MYCOSIS FUNGOIDES SHEIKHA

MYCOSIS FUNGOIDES SHEIKHA

Dermatology is NOTHING

BUT An External Hematology

New England Journal of Medicine
MYCOSIS FUNGOIDES SHEIKHA Dermatology is Nothing … BUT AN EXTERNAL HEMATOLOGY New England Journal of Medicine

Hematology is Nothing BUT AN INTERNAL DERMATOLOGY
MYCOSIS FUNGOIDES SHEIKHA Hematology is Nothing BUT AN INTERNAL DERMATOLOGY

HEMATOLOGY DERMATOLOGY The two sides of the same coin

Anwar Sheikha Professor Senior Consultant Clinical & Lab. Hematologist
MD, FRCP, FRCPath., FCAP, FRCPA, FRCPI, FACP Senior Consultant Clinical & Lab. Hematologist Clinical Professor of Hematology University of Mississippi Medical Center, Jackson, Mississippi Professor of Hematology, University of Salahaddin, Erbil, Kurdistan, IRAQ Owner & C.E.O., Raziana Company for Health Services, Hawler, IRAQ

كومبانياى ﺮﺍﺰﯿﺎﻨﻪ پروژﻩى
نه خوشخانه و شيرپه نجه خانه ى ميديا دروست دەكات MEDIA MEDICAL & CANCER CENTER ﭘﺮﻮﮊﻩﻜﻪ ﺑﺮﯾﺘﯾﻪ ﻠﻪ ۲۰۰ ﮊﻮﺮﻯ ﻧﻪﺧﻮﺵ ﻮ ﻛﻮﻣﻪﻠﮕﺎﻯ ﻛﻠﻳﻧﻳﻛﻮ ﻣﺎﻠﻰ ﭙﺰﻳﺷﮑﺎﻥ

MEDYA MEDICAL & CANCER CENTER
“RAZIANA COMPANY ” MEDYA MEDICAL & CANCER CENTER MEDIA MEDICAL & CANCER CENTER A 200 BED HOSPITAL, MEDICAL OFFICE BUILDINGS & A RESIDENCE VILLAGE AT A COST OF ~ $50 MILLION

MF is a cutaneous lymphoma of mature CD4+ T cells
MYCOSIS FUNGOIDES SHEIKHA MF is a cutaneous lymphoma of mature CD4+ T cells The commonest cutaneous T-cell lymphoma It has unique clinical & histologic features Not all cutaneous T-cell lymphomas are MF MYCOSIS FUNGOIDES SÉZARY SYNDROME MF/SZ

LYMPHOMA IS THE MOST CONFUSING PART OF MEDICINE

Professor Lennert, Keil Classification

* W.H.O. B NHL HD T & NK CLASSIFICATION OF LYMPHOID NEOPLASMS
Precursor T-cell neoplasms Precursor B-cell neoplasms * Mature (Peripheral) B-cell neoplasms Mature (Peripheral) T-cell neoplasms Nodular Lymphocyte-Predominant Hodgkin Lymphoma Classical Hodgkin Lymphoma HD

NHL T & NK *T-cell Prolymphocytic Leukemia
W.H.O. CLASSIFICATION OF LYMPHOID NEOPLASMS *T-cell Prolymphocytic Leukemia *T-cell Granular Lymphocytic Leukemia *Aggressive NK-cell Leukemia *Adult T-cell Leukemia/Lymphoma (HTLV1) *Extranodal NK/T-cell Lymphoma. Nasal type *Entropathy-type T-cell Lymphoma *Hepatosplenic γδ T-cell Lymphoma *Subcutaneous Panniculitis-like T Lymphoma *Mycosis Fungoides /Sézary Syndrome *Anaplastic Large-cell Lymphoma/T/null, skin type *Peripheral T-cell Lymphoma, not otherwise characterized *Angioimmunoblastic T-cell Lymphoma *Anaplastic Large-cell Lymphoma/T/null, systemic type NHL T & NK Mature (Peripheral) T-cell neoplasms *

Incidence: 3 per million (0.29 per 100,000 population in USA)
MYCOSIS FUNGOIDES SHEIKHA Incidence: per million (0.29 per 100,000 population in USA) 2% of all new cases of NHL Age: Older adults (55 to 60) Male/Female: 2/1 TUMOR STAGE PATCH STAGE PLAQUE STAGE ERYTHRODERMA “SÉZARY” Cerebriform “Sézary” Cells Epidermo- tropism

Patch Plaque Tumor Stage MF patches are usually distributed in sun-shielded areas such as those covered by a bathing suit or intertriginous regions. Sézary Syndrome

