Successful treatment of “accelerated” chronic lymphocytic leukemia with single agent ibrutinib: A report of two cases.

Xie, John; Jang, Albert; Vegel, Andrew; Hajja, Yasmin; Mouawad, Yara; Baghian, Ali; Berbari, Bachir; Schmid, Janet L.; Socola, Francisco; Safah, Hana; Saba, Nakhle S.

doi:10.1016/j.lrr.2021.100247

Cited by 4 publications

(5 citation statements)

References 22 publications

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“…However, it is most likely an under diagnosed entity due to its rarity and the non standardized practice of lymph node biopsy in CLL. Moreover, the diagnosis of accelerated CLL is challenging because of the overlapping clinical features between A-CLL and RT [6]. Adequate diagnosis in patients with A-CLL is important for the management and the treatment choice.…”

Section: Discussionmentioning

confidence: 99%

“…RT is characterized by a diffuse proliferation of large cells paraimmublasts mimicking centroblasts and more rarely immunoblasts, the expression of Ki 67 is more diffuse and is not limited to the proliferation centers [6] A-CLL was defined as an intermediate subtype as it has features of both typical CLL and RT with a monotonous proliferation of small lymphocytes. In a study conducted by Gine and al.…”

Section: Discussionmentioning

confidence: 99%

“…A complete response was achieved in 50% of cases and the median survival was short [17] [18] [19]. In a report of two cases, ibrutinib was used as a single agent treatment for A-CLL and managed to obtain a partial response for both patients [6].…”

Section: Discussionmentioning

confidence: 99%

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A Rare Entity of Accelerated Chronic Lymphocytic Leukemia: A Report of Two Cases and Review of Literature

Kmira,

Noura,

Wafa

et al. 2023

Health

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Background: Accelerated-chronic lymphocytic leukemia (A-CLL) is a rare disease entity as it represents less than 1% of all reported cases of chronic lymphoid leukemia (CLL). Moreover, it is most likely an under diagnosed entity due to its rarity and the non-standardized practice of lymph node biopsy in CLL. Purpose: The aims of our work are to establish the diagnosis of A-CLL and to study the prognosis and treatment of this rare entity. Method: here, we report the clinical presentation and the follow up of two cases of A-CLL. Results: Distinguishing Richter transformation (RT) from A-CLL is important as it may result in a major change in disease management. The prognosis of A-CLL is intermediate between CLL and RT. The prognosis is mainly poor due to a predominance of poor prognostic markers including an increasing number of p53-positive cases. Conclusion: To this date, no prospective study has been led to define the best treatment for A-CLL. The shorter survival of A-CLL when compared to typical CLL implies the need of a more aggressive treatment.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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A Rare Entity of Accelerated Chronic Lymphocytic Leukemia: A Report of Two Cases and Review of Literature

Kmira,

Noura,

Wafa

et al. 2023

Health

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“…It is noteworthy that, following a suboptimal response to CHOP, she demonstrated an excellent response to Imbruvica, which was remarkable because Bruton's tyrosine kinase inhibitors have yet to demonstrate their utility in aCLL. Aside from the two recently reported cases, 5 the lack of experience with Imbruvica in aCLL prompted the administration of the reduced dose (140 mg/day), but even such a substandard dose triggered a pronounced peripheral lymphocytosis (tumor flare) that, similarly to “non‐accelerated” CLL, was transient.…”

Section: Discussionmentioning

confidence: 99%

Atypical “accelerated” chronic lymphocytic leukemia with abnormal lymphocyte chromatin clumping, bone involvement, and exceptional response to Imbruvica

Yavorkovsky

2022

Cancer Reports

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Background The “accelerated” chronic lymphocytic leukemia (aCLL) is a relatively rare form of CLL progression. The expanded proliferation centers in aCLL have been associated with adverse prognostic features and propensity to more aggressive behavior with shorter survival. Case An atypical case of aCLL with distinct features is described. A 66‐year‐old female presented with a marrow replacing process associated with multiple osseous metastases and trivial lymphadenopathy. Bone biopsy revealed an unspecified low‐grade B cell lymphoproliferative disorder that demonstrated a suboptimal response to standard chemotherapy. Subsequent lymph node biopsy demonstrated findings consisted with aCLL. The distinguishing features of the case were, in addition to bone involvement, the lagging peripheral lymphocytosis and a striking pattern of the chromatin clumping with a prominent “shattered” appearance reminiscent of Pelger‐Huet‐like dysplastic anomaly. A targeted next‐generation sequencing (NGS) assay detected pathogenic mutations in TP53 and SF3B1 . In contrast to chemotherapy, the case demonstrated an excellent response to imbruvica. Conclusion The noted peculiarities could potentially distinguish this case as a novel, rare variant of aCLL.

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“…Data are very limited regarding the role of targeted therapies for A-CLL. Successful treatment of A-CLL has been reported with ibrutinib [11, 12] and acalabrutinib [13]. One case report of leukemia cutis in A-CLL was successfully treated with venetoclax and rituximab [14].…”

Section: Accelerated Chronic Lymphocytic Leukemiamentioning

confidence: 99%

Accelerated Chronic Lymphocytic Leukemia and Richter Transformation in the Era of Novel Agents

Levy Yurkovski,

Tadmor

2023

Acta Haematol

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Background: Tremendous developments in the field of chronic lymphocytic leukemia (CLL) in recent years have led to a revolutionary change in the treatment approach, which today is based on targeted treatments with a good response and optimal prognosis. Nevertheless, CLL can present or progress to “accelerated CLL” (A-CLL) or to “Richter transformation” (RT) and these two entities have a more aggressive course and are still characterized by challenges in the fields of diagnosis and therapy. In the current review, we summarized the latest knowledge in terms of diagnostic approaches to A-CLL, available treatments and clinical trials, for both A-CLL and RT which still pose an unmet need and require additional basic and clinical investigations. Summary: A-CLL is a rare and underdiagnosed entity that probably stands in the “gray zone” between CLL and RT, generally holding an intermediate prognosis. Its diagnosis is mainly based on histological findings including expanded proliferation centers, increased mitotic activity, and/or high Ki-67 index. Due to its rarity, its treatment approach has still not been defined, but it seems that novel agents, especially Bruton tyrosine kinase inhibitors (BTKi), are effective. As for RT, the standard therapy still consists of chemo-immunotherapy followed by stem-cell transplantation for fit responders with a dismal prognosis. New approaches are recently adopted including B-cell inhibition via novel agents (BTKi, venetoclax), T-cell engagers (checkpoint inhibitors, bispecific antibodies [BiTe] or the chimeric antigen receptor [CAR] technology), antibody-drug conjugates, or drug combinations. Although both CAR-T and BiTe seem promising, especially when combined with BTKi, evidence is still insufficient, and patients should generally be recruited in clinical trials. Key Messages: The field of CLL has been a subject of major advances in recent years, but A-CLL and RT remain topics of “unmet need” and require further studies to identify the best diagnostic approach and a more effective treatment.

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Successful treatment of “accelerated” chronic lymphocytic leukemia with single agent ibrutinib: A report of two cases.

Cited by 4 publications

References 22 publications

A Rare Entity of Accelerated Chronic Lymphocytic Leukemia: A Report of Two Cases and Review of Literature

A Rare Entity of Accelerated Chronic Lymphocytic Leukemia: A Report of Two Cases and Review of Literature

Atypical “accelerated” chronic lymphocytic leukemia with abnormal lymphocyte chromatin clumping, bone involvement, and exceptional response to Imbruvica

Accelerated Chronic Lymphocytic Leukemia and Richter Transformation in the Era of Novel Agents

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