Abstract:Aims
To report a series of acute lymphoblastic leukemia (ALL) cases with spontaneous remission and provide presenting clinical and pathologic information and details of clinical course to raise awareness among oncologists and patients.
Methods
We identified and analyzed nine patients with ALL and spontaneous remission. Review of literature reveals an additional nine previously reported cases with similar clinical course.
Results
All of these patients, ranging in age from 2 to 12 years of age, presented with in… Show more
“…SR; however, has been reported more commonly in children with AML, and the interval range to relapse is 0-16 months with a mean of seven months [13]. A review of the literature regarding SR in ALL suggests a similar mean time to relapse although the size of the studies is smaller [2][3][4][7][8][9][10]12]. Given that all reported SR cases resulted in a relapse, which worsens the prognosis of ALL, we contemplate it would be favorable to implement low-dose maintenance chemotherapy following SR such as methotrexate or 6-mercaptopurine.…”
Section: Discussionmentioning
confidence: 99%
“…In leukemia specifically, the presumed immunologic mechanism of cytokine storm stems from the recent or concurrent history of febrile illness with bacterial or viral septicemia. Cytokines, including TNF-a, IFN-g, and IL-2, have been demonstrated to have anti-tumor effects in vitro [ 8 ]. It has been proposed that fungal toll-like receptors (e.g., TLR2/TLR9), especially when compounded by a cytokine storm, may shift the immune system into an activated tumor microenvironment stimulating antileukemic effects.…”
Section: Discussionmentioning
confidence: 99%
“…fungal antigens, which may cross-react with antigens on leukemic blasts leading to SR [ 11 ]. Finally, hormonal involvement has been proposed as a mechanism of SR, as profound increases in adrenocorticotropic hormone (ACTH) and cortisol are known to be released as a stress response to septicemia, and exogenous corticosteroids have been shown to aid in remission of leukemia [ 8 ]. In our case, dexamethasone was used during induction although it was discontinued at the onset of her acute deterioration.…”
Spontaneous remission (SR) in acute lymphoblastic leukemia (ALL) is a poorly understood phenomenon that has been sporadically reported in medical literature for over a century, and the molecular and immunologic mechanisms of remission pose interesting clinical questions. Furthermore, the often-transient nature of these remissions poses a challenge to physicians in formulating an approach to treatment. We report on a rare case of
Candida tropicalis
sepsis in a three-year-old female with high-risk ALL who received less than two months of treatment prior to sepsis and subsequent SR.
“…SR; however, has been reported more commonly in children with AML, and the interval range to relapse is 0-16 months with a mean of seven months [13]. A review of the literature regarding SR in ALL suggests a similar mean time to relapse although the size of the studies is smaller [2][3][4][7][8][9][10]12]. Given that all reported SR cases resulted in a relapse, which worsens the prognosis of ALL, we contemplate it would be favorable to implement low-dose maintenance chemotherapy following SR such as methotrexate or 6-mercaptopurine.…”
Section: Discussionmentioning
confidence: 99%
“…In leukemia specifically, the presumed immunologic mechanism of cytokine storm stems from the recent or concurrent history of febrile illness with bacterial or viral septicemia. Cytokines, including TNF-a, IFN-g, and IL-2, have been demonstrated to have anti-tumor effects in vitro [ 8 ]. It has been proposed that fungal toll-like receptors (e.g., TLR2/TLR9), especially when compounded by a cytokine storm, may shift the immune system into an activated tumor microenvironment stimulating antileukemic effects.…”
Section: Discussionmentioning
confidence: 99%
“…fungal antigens, which may cross-react with antigens on leukemic blasts leading to SR [ 11 ]. Finally, hormonal involvement has been proposed as a mechanism of SR, as profound increases in adrenocorticotropic hormone (ACTH) and cortisol are known to be released as a stress response to septicemia, and exogenous corticosteroids have been shown to aid in remission of leukemia [ 8 ]. In our case, dexamethasone was used during induction although it was discontinued at the onset of her acute deterioration.…”
Spontaneous remission (SR) in acute lymphoblastic leukemia (ALL) is a poorly understood phenomenon that has been sporadically reported in medical literature for over a century, and the molecular and immunologic mechanisms of remission pose interesting clinical questions. Furthermore, the often-transient nature of these remissions poses a challenge to physicians in formulating an approach to treatment. We report on a rare case of
Candida tropicalis
sepsis in a three-year-old female with high-risk ALL who received less than two months of treatment prior to sepsis and subsequent SR.
“…Acute lymphoblastic leukemia (ALL) is the most common pediatric malignancy, accounting for approximately 25% of all cancers in children up to 15 years old. When left untreated, its natural history is that of uncontrolled proliferation of neoplastic lymphoblasts, resulting in decreased production of normal functional hematopoietic cells, ultimately leading to progressively worsening anemia, bleeding, infection, and fatal organ failure [1].…”
Spontaneous remission of acute lymphoblastic leukemia is an extremely rare phenomenon. It usually occurs in an aleukemic prodromic setting associated with cytopenias and fever. Nonetheless, this remission status, as far as we know, is always transient, the longest one de-scribed lasting one year. In this case report, we present a 25-months year-old boy with de novo bruises. His complete blood count displayed thrombocytopenia and leukopenia, and biochemistry analysis an increased lactate dehydrogenase. Although bone marrow aspirate was unremarkable, trephine biopsy was suggestive of B-cell acute lymphoblastic leukemia. Within three months, however, he showed a spontaneous clinical and analytical recovery. A repeated biopsy performed two years after was also completely normal. At the time of the writing of this paper, seven years later, the child remains free of progression. It might be the longest spontaneous remission ever described. Opposingly, some histology reports added a differential diagnosis - that the 24.49% immature B-cell infiltrate could, as well, resemble B-hematogones. Hence, we herein try to unveil the mechanism under this particular phenomenon. More efforts should be directed towards research about pre-acute lymphoblastic leukemia and spontaneous remission, so that pediatricians and pathologists can be more comfortable with these rare, but very challenging, situations.
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