PMID- 33994430
OWN - NLM
STAT- MEDLINE
DCOM- 20210621
LR  - 20210715
IS  - 1880-9952 (Electronic)
IS  - 1346-4280 (Print)
IS  - 1346-4280 (Linking)
VI  - 61
IP  - 2
DP  - 2021 Jun 5
TI  - Hemophagocytic lymphohistiocytosis associated with Epstein-Barr virus-positive 
      diffuse large B-cell lymphoma, NOS of bone marrow-liver-spleen type: an autopsy 
      case report.
PG  - 102-108
LID - 10.3960/jslrt.20053 [doi]
AB  - Lymphoma-associated hemophagocytic lymphohistiocytosis (HLH) has a significantly 
      poor prognosis among secondary HLH. We describe the rare case of a 74-year-old 
      female with secondary HLH presenting with a rapidly fatal course. Post-mortem 
      examination revealed Epstein-Barr virus (EBV) -positive diffuse large B-cell 
      lymphoma (DLBCL). We were unable to make a definite antemortem diagnosis because 
      the patient did not exhibit lymphadenopathy and bone marrow biopsy demonstrated 
      hemophagocytosis without evidence of lymphoma. She died of multiple organ failure 
      on the twelfth day of hospitalization despite a temporary response to steroids. 
      Autopsy revealed diffuse lymphoma cell infiltration of the bone marrow, liver and 
      spleen, suggesting "bone marrow-liver-spleen" (BLS)-type large B-cell lymphoma 
      (LBCL). BLS-type LBCL is a rare and clinically aggressive lymphoma, usually 
      associated with fever, cytopenia and HLH. The disease has a high mortality rate 
      due to the delay in diagnosis and a highly aggressive clinical course. Further 
      studies are required to improve our understanding of this rare extranodal DLBCL.
FAU - Iizuka, Hiroko
AU  - Iizuka H
AD  - Department of Hematology, Juntendo University Shizuoka Hospital, Shizuoka, Japan.
AD  - Department of Hematology, Juntendo University Urayasu Hospital, Chiba, Japan.
FAU - Mori, Yosuke
AU  - Mori Y
AD  - Department of Hematology, Juntendo University Shizuoka Hospital, Shizuoka, Japan.
AD  - Department of Hematology, Juntendo University School of Medicine, Tokyo, Japan.
FAU - Iwao, Noriaki
AU  - Iwao N
AD  - Department of Hematology, Juntendo University Shizuoka Hospital, Shizuoka, Japan.
FAU - Koike, Michiaki
AU  - Koike M
AD  - Department of Hematology, Juntendo University Shizuoka Hospital, Shizuoka, Japan.
FAU - Noguchi, Masaaki
AU  - Noguchi M
AD  - Department of Hematology, Juntendo University Urayasu Hospital, Chiba, Japan.
LA  - eng
PT  - Case Reports
PT  - Journal Article
DEP - 20210514
PL  - Japan
TA  - J Clin Exp Hematop
JT  - Journal of clinical and experimental hematopathology : JCEH
JID - 101141257
SB  - IM
MH  - Aged
MH  - Autopsy
MH  - Biopsy
MH  - Bone Marrow/*pathology
MH  - Epstein-Barr Virus Infections/*complications/pathology
MH  - Female
MH  - Humans
MH  - Liver/*pathology
MH  - Lymphohistiocytosis, Hemophagocytic/*complications/pathology
MH  - Lymphoma, Large B-Cell, Diffuse/*complications/pathology
MH  - Spleen/*pathology
PMC - PMC8265493
OTO - NOTNLM
OT  - Epstein-Barr virus-positive diffuse large B-cell lymphoma
OT  - autopsy
OT  - bone marrow-liver-spleen-type large B-cell lymphoma
OT  - hemophagocytic lymphohistiocytosis
COIS- CONFLICT OF INTEREST The authors have no significant relationships with or 
      financial interests in any commercial companies pertaining to this article.
EDAT- 2021/05/18 06:00
MHDA- 2021/06/22 06:00
PMCR- 2021/05/14
CRDT- 2021/05/17 05:52
PHST- 2021/05/18 06:00 [pubmed]
PHST- 2021/06/22 06:00 [medline]
PHST- 2021/05/17 05:52 [entrez]
PHST- 2021/05/14 00:00 [pmc-release]
AID - 20053 [pii]
AID - 10.3960/jslrt.20053 [doi]
PST - ppublish
SO  - J Clin Exp Hematop. 2021 Jun 5;61(2):102-108. doi: 10.3960/jslrt.20053. Epub 2021 
      May 14.