Gammadelta T-cell large granular lymphocyte (LGL) leukemia with spontaneous remission

Abstract

T-cell large granular lymphocyte (LGL) leukemia is a clonal disorder with an indolent clinical course. In July 1995, a 46-year-old Japanese man was admitted to our hospital because his anemia had progressed. He had a white blood cell count of 3.9 x 10(9)/L with 75% lymphocytes, which were intermediate to large and had almost round nuclei and azurophilic granules, and anemia with a red blood cell count (RBC) of 2.69 x 10(12)/L, hemoglobin (Hb) of 9.5 g/dL, and hematocrit (Hct) of 28.3%. Electron microscopic examination showed that most of the lymphocytes had a parallel tubular array and dense core granules in their cytoplasm. Flow cytometry and Southern blotting of the T-cell antigen receptor (TCR) genes using the peripheral blood species showed monoclonal proliferation of LGLs with a CD3+, TCRgammadelta+, CD4-, CD8-, CD16+, CD56-, CD57-, HLA-DR+ phenotype, and a TCR gamma gene rearrangement, respectively, suggesting that the patient was diagnosed as having gammadelta T-cell LGL leukemia. He had no symptoms, organomegaly, or skin lesions. About 1.5 years after diagnosis, the anemia gradually improved with disappearance and appearance of a rearranged band in the TCR-gamma gene and TCR-beta gene, respectively. About 7 years after diagnosis, the anemia improved completely with a RBC of 5.01 x 10(12)/L, Hb of 14.8 g/dL, and Hct of 44.3%, and he was in complete remission without TCR-beta and -gamma gene rearrangements. He had received no therapy. This is the first report of spontaneous remission of gammadelta T-cell LGL leukemia.

Case Details

Disease Location

Bone marrowithblood

Personal Characteristics

46 -year-old male, japanese

Clinical Characteristics

Referred in january 1994 for anemia evaluation slight anemia was found. He had no subjective symptoms in july 1995 he was admitted because anemia progressed, his wbc count was 3.9x10^9 with 75% lymphocytes, rbc was 2.69x10^12/l, hb was 9.5g/dl, hct was 28.3% pb blood lymphocytes were intermediate to large and had almost round nuclei and azurophilic granules electron microscope showed most of the lymphocytes had a parallel tubular array and dense core granules in the cytoplasm serum ldh was 504iu/l bm aspirate demonstrated infiltration of 33.2% lymphocytes with the same morphology described above flowcytometry of the pb mononuclear cells showed that 97.1% were positive for CD2, 3, tcr alpha beta, tcr gamma delta, CD4, 5, 7, 8, 16, 11b, 56, 57,and HLA-dr, suggesting leukemic cells had gamma delta t-cell phenotypes chromosomal analysis showed 46,xy (20/20) a rearranged band in the tcr-gamma chain gene was detected patient was diagnosed with gamma delta t-cell lgl leukemia patient was discharged in october 1995

Remission Characteristics

After discharge in oct 1995, the number of rbcs and concentration of hb improved gradually over about 4 years without therapy -- in april 1999, wbc was 3.3x10^9/l with 48% lymphocytes, rbc was 4.52x10^12/l, hb was 11.9g/dl, hct was 36.2% flow cytometry of pb monthsoculear cells showed a change of immunophenotype with a higher % of CD7, slightly higher CD8, lower CD16 and lower HLA-dr in january 1997 and april 1999, rearranged bands in the tcr-beta chain but not gamma gene were found there after, hematological parameters improved more july 2002, all blood counts were within normal limits, pb mononuclear cells showed improvement of the immunophenotype, including CD4/8 ratio (further values in article) both rearrangements bands for tcr-beta and gamma disappeared in july 2002 and january 2003 the patient remained asymptomatic duing the entire period complete sr 7 years after diagnosis anemia also gradually improved spontaneously as well

Treatment & Mechanisms

Proposed Remission Mechanisms

May have been associated with features of the cells themselves or with some change of immunological conditions

Clinical Treatment

None reported

Non-Clinical Treatment

None reported