Dermatology
Cutaneous T Cell Lymphoma
Built from Dermatology, 5th Edition

What’s inside
11 sections · 135 slides
Overview
- What this topic covers
- Cutaneous T cell lymphoma (CTCL): the big picture
What Cutaneous T Cell Lymphoma Is
A short history of the concept, and how it came to be classified
- A skin-based cancer, diagnosed at the bedside
- A brief history of mycosis fungoides
- How the CTCL concept was born
- Why "primary cutaneous" lymphomas needed their own rules
- Three main categories of CTCL
- From EORTC to the 2018 WHO-EORTC classification
- Classification algorithm for cutaneous T cell lymphoma
- WHO-EORTC classification: frequency and 5-year survival by CTCL type
Diagnosis, Classification, and Staging
How a biopsy, an antibody panel, and the clinical picture combine into one diagnosis
- Step one: is this a lymphoma at all?
- Immunophenotyping: reading the antibody panel
- T cell receptor (TCR) gene rearrangement testing
- High-throughput TCR sequencing (HTS-TCR)
- One biopsy picture, several possible diagnoses
- Differential diagnosis by histologic pattern
- Differential diagnosis of common histologic patterns in cutaneous T cell lymphoma (CTCL)
- Differential diagnosis of common histologic patterns in cutaneous T cell lymphoma (CTCL) (continued)
- Differential diagnosis of common histologic patterns in cutaneous T cell lymphoma (CTCL) (continued)
- Differential diagnosis of common histologic patterns in cutaneous T cell lymphoma (CTCL) (continued)
- A practical three-step approach to classification
- Staging: how much work-up does each type need?
Mycosis Fungoides
The most common CTCL - classic patch, plaque, and tumor stage disease
- Mycosis fungoides (MF): definition and how common it is
- Who gets mycosis fungoides
- Genetic changes behind mycosis fungoides
- Staphylococcus aureus as a tumor accelerator
- Environmental exposure: an unproven trigger
- The immune system's own fight against the tumor
- When the tumor's immune balance tips the wrong way
- The classic course: patch, then plaque, then tumor
- Limited patch stage disease (stage IA)
- Hypopigmented mycosis fungoides
- Generalized patch/plaque stage disease (stage IB)
- Tumor stage mycosis fungoides
- Mycosis fungoides, additional stage IB presentation
- Hypopigmented mycosis fungoides, additional example
- Mycosis fungoides in an adult with widespread hypopigmentation
- Hypopigmented mycosis fungoides in a child
- Large cell transformation
- Erythrodermic mycosis fungoides with large cell transformation
- Mycosis fungoides with large cell transformation, before and after transplant
- Immunophenotype of the tumor cells
- What else can look like mycosis fungoides
- Staging mycosis fungoides: the TNMB system
- TNMB classification of mycosis fungoides and Sézary syndrome
- TNMB classification of mycosis fungoides and Sézary syndrome (continued)
- TNM-based clinical stage groups for mycosis fungoides and Sezary syndrome
- What the staging work-up actually involves
- 96%
- Treatment principle: match therapy to stage
- Escalation ladder for mycosis fungoides treatment
- Topical corticosteroids and topical chemotherapy
- Radiotherapy: total skin electron beam (TSEB)
- Local radiotherapy for a single lesion
- Phototherapy: PUVA and narrowband UVB
- Systemic biologic therapies: interferon and retinoids
- Targeted systemic agents
- Systemic chemotherapy and stem cell transplant
- Treatment of mycosis fungoides
- Treatment of mycosis fungoides (continued)
- Treatment of mycosis fungoides (continued)
Variants of Mycosis Fungoides
Folliculotropic MF, pagetoid reticulosis, and granulomatous slack skin
- Folliculotropic mycosis fungoides
- Infiltrated eyebrow plaque with hair loss
- Other clinical patterns of folliculotropic MF
- Acneiform folliculotropic mycosis fungoides of the face
- Pathology and treatment of folliculotropic MF
