Journal of the Formosan Medical Association
Volume 109, Issue 3 , Pages 185-191, March 2010

Intravascular Large B cell Lymphoma in Taiwan: An Asian Variant of Non-germinal-center Origin

  • Min-Shu Hsieh

      Affiliations

    • Department of Pathology, National Taiwan University Hospital, Yun-Lin Branch, Yun-Lin, Taipei, Taiwan
    • ,
  • Yi-Chen Yeh

      Affiliations

    • Department of Pathology, Taipei Veterans General Hospital, Taipei, Taiwan
    • ,
  • Yueh-Hung Chou

      Affiliations

    • Department of Pathology, Far Eastern Memorial Hospital, Taipei, Taiwan
    • ,
  • Chung-Wu Lin

      Affiliations

    • Department of Pathology, National Taiwan University Hospital, Taipei, Taiwan
    • Corresponding Author InformationCorrespondence to: Dr Chung-Wu Lin, Department of Pathology, National Taiwan University Hospital, 7 Chung-Shan South Road, Taipei, Taiwan

Received 17 February 2009; received in revised form 20 May 2009; accepted 22 July 2009.

Article Outline

Background/Purpose

Intravascular large B cell lymphoma (IVLBCL) is a rare variant of diffuse large B cell lymphoma. We reported the clinical and immunohistochemical characteristics of 10 cases of IVLBCL from Taiwan between 1995 and 2008.

Methods

Clinical data were reviewed and immunoperoxidase stains were performed with antibodies against CD20, CD10, Bcl-6, MUM1, and CD5.

Results

There were eight males and two females with a median age of 59 years. Patients presented with dyspnea (5/10), fever (7/10), splenomegaly (5/10), and bone marrow involvement (8/10). Anemia (9/10), thrombocytopenia (6/10), and elevated serum lactate dehydrogenase or ferritin levels (8/10) were also common. Nine cases were CD20+CD10−Bcl-6−, similar to non-germinal center B cells. Six out of seven patients survived after chemotherapy, but three cases with thrombocytopenia that precluded chemotherapy died within 2 months.

Conclusion

Our cases of IVLBCL had a non-germinal center B origin and belonged to the Asian variant of this disease. The liver, spleen, and bone marrow, but rarely the skin or brain, were involved. Thrombocytopenia is a major risk factor for mortality in these cases.

Key Words:  angiotropic lymphoma , fever , intravascular large B cell lymphoma

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References 

  1. Ffleger VL , Tappeiner J . Zur Kenntnis der systemisierten Endotheliomatose der cutanen Blutgefasse . Hautarzt . 1959;10:359–363
  2. Nakamura S , Ponzoni M , Campo E . Intravascular large B-cell lymphoma . In:  Swerdlow SH ,  Campo E ,  Harris NL , et al. editor. World Health Organization classification of tumors of haematopoietic and lymphoid tissues . Lyon: IARC press; 2008;p. 252–253
  3. Murase T , Yamaguchi M , Suzuki R , et al.   Intravascular large B-cell lymphoma (IVLBCL): a clinicopathologic study of 96 cases with special reference to the immunophenotypic heterogeneity of CD5 . Blood . 2007;109:478–485
  4. Ferreri AJ , Dognini GP , Campo E , et al.   Variations in clinical presentation, frequency of hemophagocytosis and clinical behavior of intravascular lymphoma diagnosed in different geographical regions . Haematologica . 2007;92:486–492
  5. Ponzoni M , Ferreri AJ , Campo E , et al.   Definition, diagnosis, and management of intravascular large B-cell lymphoma: proposals and perspectives from an international consensus meeting . J Clin Oncol . 2007;25:3168–3173
  6. Bea S , Zettl A , Wright G , et al.   Diffuse large B-cell lymphoma subgroups have distinct genetic profiles that influence tumor biology and improve gene-expression-based survival prediction . Blood . 2005;106:3183–3190
  7. Hunt KE , Reichard KK . Diffuse large B-cell lymphoma . Arch Pathol Lab Med . 2008;132:118–124
  8. Matsue K , Asada N , Takeuchi M , et al.   A clinicopathological study of 13 cases of intravascular lymphoma: experience in a single institution over a 9-yr period . Eur J Haematol . 2008;80:236–244
  9. Sheibani K , Battifora H , Winberg CD , et al.   Further evidence that “malignant angioendotheliomatosis” is an angiotropic large-cell lymphoma . N Engl J Med . 1986;314:943–948
  10. Molina A , Lombard C , Donlon T , et al.   Immunohisto-chemical and cytogenetic studies indicate that malignant angioendotheliomatosis is a primary intravascular (angiotropic) lymphoma . Cancer . 1990;66:474–479
  11. Yamaguchi M , Seto M , Okamoto M , et al.   De novo CD5 diffuse large B-cell lymphoma: a clinicopathologic study of 109 patients . Blood . 2002;99:815–821
  12. Murase T , Nakamura S , Kawauchi K , et al.   An Asian variant of intravascular large B-cell lymphoma: clinical, pathological and cytogenetic approaches to diffuse large B-cell lymphoma associated with haemophagocytic syndrome . Br J Haematol . 2000;111:826–834
  13. Kanda M , Suzumiya J , Ohshima K , et al.   Intravascular large cell lymphoma: clinicopathological, immunohisto-chemical and molecular genetic studies . Leuk Lymphoma . 1999;34:569–580
  14. Hsu YH , Tseng BY , Shyu WC , et al.   Intravascular lymphomatosis mimicking acute disseminated encephalomyelitis: a case report . Kaohsiung J Med Sci . 2005;21:93–97
  15. Chen SH , Yu KH , Lin TL , et al.   Gallium scan-prompted skin biopsy revealed intravascular large B-cell lymphoma in a patient who presented with fever of unknown origin . Clin Nucl Med . 2009;34:318–320

PII: S0929-6646(10)60041-1

doi:10.1016/S0929-6646(10)60041-1

Journal of the Formosan Medical Association
Volume 109, Issue 3 , Pages 185-191, March 2010

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