&lt;p&gt;&lt;em&gt;BRAF&lt;/em&gt; V600E-positive monomorphic epitheliotropic intestinal T-cell lymphoma complicating the course of hairy cell leukemia&lt;/p&gt;

Broccoli, Alessandro; Bertuzzi, Clara; Fiorentino, Michelangelo; Morigi, Alice; Stefoni, Vittorio; Agostinelli, Claudio; Argnani, Lisa; Cavo, Michèle; Zinzani, Pier Luigi

doi:10.2147/ott.s202061

Cited by 4 publications

(1 citation statement)

References 15 publications

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“…A second patient maintained a FA of 2.46% on BM after the failure of repeated lines of cladribine, and ultimately died of HCL. The third patient showed progressive disease after an initial treatment with subcutaneous cladribine, with a FA on BM of 10.33%: concomitantly, he developed a BRAF V600E ‐positive monomorphic epitheliotropic intestinal T‐cell lymphoma, which caused his death a few months later 19 …”

Section: Resultsmentioning

confidence: 99%

Droplet digital polymerase chain reaction for the assessment of disease burden in hairy cell leukemia

Broccoli

Terragna

Nanni

et al. 2021

Hematological Oncology

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BRAFV600E mutation is the pathogenic driver of hairy cell leukemia (HCL) found in the vast majority of cases both at onset and during recurrences. The identification of the mutated allele in blood and marrow correlates with the presence of neoplastic cells and can be considered a marker of active disease. Likewise, the absence of the mutation after treatment may indicate a state of deep response. The BRAFV600E burden was measured by droplet digital polymerase chain reaction (ddPCR) and expressed as fractional abundance in 35 HCL patients at different stages of disease (onset, relapse, complete response [CR] after treatment, long‐term remission) in peripheral blood and/or bone marrow (when available). Mean values of fractional abundance for patients at diagnosis, relapse and response, respectively, were 12.26%, 16.52% and 0.02% in peripheral blood and 23.51%, 13.96% and 0.26% in bone marrow. Four patients out of 6 evaluated at response were molecularly negative for BRAFV600E in peripheral blood. Mean fractional abundance in peripheral blood tested in 14 patients with long lasting CR was 0.05%, and 10 patients were BRAFV600E negative. These preliminary results suggest that ddPCR permits to assess the active tumor burden in HCL at different disease phases and support the hypothesis that some patients in CR qualify for a molecular CR.

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Section: Resultsmentioning

confidence: 99%

Droplet digital polymerase chain reaction for the assessment of disease burden in hairy cell leukemia

Broccoli

Terragna

Nanni

et al. 2021

Hematological Oncology

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Cladribine/methylprednisolone

2019

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Author comment: "Tumoral reprogramming of an initiating HCL BRAF-mutated cell may also account for tumor S Cladribine/methylprednisolone cell plasticity, ending up with a change of the initial neoplastic phenotype and the development of a secondary Transformation of hairy cell leukaemia to lymphoid disease, possibly favored by the setting of profound monomorphic epitheliotropic intestinal T-cell immune suppression due to HCL itself, its treatment and lymphoma: case report complications." A 65-year-old man developed transformation of hairy cell leukaemia (HCL) to monomorphic epitheliotropic intestinal T-Broccoli A, et al. BRAF V600E-positive monomorphic epitheliotropic intestinal Tcell lymphoma complicating the course of hairy cell leukemia. OncoTargets and

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How molecular advances may improve the diagnosis and management of PTCL patients

2023

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Peripheral T-cell lymphomas (PTCL) comprised more than 30 rare heterogeneous entities, representing 10 to 15% of adult non-Hodgkin lymphomas. Although their diagnosis is still mainly based on clinical, pathological, and phenotypic features, molecular studies have allowed for a better understanding of the oncogenic mechanisms involved and the refinement of many PTCL entities in the recently updated classifications. The prognosis remains poor for most entities (5-year overall survival < 30%), with current conventional therapies based on anthracyclin-based polychemotherapy regimen, despite many years of clinical trials. The recent use of new targeted therapies appears to be promising for relapsed/refractory patients, such as demethylating agents in T-follicular helper (TFH) PTCL. However further studies are needed to evaluate the proper combination of these drugs in the setting of front-line therapy. In this review, we will summarize the oncogenic events for the main PTCL entities and report the molecular targets that have led to the development of new therapies. We will also discuss the development of innovative high throughput technologies that aid the routine workflow for the histopathological diagnosis and management of PTCL patients.

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<p><em>BRAF</em> V600E-positive monomorphic epitheliotropic intestinal T-cell lymphoma complicating the course of hairy cell leukemia</p>

Cited by 4 publications

References 15 publications

Droplet digital polymerase chain reaction for the assessment of disease burden in hairy cell leukemia

Droplet digital polymerase chain reaction for the assessment of disease burden in hairy cell leukemia

Cladribine/methylprednisolone

How molecular advances may improve the diagnosis and management of PTCL patients

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