Primary cutaneous diffuse large B cell lymphoma (other) C85.1

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 08.11.2021

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Synonym(s)

Cutaneous large cell B-cell lymphoma; Large cell B-cell lymphoma of the skin; primary cutaneous large B-cell lymphoma.

Definition
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Group of very rare, large cell lymphomas of the skin that are not localized to the legs (other) and are therefore not included in the significantly more common large cell cutaneous B-cell lymphomas of the leg (see below Primary cutaneous diffuse large B-cell lymphoma leg type). They are addressed as a separate entity. This group also includes morphologic variants of diffuse large B-cell lymphoma, such as anaplastic or immunoplastic subtypes and T-cell-rich or histiocyte-rich large B-cell lymphomas. See"Diffuse large B-cell lymphoma" (Internal Medicine).

Manifestation
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More likely to occur in older people.

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Clinical features
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Red, brownish-red or blue-red, smooth knots or plaques.

Histology
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Diffuse infiltrates in the area of the entire dermis and the upper subcutis. The epidermis remains predominantly free of infiltrates. The histopathological pattern shows predominantly large cells with the characteristics of centrocytes and centroblasts. Numerous mitoses.

Differential diagnosis
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Clinical differential diagnoses: S. and lymphoma, cutaneous B-cell lymphoma.

Therapy
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Primarily radiotherapy, if necessary excision (in case of solitary occurrence).

Alternative: Interferon alfa (low dose) or monoclonal anti-CD20 AK ( Rituximab).

Alternative: polychemotherapy (e.g. CHOP regimen).

In nodal large B-cell lymphomas, combination therapy of CHOP and rituximab proved superior to CHOP therapy alone in terms of remissions and overall survival in large numbers of cases.

Progression/forecast
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These lymphomas seem to have a rather good prognosis.

Literature
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  1. Cerroni L, Kerl H (2003) Cutaneous B-cell lymphomas. In: H. Kerl et al (eds.) Histopathology of the skin. Springer, Berlin Heidelberg New York S. 911-912
  2. Coiffier B et al (2002) CHOP chemotherapy plus rituximab compared with CHOP alone in elderly patients with diffuse large-B-cell lymphoma. N Engl J Med 246: 1830-1831
  3. Fernandez-Flores A et al (2011) The differential diagnosis between primary cutaneous large B-cell lymphoma and cutaneous follicular lymphoma: prognostic and therapeutic implications. At J Dermatopathol 33:819-826
  4. Kamath NV et al (2001) Primary cutaneous large B-cell lymphoma of the leg relapsing as cutaneous intravascular large B-cell lymphoma. Arch Dermatol 137: 1657-1658
  5. Paulli M et al (2002) Primary cutaneous large B-cell lymphoma of the leg: histogenetic analysis of a controversial clinicopathologic entity. Hum Pathol 33: 937-943
  6. Plaza JA et al (2011) The histomorphologic spectrum of primary cutaneous diffuse large B-cell lymphoma: a study of 79 cases. At J Dermatopathy 33:649-655
  7. Willemze R et al (2005) WHO-EORTC classification for cutaneous lymphomas. Blood 105: 3768-3785
  8. Yang SFet al (2011) Composite primary cutaneous peripheral T-cell lymphoma and Epstein-Barr virus-positive large B-cell lymphoma. Leuk lymphoma 52:2027-2030.

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

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Last updated on: 08.11.2021