Splenic marginal zone lymphoma

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Splenic marginal zone lymphoma
SpecialtyHematology, oncology

Splenic marginal zone lymphoma (SMZL) is a type of

lymph nodes are involved along with the peripheral blood. The neoplastic cells circulating in the peripheral blood are termed villous lymphocytes due to their characteristic appearance.[1]

Cause

The cell of origin is postulated to be a post-germinal center B-cell with an unknown degree of differentiation.[1] SMZL is a form of cancer known to be associated with Hepatitis C virus infection.[citation needed]

Molecular biology

Immunophenotype

Antigen Status
CD20 Positive
CD79a
Positive
CD5 Negative
CD10
Negative
CD23 Negative
CD43 Negative
cyclin D1 Negative

The relevant markers that define the immunophenotype for SMZL are shown in the adjacent table.[2] [3] The lack of

cyclin-D1 expression.[4]

Genetics

Clonal rearrangements of the

immunoglobulin genes (heavy and light chains) are frequently seen.[5]
The deletion 7q21-32 is seen in 40% of SMZL patients, and translocations of the CDK6 gene located at 7q21 have also been reported.[6]

Diagnosis

lymph nodes adjacent to the spleen, if involved, show an effaced architecture without preservation of the marginal zone seen in the spleen.[1]

Circulating lymphoma cells are sometimes present in peripheral blood, and they occasionally show short villi at the poles of cells and plasmacytoid differentiation.[1][8]

hyperviscosity.[9][10]

blasts.[10][11] The red pulp is always involved, with both nodules of larger neoplastic cells and sheets of the small neoplastic lymphocytes. Other features that may be seen include sinus invasion, epithelial histocytes, and plasmacytic differentiation of neoplastic cells.[citation needed
]

Prognosis

Three-quarters of patients survive five or more years; more than half of patients with SMZL survive more than a decade after diagnosis.[12]

Patients who have a hemoglobin level of less than 12 g/dL, a lactate dehydrogenase level higher than normal, and/or a blood serum albumin levels of less than 3.5 g/dL are likely to have more an aggressive disease course and a shorter survival.[12] However, even high-risk patients have even odds of living for five years after diagnosis.[12]

Some genetic mutations, such as mutations in NOTCH2, are also correlated with shorter survival.[citation needed]

Epidemiology

Less than 1% of all lymphomas are splenic marginal zone lymphomas[13] and it is postulated that SMZL may represent a large fraction of unclassifiable CD5- chronic lymphocytic leukemias.[1] The typical patient is over the age of 50, and gender preference has been described.[9]

Synonyms

Under older classification systems, the following names were used:[1]

Classification system Name
Rappaport well-differentiated lymphocytic lymphoma
Lukes-Collins small lymphocytic lymphoma
Working Formulation small lymphocytic lymphoma
FAB splenic lymphoma with circulating villous lymphocytes

See also

References

  1. ^
    ISBN 978-92-832-2411-2.{{cite book}}: CS1 maint: multiple names: authors list (link
    )
  2. ^ Isaacson PG, Matutes E, Burke M, Catovsky D (1 December 1994). "The histopathology of splenic lymphoma with villous lymphocytes". Blood. 84 (11): 3828–34.
    PMID 7949139
    .
  3. .
  4. ^ Savilo E, Campo E, Mollejo M, et al. (July 1998). "Absence of cyclin D1 protein expression in splenic marginal zone lymphoma". Mod. Pathol. 11 (7): 601–6.
    PMID 9688179
    .
  5. .
  6. ^ Corcoran MM, Mould SJ, Orchard JA, et al. (November 1999). "Dysregulation of cyclin dependent kinase 6 expression in splenic marginal zone lymphoma through chromosome 7q translocations". Oncogene. 18 (46): 6271–7.
    PMID 10597225
    .
  7. .
  8. .
  9. ^ .
  10. ^ .
  11. .
  12. ^ .
  13. PMID 9704731. Archived from the original
    on 2013-04-15.

External links