality including structural abnormalities of 14q
Clinical Treatment
None reported
Non-Clinical Treatment
None reported