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 language | English |
|---|---|
| Pages (from-to) | 785-795 |
| Number of pages | 11 |
| Journal | Histopathology |
| Volume | 57 |
| Issue number | 6 |
| DOIs | |
| Publication status | Published - 2010 Dec 1 |
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All Science Journal Classification (ASJC) codes
- Pathology and Forensic Medicine
- Histology
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Large B cell lymphoma presenting initially in bone marrow, liver and spleen : An aggressive entity associated frequently with haemophagocytic syndrome. / Yeh, Yu Min; Chang, Kung Chao; Chen, Ya Ping; Kao, Lin Yuan; Tsai, Huey Pin; Ho, Chung Liang; Wang, Jen Ren; Jones, Dan; Chen, Tsai Yun.
In: Histopathology, Vol. 57, No. 6, 01.12.2010, p. 785-795.Research output: Contribution to journal › Article
TY - JOUR
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.
UR - http://www.scopus.com/inward/record.url?scp=78650247363&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=78650247363&partnerID=8YFLogxK
U2 - 10.1111/j.1365-2559.2010.03709.x
DO - 10.1111/j.1365-2559.2010.03709.x
M3 - Article
C2 - 21166693
AN - SCOPUS:78650247363
VL - 57
SP - 785
EP - 795
JO - Histopathology
JF - Histopathology
SN - 0309-0167
IS - 6
ER -