Various Cutaneous Manifestations of Mycosis Fungoides
Figure 1. Various Cutaneous Manifestations of Mycosis Fungoides. Panel A shows patch-or-plaque mycosis fungoides affecting the lower trunk. The patches are thin, slightly scaly, erythematous lesions typically greater than 4 cm in diameter and distributed in sun-shielded areas such as those covered by a bathing suit or intertriginous regions. Plaques are thicker than patches. Panel B shows pagetoid reticulosis, a variant of mycosis fungoides that typically consists of a single patch or plaque located in an acral area. Panel C shows syringotropic mycosis fungoides, which is manifested as papules 1 to 3 mm in diameter distributed in the eccrine ducts, indicating the propensity of lymphoma cells to accumulate in these locations. Panel D shows follicular mycosis fungoides, in which lesions characterized by alopecia develop. In a similar variant, there is mucin deposition in the follicles. Panel E shows hypopigmented mycosis fungoides. This variant is more noticeable in persons with dark pigmentation and may be more common in childhood and adolescence than in adulthood. Hypopigmentation to full depigmentation occurs in patches. Panel F shows erythrodermic mycosis fungoides. This variant may evolve from patch-or-plaque mycosis fungoides and eventually involve more than 80 percent of the body-surface area. It may also arise spontaneously, as in the Sezary syndrome. Panel G shows the Sezary syndrome. In its most florid form, the diffuse infiltration of the skin may produce the exaggerated facial lines, resulting in "leonine facies." The Sezary syndrome is also associated with atypical lymphocytes on the blood smear. Panel H shows a mycosis fungoides tumor. Such tumors define the T3 stage of disease and may arise at the site of plaques or appear on their own, without being preceded by a patch-or-plaque lesion. The vertical growth phase is accelerated, and tumors tend to appear more quickly than plaques. Tumors not characterized by epidermotropism or previous mycosis fungoides are sometimes called "non-mycosis fungoides cutaneous T-cell lymphoma."

EPIDERMOTROPIC The cardinal features of MF is infiltration of epidermis and then dermis by Atypical Cerebriform Lymphoid Cells

Mycosis Fungoides: A Cancer of Skin-Homing T Cells
Figure 2. Mycosis Fungoides: A Cancer of Skin-Homing T Cells. In cutaneous T-cell lymphoma, cells home to the skin by virtue of interactions with dermal capillary endothelial cells. Circulating lymphoma cells bearing cutaneous lymphocyte antigen (CLA) roll along endothelial cells expressing E-selectin. Chemokine receptors (e.g., CC chemokine receptor 4 [CCR4]) on the malignant T cells recognize chemokines (e.g., CC chemokine ligand 17 [CCL17]) that have emanated from the epidermis and bound to the luminal side of endothelial cells, greatly facilitating the binding of leukocyte-function-associated antigen type 1 on the lymphoma cells to intercellular adhesion molecule 1 on the endothelial cells and subsequent extravasation into the dermis. From there, the lymphoma cells often display an affinity for epidermal cells and cluster around Langerhans' cells, forming Pautrier's microabscesses, which can be observed on histologic examination. This process is principally guided by the interactions of lymphoma-cell integrin {alpha}E{beta}7, CCR4, and the CD4 T-cell receptor complex with E-cadherin, CCL22, and major-histocompatibility-complex class II (MHC-II) molecules, respectively. TCR denotes T-cell receptor. Girardi M et al. N Engl J Med 2004;350:

Multiple discrete & confluent plaques of cutaneous T-cell lymphoma “MF”

Multiple plaques of cutaneous T-cell lymphoma with tumor formation “MF”

PATCH STAGE Mild epidermal hyperplasia with perivascular or band-like infiltrate of small- to medium-sized atypical lymphocytes with cerebriform nuclear convolution. EPIDERMOTROPISM: Cerebriform lymphocytes exhibit epidermotropism and are arranged along the dermal-epidermal junction in a single-file pattern or scattered in the epidermis. Pautrier’s microabscesses are small intra-epidermal collections of cerebriform lymphocytes & are pathognomonic for MF. They might not be present in early stages of MF MF PATCHES Eczematoid

2. PLAQUE STAGE: The density of the neoplastic cells within dermis increases Exaggerated epidermotropism Psoriasiform

The density of the neoplastic cells within dermis increases
2. PLAQUE STAGE: The density of the neoplastic cells within dermis increases Exaggerated epidermotropism Plaque Stage: A broad band-like cellular infiltrate in the upper dermis Pautrier Psoriasiform

“d’emblee” ﺩﻮﻤﻪﻞ 3. TUMOR STAGE: VERTICAL GROWTH
Very dense dermal infiltrate involving the full breadth of the dermis & extending to the subcutaneous fat. Epidermotropism diminishes de novo tumor “d’emblee” ﺩﻮﻤﻪﻞ Tumors could get infected  sepsis  death

Sézary Syndrome ↑CD4 to CD8 ratio >10:1 T-cell Receptor gene
ERYTHRODERMA Pathology similar to Patch stage but infiltrate is more sparse Generalized erythroderma with Sézary cells “with cerebriform nuclei” in blood of >1,000/uL  Sézary Syndrome ↑CD4 to CD8 ratio >10:1 T-cell Receptor gene rearrangement

Intensely symptomatic from pruritus & scaling
Usually have lymphadenitis Sézary Syndrome = Generalized erythroderma Lympha- denopathy Sézary cells

Pautrier Abscesses

LYMPH NODE INVOLVEMENT IN MF or SZS DERMATOPATHIC LYMPHADENITIS = DL
MYCOSIS FUNGOIDES SHEIKHA LYMPH NODE INVOLVEMENT IN MF or SZS DERMATOPATHIC LYMPHADENITIS = DL CATEGORY III “LN4” CATEGORY II “LN3” CATEGORY I “LN0-LN2” Partial or complete effacement of LN architecture by cytologically atypical lymphocyte Clusters of 10 or more atypical LC confined to the paracortex ± DL LN0 = DL/ No atypical LC LN1 = Scattered atypical cerebriform LC (not in clusters) ± DL LN2 = Small clusters < 6 cells ± DL

CD7- CD30- CD4+ MYCOSIS FUNGOIDES SHEIKHA IMMUNOPHENOTYPE in MF/SZS
Molecular Diagnosis: PCR of T-cell Receptor γ rearrangement, especially in early patch stages CD5+ CD25 -/+ Cell of Origin: CD4+ T lymphocyte with skin homing “epidermotropic” properties

