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Hematologic And Molecular Spontaneous Remission Following Sepsis In Acute Monoblastic Leukemia With Translocation (9;11): A Case Report And Review Of The Literature

Müller et al., 2004Leukemia

Müller, C. I., Trepel, M., Kunzmann, R., Lais, A., Engelhardt, R., & Lübberight, M. (2004). Hematologic and molecular spontaneous remission following sepsis in acute monoblastic leukemia with translocation (9;11): a case report and review of the literature. European journal of haematology, 73(1), 62–66. https://doi.org/10.1111/j.1600-0609.2004.00248.x

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Abstract

Spontaneous remission in patients with acute myeloid leukemia (AML) is a rarely reported phenomenon of usually short duration. The etiology remains unclear, but an association with preceding blood transfusions or bacterial infections has been reported. Triggered immune responses are suggested to play a potential role in the development of spontaneous remission. Acute monocytic leukemia was diagnosed in a 61-yr-old male patient. Cytogenetic analysis revealed a sole translocation (9;11) (q22;q23) and RT-PCR the MLL/AF9 fusion gene. As a result of the patient's reduced performance status and septic condition, cytostatic therapy was withheld. No microorganisms could be detected. Hematologic and molecular remission occurred after initiating antibiotic therapy without any cytostatic treatment; 29 months after the initial diagnosis, he is in complete remission, and excellent physical condition. Our report includes a review of the literature since 1985, reporting cases of patients with AML and spontaneous remission together with informative cytogenetics. Balanced translocations such as in core binding factor (CBF) leukemias appear somewhat overrepresented. We speculate that AML-specific T cells might be relevant for induction of spontaneous remission and need to be further investigated.

Case Details

Disease Location

Bone marrowithblood

Personal Characteristics

61 -year-old male

Clinical Characteristics

Presented on march 2001 with persisting fever of 5 day duration, headache and cough; he reported decreasing physical strength in the previous months physical revealed a markedly reduced performance status (ecog4) with signs of exsiccosis and fever on admission, wbc count was 900/ul, hemoglobin was 11.9g/dl, and platelet was 97,000/ul, differential count of the peripheral blood revealed 11% blasts, 1% metamyelocytes, 31% neutrophils, 50% lymphocytes, sldh was strongly elevated, coagulation status showed a 24% quick's value, inr was 2.3 and py was 47s. Crp was highly elevated (24.2mg/dl) marrow showed 90% pox-negative undifferentiated large blast cells with wide cytoplasm and large cytoplasmatic vacuoles, resembling monoblasts, which almost completely displaced regular hematopoiesis with some signs of dysplasia and of the erythropoiesis immunophenotyping revealed positivity for the blasts for CD13, 33, 15, 65, 11c, HLA-dr, bcl-2 and lysozyme marrow histology supported the cytological diagnosis of acl m5a, karyotype analysis of marrow demonstrated a translocation (9;11)(q22;q23) confirmed by right-pcr detection of mll/af9 fusion cdna the patient was in a septic condition, antibiotic therapy was initiated after discharge, the patient returned 11 days later with re-onset of septic signs caused by an infection of the central venous line the wbc count dropped to 1500/ul and ldh and crp were markedly increased antibiotics were administered again after staphylococcus aureus was detected on the central venous line (table 1 gives time course of lab values until 864 days after initial diagnosis)

Remission Characteristics

Antibiotics resulted in prompatient defervescence and a remarkable clinical improvement of the patient within a few days, wbcs increased, platelet counts normalized and complete clearance of blasts from peripheral blood was observed, ldh and crp returned to almost normal values patient was discharged 13 days after admission after in-initiation of antibiotics, crp and ldh values returned to normal 17 days after the second admission in june 2001, peripheral blood, marrow, all revealed complete remission of acute myeloid leukemia, there was no indication of a reversal to a pre-existing mds or a 'clonal remission' right-pcr of peripheral blood cells in august 2002 and aug 2003 count not detect mll/af9 fusion cdna at present, 29 months after initial diagnosis, the patient is well and in excellent physical condition, blood counts are normal, no blasts are seen in peripheral blood

Treatment & Mechanisms

Proposed Remission Mechanisms

A leukemia-specific t-cell response might've played a role in such cases of acute myeloid leukemia and a 'gain-of-function' blanaced translocation followed treatment for sepsis

Clinical Treatment

Antibiotics (ceftazidim and vancomycin) 2nd antibiotics (ceftazidim and vancomycin then changed to flucloxacillin and imipenem/cilastatin)

Non-Clinical Treatment

None reported