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MYCOSIS FUNGOIDES

MYCOSIS FUNGOIDES SHEIKHA. MYCOSIS FUNGOIDES. MYCOSIS FUNGOIDES SHEIKHA. Dermatology is NOTHING.

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MYCOSIS FUNGOIDES

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  1. MYCOSIS FUNGOIDES SHEIKHA MYCOSIS FUNGOIDES

  2. MYCOSIS FUNGOIDES SHEIKHA Dermatology is NOTHING

  3. MYCOSIS FUNGOIDES SHEIKHA BUT An External Hematology

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

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

  6. HEMATOLOGY DERMATOLOGY The two sides of the same coin

  7. Professor Anwar Sheikha 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

  8. كومبانياىﺮﺍﺰﯿﺎﻨﻪپروژﻩى 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

  9. “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

  10. 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

  11. LYMPHOMA IS THE MOST CONFUSING PART OF MEDICINE

  12. MYCOSIS FUNGOIDES SHEIKHA Professor Lennert, Keil Classification

  13. MYCOSIS FUNGOIDES SHEIKHA

  14. W.H.O. CLASSIFICATION OF LYMPHOID NEOPLASMS B T& NK NHL 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

  15. 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 *

  16. MYCOSIS FUNGOIDES SHEIKHA

  17. MYCOSIS FUNGOIDES SHEIKHA Incidence: 3 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

  18. MYCOSIS FUNGOIDES SHEIKHA 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

  19. Various Cutaneous Manifestations of Mycosis Fungoides

  20. MYCOSIS FUNGOIDES SHEIKHA EPIDERMOTROPIC The cardinal features of MF is infiltration of epidermis and then dermis by Atypical Cerebriform Lymphoid Cells

  21. Mycosis Fungoides: A Cancer of Skin-Homing T Cells Girardi M et al. N Engl J Med 2004;350:1978-1988

  22. MYCOSIS FUNGOIDES SHEIKHA

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

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

  25. 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

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

  27. 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

  28. 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

  29. 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

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

  31. MYCOSIS FUNGOIDES SHEIKHA

  32. Pautrier Abscesses

  33. MYCOSIS FUNGOIDES SHEIKHA LYMPH NODE INVOLVEMENT IN MF or SZS DERMATOPATHIC LYMPHADENITIS = DL

  34. MYCOSIS FUNGOIDES SHEIKHA IMMUNOPHENOTYPE in MF/SZS CD2+ CD7- CD30- CD3+ CD4+ 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

  35. MYCOSIS FUNGOIDES SHEIKHA 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

  36. 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

  37. MYCOSIS FUNGOIDES SHEIKHA OTHER FEATURES OF MF 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.

  38. Various Cutaneous Manifestations of Mycosis Fungoides

  39. MYCOSIS FUNGOIDES SHEIKHA STAGING OF MF

  40. MYCOSIS FUNGOIDES SHEIKHA Tumor- Node- Metastasis- Blood Classification For MF

  41. MYCOSIS FUNGOIDES SHEIKHA CLINICAL STAGING SYSTEM FOR MF B CLASSIFICATION (SEZARY CELLS) DOES NOT ALTER CLINICAL STAGE

  42. MYCOSIS FUNGOIDES SHEIKHA Tumor-Node-Metastasis-Blood & Clinical Staging Classification

  43. MYCOSIS FUNGOIDES SHEIKHA 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.

  44. MYCOSIS FUNGOIDES SHEIKHA 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.

  45. MYCOSIS FUNGOIDES SHEIKHA 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

  46. MYCOSIS FUNGOIDES SHEIKHA TOPICAL CHEMO- THERAPY TREATMENT OF MF 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

  47. MYCOSIS FUNGOIDES SHEIKHA 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

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