CLINICAL PRESENTATION OF MF MF often has a long natural history Median duration from onset to diagnosis may be 5 years or more Usually starts with scaly skin lesions that wax & wane over years Biopsy at this stage is usually non-diagnostic Patient may respond at this stage to topical steroid Repeated biopsy is warranted if MF is suspected and biopsy is negative

PRURITUS T1 T2 T3 T4 Commonest symptom of MF MYCOSIS FUNGOIDES SHEIKHA
CLINICAL PRESENTATION OF MF 30% Limited patch or plaque stage <10% BSA T1 35-40% Generalized patch or plaque stage >10% BSA T2 15-20% Tumorous stage <10% BSA T3 PRURITUS Commonest symptom of MF 15% Erythro- derma T4 Only 15% of MF patients show extracutaneous disease. Lymph nodes; Visceral disease, etc

OTHER FEATURES OF MF MYCOSIS FUNGOIDES SHEIKHA
Skin Hair Follicles could be extensively infiltrated. Mucin might be deposited  Follicular MF Pagetoid reticulosis is a verrucous variant of MF Affecting acral sites like hands & feet. Extreme atypical LC epidermotropism verrucae Granulomatous slack skin  pendulous folds of slack or lax skin “macrophage-mediated destruction of dermal elastic fibers” Many MF have only skin problems. 15% have extracutaneous disease; LN, Visceral sites “Lung, Oral cavity, CNS, etc” could be affected.

Figure 1. Various Cutaneous Manifestations of Mycosis Fungoides. Panel A shows patch-or-plaque mycosis fungoides affecting the lower trunk. The patches are thin, slightly scaly, erythematous lesions typically greater than 4 cm in diameter and distributed in sun-shielded areas such as those covered by a bathing suit or intertriginous regions. Plaques are thicker than patches. Panel B shows pagetoid reticulosis, a variant of mycosis fungoides that typically consists of a single patch or plaque located in an acral area. Panel C shows syringotropic mycosis fungoides, which is manifested as papules 1 to 3 mm in diameter distributed in the eccrine ducts, indicating the propensity of lymphoma cells to accumulate in these locations. Panel D shows follicular mycosis fungoides, in which lesions characterized by alopecia develop. In a similar variant, there is mucin deposition in the follicles. Panel E shows hypopigmented mycosis fungoides. This variant is more noticeable in persons with dark pigmentation and may be more common in childhood and adolescence than in adulthood. Hypopigmentation to full depigmentation occurs in patches. Panel F shows erythrodermic mycosis fungoides. This variant may evolve from patch-or-plaque mycosis fungoides and eventually involve more than 80 percent of the body-surface area. It may also arise spontaneously, as in the Sezary syndrome. Panel G shows the Sezary syndrome. In its most florid form, the diffuse infiltration of the skin may produce the exaggerated facial lines, resulting in "leonine facies." The Sezary syndrome is also associated with atypical lymphocytes on the blood smear. Panel H shows a mycosis fungoides tumor. Such tumors define the T3 stage of disease and may arise at the site of plaques or appear on their own, without being preceded by a patch-or-plaque lesion. The vertical growth phase is accelerated, and tumors tend to appear more quickly than plaques. Tumors not characterized by epidermotropism or previous mycosis fungoides are sometimes called "non-mycosis fungoides cutaneous T-cell lymphoma."

STAGING OF MF

(SKIN) T T1 T2 Tumors T3 T4 M M0 M1 B B0 B1 N N0 N1 N2 N3 Tumor- Node-
MYCOSIS FUNGOIDES SHEIKHA (SKIN) T Limited patch/plaque (<10% total skin surface) T1 Generalized patch/plaque (>10% total skin surface) T2 Tumors T3 Generalized Erythroderma T4 (VISCERA) M No Visceral involvement M0 Visceral M1 Tumor- Node- Metastasis- Blood Classification For MF (BLOOD) B No Sézary cells (<5% of LC) B0 Circulating Sézary cells (>5% of LC) B1 (LYMPH NODES) N LN Clinically uninvolved N0 Enlarged; histologically uninvolved (reactive & dermatopathic) N1 LN Clinically uninvolved; histologically involved N2 LN enlarged & involved N3

CLINICAL STAGING SYSTEM FOR MF
MYCOSIS FUNGOIDES SHEIKHA CLINICAL STAGING SYSTEM FOR MF M N T Clinical stages M0 N0 T1 IA NO T2 IB N1 T1-2 IIA N0-1 T3 tumor IIB T4 IIIA Erythroderma IIIB Erythroderma N2-3 histology T1-4 IVA M1 N0-3 IVB B CLASSIFICATION (SEZARY CELLS) DOES NOT ALTER CLINICAL STAGE

Tumor-Node-Metastasis-Blood & Clinical Staging Classification
MYCOSIS FUNGOIDES SHEIKHA (SKIN) T Limited patch/plaque (<10% total skin surface) T1 Generalized patch/plaque (>10% total skin surface) T2 Tumors T3 Generalized Erythroderma T4 (VISCERA) M No Visceral involvement M0 Visceral involvement M1 (BLOOD) B No Sezary cells (<5% of LC) B0 Circulating cells (>5% of LC) B1 M N T Clinical stages M0 N0 T1 IA NO T2 IB N1 T1-2 IIA N0-1 T3 IIB T4 IIIA IIIB N2-3 T1-4 IVA M1 N0-3 IVB (LYMPH NODES) N LN Clinically uninvolved N0 Enlarged; histologically uninvolved (reactive & dermatopathic) N1 LN Clinically uninvolved; histologically involved N2 LN enlarged & involved N3 Tumor-Node-Metastasis-Blood & Clinical Staging Classification

