Rapidly Progressive Mycosis Fungoides

in a young woman

presented by

Choon Siew Eng MBBS FRCP

Johor Bahru, Malaysia

January 21, 2010

Senior Consultant Dermatologist, Department of Dermatology, Hospital Sultanah Aminah, Johor Bahru, Malaysia

Abstract A 30-year-old woman presented with a rapidly progressive mycosis fungoides. The various treatment options were discussed.
30-year-old woman
2 years
trunk and extremities
Clinical History

A 30-year-old Chinese lady presented in May 2008 with a 6-month history of occasionally pruritic plaques affecting about 10% BSA. Clinically there was no lymphadenopathy or organomegaly. Skin biopsy of a lesion on right thigh showed hyperkeratosis, focal parakeratosis and irregular epidermal hyperplasia with Pautrier microabscesses. A dense band-like and perivascular infiltrate comprising of small atypical CD4 positive cells are seen confirming a diagnosis of mycosis fungoides.

Her CXR, CT scan from neck to pelvis were normal. Her blood biochemistry including LDH, full blood count and peripheral blood film were normal. She was diagnosed as Stage 1B mycosis fungoides and responded to topical clobetasol proprionate but lesions recur promptly on stopping clobetasol.

She defaulted in Oct 08, claiming that she was completely well but returned in April 09 after seeing a dermatologist in Singapore for relapse. Biopsies in Singapore showed similar findings except for 45% population of CD30 positive cells. Clinically, only plaques seen and RePUVA or total body electron beam therapy was suggested to her but she opted for UVB with Roaccutane (isotretinoin) since she was getting married in Oct 09. She stopped Roaccutane in mid-Nov but continue with phototherapy. However,she developed a single tumour about 1.5cm by 1.5cm on her buttock which responded to local radiotherapy.

Unfortunately, the past one month she rapidly develop multiple tumours on limbs and trunk. Repeat CT scans, blood investigations all normal, no sezary cells. LDH normal. Our haematologist suggested extracorporeal photopheresis in Kuala Lumpur but patient and husband declined this option because they have to travel to KL. Hence, a bone marrow is scheduled on Jan 27, 2010 before starting patient on weekly methotrexate 10mg.


At presentation in May 2008

Lesions had rapidly progressed in January 2010

Laboratory Data

Blood counts and biochemistry were normal

Periheral blood film normal (No Sezary cells)

Serum LDH normal

Repeat CT Scans normal


Skin biopsy of a lesion on right thigh showed hyperkeratosis, focal parakeratosis and irregular epidermal hyperplasia with Pautrier microabscesses. A dense band-like and perivascular infiltrate comprising of small atypical CD4 positive cells are seen confirming a diagnosis of mycosis fungoides.


Mycosis Fungoides

Reason for presentation

The patient came to see me with her sister yesterday seeking advice on best treatment option. Her family is willing to send her to Kuala Lumpur, Malaysia if photopheresis is the best treatment option. To me, it is not feasible since complete remission rate is so low about 26% out of 19 cases. She asked me several questions that I have lots of difficulties answering and would appreciate your views and comments.

  1. When she first presented, she was stage 1B and I told her about the good prognosis. Her question: Only 10-20% progresses to tumour stage, a) why her? b) Are there any reasons why she progresses so fast? Ventricular septal defect that had been successfully repaired. c)Any tests available to determine why?
  2. What is her prognosis now? " I have not given birth yet". Her main reason for marrying her long time boyfriend was to start a family.
  3. She hated procedures including skin biopsies and wanted to know is bone marrow necessary?
  4. Should she go for extracorporeal photopheresis or MTX or electron beam therapy?

I wish to know whether our colleagues have experience in managing such a young patient with such a rapid disease progression.

What is her prognosis?

Will she live long enough to see her kids grow and what is her best treatment option?




mycosis fungoides

Comments from Faculty and Members

Robert I. Rudolph, M.D., FACP, Clinical Professor of Dermatology
University of Pennsylvania, Philadelphia, PA, USA
on January 21, 2010

Obviously needs full scanning and cell flow cytometry and followup with derm and oncology.

Electron Beam therapy (total body) and/or  Ontak and/ or interferons for systemic therapy.

Stephen Stone M.D. Professor of Dermatology, Southern Il University, Springfield, IL, USA on January 21, 2010

Tough case. If the patient really is that focused on raising a family, I think the most reasonable approach would be electron beam. In my experience, the prognosis at this point is not good, and she might want to rethink getting pregnant.

Chang Choong Chor MBBS, MRCP, MMed, Consultant Dermatologist, Department of Dermatology, Hospital Kuala Lumpur, Malaysia on January 21, 2010

We managed a similar patient 2 years ago. He was a 35-year-old Malay gentleman who presented initially with 1-year history of plaque stage MF (biopsy confirmed) involving about 40% BSA with inguinal node. Lymph node biopsy revealed no atypical cells. (Stage IIA) He achieved partial remission (~80%) with oral PUVA and topical steroids. However, he relapsed shortly and developed disseminated tumours. Electron beam therapy was attempted twice, but his tumours relapsed within 2 months after each treatment course, and thereafter progressed rapidly into multiple large ulcerated tumours. His peripheral blood and bone marrow had no significant abnormal cells. (Stage IIB) The hematologists attempted extracorporeal photophoresis weekly for 2 months with no success. He was subsequently treated with high-dose cytotoxic chemotherapy. He achieved complete remission for about 6 months, but unfortunately sucumbed to septicaemia. On the hind side, we thought perhaps a combination of electron beam therapy and interferon might be a less risky option compared to polychemotherapy.

