Subcutaneous panniculitis-like t-cell lymphoma C84.5

Author: Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 29.10.2020

Dieser Artikel auf Deutsch

Synonym(s)

CTCL-panniculitis-like; Panniculitis-like cutaneous T-cell lymphoma; Panniculitis-like T-cell lymphoma of the skin; Subcutaneous panniculitis-like T-cell lymphoma

History
This section has been translated automatically.

Gonzales 1991

Definition
This section has been translated automatically.

Very rare T-cell lymphoma (CTCL) of elderly people (>50 years) with primary location in subcutaneous fatty tissue.

Manifestation
This section has been translated automatically.

Preferably persons > 50 years. Rarely described in childhood. No gender preference.

Localization
This section has been translated automatically.

especially the lower extremity.

Clinical features
This section has been translated automatically.

Begin with a clinical picture "reminiscent of an infection" with a pronounced general feeling of illness, fever, weight loss.

The skin findings are untypical for a cutaneous T-cell lymphoma. There are mostly symptomless or slightly painful, 5.0-20.0 cm in diameter, subcutaneous, red (inflammatory), plate-like or nodular indurations, so that the first clinical impression is more reminiscent of a nodosum erythema or an atypically localized erythema induratum. Long lasting recurrent courses with spontaneous remissions are the rule. Ulcerations are the exception (in about 6% of patients).

If a hemophagocytosis syndrome is detected (approximately in 17% of patients), the prognostically unfavorable subcutaneous form of systemic gamma/delta T-cell lymphoma must be considered. This can lead to sepsis-like clinical pictures.

Histology
This section has been translated automatically.

Dense, nodular infiltrate in the subcutis, with very pleomorphic lymphoid cells of different sizes, which often accumulate in a ring around lipocytes (so-called rimming). General view as in lobular panniculitis. Angiocentric growth pattern with fat tissue necroses. Granulomatous secondary reaction, erythrophagocytosis in 30% of cases.

Immunohistology: Tumor cells express T-cell markers: CD3, CD8, CD43, CD45RO, CD4 is not expressed! Tumor cells express cytotoxic proteins such as Perforin, TIA-1 and Granzym.

Clonal rearragenment of TZR genes can be detected in most cases.

Immunocytogenetic. Expression of the alpha/beta T cell receptor (TZR). Expression of the beta/gamma chain of the T cell receptor indicates subcutaneous infiltration of the systemic form of gamma/delta T cell lymphoma (GD-TZL) with poor prognosis.

Differential diagnosis
This section has been translated automatically.

Clinical:

  • Panniculitis: Mostly subacute, mostly painful nodular subcutaneous infiltrates.
  • Profound circumscribed scleroderma: Slow, non painful, firm indurations. Histology is diagnostic.

Histological:

  • Subcutaneous form of gamma/delta T-cell lymphoma: expression of the gamma/delta chain of TZR. Important DD(!) because systemic gamma/delta T-cell lymphoma has a significantly worse prognosis.
  • Lupus panniculitis: infiltration by small lymphocytes, no cell atypia.
  • Extranodal NK/T-cell lymphoma: expression of CD56; association with EBV.

Progression/forecast
This section has been translated automatically.

Mostly slow progressive course. 5-year survival rate of 80%.

Case report(s)
This section has been translated automatically.

The 48-year-old patient reported a changeable clinical picture with little pain, plate-like indurations, which receded under leaving behind deeply indented, also extensive "scarring" of skin and subcutis. Since 1 year his analogous changes also occurred in the lower abdomen.

The general condition of the patient was unremarkable.

Laboratory: unremarkable findings for blood and differential blood count. Liver enzymes, lipase, amylase, alpha-1-antitrypsin, kidney values o.B. No pathological immune parameters. Borrelia serology normal.

Imaging procedures: o.B.

Histopathological and molecular biological findings: Dense, nodular infiltrate in the subcutis, with very pleomorphic lymphoid cells of different sizes, which often accumulate in a ring around lipocytes (so-called rimming). General view as in lobular panniculitis. Immunohistology: Tumor cells express T cell markers: CD3, CD8, CD43, CD45RO, CD4 is not expressed! Clonal rearragenment of TZR genes was detectable.

Ther: Initiation of a steroidal monotherapy (prednisolone 1mg/kg/KG i.v.). After 8 weeks complete regression of the indurations. Maintenance dose 5mg/p.o. After 6 months local recurrence. Now radiotherapy (GD 36 Gy, fractionation 5 x/week 2.0Gy). Including complete regression and 2 years without recurrence.

Literature
This section has been translated automatically.

  1. Go RS et al (2003) Subcutaneous panniculitis-like T-cell lymphoma: complete remission with fludarabine. Ann Hematol 82: 247-250
  2. Gonzalez CL et al (1991) T-cell lymphoma involving subcutaneous tissue. A clinicopathologic entitycommonly
    associated with hemophagocytic syndrome.
    On the J Surg catheter 15:17-27.
  3. Guizzardi M et al (2003) Cytotoxic gamma/delta subcutaneous panniculitis-like T-cell lymphoma: report of a case with pulmonary involvement unresponsive to therapy. J Eur Acad Dermatol Venereol 17: 219-222
  4. Hoque SR et al (2003) Subcutaneous panniculitis-like T-cell lymphoma: a clinicopathological, immunophenotypic and molecular analysis of six patients. Br J Dermatol 148: 516-525
  5. Jaffe ES et al (2003) Classification of cytotoxic T-cell and natural killer cell lymphomas. Semin Hematol 40: 175-184
  6. Kempf W, Cerroni L (2016) Cutaneous lymphomas. In: Cerroni L et al Histopathology of the skin. Springer-Verlag Berlin Heidelberg New-York S. 915-917
  7. Kim YC et al (2002) Extranodal NK/T-cell lymphoma with extensive subcutaneous involvement, mimicking subcutaneous panniculitis-like T cell lymphoma. Int J Dermatol 41: 919-921
  8. Kumar S et al (1989) Subcutaneous panniculitis T-cell-lymphoma is a tumor of cytotoxic T-lymphocytes. Hum Pathol 29: 397-403
  9. Marzano AV (2000) Cytophagic histiocytic panniculitis and subcutaneous panniculitis-like T-cell lymphoma: report of 7 cases. Arch Dermatol 136: 889-896
  10. Mukai HY et al (2003) Successful treatment of a patient with subcutaneous panniculitis-like T-cell lymphoma with high-dose chemotherapy and total body irradiation. Eur J Haematol 70: 413-416
  11. Reimer P et al (2003) Subcutaneous panniculitis-like T-cell lymphoma during pregnancy with successful autologous stem cell transplantation. Ann Hematol 82: 305-309
  12. Wagner G et al (2016) Subcutaneous panniculitis-like T-cell lymphoma. dermatologist 67: 399-402
  13. Yamashita Y et al (1999) A case of natural killer/T cell lymphoma of the subcutis resembling subcutaneous panniculitis-like T cell lymphoma. Catholic Int 49: 241-246

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

Authors

Last updated on: 29.10.2020