What is Cutaneous Lymphoma

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1 What is Cutaneous Lymphoma Dermatologist Chief of Cutaneous Lymphoma Clinic Jewish General Hospital - McGill University Montréal 13 septembre 2014 What is Cutaneous Lymphoma 1

2 Acknowledgements Parts of this talk modified from information provided by other experts in this field: US National Cancer Institute Dr. Pierluigi Porcu (Ohio State University) Dr. Lisa Grandinetti (University of Pittsburgh) US Library of Congress for image of elephant Montréal 13 septembre 2014 What is Cutaneous Lymphoma 2

3 Definition: Cutaneous Lymphoma A cancer or malignancy that comes from a clonal expansion of lymphocytes, localizing to the skin. Montréal 13 septembre 2014 What is Cutaneous Lymphoma 3

4 Say what??? Okay, let s look at this one word at a time. Montréal 13 septembre 2014 What is Cutaneous Lymphoma 4

5 Cancer/malignancy (1) Diseases in which abnormal cells divide without control and can invade other tissues. Cancer cells can spread to other parts of the body. Cancer is not just one disease but many diseases; there are more than 100 different types of cancer. Montréal 13 septembre 2014 What is Cutaneous Lymphoma 5

6 Cancer/malignancy (2) Normal cells differentiate (e.g. become a certain type of cell), and divide/multiply to just the right number to work together with all the other cells in your body to do whatever they need to do to make your body work as one. Cancer cells multiply without limit and with out regard for what is good for you body as a whole. They may also de-differentiate become more primitive and less specific. Montréal 13 septembre 2014 What is Cutaneous Lymphoma 6

7 Note: not all tumors are cancer. Tumor means any growth. Benign tumors aren't cancer. The cells are normal cells, and cannot spread to other parts of the body. Malignant tumors are cancer. These cells are abnormal and can invade nearby tissues or spread to other parts of the body. The spread of cancer from one part of the body to another is called metastasis. Montréal 13 septembre 2014 What is Cutaneous Lymphoma 7

8 Clonal Science fiction movies aside, a clone means it all started from one cell, which multiplied and made exact copies of itself. Montréal 13 septembre 2014 What is Cutaneous Lymphoma 8

9 Montréal 13 septembre 2014 What is Cutaneous Lymphoma 9

10 Lymphoma Most cancers are named for the organ or type of cell in which they start. Lymphoma starts as a type of immune cell called a Lymphocyte. Immune cells, including lymphocytes, are part of your body s defense system. They exist to fight infection (and cancer). Montréal 13 septembre 2014 What is Cutaneous Lymphoma 10

11 Localizing to the skin Lymphocytes circulate in the blood. They are on patrol, looking for invaders. Subsets of lymphocytes home in on, and move through, other tissue. In our case, we are interested in cutaneous lymphocytes, e.g. those that home in on the skin. Montréal 13 septembre 2014 What is Cutaneous Lymphoma 11

12 So. A Cutaneous Lymphoma is a cancer or malignancy that comes from a clonal expansion of lymphocytes, localizing to the skin. That is to say, a population of the body s defensive cells, meant to patrol and respond to infection in the skin, multiplying without control, and able to spread to other tissues. Montréal 13 septembre 2014 What is Cutaneous Lymphoma 12

13 Why is this bad? If these are defensive cells, isn t having more of them a good thing? No: (1) They don t do their job of defending, and since they multiply without control, they crowd out the good defensive cells. (2) They crowd out everything else, blocking and pressing on vital tissues, and using up your energy/food supply. Montréal 13 septembre 2014 What is Cutaneous Lymphoma 13

14 What types of cutaneous lymphomas are there (1)? Lots, but grouped into: T/NK-cell (2/3 of all cutaneous lymphomas) B-cell (1/3 of all cutaneous lymphomas) Normally B-Cells produce antibodies and T- Cells either directly attack invaders or organize the actions of other cells. Montréal 13 septembre 2014 What is Cutaneous Lymphoma 14

15 What types of cutaneous lymphomas are there (2)? Classified by WHO-EORTC Published in Blood 2005:105(10) Don t worry, no quiz Montréal 13 septembre 2014 What is Cutaneous Lymphoma 15

16 Why is it important? Gives us a handle to help understand. Approach to treatment different. Prognosis different. (But in the end, whatever you have, we treat.) Montréal 13 septembre 2014 What is Cutaneous Lymphoma 16

