Treatment Outcomes and Clinical Relevance in Patients With Double Expressor DLBCL

Rungwittayatiwat, Sirapat; Boonsakan, Paisarn; Chantrathammachart, Pichika; Puavilai, Teeraya; Pukiat, Sulada; Phusanti, Sithakom; Boonyawat, Kochawan; Wacharapornin, Pathawut; Angchaisuksiri, Pantep; Ungkanont, Artit; Chuncharunee, Suporn; Niparuck, Pimjai

doi:10.4084/mjhid.2021.063

Cited by 3 publications

(1 citation statement)

References 17 publications

(40 reference statements)

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“…22 This is clinically relevant as patients with double-expressor lymphoma have been shown to have worse 3-year overall survival with R-CHOP, the first-line chemoimmunotherapy regimen for DLBCL, when compared with DLBCL with single or no protein overexpressors. [22][23][24] Dysregulation of the MYC oncogene has been demonstrated to play an important role in lymphomagenesis, being associated with several aggressive lymphomas. 25 While MYC translocations are rare in CLL, they likely play a role in the development of RS that is clonally related to the previous CLL.…”

Section: Discussionmentioning

confidence: 99%

Richter Syndrome Presenting as Subcutaneous Nodules and a Dermal Plaque

Nickles,

Hunt,

Turcios-Escobar

et al. 2024

The American Journal of Dermatopathology

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Richter syndrome (RS) describes a phenomenon in which a patient with chronic lymphocytic leukemia (CLL) develops an aggressive lymphoma, most commonly diffuse large B-cell lymphoma (DLBCL). Reports of cutaneous RS remain exceedingly rare. We report a 61-year-old woman with relapsed/refractory CLL presenting with several subcutaneous nodules on her arms and legs and a single dermal plaque on her abdomen. Skin biopsy revealed a diagnosis of DLBCL, ABC-type, and her clinical status rapidly deteriorated following diagnosis. We review the variety of clinical presentations of cutaneous RS, its association with CLL, risk factors for RS development in CLL patients, and the distinctive histopathologic and immunophenotypic features of DLBCL. We hope to highlight the importance of prompt skin biopsy in patients with CLL presenting with progressive skin lesions and increase awareness of this aggressive clinical syndrome.

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Section: Discussionmentioning

confidence: 99%

Richter Syndrome Presenting as Subcutaneous Nodules and a Dermal Plaque

Nickles,

Hunt,

Turcios-Escobar

et al. 2024

The American Journal of Dermatopathology

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Expression of MYD88 L265P Mutation in Subtypes of Diffuse Large B-Cell Lymphoma in the Pakistani Population

Shabir,

Din,

Mahmood

et al. 2024

Applied Immunohistochemistry &Amp; Molecular Morphology

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MYD88 L265P mutation is a gain-of-function driver mutation. It is observed in a significant proportion of Waldenstrom macroglobulinemia and activated B-cell subtype of diffuse large B-cell lymphoma (DLBCL; non-germinal center subtype). The incidence of this mutation in the subtypes of DLBCL has not yet been documented in the Pakistani population. This study aimed to ascertain the frequency and association of MYD88 L265P mutation within 2 subtypes of DLBCL, germinal center B-cell-like (GCB) and non-GCB B-cell lymphoma (non-GCB), in the local population. This cross-sectional study was conducted at the Armed Forces Institute of Pathology, Punjab, Pakistan. All newly diagnosed cases of DLBCL were included in the study. We analyzed 82 biopsy-proven cases of DLBCL (28 cases of GCB subtype and 54 cases of non-GCB subtype). DNA was extracted from formalin-fixed paraffin-embedded tissue blocks, and a conventional polymerase chain reaction was used to detect the MYD88 L265P mutation. The MYD88 L265P mutation was detected in 01 of 28 (3.6%) cases of the GCB subtype (95% CI: 0%-10%) and in 12 of 54 (22.2%) cases of the non-GCB subtype (95% CI: 11%-33%). Pearsos χ2 test revealed a statistically significant association of MYD88 L265P mutation with non-GCB subtype of DLBCL (P = 0.024). This association will assist in identifying a target population that may benefit from MYD88-specific treatment regimens. This may exponentially improve the outcome of patients with DLBCL harboring this mutation.

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“Double expressor” diffuse large B-cell lymphoma: A case report and literature review

Terzić

Otašević²,

Vukovic

et al. 2022

Med pregl

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Diffuse large B-cell lymphoma, not otherwise specified, is the most common type of non-Hodgkin lymphoma worldwide, accounting for 30-40% of all lymphomas. It represents a collection of morphologically, genetically and clinically different diseases. Therefore, it can be subdivided into morphological variants, phenotypic subtypes, and molecular or genetic categories. More recently, diffuse large B-cell lymphoma has witnessed advances in molecular profiling and treatment of patients with refractory and relapsed disease. The optimal management requires integrated morphological and immunophenotypic analysis of cell and tissue, along with chromosome and molecular analyses. Double-expressor lymphoma, defined as overexpression of MYC and BCL2 proteins not related to underlying chromosomal rearrangements, accounts for 20% to 30% of Diffuse large B-cell lymphoma cases. In the latest, 5th edition of the World Health Organization Classification of Hematolymphoid Tumors-lymphoid neoplasms, double-expressor lymphoma is not defined as an independent entity, but it has been proven to be a marker for poor outcome in diffuse large B-cell lymphoma. However, the degree of adverse prognosis is lesser than in double-hit lymphomas. Although double-expressor lymphoma feature is confirmed as adverse prognostic marker for diffuse large B-cell lymphoma patients, currently no sufficient data is available to support treatment intensification over standard rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone regimen. Well-designed randomized clinical trials are mandatory in order to properly respond to this substantial clinical dispute.

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Treatment Outcomes and Clinical Relevance in Patients With Double Expressor DLBCL

Cited by 3 publications

References 17 publications

Richter Syndrome Presenting as Subcutaneous Nodules and a Dermal Plaque

Richter Syndrome Presenting as Subcutaneous Nodules and a Dermal Plaque

Expression of MYD88 L265P Mutation in Subtypes of Diffuse Large B-Cell Lymphoma in the Pakistani Population

“Double expressor” diffuse large B-cell lymphoma: A case report and literature review

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