Pyothorax-associated lymphoma: a peculiar clinicopathologic entity derived from B cells at late stage of differentiation and with occasional aberrant dual B- and T-cell phenotype.
We report 12 European cases of pyothorax-associated lymphomas occurring 30-67 years following artificial pneumothorax for pleuropulmonar tuberculosis. Eleven patients presented with a localized pleural tumor mass, whereas one patient also had liver involvement. Histologic examination showed a diffuse proliferation of large lymphoid cells with frequent plasmacytoid differentiation (n = 8), expressing CD20 (n = 10), CD79a (n = 11), and/or CD138 (n = 5) B-cell antigens. Aberrant expression of T-cell markers (CD2, CD3, CD4) was noted in five cases. The B-cell origin of lymphoma cells was confirmed by the demonstration of immunoglobulin light chain restriction or clonal B cell population in six cases. In 11 of 12 cases in situ hybridization disclosed Epstein-Barr virus genome in most tumor cells and immunohistochemistry a type III LMP-1+/ EBNA-2+ latency profile. HHV-8/ORF73 antigen was not detected in all tested cases (n = 11). All investigated cases (10 of 10) disclosed a uniform CD10-/BCL-6-/MUM1+/CD138+/- phenotype, consistent with a derivation from late germinal center (GC)/post-GC B cells. Clinical outcome was poor with a median survival time of 5 months. Only one patient was in complete remission after 34 months. This study further confirms that pyothorax-associated lymphoma represents a distinct clinicopathologic entity among diffuse large B-cell lymphoma, which is characterized by a peculiar clinical presentation, frequent plasmacytoid features, and a strong association with EBV. Moreover, we show that this lymphoma entity likely originates from B cells at a late stage of differentiation and occasionally shares an aberrant dual B/T phenotype.
Petitjean
Bruno
B
Département de Pathologie and EA2348, Hôpital Henri Mondor, AP-HP, Créteil, France.
Jardin
Fabrice
F
Joly
Bertrand
B
Martin-Garcia
Nadine
N
Tilly
Hervé
H
Picquenot
Jean-Michel
JM
Brière
Josette
J
Danel
Claire
C
Mehaut
Sylvie
S
Abd-Al-Samad
Issam
I
Copie-Bergman
Christiane
C
Delfau-Larue
Marie-Hélène
MH
Gaulard
Philippe
P
D000368:Aged; D000369:Aged, 80 and over; D001402:B-Lymphocytes / Q000473:pathology*; D014408:Biomarkers, Tumor / Q000378:metabolism; D001706:Biopsy; D002454:Cell Differentiation; D016724:Empyema, Pleural / Q000150:complications* / Q000473:pathology / Q000821:virology; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000473:pathology; D005260:Female; D018858:Germinal Center / Q000473:pathology / Q000821:virology; D004854:Herpesvirus 4, Human / Q000302:isolation & purification / Q000502:physiology; D006801:Humans; D007124:Immunoenzyme Techniques; D017403:In Situ Hybridization; D016393:Lymphoma, B-Cell / Q000150:complications* / Q000473:pathology / Q000821:virology; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D010997:Pleural Neoplasms / Q000150:complications* / Q000473:pathology / Q000821:virology; D011031:Pneumothorax, Artificial / Q000009:adverse effects; D013601:T-Lymphocytes / Q000473:pathology*
D014408:Biomarkers, Tumor