Splenic marginal zone lymphoma with or without villous lymphocytes: Hematologic findings and outcomes in a series of 57 patients

Vito Franco, Ada Maria Florena, Claudio Tripodo, Emilio Iannitto, Maura Colosio, Achille Ambrosetti, Giovanni Pizzolo, Fabio Menestrina

Risultato della ricerca: Article

53 Citazioni (Scopus)

Abstract

BACKGROUND. Splenic marginal zone lymphoma (SMZL) is a well defined pathologic entity. However, questions regarding the bone marrow infiltration rate, the minimal diagnostic data set, and therapy remain unanswered. METHODS. Clinical-pathologic features and outcomes of 57 consecutive patients who had splenomegaly with no clinically significant lymphadenomegaly and who were diagnosed with SMZL with or without (±) villous lymphocytes (VL) were reviewed. RESULTS. SMVL ± VL occurred mostly in elderly males (median age, 62 years ±10 years; male-to-female ratio, (1.85). Anemia was recorded in 49% of patients, and 30% of patients had moderate thrombocytopenia. Leukocytosis and leukopenia were found in 33% and 14% of patients, respectively, and typical VL were found in 84% of patients. Serology for hepatitis C virus infection was positive in 16% of patients, and a small monoclonal component was detected in 36% of patients. The bone marrow was infiltrated with an intrasinusoidal component in all patients. Thirteen patients were monitored using a watch-and-see policy, and they remained alive 1-5 years after diagnosis. Overall, 21 patients (36%) underwent splenectomy; and, in all patients, the diagnosis of SMZL was confirmed histologically in the surgical specimens. Twenty-five patients received single-agent therapy, which included either alkylators or pentostatine, and they achieved an overall response rate (ORR) of 65% and 87%, respectively: Polychemotherapy was administered to 6 patients (ORR, 83%). The median survival for all patients in the series was not reached, and it is expected that 70% of patients will be alive at 5 years. CONCLUSIONS. Up to 20% of patients who had SMZL ± VL could be monitored using a watch-and-wait policy. The bone marrow intrasinusoidal infiltration pattern may be a valuable diagnostic hallmark, thus obviating diagnostic splenectomy. The issues regarding prognostic stratification and the best therapeutic strategy need to be addressed in properly designed, prospective trials. © 2004 American Cancer Society.
Lingua originaleEnglish
pagine (da-a)2050-2057
Numero di pagine8
RivistaCancer
Volume101
Stato di pubblicazionePublished - 2004

Fingerprint

Lymphoma
Lymphocytes
Bone Marrow
Splenectomy
Alkylating Agents
Splenomegaly
Leukocytosis
Leukopenia
Virus Diseases
Serology
Combination Drug Therapy
Hepacivirus
Thrombocytopenia
Anemia
Therapeutics

All Science Journal Classification (ASJC) codes

  • Oncology
  • Cancer Research

Cita questo

Splenic marginal zone lymphoma with or without villous lymphocytes: Hematologic findings and outcomes in a series of 57 patients. / Franco, Vito; Florena, Ada Maria; Tripodo, Claudio; Iannitto, Emilio; Colosio, Maura; Ambrosetti, Achille; Pizzolo, Giovanni; Menestrina, Fabio.

In: Cancer, Vol. 101, 2004, pag. 2050-2057.

Risultato della ricerca: Article

Franco, Vito ; Florena, Ada Maria ; Tripodo, Claudio ; Iannitto, Emilio ; Colosio, Maura ; Ambrosetti, Achille ; Pizzolo, Giovanni ; Menestrina, Fabio. / Splenic marginal zone lymphoma with or without villous lymphocytes: Hematologic findings and outcomes in a series of 57 patients. In: Cancer. 2004 ; Vol. 101. pagg. 2050-2057.
@article{a1b2d4cef1624654b0d67a71921fd58c,
title = "Splenic marginal zone lymphoma with or without villous lymphocytes: Hematologic findings and outcomes in a series of 57 patients",
abstract = "BACKGROUND. Splenic marginal zone lymphoma (SMZL) is a well defined pathologic entity. However, questions regarding the bone marrow infiltration rate, the minimal diagnostic data set, and therapy remain unanswered. METHODS. Clinical-pathologic features and outcomes of 57 consecutive patients who had splenomegaly with no clinically significant lymphadenomegaly and who were diagnosed with SMZL with or without (±) villous lymphocytes (VL) were reviewed. RESULTS. SMVL ± VL occurred mostly in elderly males (median age, 62 years ±10 years; male-to-female ratio, (1.85). Anemia was recorded in 49{\%} of patients, and 30{\%} of patients had moderate thrombocytopenia. Leukocytosis and leukopenia were found in 33{\%} and 14{\%} of patients, respectively, and typical VL were found in 84{\%} of patients. Serology for hepatitis C virus infection was positive in 16{\%} of patients, and a small monoclonal component was detected in 36{\%} of patients. The bone marrow was infiltrated with an intrasinusoidal component in all patients. Thirteen patients were monitored using a watch-and-see policy, and they remained alive 1-5 years after diagnosis. Overall, 21 patients (36{\%}) underwent splenectomy; and, in all patients, the diagnosis of SMZL was confirmed histologically in the surgical specimens. Twenty-five patients received single-agent therapy, which included either alkylators or pentostatine, and they achieved an overall response rate (ORR) of 65{\%} and 87{\%}, respectively: Polychemotherapy was administered to 6 patients (ORR, 83{\%}). The median survival for all patients in the series was not reached, and it is expected that 70{\%} of patients will be alive at 5 years. CONCLUSIONS. Up to 20{\%} of patients who had SMZL ± VL could be monitored using a watch-and-wait policy. The bone marrow intrasinusoidal infiltration pattern may be a valuable diagnostic hallmark, thus obviating diagnostic splenectomy. The issues regarding prognostic stratification and the best therapeutic strategy need to be addressed in properly designed, prospective trials. {\circledC} 2004 American Cancer Society.",
author = "Vito Franco and Florena, {Ada Maria} and Claudio Tripodo and Emilio Iannitto and Maura Colosio and Achille Ambrosetti and Giovanni Pizzolo and Fabio Menestrina",
year = "2004",
language = "English",
volume = "101",
pages = "2050--2057",
journal = "Cancer",
issn = "0008-543X",
publisher = "John Wiley and Sons Inc.",

