Page 1440 - Hematology_ Basic Principles and Practice ( PDFDrive )

1286 Part VII Hematologic Malignancies

Armitage JO, Weisenburger DD: New approach to classifying non-Hodgkin’s
Pathobiology and Differential Diagnosis lymphomas: clinical features of the major histologic subtypes. Non-
Hodgkin’s Lymphoma Classification Project. J Clin Oncol 16:2780,
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Berger F, Felman P, Thieblemont C, et al: Non-MALT marginal zone B-cell
As with SMZL, some series have reported an association with HCV lymphomas: a description of clinical presentation and outcome in 124
in up to one-fourth of NMZL cases, but with a strong geographic patients. Blood 95:1950, 2000.
variation (mostly Italian and Spanish series). 209,210,212,213 No other clear Chacon JI, Mollejo M, Munoz E, et al: Splenic marginal zone lymphoma:
association has been described. clinical characteristics and prognostic factors in a series of 60 patients.
Blood 100:1648, 2002.
Craig VJ, Arnold I, Gerke C, et al: Gastric MALT lymphoma B cells express
Histology and Immunophenotype polyreactive, somatically mutated immunoglobulins. Blood 115:581,
2010.
The histology and immunophenotype of NMZL resembles that of Escalon MP, Champlin RE, Saliba RM, et al: Nonmyeloablative allogeneic
215
ENMZL or SMZL (see Fig. 79.3C). The frequent presence of hematopoietic transplantation: a promising salvage therapy for patients
monocytoid B cells explains why this disease has been previously with non-Hodgkin’s lymphoma whose disease has failed a prior autolo-
216
called monocytoid B-cell lymphoma and NMZL with or without gous transplantation. J Clin Oncol 22:2419, 2004.
monocytoid B cells. A primary ENMZL should always be ruled out Fischbach W, Goebeler ME, Ruskone-Fourmestraux A, et al: Most patients
with appropriate studies (such as endoscopy) because 30%–40% of with minimal histological residuals of gastric MALT lymphoma after
cases presenting as NMZL may in fact represent nodal dissemination successful eradication of Helicobacter pylori can be managed safely by a
of an ENMZL of MALT type. 217,218 watch and wait strategy: experience from a large international series. Gut
56:1685, 2007.
Hancock BW, Qian W, Linch D, et al: Chlorambucil versus observation
Genetics after anti-Helicobacter therapy in gastric MALT lymphomas: results of
the international randomised LY03 trial. Br J Haematol 144:367, 2009.
Complex genetic abnormalities are usually observed in NMZL, with Hermine O, Lefrere F, Bronowicki JP, et al: Regression of splenic lymphoma
the most frequent being partial trisomies of chromosomes 3 and 18, with villous lymphocytes after treatment of hepatitis C virus infection.
affecting the same regions as in ENMZL. None of the characteristic N Engl J Med 347:89, 2002.
77
translocations of ENMZL are seen in NMZL, however. Further- Ho L, Davis RE, Conne B, et al: MALT1 and the API2-MALT1 fusion act
more, del(7q) is also not observed. More than 75% of cases have between CD40 and IKK and confer NF-κ B-dependent proliferative
mutated immunoglobulin genes. 210,219 Of interest, different VH advantage and resistance against FAS-induced cell death in B cells. Blood
immunoglobulin gene segments are predominantly involved in 105:2891, 2005.
HCV-positive and HCV-negative patients, raising the possibility that Husain A, Roberts D, Pro B, et al: Meta-analyses of the association between
distinct antigens drive different underlying chronic immune stimula- Chlamydia psittaci and ocular adnexal lymphoma and the response of
tion processes. 220 ocular adnexal lymphoma to antibiotics. Cancer 110:809, 2007.
Lecuit M, Abachin E, Martin A, et al: Immunoproliferative small intestinal
disease associated with Campylobacter jejuni. N Engl J Med 350:239, 2004.
Therapy Liu H, Ruskon-Fourmestraux A, Lavergne-Slove A, et al: Resistance of
t(11;18) positive gastric mucosa-associated lymphoid tissue lymphoma
Few data are available to guide treatment of NMZL, and most recom- to Helicobacter pylori eradication therapy. Lancet 357:39, 2001.
9
mendations are extrapolated from the management of FL. As in Malfertheiner P, Megraud F, O’Morain C, et al: Current concepts in the
other indolent lymphomas, expectant observation is appropriate for management of Helicobacter pylori infection: the Maastricht III Consensus
asymptomatic patients. Radiation therapy may be curative for early Report. Gut 56:772, 2007.
disease. Symptomatic advanced disease can be managed with the Nathwani BN, Anderson JR, Armitage JO, et al: Marginal zone B-cell lym-
same general approach described for advanced ENMZL, although phoma: a clinical comparison of nodal and mucosa-associated lymphoid
there is usually a tendency to use combination chemoimmunotherapy tissue types. Non-Hodgkin’s Lymphoma Classification Project. J Clin
identical to that for FL as first-line treatment because of the generally Oncol 17:2486, 1999.
worse outcomes with NMZL as compared with ENMZL. Olszewski AJ, Castillo JJ: Survival of patients with marginal zone lymphoma:
analysis of the Surveillance, Epidemiology, and End Results database.
Cancer 119:629, 2013.
Prognosis Rinaldi A, Mian M, Chigrinova E, et al: Genome-wide DNA profiling of
marginal zone lymphomas identifies subtype-specific lesions with an
Most series report worse prognosis for NMZL when compared with impact on the clinical outcome. Blood 117:1595, 2011.
32
the other forms of MZL. Overall survival rates at 5 years vary from Rosebeck S, Madden L, Jin X, et al: Cleavage of NIK by the API2-MALT1
55% to 70% in most series, except for those containing a greater fusion oncoprotein leads to noncanonical NF-κB activation. Science
proportion of early-stage disease, which report 5-year survivals of up 331:468, 2011.
to 80%. Five-year progression-free survival is around 30%. Relapse Ruskone-Fourmestraux A, Fischbach W, Aleman BM, et al: EGILS consensus
in extranodal sites is rare. As in other lymphomas, the IPI correlates report. Gastric extranodal marginal zone B-cell lymphoma of MALT. Gut
with outcomes, as does the Follicular Lymphoma International 60:747, 2011.
221
Prognostic Index (FLIPI) (see Chapter 80). In a series of 47 Salido M, Baro C, Oscier D, et al: Cytogenetic aberrations and their prog-
patients, those with low-, intermediate-, and poor-risk FLIPI scores nostic value in a series of 330 splenic marginal zone B-cell lymphomas:
had a 5-year overall survival of approximately 90%, 70% and 35%, a multicenter study of the Splenic B-Cell Lymphoma Group. Blood
respectively. 209 116:1479, 2010.
Schechter NR, Portlock CS, Yahalom J: Treatment of mucosa-associated
lymphoid tissue lymphoma of the stomach with radiation alone. J Clin
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Seligmann M, Danon F, Hurez D, et al: Alpha-chain disease: a new immu-
Al-Saleem T, Al-Mondhiry H: Immunoproliferative small intestinal disease noglobulin abnormality. Science 162:1396, 1968.
(IPSID): a model for mature B-cell neoplasms. Blood 105:2274, Senff NJ, Noordijk EM, Kim YH, et al: European Organization for Research
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