Dear Professor Hoppe, I am writing on behalf of a strong-willed 32 year old Iraqi Kurdish patient with the diagnosis of mycosis fungoides, involving almost 20% of her body surface area. Patient gives three years history of scaly skin lesions, not responding to topical dermatological treatment. Recent histology sections showed the diagnosis of early stage mycosis fungoides. Patient desires consultation from Stanford, specifically asking for yourself. I truly appreciate having an appointment for January 2007 with a formal letter indicating the detail of the visit and the cost associated with the treatment. After having the appointment letter from your hospital, we usually need few months to process the visa to USA. Looking forward to hearing from you. Professor Anwar Sheikha, MD, FRCP, FRCPath.

Dear Professor Sheikha, Thank you for your inquiry regarding possible consultation and treatment for mycosis fungoides. We would be happy to see your patient at Stanford. We have a comprehensive multi-disciplinary cutaneous lymphoma clinic that includes dermatologists (Dr. Y. Kim is the Director), radiation oncologists, medical oncologists, and dermatopathologists. Our preference is to see all new patients in this clinic before making specific recommendations for therapy. We employ a variety of topical and systemic therapies for management and one of our major treatment programs is with total skin irradiation. Considering the special circumstances for this patient, to facilitate her visit and treatment, it would probably be best while we are waiting for visa clearance, etc. to be able to review the biopsy material. It might be simplest if you request the slides and then forward them to me (R. Hoppe, Department of Radiation Oncology, Room CC-G224, 875 Blake Wilbur Drive, Stanford CA 94305). I will then obtain review from Stanford Pathology.

Assuming we confirm the diagnosis, we would be able to save some time once the patient arrives. It is possible we may recommend treatment other than irradiation (e.g., phototherapy or topical agents) that does not require staying at Stanford. If that is the case, the expense would probably not be greater than several hundred to a few thousand dollars, depending on other examinations (e.g., radiology, etc.) that we may recommend. If we recommend a course of total skin irradiation, the "list price" would total ~$76,000. Our clinic will be meeting in January on January 11, 25, and 26 and we could arrange an appointment for any of those dates. In addition, I have taken the liberty of contacting Barbara Ralston in our Office of Special Patient Services to facilitate these arrangements. If you have any other questions, please feel free to contact me. Sincerely, Rich Hoppe Chair, Radiation Oncology

TREATMENT OF MF MYCOSIS FUNGOIDES SHEIKHA TOPICAL CHEMO- THERAPY
Effective TOPICAL NITROGEN MUSTARD “MECHLORETHAMINE" Mechanism ?? AQUEOUS SOLUTION OINTMENT 10 to 20 mg Per 100 cc = Choice of aqueous or ointment depends on convenience, preference & cost Hypersensitivity is 30% with Aqueous solution & < 5% with ointment

Topical N2-Mustard is applied locally or to the entire skin at least daily during the clearing phase. After few weeks treatment may be applied to the affected region. N2-Mustard may only be applied to the affected anatomical site if the disease is really limited. Treatment is continued on daily basis until the lesions are cleared (6 months+)  3 to 6 months of maintenance therapy If response is slow; increase N2-Mustard concentration or frequency of application Half will relapse after discontinuation of R/ but respond again CR rate for limited patch or plaque stage “T1” is 70% to 80% The median time to skin clearance is 6 to 8 months 20% to 25% have durable CR of > 10 years Local Radiation to Refractory local lesions

TREATMENT OF MF MYCOSIS FUNGOIDES SHEIKHA TOPICAL CHEMO- THERAPY
TOPICAL Carmustine “BCNU" Similar efficacy to N2- Mustard but it could be absorbed & cause myelosuppression, thus limiting its long-term use. BCNU use could cause telangiectasias in areas exposed to the drug

Less response for erythrodermic or tumor stage
MYCOSIS FUNGOIDES SHEIKHA PHOTO- THERAPY TREATMENT OF MF Ultraviolet Light (UV)  UVA or UVB wavelength ± Psoralen = PUVA Psoralen is a photosensitizing agent The long-wave UVA has greater dermal penetration power For early Limited disease UVB alone or Home UV phototherapy (UVA & UVB) could be effective PUVA is the most commonly used form of therapy for MF & SZS It is effective in Psoriasis but has also been found to be effective in MF PUVA is used 2-3 times/week during the clearance phase ( >6 months) Reduce frequency in maintenance phase. For recurrence ↑ frequency again Complete clearance rate with PUVA is 50% to 90% for patch & plaque stage Less response for erythrodermic or tumor stage

PUVA COMPLICATIONS ACUTE: Nausea Phototoxic reactions such as erythroderma, blistering & dryness Shield eyes & skin from sun for 24 hrs after Psoralen ingestion LONG TERM: Cataract (use UVA opaque goggles during therapy) Secondary cutaneous malignancy

Bexarotene MYCOSIS FUNGOIDES SHEIKHA TOPICAL RETINOIDS “Targretin”
1% Gel Overall Response Rate is % Complete Response rate is 21% Because of the irritant effect, it is only used for discrete patch or plaque stage Not applicable in generalized disease Apply thin over the lesions twice daily Irritation is a rule. Withhold for few weeks if erythema

