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Selective IgM deficiency in CD30+ cutaneous lymphoproliferative disorder
Surbhi Saini1, Andrew J Dettore, Kanta J Bhambhani
1Division of Hematology/Oncology, Children's Hospital of Michigan, Wayne State University, Detroit, MI, USA.
Journal of Pediatric Hematology/Oncology
|April 26, 2011
Summary
A boy with cutaneous anaplastic large cell lymphoma and lymphomatoid papulosis developed selective immunoglobulin M deficiency post-chemotherapy. Low IgM+ and memory B cells suggest a link between this deficiency and chronic CD30+ skin disorders.
Area of Science:
- Immunology
- Dermatology
- Pediatric Oncology
Background:
- Primary cutaneous anaplastic large cell lymphoma (CALCL) is a rare T-cell lymphoma.
- Lymphomatoid papulosis (LyP) is a benign lymphoproliferative disorder that can be associated with other lymphomas.
Observation:
- A 13-year-old boy presented with an ulcerative facial lesion diagnosed as CALCL with lymph node involvement.
- He had a history of recurrent, self-limited LyP lesions.
- Following chemotherapy, the CALCL tumor regressed, but LyP lesions persisted, exhibiting a characteristic healing halo.
Findings:
- The patient developed selective immunoglobulin M (IgM) deficiency, with persistently low IgM levels four years post-chemotherapy.
- Low peripheral blood IgM+ B cells and memory B cells were observed.
- No recurrent infections were noted despite the IgM deficiency.
Implications:
- This case suggests a potential cause-effect relationship between selective IgM deficiency and chronic CD30+ cutaneous lymphoproliferative disorders like LyP and CALCL.
- Further research is warranted to explore the immunological mechanisms underlying this association.
- Understanding this link may inform future diagnostic and therapeutic strategies for pediatric patients with these conditions.