17 Cutaneous T- & NK-cell lymphomas (1) Mycosis fungoides incl. variants and subtypes About 2/3 of T/NK-Cell Lymphomas, so About 1/2 of all cutaneous Lymphomas Folliculotropic MF Pagetoid reticulosis Granulomatous slack skin Montréal 13 septembre 2014 What is Cutaneous Lymphoma 17

18 Cutaneous T- & NK-cell lymphomas (2) (* P.C. = Primary Cutaneous) Sézary syndrome P.C. CD30+ lymphoproliferative disorders: P.C. anaplastic large cell lymphoma Lymphomatoid papulosis Subcutaneous panniculitis-like T-cell lymphoma Adult T-cell leukemia/lymphoma Extranodal NK/T-cell lymphoma, nasal type Provisional entities: P.C. Aggressive epidermotropic CD8+ T-cell lymphoma P.C. Gamma-Delta (γ/δ) T-cell lymphoma P.C. CD4+ small/medium-sized pleomorphic T-cell lymphoma P.C. peripheral T-cell lymphoma, unspecified Montréal 13 septembre 2014 What is Cutaneous Lymphoma 18

19 Cutaneous B-cell lymphomas (P.C. = Primary Cutaneous) P.C. marginal zone B-cell lymphoma P.C. follicle center lymphoma P.C. diffuse large B-cell lymphoma, leg type P.C. diffuse large B-cell lymphoma, other Some newer classifications combine these two Intravascular large B-cell lymphoma Montréal 13 septembre 2014 What is Cutaneous Lymphoma 19

20 What types of cutaneous lymphomas are there (3)? Even within types, can have different stages. More advanced stages are worse. Details next talk. (But in the end, whatever you have, we treat.) Montréal 13 septembre 2014 What is Cutaneous Lymphoma 20

21 How do we diagnose (1)? Not always easy. Classic story, especially for the most common type (MF) is that patient has a some sort of rash for years before figure out what it is. Team approach. No one magic piece of information, have to put together the puzzle Montréal 13 septembre 2014 What is Cutaneous Lymphoma 21

22 Montréal 13 septembre 2014 What is Cutaneous Lymphoma 22

23 How do we diagnose (2)? Examine your patient! Skin Biopsy Blood tests Lymph Node Biopsy (for prognosis & staging; not important for diagnosis) Scans/X-Rays (for prognosis & staging; not important for diagnosis) Montréal 13 septembre 2014 What is Cutaneous Lymphoma 23

24 How do we diagnose (3)? Skin Biopsy (sent to Pathology): Take a small piece of skin. Sometimes can be preserved in formaldehyde (basic test), sometimes might need fresh (special test). Some is stained different colors and look at it under the microscope (basic test) Some is immuno- or molecular pathology to check certain protein markers on the cell surfaces (intermediate and advanced testing). Montréal 13 septembre 2014 What is Cutaneous Lymphoma 24

25 How do we diagnose (4)? Blood tests: Basic tests like CBC & Liver Function tests CBC = Complete Blood Count counting different types of white blood cells, red blood cells, platelets Flow and molecular cytology Like with fresh tissue biopsy, looking at markers on cell surfaces Montréal 13 septembre 2014 What is Cutaneous Lymphoma 25

26 How do we diagnose (5)? Lymph Node Biopsy Not important for diagnosis Only if LN clinically involved, gives more information about stage & prognosis Scans/X-Rays Not important for diagnosis Only if LN clinically involved, gives more information about stage & prognosis Montréal 13 septembre 2014 What is Cutaneous Lymphoma 26

27 Key Words about Lymphoma (1) Clinically Indolent vs Aggressive Indolent Grows slowly. Often no change life expectancy Treatment may not be necessary Usually not curable Aggressive Grows rapidly Treatment necessary for survival Often curable Montréal 13 septembre 2014 What is Cutaneous Lymphoma 27

28 Key Words about Lymphoma (2) Biopsy: Low Grade vs High Grade Low Grade (this is good) Cancer cells look small & monotonous Most of them are resting High Grade Cancer cells look big & ugly Most of them are dividing & growing Montréal 13 septembre 2014 What is Cutaneous Lymphoma 28

29 Key Words about Lymphoma (3) Referring to results of treatment Remission cancer not detectable Cure cancer never comes back Relapse cancer come back after remission Survival how long live after diagnosis Progression cancer starts growing after had been static Montréal 13 septembre 2014 What is Cutaneous Lymphoma 29

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