TREATMENT OF MF RADIATION THERAPY MYCOSIS FUNGOIDES SHEIKHA
MF is an exquisitely radiosensitive neoplasm Irradiation may be exploited in several ways Individual plaques or tumors of MF may be treated to total doses of 15 to 25 Gy in 1 to 3 weeks, with a high likelihood of achieving long-term local control. For the unusual patient with with unilesional or localized MF, local electron beam therapy achieves the most efficient & complete clearance of the disease Depth of penetration of electrons is controllable; this is of major advantage in MF Depth of R/ with TSEBT is better than N2-Mustard or PUVA

TOTAL SKIN ELECTRON BEAM THERAPY
MYCOSIS FUNGOIDES SHEIKHA TOTAL SKIN ELECTRON BEAM THERAPY “Stanford Technique” A full cycle takes 2 days 2 Gy is given per cycle Total dose of around 36 Gy is given over 10 weeks; Give a week rest in middle to give relief from erythema OVERALL RESPONSE RATE 100% COMPLETE RESPONSE RATE 98% 50% OF T1 & 25% OF T2 ARE FREE OF DISEASE 5 YEARS AFTER A SINGLE COURSE Local N2-Mustard is indicated for 6 months after TSEBT Complications: Erythema Dry desquamation Alopecia Nail loss Sweating problems Indications: Very thick plaques Recent rapid progression Other local therapy are ineffective

TREATMENT OF MF MYCOSIS FUNGOIDES SHEIKHA SYSTEMIC CHEMO- THERAPY
Only for Extracutaneous MF 80% to 100% Complete or Partial Response Duration of Response is usually < 1 year CHOP COP CAVE COMP

TREATMENT OF MF MYCOSIS FUNGOIDES SHEIKHA OTHER TREATMENTS
Extracorporeal Photopheresis Interferon-α Systemic Retinoids Recombinant Fusion Proteins IL-2-diphtheria toxin (Ontak; denileukin diftitox) For IL-2 receptor “CD25+” MF

OUTCOME STAGE IA (Limited Patch or Plaque, T1) Disease Excellent Prognosis with conventional Treatment Life Expectancy = Age Matched Population Only 9% progress to more advanced stages Aggressive Therapy has no Survival Advantage Do not over treat

OUTCOME STAGE IB/IIA (Generalized Patch or Plaque, T2) Disease Median Survival of 11 years 25% MR from MF

OUTCOME STAGE IIB (Tumorous) Disease Median Survival of 3.2 years Majority die of MF

OUTCOME STAGE III (Erythrodermic, T4) Disease Total Skin Electron Beam Therapy is not recommended Survival is variable

OUTCOME STAGE IV (Extracutaneous) Disease Poor Prognosis Median Survival 13 months

REVISON

Panel A shows patch-or-plaque MF affecting the lower trunk. The patches are thin, slightly scaly, erythematous lesions typically greater than 4 cm in diameter and distributed in sun-shielded areas such as those covered by a bathing suit or intertriginous regions. Plaques are thicker than patches. Figure 1. Various Cutaneous Manifestations of Mycosis Fungoides. Panel A shows patch-or-plaque mycosis fungoides affecting the lower trunk. The patches are thin, slightly scaly, erythematous lesions typically greater than 4 cm in diameter and distributed in sun-shielded areas such as those covered by a bathing suit or intertriginous regions. Plaques are thicker than patches. Panel B shows pagetoid reticulosis, a variant of mycosis fungoides that typically consists of a single patch or plaque located in an acral area. Panel C shows syringotropic mycosis fungoides, which is manifested as papules 1 to 3 mm in diameter distributed in the eccrine ducts, indicating the propensity of lymphoma cells to accumulate in these locations. Panel D shows follicular mycosis fungoides, in which lesions characterized by alopecia develop. In a similar variant, there is mucin deposition in the follicles. Panel E shows hypopigmented mycosis fungoides. This variant is more noticeable in persons with dark pigmentation and may be more common in childhood and adolescence than in adulthood. Hypopigmentation to full depigmentation occurs in patches. Panel F shows erythrodermic mycosis fungoides. This variant may evolve from patch-or-plaque mycosis fungoides and eventually involve more than 80 percent of the body-surface area. It may also arise spontaneously, as in the Sezary syndrome. Panel G shows the Sezary syndrome. In its most florid form, the diffuse infiltration of the skin may produce the exaggerated facial lines, resulting in "leonine facies." The Sezary syndrome is also associated with atypical lymphocytes on the blood smear. Panel H shows a mycosis fungoides tumor. Such tumors define the T3 stage of disease and may arise at the site of plaques or appear on their own, without being preceded by a patch-or-plaque lesion. The vertical growth phase is accelerated, and tumors tend to appear more quickly than plaques. Tumors not characterized by epidermotropism or previous mycosis fungoides are sometimes called "non-mycosis fungoides cutaneous T-cell lymphoma."

