title: Blastic marginal zone lymphoma: a clinical and pathological study of 8 cases and review of the literature.
abstract: BACKGROUND
  Blastic transformation (BT) of marginal zone lymphoma or mucosa-associated lymphoid tissue lymphoma has been mainly reported in the spleen and stomach. Primary cutaneous marginal zone lymphoma that undergoes BT is rare and not well documented. We describe 8 patients with blastic primary cutaneous marginal zone lymphoma and compare the clinical, pathologic, and molecular findings of these patients with 10 cases previously reported in the literature.
  
  
  RESULTS
  The cases of blastic marginal zone lymphoma could be categorized into cases of de novo blastic marginal zone lymphoma and large-cell transformation arising in a background of a history of biopsy proven marginal zone lymphoma. The cases of de novo blastic marginal zone lymphoma occurred in elderly patients without any medical history. In each of the cases, the lesions were radiated, not treated, or treated with complete excision without any death due to lymphoma nor was there any evidence of extracutaneous dissemination. Large-cell transformation arising in background of marginal zone lymphoma typically occurred in patients who were younger; 2 of the 4 cases were immunocompromised. The clinical course in each of the cases was aggressive with 3 of the 4 patients succumbing to disseminated disease while 1 patient developed extracutaneous nodal disease. Phenotypically, there was an expression of CD5 in a total of 3 of the 8 cases and CD23 in 3 of the 8 cases. Commonality of B-cell clones was demonstrated in 2 cases where biopsies were available of both the less aggressive appearing marginal zone lymphoma and the transformed biopsies. Cytogenetic abnormalities associated with BT included a deletion of chromosome 7q in all cases tested.
  
  
  CONCLUSIONS
  Large-cell transformation arising in a patient with a history of marginal zone lymphoma portends a worse prognosis, including death from disseminated disease, whereas a de novo presentation of blastic marginal zone lymphoma may define a clinical course similar to other forms of low-grade cutaneous B-cell lymphoma. The expression of CD5 and CD23 may define a phenotypic profile associated with BT. It is possible that marginal zone lymphomas associated with CD5 and CD23 positivity should be followed more closely and/or treated with radiation and/or complete excision.
authors.affiliation: Department of Pathology and Laboratory Medicine, Weill Medical College of Cornell University, New York, NY 10065, USA. cym2003@med.cornell.edu
authors.forename: Cynthia M
authors.initials: CM
authors.lastname: Magro
authors.forename: Amy
authors.initials: A
authors.lastname: Yang
authors.forename: Garth
authors.initials: G
authors.lastname: Fraga
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001402:B-Lymphocytes / Q000276:immunology / Q000473:pathology*; D014408:Biomarkers, Tumor / Q000032:analysis; D001706:Biopsy; D018956:CD5 Antigens / Q000032:analysis; D002471:Cell Transformation, Neoplastic / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D002872:Chromosome Deletion; D002897:Chromosomes, Human, Pair 7; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping; D008213:Lymphocyte Activation; D018442:Lymphoma, B-Cell, Marginal Zone / Q000235:genetics / Q000276:immunology / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D011237:Predictive Value of Tests; D011379:Prognosis; D017455:Receptors, IgE / Q000032:analysis; D012307:Risk Factors; D012878:Skin Neoplasms / Q000235:genetics / Q000276:immunology / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D013997:Time Factors; D055815:Young Adult
chemical_list: D014408:Biomarkers, Tumor; D018956:CD5 Antigens; D017455:Receptors, IgE