Impact of B-cell count and imaging screening in cMBL: any need to revise the current guidelines?

Abstract

one intensification cycle of high-dose cytarabine and etoposide (HAE). The patient received a prophylactic cranial irradiation and intrathecal therapy with cytarabine. Allogeneic stem cell transplantation was not performed because of the lack of a HLAidentical sibling donor. A maintenance therapy with 6-thioguanine and cytarabine was given. The patient remained in remission and was discharged from hospital after 8 months of treatment. After 6 months, the patient was hospitalized because of weight loss, thrombocytopenia and hepatosplenomegaly. Repeated bone marrow examinations revealed no evidence for AML relapse. In the following, the patient suffered from a severe aspergillus sepsis and died due to a septic multiple organ failure with bleeding complications.To our knowledge, this is the first report to describe a somatic EZH2 mutation in pediatric AML. Although EZH2 mutations seem to be rare in childhood AML, our findings indicate that EZH2 mutations might contribute to the disease in specific cases. The incidence of EZH2 mutations in childhood AML needs to be assessed in a larger cohort of patients particularly in children with t(8;21).

Total body computed tomography scan in the initial work‐up of Binet stage A chronic lymphocytic leukemia patients: Results of the prospective, multicenter O‐CLL1‐GISL study

“…Accordingly, Rossi et al reported that 15% of cMBLs progressed to SLLs, suggesting that some cMBLs are SLLs with a very low tumor burden at diagnosis [25]. Scarf o et al found lymphadenopathy and/or hepato-/spleganomegaly in only 6% of cMBL by radiologic evaluation, although the criteria utilized for defining lymphadenopathies were not clearly outlined in the study [26]. Overall, our and other data support the notion that TB-CT scan could correctly distinguish between cMBLs and SLLs.…”

Monoclonal B-cell lymphocytosis: right track or red herring?

“…30 This would allow some rare stage 0 patients who do not progress to be demoted into an MBL state (where they conceptually belong). [31][32][33] That would perhaps allow a more proper classification of MBL and possibly reassure some rare patients. However, because clinically it would be retrospective, it would not lead to an understanding of which MBL will progress and which will not.…”

“…The most sensible conclusion is that for clinical MBL we have to consider a follow-up MGUS-style, 21 first reassuring the affected persons that their risk of progression toward a full-blown leukemia is in the range of 1% to 2% per year and then suggesting a yearly hematologic consultation with a complete blood cell count and whenever deemed useful abdominal ultrasound/chest x-ray, with no invasive investigations whatsoever. 33 For populationscreening MBL, based on the most recent evidence, 20 the risk of progressing and developing CLL is very low, if any, and likely not different from that of the general population. Therefore, the most reasonable attitude is to continue investigating these subjects to establish the genetic architecture of MBL as this would allow defining which genes are critically involved in evolution, which critical pathways are involved, and how they relate to microenvironmental influences.…”

Immunogenetics shows that not all MBL are equal: the larger the clone, the more similar to CLL

“…28 This indicates that both entities comprise a mixture of cases with distinct overall propensity to progression and suggests the need to identify molecular or clinical signatures besides the official threshold of 5 3…”