Primary effusion lymphoma in Taiwan shows two distinctive clinicopathological subtypes with rare human immunodeficiency virus association

Diagnostic Cytopathology

et al. 2020

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BackgroundLymphomas presenting in effusions could either be primary or secondary, with very limited data from Taiwan.MethodsWe retrospectively reviewed effusion lymphomas from our archives in a tertiary center from July 2011 to June 2019.ResultsWe identified 59 specimens from 43 patients, including 7 cases with primary effusion lymphoma (PEL) and 36, secondary effusion involvement. Half of the secondary cases presented concurrently with effusion lymphoma, while the remaining half‐experienced effusion lymphoma during disease progression. All patients with PELs were males with a median age of 77 and presented with massive pleural effusion. None was HIV‐related. Two (29%) PEL cases were positive for human herpes virus 8 (HHV8). The only case with plasmablastic phenotype in the PEL group was positive for both HHV8 and EBV. Four patients died shortly after diagnosis; while the remaining three were alive at the last follow‐up (two at 13 months and one at 99 months). Of the secondary cases, diffuse large B‐cell lymphoma/high grade B‐cell lymphoma was the most common (n = 16, 44%), followed by mantle cell lymphoma (n = 5, 14%). Only 8 cases (22%) were T‐cell neoplasms. Prognosis for patients with secondary effusion involvement was dismal, with 1‐ and 2‐year overall survival rates at 17% and 8%, respectively.ConclusionWe found a wide cytopathological spectrum of effusion lymphoma in Taiwan. Most of our PEL cases were distinct from that defined in the World Health Organization scheme by a B‐cell phenotype, HHV8‐negativity, and absence of immunodeficiency. As compared to PEL cases, the prognosis of those with secondary involvement was extremely poor.

Section: Methodsmentioning

confidence: 99%

Section: Clinicocytological Features Of Patients With Pelsmentioning

confidence: 99%

The cytopathological spectrum of lymphomas in effusions in a tertiary center in Taiwan

Wang

Diagnostic Cytopathology

et al. 2020

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“…that distinguish it from PEL 33. In our recent study of PEL in Taiwan with a literature review of 256 cases, we identified a group of patients with HHV8-negative EBL and with rare HIV infection, in contrast to the HIV-and HHV8-double positive classical PEL in the WHO classification 34. Interestingly from our current study, there might be a small number of PEL or EBL comprising of ENKTL.…”

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confidence: 55%

Malignant effusions from extranodal NK/T‐cell lymphomas are frequently of anaplastic morphology with azurophilic granules and of T‐cell lineage

Liu

Diagnostic Cytopathology

Chuang

2020

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Background Extranodal natural killer/T‐cell lymphoma (ENKTL) nasal type is an extranodal lymphoma, with most cases arising from the prototypic nasal/upper aerodigestive tract and the cell of origin (COO) is frequently of NK cells. Effusion involvement by ENKTL is rare. The cytomorphological and immunophenotypical features and COO of effusion ENKTL are elusive. Design We report four new cases of effusion ENKTL, reviewing the cytomorphological features and investigating their COO by immunohistochemistry for T‐cell receptor (TCR) protein expression and clonality assay for TCR gene rearrangement. We also reviewed 12 additional cases of effusion ENKTL from the literature. Results All our four cases showed anaplastic tumor cells with fine to coarse azurophilic granules, with the primary tumor organs from the prototypic sites (2), small intestine (1), or indeterminate (1). The COO was either T‐cell (3) or NK‐cell (1). Compiling our four cases with that from the literature, all 16 effusion ENKTL cases were Epstein‐Barr virus‐associated and the majority (83%; 10/12) originated from the nonprototypic sites. Five of six (83%) effusion ENKTL cases were of T‐cell lineage. Prognosis was poor with a median survival time of only 2.4 months. Conclusions Our study revealed that ENKTL in the effusion might be characterized by anaplastic large cell morphology, cytoplasmic azurophilic granules, and a higher proportion of T‐cell lineage as compared to de novo tumors arising from the prototypic sites. ENKTL rarely presented in effusion, which is associated with dismal outcome. Large international studies are warranted to validate these findings.

“…rare cases of primary effusion lymphoma might resolve after pleurocentesis without chemotherapy. 6,7 Improvements in the understanding and diagnosis of CML are considered one of the most successful cancer medicine stories of the past century. 2 Instead of cytoreduction chemotherapy in the past, nowadays TKI is the standard treatment modality for CML.…”

Section: Discussionmentioning

confidence: 99%

Dasatinib‐related effusion lymphoma in a patient treated for chronic myeloid leukaemia

Miyagi

et al. 2020

Cytopathology

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This report presents the clinical, cytological and molecular features of pleural effusion lymphoma which developed in a patient treated for chronic myeloid leukaemia after five years of dasatinib therapy. The effusion lymphoma recurred three times but largely resolved after pleurocentesis and steroid treatment. More reports on such unusual cases may improve our understanding and management of patients with this rare adverse effect associated with dasatinib.