spontaneous remission of B-cell chronic lymphocytic leukemia associated with T lymphocytic hyperplasia in bone marrow

Abstract

Spontaneous remissions in B-cell chronic lymphocytic leukemia (B-CLL) are rare and none of them has been studied with immunophenotyping (by flow cytometry and immunohistochemistry) and genotyping. In this patient, studied after spontaneous remission had occurred, there was a residual T-lymphocytic hyperplasia in the bone marrow with a normal CD4:CD8 ratio. Absolute CD4 and CD8 counts and CD4:CD8 ratio in the peripheral blood were normal. Flow cytometry revealed no B-CLL cells in the peripheral blood and less than 2% B-CLL cells in the bone marrow.

Case Details

Disease Location

Bone marrowithblood

Personal Characteristics

83 -year-old male

Clinical Characteristics

In may 1992, the patient had evaluation of extensive bilateral cervical, axillary, and inguinal adenopathy (up to 2.0 cm in diameter) present since september 1991 wbc was 13,300/mm^3 with 59% mature lymphocytes inguinal lymph node biopsy revealed a diffuse well-differentiated b-cll (small lymphocytic lymphoma/cll) flow cytometry discllosed a monoclonal population of b lymphocytes positive for CD20 with coexpression of CD5 diagnosis of b-cll binet stage b, rai stage i was established the patient received follow ups every 1-3 months over the next several years, he remained asymptomatic after resolution of adenopathies, the absolute lymphocyte count gradually rose to 36,200/mm^3 in oct 1994 in aug and nov 1998, marrow aspirates showed a focal lymphoctyosis, the majority of the lymphocytes being small and mature with a minor component of small lymphocytes with irregular nuclei marrow core biopsy showed focal random and paratrabecular aggregates of small lymphoid cells comprising about 20% of the marrow ihc showed the lymphoid aggregates were primarily t lymphocytes with infrequent individually distributed b lymphocytes (t-lymphocyte hyperplasia) cell suspension immunophenotyping showed only a minor population (<2%) of monoclonal b lymphocytes indicative of residual b-cll, confirmed by genotyping with a faintly positive immunoglobulin heavy-chain gene rearrangement these cells showed a typical of b-cll with positivity for CD5, 23, 43, 19 expression greater than 20 and weak surface monoclonal lambda light chains t lymphocytes were polyclonal, t-cell receptor gene showed a germline configuration

Remission Characteristics

Bilateral cervical, axillary and inguinal adenopathy remained stable for several months then in 1993 the nodes started receding spontaneously and by march 1994 only a few small nodes (1 - 1.5cm in diameter) remained, limited to inguinalregions subsequently, bilateral inguinal adenopathy disappeared completely and CT in 1998 confirmed complete resolution of all adenopathy after the peak absolute lymphocyte count in oct 1994, it fell spontaneous to the range of 23,00 - 27,000/mm^3 where it remained for several months, then in jan 1996 the absolute lymphocyte count fell spontaneously again by aug 1998 was 1,100/mm^3 cytogenetic study on marrow in august 1998 were normal another study in nov 1998 showed loss of y chromosome in 4/20 metaphases (sign of aging in men) flow cytometry of peripheral blood in dec 1998 showed an absolute CD4 lymphocyte count of 740/mm^3 and an absolute CD8 lymphocyte count of 384/mm^3 with a normal 4:8 ration patient was seen in 2000 for a follow up and b-cll was still in remission, there was no palpable adenopaty or hepatosplenomegaly, absolute lymphocyte count was 1100/mm^3, lymphocytes also appeared small, mature, and normal in appearance

Treatment & Mechanisms

Proposed Remission Mechanisms

Possibility that the preservation of the CD4 helper cells prevented t-cell anergy toward the neoplastic b cells and contributed to the sr an imporightant remission-associated event was an expanded normal t-lymphocyte population in the bone marrow

Clinical Treatment

None reported

Non-Clinical Treatment

None reported