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CD5-Positive Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type: An Unusual Presentation.

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CD5-positive primary cutaneous diffuse large B-cell lymphoma-leg type (PCDLBCL-LT) is rare. This case report details a patient

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R-CHOPprimary cutaneous diffuse large B-cell lymphoma–leg typerituximab–bevacizumabskin

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Area of Science:

  • Hematology
  • Oncology
  • Dermatopathology

Background:

  • Primary cutaneous diffuse large B-cell lymphoma-leg type (PCDLBCL-LT) is an aggressive non-Hodgkin lymphoma.
  • CD5 expression in PCDLBCL-LT is rare and complicates diagnosis, broadening the differential to include other B-cell lymphomas.
  • Accurate diagnosis is crucial for appropriate patient management and monitoring.

Purpose of the Study:

  • To report a rare case of CD5-positive PCDLBCL-LT in an elderly female patient.
  • To highlight the diagnostic challenges and management of this distinct PCDLBCL-LT subgroup.
  • To emphasize the importance of CD5 testing in PCDLBCL-LT diagnosis.

Main Methods:

  • Case report of a 70-year-old female with a lower extremity cutaneous nodule.
  • Histopathological and immunohistochemical analysis including CD5, CD20, MUM1, BCL2, IgM, and cyclin D1.
  • Fluorescence in situ hybridization (FISH) for MYC, BCL2, and BCL6 rearrangements.
  • Treatment with R-CHOP chemotherapy followed by rituximab-bevacizumab for relapse.

Main Results:

  • Histopathology revealed a pandermal diffuse infiltrate of atypical large B-cells.
  • Immunohistochemistry confirmed a CD5+, CD20+, MUM1+, BCL2+, IgM+, cyclin D1- profile, supporting CD5+ PCDLBCL-LT.
  • FISH results were negative for MYC, BCL2, and BCL6 rearrangements, ruling out double-hit/triple-hit lymphoma.
  • The patient achieved initial complete response with R-CHOP, followed by relapse and a second complete remission with rituximab-bevacizumab.

Conclusions:

  • CD5 positivity defines a distinct subgroup of PCDLBCL-LT requiring careful diagnostic consideration.
  • Routine CD5 testing is essential for accurate diagnosis and differential diagnosis of PCDLBCL-LT.
  • This case underscores the importance of vigilant monitoring and tailored treatment strategies for CD5+ PCDLBCL-LT.