 |
|
Cytophagic Histiocytic Panniculitis and Subcutaneous Panniculitis-like T-Cell Lymphoma
Report of 7 Cases
Angelo V. Marzano, MD;
Emilio Berti, MD;
Marco Paulli, MD;
Ruggero Caputo, MD
Arch Dermatol. 2000;136:889-896.
Background Cytophagic histiocytic panniculitis (CHP) is a rare subtype of panniculitis that usually follows a fatal course, with a terminal hemophagocytic syndrome. Recent reports on a subset of peripheral T-cell lymphoma named subcutaneous panniculitis-like T-cell lymphoma (SPTL) raised the question about the relationship between these entities.
Observations We describe 7 patients in the study: 1with fatal CHP, 4 with SPTL, and 2 with long-term CHP. The 5 patients with fatal CHP and SPTL died of complications of hemophagocytic syndrome, with a disease duration ranging from 8 to 74 months. The other 2 patients were still alive 6 and 41 years after disease onset. Immunohistochemical results proved that 2 of the SPTL cases were type  / and expressed the cytotoxic/suppressor antigen CD8, while the other 2 were type  / and were positive for the natural killer–associated antigen CD56. In these 4 cases, molecular biology studies by polymerase chain reaction detected T-cell receptor gene rearrangement, indicating a clonal process. In contrast, in the 2 patients who had long-term CHP, the polymerase chain reaction results failed to disclose clonality. In the subject with fatal CHP, genotypic analysis was not performed.
Conclusion Our observations suggest that CHP and SPTL may span a clinicopathologic spectrum in which there is a natural disease progression from CHP to SPTL.
From the Institute of Dermatological Sciences, University of Milan, and IRCCS Ospedale Maggiore, Milan (Drs Marzano, Berti, and Caputo) and Anatomic Pathology, IRCCS Policlinico S. Matteo and University of Pavia, Pavia (Dr Paulli), Italy.
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati  Twitter
What's this?
RELATED ARTICLES
Cytophagic Histiocytic Panniculitis—a Critical Reappraisal
Mark R. Wick and James W. Patterson
Arch Dermatol. 2000;136(7):922-924.
EXTRACT
| FULL TEXT
Archives of Dermatology Reader's Choice: Continuing Medical Education
Arch Dermatol. 2000;136(7):946-947.
FULL TEXT
THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES
|
Subcutaneous panniculitis-like T-cell lymphoma: definition, classification, and prognostic factors: an EORTC Cutaneous Lymphoma Group Study of 83 cases
Willemze et al.
Blood 2008;111:838-845.
ABSTRACT
| FULL TEXT
Cytophagic histiocytic panniculitis with fatal haemophagocytic lymphohistiocytosis in a paediatric patient with perforin gene mutation
Chen et al.
J. Clin. Pathol. 2007;60:1168-1169.
FULL TEXT
T-cell non-Hodgkin lymphoma
Rizvi et al.
Blood 2006;107:1255-1264.
ABSTRACT
| FULL TEXT
Fever and Necrotic Nodules on the Extremities--Diagnosis
Arch Dermatol 2005;141:1035-1040.
FULL TEXT
WHO-EORTC classification for cutaneous lymphomas
Willemze et al.
Blood 2005;105:3768-3785.
ABSTRACT
| FULL TEXT
CD56+ hematological neoplasms presenting in the skin: a retrospective analysis of 23 new cases and 130 cases from the literature
Bekkenk et al.
Ann Oncol 2004;15:1097-1108.
ABSTRACT
| FULL TEXT
CD56+ Lymphoma With Skin Involvement: Clinicopathologic Features and Classification
Arch Dermatol 2004;140:427-436.
Gamma-delta T-cell phenotype is associated with significantly decreased survival in cutaneous T-cell lymphoma
Toro et al.
Blood 2003;101:3407-3412.
ABSTRACT
| FULL TEXT
T Cell and NK Cell Lymphoproliferative Disorders
Greer et al.
ASH Education Book 2001;2001:259-281.
ABSTRACT
| FULL TEXT
Cytophagic Histiocytic Panniculitis--a Critical Reappraisal
Wick and Patterson
Arch Dermatol 2000;136:922-924.
FULL TEXT
|
