Marginal Zone Lymphomas: Management of Nodal, Splenic, and MALT NHL

Kahl, Brad S.; Yang, David T.

doi:10.1182/asheducation-2008.1.359

Cited by 45 publications

(55 citation statements)

References 42 publications

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“…Usually, guidelines for follicular lymphoma or chronic lymphocytic leukemia/ small lymphocytic lymphoma are adopted. 99 Different studies have reported different (combinations of) treatments, including watchful waiting, surgery, radiotherapy, different chemotherapeutic regimens, rituximab, and autologous stem cell transplantation. 9,[13][14][15][16][17][18][19] However, no optimal treatment strategy can be deduced from these studies.…”

Section: Treatment and Prognosismentioning

confidence: 99%

Recognizing nodal marginal zone lymphoma: recent advances and pitfalls. A systematic review

Brand

Krieken

2013

Haematologica

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The diagnosis of nodal marginal zone lymphoma is one of the remaining problem areas in hematopathology. Because no established positive markers exist for this lymphoma, it is frequently a diagnosis of exclusion, making distinction from other low-grade B-cell lymphomas difficult or even impossible. This systematic review summarizes and discusses the current knowledge on nodal marginal zone lymphoma, including clinical features, epidemiology and etiology, histology, and cytogenetic and molecular features. In particular, recent advances in diagnostics and pathogenesis are discussed. New immunohistochemical markers have become available that could be used as positive markers for nodal marginal zone lymphoma. These markers could be used to ensure more homogeneous study groups in future research. Also, recent gene expression studies and studies describing specific gene mutations have provided clues to the pathogenesis of nodal marginal zone lymphoma, suggesting deregulation of the nuclear factor kappa B pathway. Nevertheless, nodal marginal zone lymphoma remains an enigmatic entity, requiring further study to define its pathogenesis to allow an accurate diagnosis and tailored treatment. However, recent data indicate that it is not related to splenic or extranodal lymphoma, and that it is also not related to lymphoplasmacytic lymphoma. Thus, even though the diagnosis is not always easy, it is clearly a separate entity. ABSTRACT© F e r r a t a S t o r t i F o u n d a t i o n . Epidemiology and etiology NMZL is a rare lymphoma, accounting for 1.5-1.8% of lymphoid neoplasms. 9,10 The incidence of NMZL appears to be increasing, which is most likely due to a 'real' increase, rather than better recognition.11 Most studies report a median age around 60 years with a wide age distribution ranging from adolescence to patients over 90 years old. [9][10][11][12][13][14][15][16][17][18][19][20] Both sexes are affected with approximately equal frequency.Morphologically, NMZL resembles EMZL. EMZL is well known for its association with conditions that provide a chronic stimulation to the immune system, including chronic infections (e.g. Helicobacter pylori infection in gastric EMZL) and autoimmune conditions (e.g. salivary gland EMZL in Sjögren's syndrome). From this, one could hypothesize that NMZL, or a subset of NMZLs, might also be caused by specific chronic inflammatory conditions. Indeed, infections and autoimmune disorders have been reported in association with NMZL, but this evidence remains far from sufficient to establish a definitive role for these stimuli in lymphomagenesis. Recently, we encountered a case of what we had referred to as NMZL lymphoma in an axillary lymph node. However, we detected H. pylori by PCR analysis and a gastric biopsy revealed EMZL. This case implies that without extensive workup, including gastric biopsies, one cannot be sure that a case of NMZL really represents this entity.Hepatitis C virus (HCV) has been reported in a subset of NMZL patients. In an early but small study, monocytoid B-cell lymph...

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Section: Treatment and Prognosismentioning

confidence: 99%

Recognizing nodal marginal zone lymphoma: recent advances and pitfalls. A systematic review

Brand

Krieken

2013

Haematologica

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“…Inmunofenotípicamente expresan CD19,CD20,CD22 y PAX5 y no expresan CD25 y CD103, lo que ayuda a diferenciarlo de la tricoleucemia 15,16 . En esta paciente, si se hubiera podido contar con la determinación del inmunofenotipo en la primera hospitalización, se habría conocido el tipo específi co de linfoma que padecía, lo que hubiera permitido haber iniciado su terapia un año antes, evitando la complicación que motivó la comunicación de este caso.…”

Section: Discussionunclassified

“…La esplenectomía es la terapia de elección ya que corrige las citopenias, alivia los síntomas y cuando ello no es sufi ciente se ha usado quimioterapia (fl udarabina, pentostatin). En ancianos o pacientes con falla renal se ha utilizado rituximab con buenos resultados 15,16,28,29 .…”

Section: Discussionunclassified

Linfoma marginal del bazo asociado a acidosis láctica tipo B: Caso clínico

Vega

Rodríguez²,

Peña

et al. 2012

Rev. méd. Chile

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“…The optimal management of extranodal marginal zone B-cell lymphomas has not yet been clearly defined, especially in H. pylori-negative MALT lymphomas and refractory B-cell lymphomas (1,7). Although chemotherapy with CHOP may not be an optimal regimen, it is the standard treatment for patients with aggressive lymphomas (9).…”

Section: Discussionmentioning

confidence: 99%