Figure 1. Various Cutaneous Manifestations of Mycosis Fungoides. Panel A shows patch-or-plaque mycosis fungoides affecting the lower trunk. The patches are thin, slightly scaly, erythematous lesions typically greater than 4 cm in diameter and distributed in sun-shielded areas such as those covered by a bathing suit or intertriginous regions. Plaques are thicker than patches. Panel B shows pagetoid reticulosis, a variant of mycosis fungoides that typically consists of a single patch or plaque located in an acral area. Panel C shows syringotropic mycosis fungoides, which is manifested as papules 1 to 3 mm in diameter distributed in the eccrine ducts, indicating the propensity of lymphoma cells to accumulate in these locations. Panel D shows follicular mycosis fungoides, in which lesions characterized by alopecia develop. In a similar variant, there is mucin deposition in the follicles. Panel E shows hypopigmented mycosis fungoides. This variant is more noticeable in persons with dark pigmentation and may be more common in childhood and adolescence than in adulthood. Hypopigmentation to full depigmentation occurs in patches. Panel F shows erythrodermic mycosis fungoides. This variant may evolve from patch-or-plaque mycosis fungoides and eventually involve more than 80 percent of the body-surface area. It may also arise spontaneously, as in the Sezary syndrome. Panel G shows the Sezary syndrome. In its most florid form, the diffuse infiltration of the skin may produce the exaggerated facial lines, resulting in "leonine facies." The Sezary syndrome is also associated with atypical lymphocytes on the blood smear. Panel H shows a mycosis fungoides tumor. Such tumors define the T3 stage of disease and may arise at the site of plaques or appear on their own, without being preceded by a patch-or-plaque lesion. The vertical growth phase is accelerated, and tumors tend to appear more quickly than plaques. Tumors not characterized by epidermotropism or previous mycosis fungoides are sometimes called "non-mycosis fungoides cutaneous T-cell lymphoma." Panel B shows pagetoid reticulosis, a variant of mycosis fungoides that typically consists of a single patch or plaque located in an acral area.

Figure 1. Various Cutaneous Manifestations of Mycosis Fungoides. Panel A shows patch-or-plaque mycosis fungoides affecting the lower trunk. The patches are thin, slightly scaly, erythematous lesions typically greater than 4 cm in diameter and distributed in sun-shielded areas such as those covered by a bathing suit or intertriginous regions. Plaques are thicker than patches. Panel B shows pagetoid reticulosis, a variant of mycosis fungoides that typically consists of a single patch or plaque located in an acral area. Panel C shows syringotropic mycosis fungoides, which is manifested as papules 1 to 3 mm in diameter distributed in the eccrine ducts, indicating the propensity of lymphoma cells to accumulate in these locations. Panel D shows follicular mycosis fungoides, in which lesions characterized by alopecia develop. In a similar variant, there is mucin deposition in the follicles. Panel E shows hypopigmented mycosis fungoides. This variant is more noticeable in persons with dark pigmentation and may be more common in childhood and adolescence than in adulthood. Hypopigmentation to full depigmentation occurs in patches. Panel F shows erythrodermic mycosis fungoides. This variant may evolve from patch-or-plaque mycosis fungoides and eventually involve more than 80 percent of the body-surface area. It may also arise spontaneously, as in the Sezary syndrome. Panel G shows the Sezary syndrome. In its most florid form, the diffuse infiltration of the skin may produce the exaggerated facial lines, resulting in "leonine facies." The Sezary syndrome is also associated with atypical lymphocytes on the blood smear. Panel H shows a mycosis fungoides tumor. Such tumors define the T3 stage of disease and may arise at the site of plaques or appear on their own, without being preceded by a patch-or-plaque lesion. The vertical growth phase is accelerated, and tumors tend to appear more quickly than plaques. Tumors not characterized by epidermotropism or previous mycosis fungoides are sometimes called "non-mycosis fungoides cutaneous T-cell lymphoma." Panel C shows syringotropic mycosis fungoides, which is manifested as papules 1 to 3 mm in diameter distributed in the eccrine ducts, indicating the propensity of lymphoma cells to accumulate in these locations.

Figure 1. Various Cutaneous Manifestations of Mycosis Fungoides. Panel A shows patch-or-plaque mycosis fungoides affecting the lower trunk. The patches are thin, slightly scaly, erythematous lesions typically greater than 4 cm in diameter and distributed in sun-shielded areas such as those covered by a bathing suit or intertriginous regions. Plaques are thicker than patches. Panel B shows pagetoid reticulosis, a variant of mycosis fungoides that typically consists of a single patch or plaque located in an acral area. Panel C shows syringotropic mycosis fungoides, which is manifested as papules 1 to 3 mm in diameter distributed in the eccrine ducts, indicating the propensity of lymphoma cells to accumulate in these locations. Panel D shows follicular mycosis fungoides, in which lesions characterized by alopecia develop. In a similar variant, there is mucin deposition in the follicles. Panel E shows hypopigmented mycosis fungoides. This variant is more noticeable in persons with dark pigmentation and may be more common in childhood and adolescence than in adulthood. Hypopigmentation to full depigmentation occurs in patches. Panel F shows erythrodermic mycosis fungoides. This variant may evolve from patch-or-plaque mycosis fungoides and eventually involve more than 80 percent of the body-surface area. It may also arise spontaneously, as in the Sezary syndrome. Panel G shows the Sezary syndrome. In its most florid form, the diffuse infiltration of the skin may produce the exaggerated facial lines, resulting in "leonine facies." The Sezary syndrome is also associated with atypical lymphocytes on the blood smear. Panel H shows a mycosis fungoides tumor. Such tumors define the T3 stage of disease and may arise at the site of plaques or appear on their own, without being preceded by a patch-or-plaque lesion. The vertical growth phase is accelerated, and tumors tend to appear more quickly than plaques. Tumors not characterized by epidermotropism or previous mycosis fungoides are sometimes called "non-mycosis fungoides cutaneous T-cell lymphoma." Panel D shows follicular mycosis fungoides, in which lesions characterized by alopecia develop. In a similar variant, there is mucin deposition in the follicles.

