A Rare Case of Spontaneous Remission of Terminal Deoxynucleotidyl Transferase Negative B-acute Lymphoblastic Leukemia

Abstract

Spontaneous remission of untreated pediatric leukemia is an extremely rare occurrence. The underlying mechanism may be because of an immune-mediated process or increased cortisol production during stress or infection. We describe a rare case of terminal deoxynucleotidyl transferase negative B-acute lymphoblastic leukemia with concurrent infection that went into remission without treatment with chemotherapy or corticosteroids. Though B-acute lymphoblastic leukemia can rarely go into spontaneous remission, these patients require close follow-up as most patients will eventually develop recurrence.

Patient with Down Syndrome and relapsed acute lymphoblastic leukemia with sustained remission despite only partial British R3 chemotherapy

“…Spontaneous or transient remission of acute lymphoblastic leukemia during severe infection has been described in a few cases and case series reports with averaged ten weeks in duration [5][6][7]20 . The proposed mechanism was due to increased cortisol production during stress or infection combined with an immunemediated process thereby generating anti-leukemic effects 5,6,21 .…”

“…DS itself was proved to be an independent prognostic factor of outcome after ALL relapse. 4 There are several case reports of patients with leukemia who attained spontaneous remission after severe infection [5][6][7] . There are no reports of a patient with relapsed leukemia with sustained remission following cessation of therapy secondary to severe infections, except an older patient with relapsed FLT3 internal tandem duplication mutant acute myeloid leukemia whose disease underwent spontaneous remission for 35 days without precipitating cause 8 .…”

Patient with Down syndrome and relapsed acute lymphoblastic leukemia with sustained remission despite only partial R3 chemotherapy

“…6 There are several case reports of patients with leukemia who attained spontaneous remission after severe infection. [7][8][9] There are no reports of a patient with relapsed leukemia with sustained remission following cessation of therapy secondary to severe infections, except an older patient with relapsed FLT3 internal tandem duplication mutant acute myeloid leukemia whose disease underwent spontaneous remission for 35 days without precipitating cause. 10 We describe a case of a 37-year-old man with Down syndrome and ALL (DS-ALL) who had a late bone marrow relapse and sustained remission for 2 years after only receiving two cycles of ALL R3 salvage chemotherapy due to severe systemic infections.…”

“…23 Spontaneous or transient remission of acute lymphoblastic leukemia during severe infection has been described in a few cases and case series reports with averaged ten weeks in duration. [7][8][9]24 The proposed mechanism was due to increased cortisol production during stress or infection combined with an immunemediated process thereby generating anti-leukemic effects. 7,8,25 Cytokines, including tumor necrosis factor-alpha, interferonalpha, and interleukin-2, released during infection can directly inhibit the residual blast proliferation or through increased activity of T lymphocytes, macrophages, and natural killer cells leading to an anti-leukemia effect.…”

“…[7][8][9]24 The proposed mechanism was due to increased cortisol production during stress or infection combined with an immunemediated process thereby generating anti-leukemic effects. 7,8,25 Cytokines, including tumor necrosis factor-alpha, interferonalpha, and interleukin-2, released during infection can directly inhibit the residual blast proliferation or through increased activity of T lymphocytes, macrophages, and natural killer cells leading to an anti-leukemia effect. 26,27 Similar phenomena and mechanisms have been discussed and reported in several cases and case series in acute myeloid leukemia patients.…”