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Primary Cutaneous Lymphoma

Primary Cutaneous Lymphoma. Heterogeneous group of T and B-cell lymphomas variation in clinical presentation histology immunophenotype prognosis Incidence 0.5-1/100,000/year. Classified according to updated Kiel Classification Working Formulation REAL Classification Treated as

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Primary Cutaneous Lymphoma

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  1. Primary Cutaneous Lymphoma • Heterogeneous group of T and B-cell lymphomas • variation in • clinical presentation • histology • immunophenotype • prognosis • Incidence 0.5-1/100,000/year

  2. Classified according to • updated Kiel Classification • Working Formulation • REAL Classification • Treated as • Lymphoma of Lymph Nodes • ? What about indolent cutaneous lymphomas

  3. Primary Cutaneous Lymphoma • Definition: patients without concurrent extracutaneous disease at the time of diagnosis • characteristic clinical and histologic features • different behavior and prognosis • different translocation and oncogenes

  4. Difficulties: • Primary cutaneous lymphomas cannot be defined properly by histologic criteria alone. • Example : • CD30-positive and CD-negative T-cell lymphomas • Lymphoid papulosis and Mycosis Fungoides • Classification needs • Histology • Clinical presentation • Immunology

  5. European Organization for Research and Treatment of Cancer “EORTC” • Clinically relevant classification • Primary cutaneous lymphoma is defined as non-Hodgkin Lymphomas presenting in the skin with no evicence of extracutaneous desease at the time of diagnosis and within the first 6 month. • Exclude secondary lymphomas, Lymphoma in immunocompromised patients, and HTLV-1 associated adult T-cell lymphoma

  6. Mycosis Fungoides • Epidermotropic CTCL characterized by a proliferation of small or medium-sized neoplastic T lymphocytes with cerebriform nuclei • Indolent clinical course • slow progression over years • patches, plaques, tumors, lymph node, internal organs

  7. Epidermotropic, band-like infiltrates involving the papillary dermis • small, medium-sized, and occasionally large mononuclear cells • hyperchromatic, indented (cerebriform) nuclei • Pautrier’s microabscesses

  8. CD3+, CD4+, CD45RO+,CD8-, CD30- • 5-year survival 87% • Therapy: • confined to skin: skin-targeted therapies, phototherapy, topical nitrogen mustard, radiotherapy • Progression to CD30+ or CD30- Large T-cell lymphoma

  9. MF specific Variants • Classical Alibert-Bazin type • Bullous, hyper or hypopigmented MF • MF with follicular mucinosis • Pagetoid reticulosis • Granulomatous slack skin

  10. MF-Associated Folliular Mucinosis • Definition: • Folliculotropic infiltrates with sparing of the epidermis • mucinous degeneration of the hair follicles • preferential involvement of the head and neck • Clinically: • follicular papules, indurated plaques, and tumor • associated with hair loss , pruritus

  11. Micro: • perivascular and periadnexal localization of the dermal infiltrates • infiltration of the follicular epithelium • medium-sized to large hyperchromatic cells with cerebriform nuclei • sparing of the epidermis • mucinous degeneration of the follicular epithelium • Survival 70% • Therapy: total skin electron beam

  12. Pagetoid Reticulosis • Localized “Woringer-Kolopp” • ?Disseminated “Ketron-Goodman” • Definition: localized patches or plaques with an intraepidermal proliferation of neoplastic T cells • Slowly growing psoriasiform or hyperkeratotic patch.

