Large B cell lymphoma presenting initially in bone marrow, liver and spleen: An aggressive entity associated frequently with haemophagocytic syndrome

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Abstract

Aims: To describe diffuse large B cell lymphoma (DLBCL) presenting initially in bone marrow, liver and spleen (BLS-type) without lymphadenopathy.Methods and results: The clinicopathological and cytogenetic features of 11 such cases (eight men, three women; mean age: 62.7 years are described). Usually presenting with fever and haemophagocytic syndrome suggesting infection and complicating timely diagnosis, bone marrow examination showed patchy and interstitial infiltration of large tumour cells without sinusoidal involvement. All cases had a high Ki-67 index (≥90%), commonly a non-germinal centre/activated B cell immunophenotype and were negative for Epstein-Barr virus and human herpesvirus 6 and 8. The more frequent cytogenetic changes involved chromosomal loci 14q32 and 9p24, as well as del(3)(q21), add(7)(p22), t(3;6), del(8)(p22), +18 and add(19)(p13). Clinical behaviour was very aggressive, with a 2-year survival rate of 18% (45% of patients died within 3 weeks). High-dose chemotherapy with haematopoietic stem cell transplantation prolonged survival in one patient.Conclusions: Although it shares with intravascular LBCL a subtle presentation and an aggressive clinical course, this primary BLS large cell lymphoma variant is distinguished by lacking an intravascular component and having different cytogenetic findings.

Original languageEnglish
Pages (from-to)785-795
Number of pages11
JournalHistopathology
Volume57
Issue number6
DOIs
Publication statusPublished - 2010 Dec 1

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Hemophagocytic Lymphohistiocytosis
B-Cell Lymphoma
Cytogenetics
Spleen
Bone Marrow
Liver
Bone Marrow Examination
Human Herpesvirus 6
Human Herpesvirus 8
Lymphoma, Large B-Cell, Diffuse
Hematopoietic Stem Cell Transplantation
Human Herpesvirus 4
Lymphoma
B-Lymphocytes
Fever
Survival Rate
Drug Therapy
Survival
Infection
Neoplasms

All Science Journal Classification (ASJC) codes

  • Pathology and Forensic Medicine
  • Histology

Cite this

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title = "Large B cell lymphoma presenting initially in bone marrow, liver and spleen: An aggressive entity associated frequently with haemophagocytic syndrome",
abstract = "Aims: To describe diffuse large B cell lymphoma (DLBCL) presenting initially in bone marrow, liver and spleen (BLS-type) without lymphadenopathy.Methods and results: The clinicopathological and cytogenetic features of 11 such cases (eight men, three women; mean age: 62.7 years are described). Usually presenting with fever and haemophagocytic syndrome suggesting infection and complicating timely diagnosis, bone marrow examination showed patchy and interstitial infiltration of large tumour cells without sinusoidal involvement. All cases had a high Ki-67 index (≥90{\%}), commonly a non-germinal centre/activated B cell immunophenotype and were negative for Epstein-Barr virus and human herpesvirus 6 and 8. The more frequent cytogenetic changes involved chromosomal loci 14q32 and 9p24, as well as del(3)(q21), add(7)(p22), t(3;6), del(8)(p22), +18 and add(19)(p13). Clinical behaviour was very aggressive, with a 2-year survival rate of 18{\%} (45{\%} of patients died within 3 weeks). High-dose chemotherapy with haematopoietic stem cell transplantation prolonged survival in one patient.Conclusions: Although it shares with intravascular LBCL a subtle presentation and an aggressive clinical course, this primary BLS large cell lymphoma variant is distinguished by lacking an intravascular component and having different cytogenetic findings.",
author = "Yeh, {Yu Min} and Chang, {Kung Chao} and Chen, {Ya Ping} and Kao, {Lin Yuan} and Tsai, {Huey Pin} and Ho, {Chung Liang} and Wang, {Jen Ren} and Dan Jones and Chen, {Tsai Yun}",
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T1 - Large B cell lymphoma presenting initially in bone marrow, liver and spleen

T2 - An aggressive entity associated frequently with haemophagocytic syndrome

AU - Yeh, Yu Min

AU - Chang, Kung Chao

AU - Chen, Ya Ping

AU - Kao, Lin Yuan

AU - Tsai, Huey Pin

AU - Ho, Chung Liang

AU - Wang, Jen Ren

AU - Jones, Dan

AU - Chen, Tsai Yun

PY - 2010/12/1

Y1 - 2010/12/1

N2 - Aims: To describe diffuse large B cell lymphoma (DLBCL) presenting initially in bone marrow, liver and spleen (BLS-type) without lymphadenopathy.Methods and results: The clinicopathological and cytogenetic features of 11 such cases (eight men, three women; mean age: 62.7 years are described). Usually presenting with fever and haemophagocytic syndrome suggesting infection and complicating timely diagnosis, bone marrow examination showed patchy and interstitial infiltration of large tumour cells without sinusoidal involvement. All cases had a high Ki-67 index (≥90%), commonly a non-germinal centre/activated B cell immunophenotype and were negative for Epstein-Barr virus and human herpesvirus 6 and 8. The more frequent cytogenetic changes involved chromosomal loci 14q32 and 9p24, as well as del(3)(q21), add(7)(p22), t(3;6), del(8)(p22), +18 and add(19)(p13). Clinical behaviour was very aggressive, with a 2-year survival rate of 18% (45% of patients died within 3 weeks). High-dose chemotherapy with haematopoietic stem cell transplantation prolonged survival in one patient.Conclusions: Although it shares with intravascular LBCL a subtle presentation and an aggressive clinical course, this primary BLS large cell lymphoma variant is distinguished by lacking an intravascular component and having different cytogenetic findings.

AB - Aims: To describe diffuse large B cell lymphoma (DLBCL) presenting initially in bone marrow, liver and spleen (BLS-type) without lymphadenopathy.Methods and results: The clinicopathological and cytogenetic features of 11 such cases (eight men, three women; mean age: 62.7 years are described). Usually presenting with fever and haemophagocytic syndrome suggesting infection and complicating timely diagnosis, bone marrow examination showed patchy and interstitial infiltration of large tumour cells without sinusoidal involvement. All cases had a high Ki-67 index (≥90%), commonly a non-germinal centre/activated B cell immunophenotype and were negative for Epstein-Barr virus and human herpesvirus 6 and 8. The more frequent cytogenetic changes involved chromosomal loci 14q32 and 9p24, as well as del(3)(q21), add(7)(p22), t(3;6), del(8)(p22), +18 and add(19)(p13). Clinical behaviour was very aggressive, with a 2-year survival rate of 18% (45% of patients died within 3 weeks). High-dose chemotherapy with haematopoietic stem cell transplantation prolonged survival in one patient.Conclusions: Although it shares with intravascular LBCL a subtle presentation and an aggressive clinical course, this primary BLS large cell lymphoma variant is distinguished by lacking an intravascular component and having different cytogenetic findings.

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