}

TY - JOUR

T1 - Splenic marginal zone lymphoma with or without villous lymphocytes: Hematologic findings and outcomes in a series of 57 patients

AU - Franco, Vito

AU - Florena, Ada Maria

AU - Tripodo, Claudio

AU - Iannitto, Emilio

AU - Colosio, Maura

AU - Ambrosetti, Achille

AU - Pizzolo, Giovanni

AU - Menestrina, Fabio

PY - 2004

Y1 - 2004

N2 - BACKGROUND. Splenic marginal zone lymphoma (SMZL) is a well defined pathologic entity. However, questions regarding the bone marrow infiltration rate, the minimal diagnostic data set, and therapy remain unanswered. METHODS. Clinical-pathologic features and outcomes of 57 consecutive patients who had splenomegaly with no clinically significant lymphadenomegaly and who were diagnosed with SMZL with or without (±) villous lymphocytes (VL) were reviewed. RESULTS. SMVL ± VL occurred mostly in elderly males (median age, 62 years ±10 years; male-to-female ratio, (1.85). Anemia was recorded in 49% of patients, and 30% of patients had moderate thrombocytopenia. Leukocytosis and leukopenia were found in 33% and 14% of patients, respectively, and typical VL were found in 84% of patients. Serology for hepatitis C virus infection was positive in 16% of patients, and a small monoclonal component was detected in 36% of patients. The bone marrow was infiltrated with an intrasinusoidal component in all patients. Thirteen patients were monitored using a watch-and-see policy, and they remained alive 1-5 years after diagnosis. Overall, 21 patients (36%) underwent splenectomy; and, in all patients, the diagnosis of SMZL was confirmed histologically in the surgical specimens. Twenty-five patients received single-agent therapy, which included either alkylators or pentostatine, and they achieved an overall response rate (ORR) of 65% and 87%, respectively: Polychemotherapy was administered to 6 patients (ORR, 83%). The median survival for all patients in the series was not reached, and it is expected that 70% of patients will be alive at 5 years. CONCLUSIONS. Up to 20% of patients who had SMZL ± VL could be monitored using a watch-and-wait policy. The bone marrow intrasinusoidal infiltration pattern may be a valuable diagnostic hallmark, thus obviating diagnostic splenectomy. The issues regarding prognostic stratification and the best therapeutic strategy need to be addressed in properly designed, prospective trials. © 2004 American Cancer Society.

AB - BACKGROUND. Splenic marginal zone lymphoma (SMZL) is a well defined pathologic entity. However, questions regarding the bone marrow infiltration rate, the minimal diagnostic data set, and therapy remain unanswered. METHODS. Clinical-pathologic features and outcomes of 57 consecutive patients who had splenomegaly with no clinically significant lymphadenomegaly and who were diagnosed with SMZL with or without (±) villous lymphocytes (VL) were reviewed. RESULTS. SMVL ± VL occurred mostly in elderly males (median age, 62 years ±10 years; male-to-female ratio, (1.85). Anemia was recorded in 49% of patients, and 30% of patients had moderate thrombocytopenia. Leukocytosis and leukopenia were found in 33% and 14% of patients, respectively, and typical VL were found in 84% of patients. Serology for hepatitis C virus infection was positive in 16% of patients, and a small monoclonal component was detected in 36% of patients. The bone marrow was infiltrated with an intrasinusoidal component in all patients. Thirteen patients were monitored using a watch-and-see policy, and they remained alive 1-5 years after diagnosis. Overall, 21 patients (36%) underwent splenectomy; and, in all patients, the diagnosis of SMZL was confirmed histologically in the surgical specimens. Twenty-five patients received single-agent therapy, which included either alkylators or pentostatine, and they achieved an overall response rate (ORR) of 65% and 87%, respectively: Polychemotherapy was administered to 6 patients (ORR, 83%). The median survival for all patients in the series was not reached, and it is expected that 70% of patients will be alive at 5 years. CONCLUSIONS. Up to 20% of patients who had SMZL ± VL could be monitored using a watch-and-wait policy. The bone marrow intrasinusoidal infiltration pattern may be a valuable diagnostic hallmark, thus obviating diagnostic splenectomy. The issues regarding prognostic stratification and the best therapeutic strategy need to be addressed in properly designed, prospective trials. © 2004 American Cancer Society.

UR - http://hdl.handle.net/10447/203779

M3 - Article

VL - 101

SP - 2050

EP - 2057

JO - Cancer

JF - Cancer

SN - 0008-543X

ER -