  13. Micro: • acanthosis • large atypical pagetoid cells • medium-sized to large with hyperchramatic cerebriform nuclei • CD3+, CD4+, CD8-, or CD3+, CD4-, CD8+ • Therapy: radio or surgery • prognosis : excellent

  14. CD30+ Lymphoproliferative Disorders • Include: • CD30+ large T-cell lymphoma • Lymphomatiod papulosis (LyP) • ? Borderline Cases

  15. CD30+ large T-cell lymphoma • Large tumor cells, CD30+ • no history or evidence of MF or LyP • most 80% has features of Anaplastic Large cell lymphoma • others immunoblastic, pleomorphic • adult M:F 3:2 • Solitary localized (ulcerating) nodule

  16. Micro: • diffuse non-epidrmotropic • Anaplastic, or pleomorphic or , immunblastic • immuno: • CD30+(>75%), CD4+, CD2-, CD3-, CD5-, CD15-,EMA- • Survival: 90% • Treatment : localized-radio, generalized-chemo

  17. Lymphomatoid Papulosis • Chronic, recurrent, self-healing papulonodular skin eruption with histologic features of CTCL • 10-20% associated with others (MF, CD30+ large T-cell lymphoma, or Hodgkin’s)

  18. Micro: • type A: wedge-shaped, initially non-epidermotropic , large atypical, CD30+ cells with extensive infiltrates of histiocytes, small lymphocytes and eosinophils • Type B: perivascular or bandlike, epidermotropic infiltrates with small to medium-sized cells with cerebriform nuclei • Type C: features suggestive of CD30+ large T-cell lymphoma.

  19. Immuno • Type A and Type C: similar to CD30+ large T-cell , CD3+, CD4+/-, CD5+/-, CD8-, CD30+, CD15-, EMA- • Type B: CD3+,CD4+, CD8-, CD30- • Survival : 100% • no cure, relapse after treatment

  20. Sezary’s Syndrome • Definition: • erythroderma, generalized lymphadenopathy, and the presence of neoplastic T cells in skin, lymph nodes and peripheral blood • Micro: • may be similar to MF • more often monotonous cells • CD3+, CD4+, CD45RO+, CD8-, CD30- • Survival: 11%, Chemo is the treatment

  21. CD30- Large Cell Lymphoma • CD30- large neoplastic cells without MF • solitary, localized, or generalized plaques, nodules, or tumors. • Micro: • medium-sized to large pleomorphic T cells with or without cerebriform nuclei and immunoblasts • CD4+ only, CD30- • Survival : 15% • Chemo is recommended

  22. Granulomatous Slack Skin • Definition: • slow development of folds of lax skin and a granulomatous infiltrated with clonal T cells • axillae groins , Male predominant • ?association with Hodgkin’s , and MF • Micro: • dense granulomatous dermal infiltrates • atypical T cells with cerebriform nuclei • CD3+ , CD4+, CD8- • Survival : indolent course

  23. Pleomorphic Small/Medium-Sized CTCL • Definition: • neoplastic proliferation of pleomorphic small/medium-sized Tcells • clinical picture different form MF • Micro: • dense, diffuse or nodular infiltrates of small/medium pleopmorphic neoplastic T cells • CD4+, CD8+(some), loss of pan Tcell markers • Favorable prognosis

  24. Subcutanious Panniculitis-Like T-Cell Lymphoma • Definition • subcutaneous infiltrates of small, medium-sized or large pleomophic T cells and many macrophages • Clinically • subcutaneous nodules mainly on legs • systemic symptoms fever, weight loss.. • Hemophagocytic syndrome

  25. Micro: • panniculitis-like infiltrates with neoplastic T cells and macrophages • tumor cell necrosis, karyorrhexis , erythrophagocytosis • CD3+, CD4+, CD8- • or CD3+, CD4-, CD8+ • Poor prognosis

  26. Follicular Center Cell Lymphoma • Definition: • follicle center cells, centrocytes (small and large cleaved) and cetroblasts (large with prominent nucleoli) • Clinically: • non scaling , solitary or grouped papules, plaques or tumors • Head , neck and trunk • dissemination is uncommon

  27. Micro: • nodular of diffuse infiltrates sparing the epidermis • Early lesions: centrocytes , few centrobasts, many T cells, neoplastic follicles are rare (follicular center lymphoma) • Late lesions: monotonous infiltrates of large centrobalsts and centrocytes (large B cell lympnoma) • CD19+, CD20+, CD22+, CD79a+ • CD5-, CD10- • rarely express bcl-2 • Survival: 97%

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