Panel E shows hypopigmented mycosis fungoides. This variant is more noticeable in persons with dark pigmentation and may be more common in childhood and adolescence than in adulthood. Hypopigmentation to full depigmentation occurs in patches. Figure 1. Various Cutaneous Manifestations of Mycosis Fungoides. Panel A shows patch-or-plaque mycosis fungoides affecting the lower trunk. The patches are thin, slightly scaly, erythematous lesions typically greater than 4 cm in diameter and distributed in sun-shielded areas such as those covered by a bathing suit or intertriginous regions. Plaques are thicker than patches. Panel B shows pagetoid reticulosis, a variant of mycosis fungoides that typically consists of a single patch or plaque located in an acral area. Panel C shows syringotropic mycosis fungoides, which is manifested as papules 1 to 3 mm in diameter distributed in the eccrine ducts, indicating the propensity of lymphoma cells to accumulate in these locations. Panel D shows follicular mycosis fungoides, in which lesions characterized by alopecia develop. In a similar variant, there is mucin deposition in the follicles. Panel E shows hypopigmented mycosis fungoides. This variant is more noticeable in persons with dark pigmentation and may be more common in childhood and adolescence than in adulthood. Hypopigmentation to full depigmentation occurs in patches. Panel F shows erythrodermic mycosis fungoides. This variant may evolve from patch-or-plaque mycosis fungoides and eventually involve more than 80 percent of the body-surface area. It may also arise spontaneously, as in the Sezary syndrome. Panel G shows the Sezary syndrome. In its most florid form, the diffuse infiltration of the skin may produce the exaggerated facial lines, resulting in "leonine facies." The Sezary syndrome is also associated with atypical lymphocytes on the blood smear. Panel H shows a mycosis fungoides tumor. Such tumors define the T3 stage of disease and may arise at the site of plaques or appear on their own, without being preceded by a patch-or-plaque lesion. The vertical growth phase is accelerated, and tumors tend to appear more quickly than plaques. Tumors not characterized by epidermotropism or previous mycosis fungoides are sometimes called "non-mycosis fungoides cutaneous T-cell lymphoma."

Figure 1. Various Cutaneous Manifestations of Mycosis Fungoides. Panel A shows patch-or-plaque mycosis fungoides affecting the lower trunk. The patches are thin, slightly scaly, erythematous lesions typically greater than 4 cm in diameter and distributed in sun-shielded areas such as those covered by a bathing suit or intertriginous regions. Plaques are thicker than patches. Panel B shows pagetoid reticulosis, a variant of mycosis fungoides that typically consists of a single patch or plaque located in an acral area. Panel C shows syringotropic mycosis fungoides, which is manifested as papules 1 to 3 mm in diameter distributed in the eccrine ducts, indicating the propensity of lymphoma cells to accumulate in these locations. Panel D shows follicular mycosis fungoides, in which lesions characterized by alopecia develop. In a similar variant, there is mucin deposition in the follicles. Panel E shows hypopigmented mycosis fungoides. This variant is more noticeable in persons with dark pigmentation and may be more common in childhood and adolescence than in adulthood. Hypopigmentation to full depigmentation occurs in patches. Panel F shows erythrodermic mycosis fungoides. This variant may evolve from patch-or-plaque mycosis fungoides and eventually involve more than 80 percent of the body-surface area. It may also arise spontaneously, as in the Sezary syndrome. Panel G shows the Sezary syndrome. In its most florid form, the diffuse infiltration of the skin may produce the exaggerated facial lines, resulting in "leonine facies." The Sezary syndrome is also associated with atypical lymphocytes on the blood smear. Panel H shows a mycosis fungoides tumor. Such tumors define the T3 stage of disease and may arise at the site of plaques or appear on their own, without being preceded by a patch-or-plaque lesion. The vertical growth phase is accelerated, and tumors tend to appear more quickly than plaques. Tumors not characterized by epidermotropism or previous mycosis fungoides are sometimes called "non-mycosis fungoides cutaneous T-cell lymphoma." Panel F shows erythrodermic mycosis fungoides. This variant may evolve from patch-or-plaque mycosis fungoides and eventually involve more than 80 percent of the body-surface area. It may also arise spontaneously, as in the Sézary syndrome.

Figure 1. Various Cutaneous Manifestations of Mycosis Fungoides. Panel A shows patch-or-plaque mycosis fungoides affecting the lower trunk. The patches are thin, slightly scaly, erythematous lesions typically greater than 4 cm in diameter and distributed in sun-shielded areas such as those covered by a bathing suit or intertriginous regions. Plaques are thicker than patches. Panel B shows pagetoid reticulosis, a variant of mycosis fungoides that typically consists of a single patch or plaque located in an acral area. Panel C shows syringotropic mycosis fungoides, which is manifested as papules 1 to 3 mm in diameter distributed in the eccrine ducts, indicating the propensity of lymphoma cells to accumulate in these locations. Panel D shows follicular mycosis fungoides, in which lesions characterized by alopecia develop. In a similar variant, there is mucin deposition in the follicles. Panel E shows hypopigmented mycosis fungoides. This variant is more noticeable in persons with dark pigmentation and may be more common in childhood and adolescence than in adulthood. Hypopigmentation to full depigmentation occurs in patches. Panel F shows erythrodermic mycosis fungoides. This variant may evolve from patch-or-plaque mycosis fungoides and eventually involve more than 80 percent of the body-surface area. It may also arise spontaneously, as in the Sezary syndrome. Panel G shows the Sezary syndrome. In its most florid form, the diffuse infiltration of the skin may produce the exaggerated facial lines, resulting in "leonine facies." The Sezary syndrome is also associated with atypical lymphocytes on the blood smear. Panel H shows a mycosis fungoides tumor. Such tumors define the T3 stage of disease and may arise at the site of plaques or appear on their own, without being preceded by a patch-or-plaque lesion. The vertical growth phase is accelerated, and tumors tend to appear more quickly than plaques. Tumors not characterized by epidermotropism or previous mycosis fungoides are sometimes called "non-mycosis fungoides cutaneous T-cell lymphoma." Panel G shows the Sézary syndrome. In its most florid form, the diffuse infiltration of the skin may produce the exaggerated facial lines, resulting in "leonine facies." The Sézary syndrome is also associated with atypical lymphocytes on the blood smear.