As for your patient, since she has developed tumours and no evidence of nodal, blood or visceral involvement, she should be stage IIB. Bone marrow is preferable but not absolutely necessary if her peripheral blood remains normal.

Extracorporeal photophoresis is more useful in Sezary's syndrome, erythrodermic MF and extensive plaque-stage MF. There is little evidence of its usefulness in tumour-stage MF. The following treatment options can be considered for her:

1) PUVA + Interferon (PUVA may not penetrate tumours though)
2) TSEB + Interferon
3) Pegylated liposomal doxorubicin

Polychemotherapy should be the last resort.

Nidal Dabbour MD, Consultant Dermatologist, al-Khobar, Saudi Arabia on January 21, 2010

First of all be sure that this is really MF, since she is Chinese, I will do HTLV IgG and CD 25 on the biopsy. HTLV looks exactly the same as MF clinically and histologically.

Allain Rook M.D., Professor and Director, Photopheresis Program, Department of Dermatology, University of Pennsylvania, Philadelphia, PA, USA on January 22, 2010

For tumor stage MF you need to consider the following:

1) If possible consider skin radiation

2) You need to consider bone marrow transplantation if possible once you have begun treatment

3) Do you have bexarotene? If yes, consider beginning 4 capsules daily all at once with a meal. You must use a statin to prophylax against hyperlipidemia. Also thyroxine replacement for hypothalamic hypothyroidism as it suppresses TSH. Therefore you begin 25 micrograms and work up with monthly serum free T4 levels. 

4) Also consider starting low dose interferon

5) You may administer bexarotene and interferon along with radiation.

6) If things fail, consider gemcitabine but you should consult Oncology regarding this. 

Omar A. Ibrahimi, M.D., Ph.D., Resident Physician, Harvard Medical School
Department of Dermatology, Massachusetts General Hospital, Boston, MA, USA
on January 22, 2010

Very sad and unfortunate that she is in the subset of MF patients that has progressed. I think she is a stage IIB (tumors but no nodes/blood invovlement). I don't see any tumor like lesions in her pictures though, so we should be sure of that before we label her a IIb versus a Ib. Survival isn't great in these folks, as far as I know not much better than erythrodermic patients.

It seems like she definitely needs electron beam therapy for her nodular/tumor lesions. I know conceiving is of importance to her but so is so young we need to treat her first. I think MTX or bexarotene would be good agents to start with. Perhaps adding a histone deacetylase inhibitor or denileukin in conjunction. Perhaps I'm missing something but getting photopheresis does not make sense to me as she has no leukemic component

Yelva Lynfield MD, Consultant Dermatologist, Mendota Heights, MN, USA on January 23, 2010

She will be treated with some very toxic drugs. If she really wants children she should consider donating eggs, in vitro fertilization, and freezing the embryos. This is similar to a man's banking sperm before being treated with toxic drugs.

el-Azhary, Rokea A., M.D., Ph.D, Professor of Dermatology, Mayo School of Medicine, Rochester, Minnesota, USA on January 27, 2010

As I look at this patient's pictures, I really see only plaque stage MF with very few small tumors all of which should respond to PUVA .  Radiation is very impractical as it does not last and we should keep it for more resistant tumors or ulcerative ones as the disease progresses.  If she wishes to get pregnant right away then NBUVB might be a better option than PUVA, temporarily, till she delivers.   All the systemic drugs mentioned are currently unnecessary and certainly photopheresis would not help MF at this stage.  Hope this helps.

Marie-France Demierre M.D., Professor and Director of the Skin Oncology and Photopheresis Programs, Department of Dermatology, Boston University School of Medicine, Boston, USA, on January 27, 2010

I would consider combination therapy first: PUVA + interferon low dose, then total skin radiation followed by low dose interferon.

Liposomal doxorubicin could also help this patient if she fails the above

To start a family, total skin electron beam, followed by NUVB for maintenance would work. My concern is that only NUVB will not treat the thick lesions, she is at risk of progressing and dying from her disease

Choon Siew Eng MBBS, FRCP, Senior Consultant Dermatologist, Department of Dermatology, Hospital Sultanah Aminah, Johor Bahru, Malaysia on January 28, 2010

My gratitude and thanks to all our colleagues for sharing their experiences and for their suggestions.

Update on the case. We were worried about adult T-Cell leukemia/lymphoma (ATLL) but Human T-lymphotrophic virus (HTLV1) is not available and we did in fact asked patient to do HIV as well as HTLV1. We do not have Bexarotene but can put patient on combination of interferon and acitretin/istretinoin. She had multiple nodules, one just behind left axilla was ulcerated. The nodule on dorsum of left hand was about 3 cm by 3cm, firm but softer and smaller after radiotherapy.


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