- Pagetoid reticulosis
- Solitary plaque of pagetoid reticulosis
- Granulomatous slack skin
- Pendulous folds of lax skin in the groin
Sezary Syndrome
Erythroderma, blood involvement, and a T cell clone shared between skin and blood
- Sezary syndrome (SS): definition
- How common Sezary syndrome is
- Erythroderma of Sezary syndrome
- Pathology of Sezary syndrome
- Sezary cell ultrastructure
- What drives Sezary syndrome
- Telling Sezary syndrome apart from other causes of erythroderma
- Treatment: extracorporeal photopheresis and systemic options
- Mogamulizumab and its skin side effect
Adult T Cell Leukemia/Lymphoma
A T cell cancer caused by a specific virus, HTLV-1
- Adult T cell leukemia/lymphoma (ATLL)
- Global distribution of HTLV-1 infection
- Skin lesions of adult T cell leukemia/lymphoma
- Pathology, diagnosis, and treatment of ATLL
Primary Cutaneous CD30-Positive Lymphoproliferative Disorders
Lymphomatoid papulosis and cutaneous anaplastic large cell lymphoma - one spectrum, two clinical faces
- A spectrum, not two separate diseases
- Diagnosis and treatment algorithm for CD30-positive lymphoproliferations
- Primary cutaneous anaplastic large cell lymphoma (C-ALCL)
- Solitary tumor of primary cutaneous anaplastic large cell lymphoma
- Immunophenotype and genetics of C-ALCL
- Treating C-ALCL
- Lymphomatoid papulosis (LyP): definition
- How common LyP is, and who gets it
- Papulonecrotic lesions of lymphomatoid papulosis
- Is LyP linked to other lymphomas?
- LyP's variable biopsy picture
- Lymphomatoid papulosis (LyP) – histologic subtypes and their differential diagnoses
- Lymphomatoid papulosis (LyP) – histologic subtypes and their differential diagnoses (continued)
- Lymphomatoid papulosis, type D
- Agminated (clustered) lymphomatoid papulosis
- Managing lymphomatoid papulosis
Subcutaneous Panniculitis-Like T Cell Lymphoma
A cytotoxic T cell lymphoma of fat tissue, sometimes complicated by macrophage overactivation
- Subcutaneous panniculitis-like T cell lymphoma (SPTCL)
- Nodular lesions of subcutaneous panniculitis-like T cell lymphoma
- Autoimmune disease overlap and the risk of HLH
- Immunophenotype and a key genetic finding
- Comparison of subcutaneous panniculitis-like T cell lymphoma (SPTCL) and primary cutaneous gamma/delta T cell lymphoma (PCGD-TCL) with subcutaneous involvement 53
- Treating SPTCL
Extranodal NK/T Cell Lymphoma, Nasal Type
An aggressive, virus-associated lymphoma that often starts in the nose
- Extranodal NK/T cell lymphoma, nasal type
- Cutaneous involvement in extranodal NK/T cell lymphoma
- Ulceronecrotic nasal mass in NK/T cell lymphoma
- Extranodal NK/T cell lymphoma with active EBV infection
- Pathology and treatment of nasal NK/T cell lymphoma
- Hydroa vacciniforme-like lymphoproliferative disorder
Rare Cytotoxic and Other CTCL Subtypes
Aggressive epidermotropic CD8+ disease, acral CD8+ disease, gamma/delta lymphoma, CD4+ small/medium disease, and PTCL NOS
- Primary cutaneous aggressive epidermotropic CD8+ cytotoxic T cell lymphoma
- Generalized skin lesions of aggressive epidermotropic CD8+ CTCL
- Primary cutaneous acral CD8+ T cell lymphoproliferative disorder
- Primary cutaneous CD8+ acral T cell lymphoproliferative disorder
- Primary cutaneous gamma/delta T cell lymphoma (PCGD-TCL)
- Plaques and nodules of primary cutaneous gamma/delta T cell lymphoma
- Primary cutaneous CD4+ small/medium T cell lymphoproliferative disorder
- Primary cutaneous peripheral T cell lymphoma, NOS
- Rapidly growing tumors of primary cutaneous peripheral T cell lymphoma, NOS
- Cutaneous nodules in peripheral T cell lymphoma, NOS
- Prognosis and treatment of PTCL, NOS
- Putting it all together
- Three ideas to take away
- References
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- References (continued)
- Dermatology, 5th Edition (2-Volume Set)