Subcutaneous T-cell lymphoma

Source: Wikipedia, the free encyclopedia.
Subcutaneous T-cell lymphoma
SpecialtyOncology, cutaneous oncology
ComplicationsHPS (hemophagocytic syndrome)
Diagnostic methodRequires biopsy confirmation by expert hemopathologist
Differential diagnosisLupus panniculitis, cutaneous gamma delta T Cell lymphoma

Subcutaneous T-cell lymphoma (also known as a "panniculitis-like T-cell lymphoma") is a cutaneous condition that most commonly presents in young adults, and is characterized by subcutaneous nodules.[1]: 739  Common symptoms include fever, fatigue, and pancytopenia.

Diagnosis

Classification

Subcutaneous panniculitis-like T-cell lymphoma is a subtype of

B-cells
and T-cells. PTCL specifically affects T-cells, and results when T-cells develop and grow abnormally.

Subcutaneous panniculitis-like T-cell lymphoma is a rare type of lymphoma that infiltrates the subcutaneous fat but does not involve the skin. There are two subtypes – alpha-beta and gamma-delta. Patients with the gamma-delta subtype have a more aggressive clinical course.[3]

It is described as

CD56 usually negative.[4]

Treatment

Historically, subcutaneous panniculitis-like T-cell lymphoma, as with other types of T-cell lymphoma, was treated similarly to B-cell lymphomas. However, in recent years, scientists have developed techniques to better recognize the different types of lymphomas, such as PTCL. It is now understood that PTCL behaves differently from B-cell lymphomas and therapies are being developed that specifically target these types of lymphoma. Currently, however, there are no drugs approved by the

Novel approaches to the treatment of PTCL in the relapsed or refractory setting are under investigation.

CHOP or CHOP-like regimens have been used.[13]

Research

Pralatrexate is one compound currently under investigation to add to the frontline treatment of PTCL. It is currently approved by the FDA for relapsed or refractory T cell lymphoma. Side effects include mouth sores, which can be severe.[citation needed]

See also

References

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  2. ^ Swerdlow SH, WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, 2008
  3. PMID 18954748
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  9. ^ d’Amore F, et al. Blood. 2006;108:A401
  10. PMID 12011124
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External links