Leukemic High Grade B Cell Lymphoma is Associated With MYC Translocation, Double Hit/Triple Hit Status, Transformation, and CNS Disease Risk: The Mayo Clinic Experience

Kuhlman, Justin J; Moustafa, Muhamad Alhaj; Jiang, Liuyan; Iqbal, Madiha; Seegobin, Karan; Wolcott, Zoe; Ayala, Ernesto; Ansell, S.; Rosenthal, Allison; Paludo, Jonas; Micallef, Ivana N.; Johnston, Patrick B.; Inwards, David J.; Habermann, Thomas M.; Kharfan‐Dabaja, Mohamed A.; Witzig, Thomas E.; Nowakowski, Grzegorz S.; Tun, Han W.

doi:10.1016/j.clml.2022.04.009

Cited by 2 publications

(3 citation statements)

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“…Bone marrow involvement by DLBCL has not been definitively proven to be associated with high CNS risk; however, leukemic manifestation with bone marrow involvement is associated with high CNS risk. [18][19][20] Unlike the second case, this patient did quite well, achieving CR for three years after R-CHOP and getting back into CR following a late bone marrow-only recurrence with non-transplant treatment.…”

mentioning

confidence: 72%

“…Leukemic HGBCL carries a high CNS risk. 18 Abnormal laboratory findings including very high LDH and ferritin supported the aggressive clinical manifestation.…”

Section: Dovepressmentioning

confidence: 99%

“…Richter's transformation presenting as leukemic HGBCL with extensive bone marrow involvement has been reported in the setting of widely disseminated disease. 18 There were two clonal B cell populations in the blood: a CD5+ and CD10-clone likely related to CLL and a CD5-and CD10+ clone related to HGBCL with c-MYC and BCL-2 translocations. These two clonal B-cell populations shared the same kappa light chain restriction.…”

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confidence: 99%

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Primary Bone Marrow Lymphoma: De Novo and Transformed Subtypes

Kimbrough¹,

Jiang²,

Parent³

et al. 2022

JBM

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Diffuse large B-cell lymphoma (DLBCL) is well known for selectively involving certain extranodal locations such as the central nervous system (CNS), testes, and skin. DLBCL or high-grade B-cell lymphoma selectively involving the bone marrow is rare and has been sparsely reported in the medical literature. We report two cases of lymphoma presenting with primary bone marrow involvement without evidence of involvement of any other sites. The first case represents de novo DLBCL. The patient achieved complete remission with initial treatment, had a bone marrow-only relapse three years later, and achieved a second complete remission following non-transplant salvage therapy. The second case had findings consistent with "double hit" Richter's transformation of chronic lymphocytic leukemia with translocation of c-MYC and BCL-2. This patient had an aggressive clinical course characterized by rapid progression with CNS involvement within three months resulting in the demise of the patient. These two cases represent two distinct subtypes of primary bone marrow lymphoma: de novo and transformed. Further research is necessary to gain a better understanding of this rare lymphoma entity and develop novel therapies. Keywords: diffuse large B-cell lymphoma, high-grade B-cell lymphoma with MYC and BCL-2 rearrangements, bone marrow involvement, cytopenias Plain Language Summary• Primary bone marrow lymphoma (PBML) is confined to the bone marrow with or without a leukemic component and represents a distinct, aggressive type of extranodal lymphoma. • PBML is very rare and has been sparsely reported in the medical literature.• PBML appears to consist of two subtypes: de novo PBML and PBML derived from transformation of bone marrow-based lowgrade lymphomas. • PBML should be distinguished from other aggressive lymphomas which can primarily involve the bone marrow such as Burkitt lymphoma, lymphoblastic lymphoma, blastoid mantle cell lymphoma, and intravascular large B-cell lymphoma. • PET imaging is useful for monitoring lymphoma activity in the bone marrow.

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mentioning

confidence: 72%

“…Leukemic HGBCL carries a high CNS risk. 18 Abnormal laboratory findings including very high LDH and ferritin supported the aggressive clinical manifestation.…”

Section: Dovepressmentioning

confidence: 99%

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confidence: 99%

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