Panel H shows a mycosis fungoides tumor. Such tumors define the T3 stage of disease and may arise at the site of plaques or appear on their own, without being preceded by a patch-or-plaque lesion. The vertical growth phase is accelerated, and tumors tend to appear more quickly than plaques. Tumors not characterized by epidermotropism or previous mycosis fungoides are sometimes called "non–mycosis fungoides cutaneous T-cell lymphoma Figure 1. Various Cutaneous Manifestations of Mycosis Fungoides. Panel A shows patch-or-plaque mycosis fungoides affecting the lower trunk. The patches are thin, slightly scaly, erythematous lesions typically greater than 4 cm in diameter and distributed in sun-shielded areas such as those covered by a bathing suit or intertriginous regions. Plaques are thicker than patches. Panel B shows pagetoid reticulosis, a variant of mycosis fungoides that typically consists of a single patch or plaque located in an acral area. Panel C shows syringotropic mycosis fungoides, which is manifested as papules 1 to 3 mm in diameter distributed in the eccrine ducts, indicating the propensity of lymphoma cells to accumulate in these locations. Panel D shows follicular mycosis fungoides, in which lesions characterized by alopecia develop. In a similar variant, there is mucin deposition in the follicles. Panel E shows hypopigmented mycosis fungoides. This variant is more noticeable in persons with dark pigmentation and may be more common in childhood and adolescence than in adulthood. Hypopigmentation to full depigmentation occurs in patches. Panel F shows erythrodermic mycosis fungoides. This variant may evolve from patch-or-plaque mycosis fungoides and eventually involve more than 80 percent of the body-surface area. It may also arise spontaneously, as in the Sezary syndrome. Panel G shows the Sezary syndrome. In its most florid form, the diffuse infiltration of the skin may produce the exaggerated facial lines, resulting in "leonine facies." The Sezary syndrome is also associated with atypical lymphocytes on the blood smear. Panel H shows a mycosis fungoides tumor. Such tumors define the T3 stage of disease and may arise at the site of plaques or appear on their own, without being preceded by a patch-or-plaque lesion. The vertical growth phase is accelerated, and tumors tend to appear more quickly than plaques. Tumors not characterized by epidermotropism or previous mycosis fungoides are sometimes called "non-mycosis fungoides cutaneous T-cell lymphoma."

Let me now examine you Figure 1. Various Cutaneous Manifestations of Mycosis Fungoides. Panel A shows patch-or-plaque mycosis fungoides affecting the lower trunk. The patches are thin, slightly scaly, erythematous lesions typically greater than 4 cm in diameter and distributed in sun-shielded areas such as those covered by a bathing suit or intertriginous regions. Plaques are thicker than patches. Panel B shows pagetoid reticulosis, a variant of mycosis fungoides that typically consists of a single patch or plaque located in an acral area. Panel C shows syringotropic mycosis fungoides, which is manifested as papules 1 to 3 mm in diameter distributed in the eccrine ducts, indicating the propensity of lymphoma cells to accumulate in these locations. Panel D shows follicular mycosis fungoides, in which lesions characterized by alopecia develop. In a similar variant, there is mucin deposition in the follicles. Panel E shows hypopigmented mycosis fungoides. This variant is more noticeable in persons with dark pigmentation and may be more common in childhood and adolescence than in adulthood. Hypopigmentation to full depigmentation occurs in patches. Panel F shows erythrodermic mycosis fungoides. This variant may evolve from patch-or-plaque mycosis fungoides and eventually involve more than 80 percent of the body-surface area. It may also arise spontaneously, as in the Sezary syndrome. Panel G shows the Sezary syndrome. In its most florid form, the diffuse infiltration of the skin may produce the exaggerated facial lines, resulting in "leonine facies." The Sezary syndrome is also associated with atypical lymphocytes on the blood smear. Panel H shows a mycosis fungoides tumor. Such tumors define the T3 stage of disease and may arise at the site of plaques or appear on their own, without being preceded by a patch-or-plaque lesion. The vertical growth phase is accelerated, and tumors tend to appear more quickly than plaques. Tumors not characterized by epidermotropism or previous mycosis fungoides are sometimes called "non-mycosis fungoides cutaneous T-cell lymphoma."

Therapeutic Options in the Management of Mycosis Fungoides and the Sezary Syndrome
Table 4. Therapeutic Options in the Management of Mycosis Fungoides and the Sezary Syndrome. Girardi M et al. N Engl J Med 2004;350:

Therapeutic Options in the Management of Mycosis Fungoides and the Sezary Syndrome

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